DIAGNOSTIC IMAGING

CHEST
II
 DIAGNOSTIC IMAGING
CHEST
Jud W. Gurney, MD, FACR J. Michael Holbert, MD, FACR
The Nebraska Medical Center Chief of Thoracic Imaging
Charles A. Dobry Professor of Radiology Scott & White Clinic and Memorial Hospital
University of Nebraska Medical Center
Associate Professor of Radiology
Omaha, Nebraska Texas A&M University Health Science Center, College of
Medicine
Temple, Texas
Helen T. Winer-Muram, MD
Professor of Radiology
Indiana University Marc V. Gosselin, MD
Indianapolis, Indiana Director of Thoracic Imaging
Associate Professor
Oregon Health and Science University
Eric J. Stern, MD Portland, Oregon
Director of Thoracic Imaging
Harborview Medical Center
Tan-Lucien H. Mohammed, MD, FCCP
Professor of Radiology, Adjunct Professor of Medicine,
Adjunct Professor Medical Education & Biomedical Informatics Section of Thoracic Imaging
University of Washington Associate Residency Program Director of Radiology
Seattle, Washington The Cleveland Clinic Foundation
Cleveland, Ohio

Tomas Franquet, MD
Kitt Shaffer, MD, PhD
Chief Thoracic Imaging
Hospital de Sant Pau Director of Undergraduate Medical Education
Cambridge Health Alliance
Associate Professor of Radiology
Universidad Aut6noma de Barcelona Associate Professor of Radiology
Barcelona, Spain Harvard Medical School
Boston, Massachusetts

James G. Ravenel, MD
Associate Professor of Radioiogy
Patricia J. Mergo, MD
Medical University of South Carolina Associate Professor of Radiology and Pediatrics
Charleston, South Carolina University of Florida College of Medicine
Gainesville, Florida

Charles S. White, MD
Director of Thoracic Imaging
Sujal R. Desai, MD, FRCp, FRCR
Professor of Radiology and Medicine Consultant Radiologist
University of Maryland School of Medicine King's College Hospital
Baltimore, Maryland Denmark Hill, London

Alexander A. Bankier, MD Phillip M. Boiselle, MD

Associate Professor of Radiology Director of Thoracic imaging and
Medical University of Vienna Director of Resident Career Development and Mentoring
Vienna, Austria Beth Israel Deaconess Medical Center
Associate Professor of Radiology
Harvard Medical School
Boston, Massachusetts

AMIRSYS
Names you know, content you trust

III
 AMIRSYS'"
Names you know, content you trust'"

First Edition
Text - Copyright Jud W. Gurney, MD, FACR 2006

Drawings - Copyright Amirsys Inc. 2006

Compilation - Copyright Amirsys Inc. 2006

All rights reserved. No part of this publication may be reproduced, stored in a retrieval system, or transmitted, in any form or media
or by any means, electronic, mechanical, photocopying, recording, or otherwise, without prior written permission from Amirsys Inc.

Composition by Amirsys Inc, Salt Lake City, Utah

Printed by Friesens, Altona, Manitoba, Canada

ISBN: 1-4160-2334-8
IS8N: 0-8089-2322-6 (International English Edition)

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Library of Congress Cataloging-in-Publication Data

Diagnostic imaging. Chest / Jud W. Gurney ... let al.].- 1st ed.
p. cm.
Includes index.
ISBN 1-4160-2334-8 - ISBN 0-8089-2322-6
1. Chest-Imaging. I. Gurney, Jud W.

RC941.DS32006
617.S'407S4-dc22
2006000191

IV
To my mother and father, good Nebraskans, good values, My dad's only word of advice: "just
try and do the best you can" - I'm still trying. And to my children Antonia (Annie) and lan,
and my loving wife Mary, you make it all possible.

jWG

I wish to thank my husband and children for their patience and unwavering support. I dedicate
this book to the memory of my late parents Riva and Leon Winer.

HWM

I dedicate this effort to my beautiful and loving wife, Karen. Her

unwavering love, support, and patience give me great strength and peace.

EjS

v
VI
DIAGNOSTIC IMAGING
CHEST

xxv
VIII
 FOREWORD

Dr. Gurney and his coauthors have put together a unique book. This is a practical text in chest imaging that will be used in
daily practice for years to come. "Chest DI" is a different type of book for the beginning of the 21st century. Diagnostic imaging
and clinical medicine are changing rapidly. The number of imaging examinations has exploded dramatically. Modern medical
practice is becoming more and more dependent on imaging for both diagnosis and management. More sophisticated imaging
technology, such as CT, MR, and PET provide new insights into diagnosis and treatment.

What the modern resident and practitioner need to know to practice radiology is truly daunting. What is needed is "just in
time" information to borrow a term from current manufacturing practice. One needs to find information quickly, not down the
hall in the office and certainly not in the department library. It needs to be available at the workstation, be easily searched,
comprehensive, and useful.

The authors have done a wonderful job in organizing this large body of information into a iogical structure. Each chapter is
succinct with a brief description of terminology and imaging features, pathology, and clinical issues. Each disease process
contains multiple illustrative cases that provide greater depth than other textbooks by the use of high-quaiity diagnostic images,
as well as excellent color illustrations.

A short synopsis of "key facts" and an up-to-date bibliography follows each section. This is not a textbook to be read on a
lazy Sunday afternoon. This is a book to be kept by the workstation. It will quickly become worn and dog-eared, because it
answers the needs of the increasingly busy radiologist.

Lawrence R. Goodman, MD, FACR

Director, Section of Thoracic Imaging
Froedtert Memorial Lutheran Hospital
Professor of Radiology
Medical College of Wisconsin
Milwaukee, Wisconsin

IX
x
 PREFACE

Many years ago I went on sabbatical and became a Neuroradiology fellow under Ric Harnsberger and Anne Osborn. What a
humbling experience. During my year they shared their vision for a new publishing company, one that would embody their
passion for education. That was their dream. Amirsys was their reality. It was my great iuck to be present at the genesis of a
publishing company, which produces not just books, but also digitai-based products that greatiy expand where and when
information is consumed.

This book follows the same format as the others in the Diagnostic Imaging series. We have tried to provide as comprehensive
a text as possible, from the common diagnosis to the rare disease, a resource for the novice and the expert. The information is
presented in formatted, bulleted text, making it easy to find the information you need, when you need it - at the workstation.
Numerous images and color graphics illustrate each diagnosis. Each chapter is packed with information, useful clues from
radiographic findings, salient clinical points, and evoiution of disease processes that shouid make this book one that is used on a
daily basis. Besides diagnoses there are chapters on patterns, an approach integral to thorax diagnoses.

My coauthors added their own international expertise, which is refjected throughout the book. I am truly indebted to their
devotion to this project. We all hope that you find this book usefui in your practice.

Jud W. Gurney, MD, FACR

The Nebraska Medical Center
Charies A Dobry Professor of Radiology
University of Nebraska Medical Center
Omaha, Nebraska

XI
XII
ACKNOWLEDGMENTS

Illustrations
Richard Coombs, MS
Lane R. Bennion, MS
Walter Stuart, MFA

Art Direction and Design

Lane R. Bennion, MS
Richard Coombs, MS

Image/Text Editing
Angie D. Mascarenaz
Kaerli Main

Medical Text Editing

Gregory L. Johnson, MD
Howard Mann, MD

Case Management
Roth LaFleur
Chris Odekirk

Production Lead
Melissa A. Hoopes

Xlli
XIV
 SECTIONS

PART I
Lung
Airspace [1]
Interstitium [2]
Airways [JJ

PART II
Mediastinum
Mediastinum [IJ
Aorta and Creat Vessels [2]
Heart and Pericardium [JJ
Pulmonary Vasculature ~

PART III
Pleura - Chest Wall - Diaphragm
Pleura [IJ
Chest Wall [2]
Diaphragm [JJ

PART IV
Special Topics
Atelectasis [l]
Trauma [2]
Lung Cancer [JJ
Portable ICU ~
Physiology [5]
Special Patients [6]

PART V
Patterns
Patterns [1]

xv
XVI
 TABLE OF CONTENTS
Goodpasture Syndrome 1-1-48
PART I Marc V. Gosselit/, MD

Lung Inflammatory - Degenerative

Eosinophilic Pneumonia 1-1-52
Philip M. Boiselle, MD
Introduction and Overview
Acute Interstitial Pneumonia 1-1-56
Secondary Pulmonary Lobule 1-0-2 SlIjal R. Desai, MD, FRCI~ FRCR
flld W. Gilmer, MD, FACR

Toxic - Metabolic
SECTION 1 Metastatic Pulmonary Calcification 1-1-60
Airspace flld W. Gllmey, MD, FACR

Alveolar Microlithiasis 1-1-64

Infectious flld W. Gllmey, MD, FACR

Bacterial Pneumonia 1-1-2 Lipoid Pneumonia 1-1-68

Tomtis Frat/qllel, MD
flld W. Gllmey, MD, FACR

Staphylococcus Pneumonia 1-1-6 Pulmonary Alveolar Proteinosis 1-1-72

Kit! Shaffer, MD, PhD
Eric f. Stem, MD

Mycobacterial Pneumonia 1-1-8 Desquamative Interstitial Pneumonia 1-1-76

Kit! Slwffer, MD, PIlD
He/en T. Winer-Muram, MD

Lung Abscess 1-1-12 Cryptogenic Organizing Pneumonia 1-1-80

SlIjal R. Desai, MD, FRCp, FRCR
Helen T. Winer-Muram, MD
Histoplasmosis 1-1-16
Helen T. Winer-Mllram, MD Neoplastic
Aspergillosis 1-1-20 Bronchioloalveolar Cell Carcinoma 1-1-84
/. MiellOel Holbert, MD, FACR Helet/ T Wit/er-Mllram, MD
Blastomycosis 1-1-24
f. Micllael Holbert, MD, FACR

Coccidioidomycosis 1-1-26
SECTION 2
f. Micllael Holbert, MD, FACR Interstitium
Parasitic Pneumonia 1-1-28
SlIjal R. Desai, MD, FRCp, FRCR
Infectious
Viral Pneumonia 1-2-2
Vascular Helet/ T Wit/er-Mllram, MD

Cardiogenic Pulmonary Edema 1-1-32 Pneumocystis Pneumonia 1-2-6

f. Micllael Holbert, MD, FACR Marc V. GOSSeli'l, MD
oncardiac Pulmonary Edema 1-1-36
SlIjal R. Desai, MD, FRCp, FRCR
Inflammatory - Degenerative
Fat Pulmonary Embolism 1-1-40
Eric /. Stem, MD
Sarcoidosis, Pulmonary 1-2-10
Helen T Winer-Muram, MD
Diffuse Alveolar Hemorrhage 1-1-44
Marc V. Gosselit/, MD

XVII
Idiopathic Pulmonary Fibrosis 1-2-14 Infectious
SlIial R. Desai, MD, FRCp, FRCR

Hypersensitivity Pneumonitis 1-2-18 Rhinoscleroma 1-3-28

Tomas Franqllet, MD Illd W. GlImey, MD, FACR

Rheumatoid Arthritis 1-2-22 Chronic Bronchitis 1-3-30

Patricia I. Mergo, MD I. Michael Holbert, MD, FACR

Scleroderma, Pulmonary 1-2-26 Bronchiectasis 1-3-34

Illd W. GlImey, MD, FACR /. Michael Holbert, MD, FACR

Polymyositis - Dermatomyositis, Pulmonary 1-2-30 Mycobacterial Avium Complex 1-3-38

Aliye A. Ozsoyoglll & Tan-LlIcien Mohammed, MD, FCCP Helen T Winer-Mllram, MD

Nonspecific Interstitial Pneumonitis 1-2-34 Laryngeal Papillomatosis 1-3-42

Helen T Wir,er-Mllram, MD Illd W. GlImey, MD, FACR

Toxic - Metabolic Inflammatory - Degenerative

Asbestosis 1-2-38 Allergic Bronchopulmonary Aspergillosis 1-3-46
Illd W. GlImey, MD, FACR Patricia /. Mergo, MD

Silicosis - Coal Worker Pneumoconiosis 1-2-42 Tracheobronchomalacia 1-3-50

Illd W. GlImey, MD, FACR Philip M. Boiselle, MD

Berylliosis 1-2-46 Relapsing Polychondritis 1-3-54

Illd W. GlImey, MD, FACR Illd W. GlImey, MD, FACR

Lung Ossification 1-2-50 Middle Lobe Syndrome 1-3-58

Eric /. Stem, MD Illd W. GlImey, MD, FACR

Saber-Sheath Trachea 1-3-60

Tan-LlIcien Mohammed, MD, FCCP
Neoplastic
Bronchiolitis Obliterans 1-3-64
Lymphangitic Carcinomatosis 1-2-52 Alexander Bankier, MD
Illd W. GlImey, MD, FACR
Asthma 1-3-68
Lymphocytic Interstitial Pneumonia 1-2-56 Alexander Hankier, MD
Illd W. GlImey, MD, FACR
Pan lobular Emphysema 1-3-72
Lymphangiomyomatosis 1-2-60 Alexander Bankier, MD
Alexander Bankier, MD

Toxic - Metabolic
Congenital
Langerhans Cell Granulomatosis, Pulmonary 1-3-76
Diffuse Pulmonary Lymphangiomatosis 1-2-64 He/en T Winer-Mllram, MD
Illd W. GlImey, MD, FACR
Respiratory Bronchiolitis 1-3-80
Alexander Bankier, MD

SECTION 3 Centrilobular Emphysema 1-3-84

Alexander Bankier, MD
Airways Amyloidosis, Pulmonary 1-3-88
Tomas Franqllet, MD
Congenital
Cystic Fibrosis, Pulmonary 1-3-2 Neoplastic
Kitt SI/(/ffer, MD, PhD
Tracheopathia Osteochondroplastica 1-3-92
Tracheobronchomegaly 1-3-6 Illd W. GlImey, MD, FACR
Helen T W;ner-Mlirarn~ MD
Carcinoid, Pulmonary 1-3-94
Immotile Cilia Syndrome 1-3-10 Marc V. Gosselill, MD
Helen T Wir,er-Mllram, MD
Kaposi Sarcoma, Pulmonary 1-3-98
Bronchial Atresia 1-3-14 Philip M. Boiselle, MD
Illd W. GlImey, MD, FACR

Anomalous Bronchi 1-3-18

Tomas Franqliet,

Alpha-1 Antiprotease
MD

Deficiency 1-3-22
PART II
Alexander Bankier, MD
Mediastinum
Paratracheal Air Cyst 1-3-26
Illd W. GlImey, MD, FACR
Introduction and Overview
Mediastinal Compartments 11-0-2
Illd W. GlImey, MD, FACR

X V 111
 SECTION 1 SECTION 2
Mediastinum Aorta and Great Vessels

Congenital Congenital
Thoracic Cysts 11-1-2 Right Aortic Arch 11-2-2
Helen T Winer-Muram, MD C/wrles S. White, MD

Aberrant Subclavian 11-2-6

Clwrles S. White, MD
Inflammatory - Degenerative
Aortic Coarctation 11-2-10
Mediastinal Fibrosis 11-1-6 Tan-Lucien Mohammed, MD, FCCI'
jud W. Gurney, MD, FACR
Intralobar Sequestration 11-2-14
Achalasia 11-1-10 Marc V. Gosselin, MD
Tonuis Franquet, MD
Left Superior Vena Cava 11-2-18
Esophageal Diverticuli 11-1-12 C/wrles S. White, MD
Tomas Franqllet, MD
Azygos Continuation of IVC 11-2-22
Hiatal and Paraesophageal Hernias 11-1-14 Charles S. White, MD
Patricia f. Mergo, MD
Azygos Fissure 11-2-26
Tomas Franquet, MD
Neoplastic
Goiter, Mediastinum 11-1-18 Inflammatory - Degenerative
James G. Ravenel, MD
Aortic Atherosclerosis 11-2-28
Hodgkin Lymphoma, Mediastinum 11-1-22 Charles S. White, MD
Tan-Lucien Mohammed, MD, FCCI'
Marfan Syndrome 11-2-32
Non-Hodgkin Lymphoma, Mediastinum 11-1-26 Suial R. Desai, MD, FRCp, FRCR
Ruchi Yadav, MD & Tan-Lucien Mohammed, MD, FCCI'
Aortic Dissection 11-2-36
Thymoma 11-1-30 jud W. Gllrney, MD, FACR
Marc V. Gosselin, MD
Aortic Aneurysm 11-2-40
Thymic Tumors Other Than Thymoma 11-1-34 Jud W. Gurney, MD, FACR
Marc V. Gosselin, MD
Takayasu Disease 11-2-44
Thymic Rebound 11-1-38 Charles S. White, MD
Marc V. Gosselin, MD
SVC Obstruction 11-2-48
Germ Cell Tumors, Mediastinum 11-1-40 Nicholas D. Krallse & Tan-Lucien Mohammed, MD, FCCI'
Marc V. Gosselin, MD

Lipomatosis, Mediastinum 11-1-44

james G. Ravenel, MD SECTION 3
Castleman Disease, Mediastinum 11-1-48 Heart and Pericardium
Marc V. Gosselin, MD

Angioimmunoblastic Lymphadenopathy 11-1-52

Marc V. Gosselin, MD
Congenital
Hemangioma - Lymphangioma, Mediastinum 11-1-54 Partial Absence Pericardium 11-3-2
Kitt Shaffer, MD, PhD Tan-LllciC/1 Mohammed, MD, FCCI'

Nerve Sheath Tumors, Mediastinum 11-1-58 Heterotaxy Syndrome 11-3-4

james G. Ravenel, MD jud W. Gurney, MD, FACR

Sympathetic Ganglion Tumors, Mediastinum 11-1-62 Pericardial Cyst 11-3-8

James G. Ravenel, MD Patricia j. Mergo, MD

Extramedullary Hematopoiesis, Mediastinum 11-1-66

jud W. Gurney, MD, FACR Inflammatory - Degenerative
Coronary Artery Calcification 11-3:12
Vascular Charles S. White, MD

Varices, Mediastinum 11-1-70 Left Atrial Calcification 11-3-16

james G. Ravenel, MD Heslwm H. Malik &: Tan-Lucien Mohammed, MD, FCCI'

Ventricular Calcification 11-3-20

Heslwm H. Malik & Tan-Lucien Mohammed, MD, FCCI'

Valve and Annular Calcification 11-3-24

Aliye A. Ozsoyoglll & Tan-Lucien Mohammed, MD, FCCI'

Aortic Valve Dysfunction 11-3-28

Charles S. White, MD

XIX
Mitral Valve Dysfunction 11-3-32
Charles S. White, MD
Vascular
Constrictive Pericarditis 11-3-36 Pulmonary Emboli 11-4-50
Patricia /. Mergo, MD Helen T Winer-Muram, MD

Postcardiac Injury Syndrome 11-3-40 Pulmonary Artery Hypertension 11-4-54

Tomas Franqllet, MD /lid W Gurney, MD, FACR

Pulmonary Artery Aneurysm 11-4-58

/lid W Gllrney, MD, FACR
Toxic - Metabolic
High Altitude Pulmonary Edema 11-4-62
Myxedema 11-3-44 /lid W Gurney, MD, FACR
Iud W Gurney, MD, FACR
Neurogenic Pulmonary Edema 11-4-66
/lid W Gllrney, MD, FACR
Neoplastic
Left Atrial Myxoma 11-3-46 Neoplastic
Kitt Shaffer, MD, PhD
Pulmonary Artery Sarcoma 11-4-70
Metastases Pericardium 11-3-50 Kill Slwffer, MD, PhD
Kitt Shaffer, MD, PhD
Tumor Emboli, Pulmonary 11-4-74
Helen T Winer-Muram, MD

SECTION 4 Capillary Hemangiomatosis, Pulmonary 11-4-78

Clwrles S. White, MD
Pulmonary Vasculature

Arteriovenous
Congenital
Malformation, Pulmonary 11-4-2
PART III
Tomas Franquet, MD Pleura - Chest Wall - Diaphragm
Partial Anomalous Venous Return 11-4-6
/lid W Gllrney, MD, FACR
Introduction and Overview
Scimitar Syndrome 11-4-8
/lid W Gurney, MD, FACR Pleural Space and Fissures 11I-0-2
/lid W Gurney, MD, FACR
Idiopathic Pulmonary Artery Dilatation 11-4-12
/lid W Gllrney, MD, FACR

Congenital Interruption Pulmonary Artery 11-4-16 SECTION 1

Iud W Gurney, MD, FACR
Pleura
Anomalous Origin Left Pulmonary Artery 11-4-20
Charles S. While, MD
Congenital
Infectious Apical Lung Hernia 11I-1-2
/. Michael Holbert, MD, FACR
Septic Emboli, Pulmonary 11-4-22
Patricia /. Mergo, MD Yellow-Nail Syndrome 11I-1-4
/lid W Gllrney, MD, FACR

Inflammatory - Degenerative
Inflammatory - Degenerative
Vasculitis, Pulmonary 11-4-26
Charles S. White, MD Exudative Pleural Effusion 11I-1-6
Helen T. Winer-Muram, MD
Wegener Granulomatosis, Pulmonary 11-4-28
/. Michael Holbert, MD, FACR Apical Pleural Cap 11I-1-10
/lid W Gllrney, MD, FACR
Veno-Occlusive Disease, Pulmonary 11-4-32
Sliial R. Desai, MD, FRCp, FRCR Systemic Lupus Erythematosus, Pulmonary 11I-1-12
Patricia /. Mergo, MD

Toxic - Metabolic
Infectious
Takosis, Pulmonary 11-4-34
/lid W Gllrney, MD, FACR Empyema 11I-1-16
Kilt Slwffer, MD, PhD
Illicit Drug Abuse, Pulmonary 11-4-38
/lid W Gllrney, MD, FACR Broncho-Pleural Fistula 11I-1-20
Kitt SJwffer, MD, PhD
Smoke Inhalation 11-4-42
/lid W Gurney, MD, FACR

Silo-Filler's Disease 11-4-46

Helen T. Winer-Muram, MD

xx
 Neoplastic SECTION 3
Metastasis, Pleural 11I-1-24 Diaphragm
Patricia I. Mergo, MD

Malignant Mesothelioma 11I-1-28

Illd W Gumey, MD, FACR
Congenital
Pancoast Tumor 11I-1-32 Eventration of Diaphragm 11I-3-2
Illd W Gllmey, MD, FACR Eric I. Stem, MD

Fibrous Tumor of Pleura 11I-1-36 Bochdalek and Morgagni Hernias 11I-3-6

Marc V. Gosselin, MD Illd W Gllmey, MD, FACR

Erdheim Chester Disease, Pleural 11I-1-40

Illd W Gllmey, MD, FACR Inflammatory - Degenerative
Phrenic Nerve Paralysis 11I-3-10
Vascular Eric I. Stem, MD

Transudative Pleural Effusion 11I-1-44

Helen T. Winer-Mllram, MD
Hemangioendothelioma
Kitt Shaffer, MD, PhD
11I-1-48 PART IV
Spedal Topi
Toxic - Metabolic
Asbestos Related Pleural Disease 11I-1-52 Introduction and Overview

';;~~~i:
SlIjal R. Desai, MD, FRCI~ FRCR Introduction to Speciai Topics IV-0-2

_____ ~_~_~_~_:_~_N_a_li 1 I ,,,' ~ G"""" MD

Congenital
Lobar Atelectasis IV-I-2
Pectus Deformity 11I-2-2 Helen T. Winer-Muram, MD
Tomas Franqllet, MD
Subsegmentai Atelectasis IV-I-6
Kyphoscoliosis 11I-2-4 Helen T Winer-Muram, MD
Helen T. Winer-Muram, MD
Round Atelectasis IV-I-lO
Poland Syndrome 11I-2-8 Helen T. Winer-Murmu, MD
Tomas Franqllet, MD

Infectious SECTION 2
Empyema Necessitatis 11I-2-10
Trauma
Helen T. Winer-Mllram, MD
Pneumomediastinum IV-2-2
Eric /. Stem, MD
Inflammatory - Degenerative Pneumothorax IV-2-6
Ankylosing Spondylitis 11I-2-]4 Eric I. Stem, MD
Alexander Bankier, MD Tracheobronchial Tear IV-2-1O
Elastoma, Fibroma, and Fibromatosis 11I-2-]8 Eric /. Stem, MD
Tomas Franqllet, MD Lung Trauma IV-2-12
Eric j. Stem, MD

Neoplastic Rib Fracture and Flail Chest IV-2-16

Eric I. Stem, MD
Lipoma, Chest Wall 11I-2-22
/. Michael Holbert, MD, FACR Aortic Transection IV-2-20
Patricia /. Mergu, MD
Lymphoma, Chest Wall 11I-2-26
Nicholas D. Krallse & Tan-Lucien Mohammed, MD, FCCP Spinal Fracture IV-2-24
Eric I. Stem, MD
Askin Tumor, Chest Wall 11I-2-28
Helen T Winer-Muram, MD Sternal Fracture IV-2-28
Tonuis Franqllet, MD

Diaphragmatic Tear IV-2-30

Eric /. Stem, MD

Esophageal Tear IV-2-34

Tomas Frallquct, MD

XXI
Splenosis IV-2-38 Cardiac Volvulus IV-4-S0
Iud W Gllrney, MD, FACR Illd W Gurney, MD, FACR

Air Embolus, Pulmonary IV-2-42

Helen T. Winer-Mllram, MD

Thoracic Duct Tear IV-2-46

SECTION 5
Nicholas D. Krallse & Tan-Lucien Mohammed, MD, FCCI' Physiology
Ventilation and Lung Volumes IV-S-2
SECTION 3 Illd W Gllrney, MD, FACR

Lung Cancer Lung Perfusion IV-S-6

Illd W Gllmey, MD, FACR

Non-Small Cell Lung Cancer IV-3-2 Gravitational Lung Gradients IV-S-lO

lames G. Ravenel, MD Illd W Gllmey, MD, FACR

Small Cell Lung Cancer IV-3-6 Aspiration IV-S-14

lames G. Ravenel, MD Tomas Franquet, MD

Staging of Lung Cancer IV-3-1O

lames G. Ravenel, MD
IV-3-14
SECTION 6
Regional Lymph Node Classification
lames G. Ravenel, MD Special Patients
Solitary Pulmonary Nodule IV-3-16
Helen T. Winer-Muram, MD Metastases, Chest IV-6-2
Illd W Gurney, MD, FACR
Lung Cancer Screening IV-3-20
Iud W Gurney, MD, FACR Drug Reaction, Intrathoracic IV-6-6
Helen T. Winer-Mllram, MD
Missed Lung Cancer IV-3-24
Charles S. White, MD Radiation-Induced Lung Disease IV-6-10
f. Michael Holbert, MD, FACR
Immunosuppressed (Not AIDS) IV-6-14
SECTION 4 Illd W Gurney, MD, FACR

Portable ICU AIDS IV-6-18

Philip M. Boiselle, MD

Non-Surgical Sickle Cell Disease IV-6-22

Helen T. Winer-Muram, MD
Normal Tubes and Catheters IV-4-2
Helen T. Winer-Muram, MD

Abnormal Tubes and Catheters

Helen T. Winer-Muram, MD
IV-4-6
PART V
Cardiac Pacemakers IV-4-1O Patterns
lames G. Ravenel, MD

Pleurodesis IV-4-14
Kitt Shaffer, MD, PhD Introduction and Overview
Introduction to Patterns V-O-2
Iud W Gurney, MD, FACR
Surgical
Median Sternotomy
Philip M. Boiselle, MD
Thoracotomy
Helen T. Winer-Muram, MD

Esophageal Resection
Tomas Franquet, MD
Thoracoplasty and Apicolysis
Iud W Gllmey, MD, FACR

Pneumonectomy
Illd W Gllmey, MD, FACR

Lung Volume Reduction and Bullectomy

f. Michael Holbert, MD, FACR
Lung Transplantation
Alexander Bankier, MD

Posttransplant Lymphoproliferative Dz
Alexander Bankier, MD

Lobar Torsion, Lung

Iud W Gurney, MD, FACR

XXII
Cystic Lung Disease V-1-30
Alexander Bank;er, MD

HRCT: Bronchocentric Pattern V-I-34

Illd W. Gllmey, MD, FACR

HRCT: Lymphatic Pattern V-I-38

Illd W. Gllmey, MD, FACR

HRCT: Ground-Glass Opacities V-I-42

Eric /. Stern, MD
HRCT: Mosaic Pattern of Lung Attenuation V-I-46
Eric /. Stem, MD

HRCT: Tree-In-Bud Pattern V-I-SO

Eric I. Stem, MD

Anterior Mediastinal Mass V-I-54

lames G. Ravenel, MD

Middle Mediastinal Mass V-I-58

lames G. Ravenel, MD

Posterior Mediastinal Mass V-I-62

lames G. Ravenel, MD

XXIII
XXIV
 PA

Introduction an Ov

Airspa(;e II]
Interstitium rn
Airways [lJ
 SECONDARY PULMONARY LOBULE
o
2
 SECONDARY PULMONARY LOBULE
o
4

Gross path%sy, section sIK7N5 diffuse puImonaty

edema uniformly thickening the intet/obular septa
(anows). lDbuIes are either edemallM (open arru.v) or
spared (anved amlW).

Enlhelm-Chester disease
Alveolar Filling Pattern
Pneumonia
Edema, cardiogenlc and noncardiogenic
Hemorrhage
Alveolar proteinosls
EosInophilIc pneumonias
Desqwunative Interstitial pneumonitis
Neoplastic (lipidic growth)
Capillary Pattern (also Called Random
Pattern)
Hematogenous metastases
Miliary Infection
Lobular Septal Pattern
Usual Interstitial pneumonitis
Chronic hypersensItivity pneumonitis
Nonspecific Interstitial pneumonitis
Asbestosis

I RELATED REFERENCES I
1. Arakawa'H et aI: Expiratory hlgh-reso1utlon en dIqnostic;
value In diffuse hmg diseases. 1JR Am J Roentgenol.
175(6):1537-43,2000
Z. TakahashI M et aI: Bronchiolar disease: spectrum and
flIdiologltal f1ndlnp.l!ur J Radlol. 35(1):15-29, ZOOO
t;otby 1V et aI: Anatomic distribution and histopathologic
patter:nt In diffuse lung disease: CXlrrelationwith mer. J
1borac ImqIng. It(I):I-Z6, 1996
HeltmWI ERet Ill:The secondary pulmonary lobule: a
practical (:Qllt1ept for interpretation of chest radiographs. I.
Roentgen anatomy of the noanaI secondary pulmonary
lobule. RadIology. 93(3):507-12, 1969
 SECONDARY PULMONARY LOBULE

I IMAGE GALLERY o
5
(l.e#'f) Axial HRCT shows
numerous localized
perivascular and subpleural
nodules. The remainder of
the lung is normal. fIliJhO
Axial HRCT 10 mm MIP
asain demonstrates
perivascul nodules but also
shows numerous other
centrIacinar nodules
(amN/IS) not visible on the 1
mm thidt. scan. DiatIfrosis:
SaICOidosis.

(l.e#'f) RaeJiosraph shows

bronchial branching pIIII1em
in secondary puItrroMry
lobule. Large COte bronchiole
(open arrow), tapers 10 3
generations at terminal
bronchioles (amNVS) and
!hen 2 respitaIOry
bronchioles (curved arrow).
~ Axial HRCT shows
small lobular branching
opacities (amNVS) and
numerous centrilobular
nodules some of which haIIe
ground-pw heJos (curved
arrows). InlTaVascular IUmor
emboli.

(l.e#'f) Axial HRCT shows

markedly irresularly (and
beIlded - arrow) /merIobuIar
septal th~ and
thicken"" of bronchiole
walls (cuned anuw) from
Iymphanslrlc IUmor. /JIWIO
Axial HRCT shows duster at
ce""iIobtMr noduIeJ
(anows) In IIdditJon 10
subpleural nodules and
~tcuwed
arrows) II.". _~mphatic
distribulion. Silicosis.
 Infectious
Bacterial Pneumonia 1-1-2
Staphylococcus Pneumonia 1-1-6
Mycobacterial Pneumonia 1-1-8
Lung Abscess 1-1-12
Histoplasmosis 1-1-16
Aspergillosis 1-1-20
Blastomycosis 1-1-24
Coccidioidomycosis 1-1-26
Parasitic Pneumonia 1-1-28

Vascular
Cardiogenic Pulmonary Edema 1-1-32
Noncardiac Pulmonary Edema 1-1-36
Fat Pulmonary Embolism 1-1-40
Diffuse Alveolar Hemorrhage 1-1-44
Goodpasture Syndrome 1-1-48

Inflammatory - Degenerative
Eosinophilic Pneumonia 1-1-52
Acute Interstitial Pneumonia 1-1-56

Toxic - Metabolic
Metastatic Pulmonary Calcification 1-1-60
Alveolar Microlithiasis 1-1-64
Lipoid Pneumonia 1-1-68
Pulmonary Alveolar Proteinosis 1-1-72
Desquamative Interstitial Pneumonia 1-1-76
Cryptogenic Organizing Pneumonia 1-1-80

Neoplastic
Bronchioloalveolar Cell Carcinoma 1-1-84
 BACTERIAL PNEUMONIA
1
2

Graphic shows how inflammatory exudate begins in Frontal radiograph shows focal lobar consolidation in
distal air spaces spreading through the airways to the left upper lobe. No complications: Pleural effusion,
adjacent alveoli. Eventually will spread to adjacent cavitation, or adenopathy. Most common community
segments through pores of Kohn. acquired pneumonia is S. pneumonia.

Basilar segments in upright position

I TERMI NOLOGY Posterior segments upper lobes and superior
Abbreviations and Synonyms segments lower lobes in supine position
Bronchopneumonia, lobar pneumonia, lung infection, Axillary upper lobe subsegments in decubitus
pneumonitis position

Definitions Radiographic Findings

Radiography
Pathogenic infection of the lung due to bacteria
o Nearly any pattern from consolidation to interstitial
o Diagnosis based on culture, gray scale does not
thickening, may be focal or diffuse (multilobar)
substitute for gram stain
o Lobar vs. bronchopneumonia
Pathologic designation rarely helpful
radiographically
I IMAGING FINDINGS Difficult to reliably identify (poor interobserver
General Features agreement)
Best diagnostic clue o High sensitivity, exceptions, may not have visible
o Focal parenchymal abnormality in patient with abnormality in
fever Immunocompromised patients, especially if
Absence of parenchymal abnormality excludes neutropenic
pneumonia (except in immunocompromised) Dehydration: Controversial; rare if it exists at all
Pattern not diagnostic of an organism, an o Lobar consolidation
individual organism may cause multiple patterns Maintain high suspicion for postobstructive
Location pneumonia
o Aspiration preferentially to dependent lung

DDx: Bacterial Pneumonia

Pulmonary Edema Aspiration Hemorrhage

 BACTERIAL PNEUMONIA
Key Facts
Terminology
Diagnosis based on culture, gray scale does not
substitute for gram stain
Imaging Findings
Absence of parenchymal abnormality excludes
pneumonia (except in immunocompromised)
Pattern not diagnostic of an organism, an individual
organism may cause multiple patterns
Time table: Expected 50% resolution 2 weeks; 66% 4
weeks; 75% 6 weeks, 90% 8 weeks
Top Differential Diagnoses
Edema
Hemorrhage
Aspiration
Atelectasis
Common organisms: S. pneumonia, S. aureus,
Klebsiella (especially with lobar expansion),
Legionella, anaerobic or gram-negative organisms
o Diffuse interstitial pattern
Unusual, more common with mycoplasma or viral
pneumonia, can be seen with S. au reus
o Hilar adenopathy
Rare, limits differential: Tuberculosis,
mycoplasma, fungi, mononucleosis, measles,
plague, tularemia, anthrax, pertussis
o Parapneumonic effusion vs. empyema
Loculation suggests empyema (simple effusions in
those with adhesions also loculate)
Large effusions suggest anaerobic, gram-negative
organisms, or S. aureus
Effusions on 1st film suggest S. aureus or S.
pneumoniae, late effusions suggest gram-negative
or anaerobic organisms
o Pneumatoceles
Develop later in course of pneumonia (classically
S. aureus or Pneumocystis jiroveci)
May persist for months, but usually spontaneously
resolve
o Cavitation (abscess)
More common in upper lobes
Common organisms: S. aureus, anaerobes or
gram-negative bacteria
o Resolution
Faster in nonsmokers and outpatients (within 2-3
weeks)
Time table: Expected 50% resolution 2 weeks; 66%
4 weeks; 75% 6 weeks, 90% 8 weeks
Delayed with advancing age, involvement of
multiple lobes, or complications (abscess,
empyema)
Recommend follow-up 6 weeks (unless symptoms
worsen)
o Recurrent pneumonia
> 90% have underlying systemic disease, when
recurrence is limited to the same lobe a focal
structural abnormality should be sought
o Interobserver agreement
1
Infarcts 3
Bronchioloalveolar Cell Carcinoma
Bronchiolitis Obliterans-Organizing Pneumonia
(BOOP)
Chronic Eosinophilic Pneumonia
Farmer's Lung
Pathology
Offending organism cultured in < 50%
Clinical Issues
Pulmonary cavity in edentulous patients = lung
cancer
5th leading cause of death
Bilateral pleural effusions and multilobar disease
associated with increased mortality
Good for detection of consolidation, multilobar
distribution and pleural effusion, poor for pattern
of abnormality and detection of air bronchograms
CT Findings
CECT
o Evaluate cause in patients with recurrent pneumonia
Recurrent pneumonias consider: Bronchogenic
carcinoma, bronchiectasis,
tracheobronchomegaly, COPD, pulmonary
alveolar proteinosis, sequestration, esophageal
diverticulum, right middle lobe syndrome
o Separate abscess from empyema
Abscess: Thick, irregular wall; round shape, narrow
contact with chest wall
Empyema: Thin, uniform wall; lenticular shape,
broad contact with chest wall, split pleura sign
Imaging Recommendations
Best imaging tool: Chest radiographs suffice for
detection and to document response to therapy, detect
complications and underlying disease masked by
infection
Protocol advice
oCT
Useful in immunocompromised with normal
chest radiographs
More sensitive and specific for complications
Useful to screen for underlying structural
abnormalities such as bronchiectasis
I DIFFERENTIAL DIAGNOSIS
Edema
Cardiomegaly and pulmonary venous hypertension
Edema will shift with position (gravitational shift test)
Hemorrhage
Patients usually anemic and often have hemoptysis
(80%)
 BACTERIAL PNEUMONIA
1 Aspiration
Organism identification
acid-fast)
with special stains (Gram or

4
Typical predisposing conditions: Esophageal motility
disorder, obtundation, alcoholism
Atelectasis IClINICAllSSUES
Fissural displacement or other signs of volume loss Presentation
Infarcts Most common signs/symptoms
Resolution: Infarcts "melting snowball" sign, in o Acute onset fever, chills, cough, sputum production
contrast pneumonia fades o Elevated white blood cell count with left-shift
Other signs/symptoms
Bronchioloalveolar Cell Carcinoma o Sputum: Bloody with Klebsiella and S. pneumonia,
Often course of antibiotics tried for pneumonia green in Pseudomonas and Haemophilus,
Suspect if consolidated lung fails to clear 6-8 weeks foul-smelling with anaerobic organisms
after antibiotic treatment o Consolidation + bacteremia = pneumonia
o Pulmonary cavity in edentulous patients = lung
Bronchiolitis Obliterans-Organizing cancer
Pneumonia (BOOP) o Empyema: May be surprisingly free of toxic
Patients often treated for pneumonia for variable symptoms
length of time o Diarrhea common in Legionella
Clinical Profile
Chronic Eosinophilic Pneumonia o Infection source
Typically peripheral upper lobe consolidation Hospital acquired: S. aureus, anaerobes, and
Will not respond to antibiotics (clinical course will gram-negative organisms
wax and wane giving the impression of antibiotic Community acquired: Pneumococcus,
response) mycoplasma, viruses, less commonly S. aureus,
Legionella, Klebsiella
Farmer's Lung o Associated conditions
Farmer's lung often mistaken as pneumonia, usually
Splenectomy: S. pneumoniae
self-limited and antibiotics assumed to be successful Cystic fibrosis: Pseudomonas aeruginosa
History of antigen exposure COPD: Hemophilus influenza, Branhamella
catarrhalis
Sickle cell: S. pneumoniae
I PATHOLOGY Aspiration: Anaerobic organisms, gram-negative
General Features bacteria, S. aureus, Actinomyces
General path comments Demographics
o Offending organism cultured in < SO% Age: Any age, elderly at increased risk
o Portal of entry inhalation or aspiration oral
secretions Natural History & Prognosis
Epidemiology: More common in winter months (due 5th leading cause of death
to viral infections) except for Legionella that is more Prognosis depends on virulence of organism,
common in summer antibiotic susceptibility and host response
Prognostic radiographic findings
Gross Pathologic & Surgical Features o Bilateral pleural effusions and multilobar disease
Lobar vs. bronchopneumonia associated with increased mortality
o Lobar
Alveolar flooding with inflammatory exudate, Treatment
especially neutrophils Pneumococcal and influenza immunization for at risk
Rapidly spreads throughout lobe, only stopped by adults
intact fissures Appropriate antibiotics
Usually originates in peripheral lung Drain empyemas, not abscesses
Produces "round pneumonia" if located within Bronchoscopy for recurrent disease in same location
center of lobe (lipidic growth)
o Bronchopneumonia
Exudate centered on terminal bronchioles I DIAGNOSTIC CHECKLIST
(centrilobular)
Respects septal boundaries Consider
Patchy: Adjacent lobules may be normal Included in differential of nearly any focal or diffuse
producing patchwork quilt pattern lung disease, especially in patient with fever

Microscopic Features
Nonspecific acute and/or chronic inflammatory cells I SElECTED REFERENCES
1. Geppert EF: Recurrent pneumonia. Chest. 98: 739-45, 1990
 BACTERIAL PNEUMONIA
I IMAGE GALLERY 1
5

(Leh) Frontal radiograph

shows lingular consolidation
(arrow) and moderate sized
le(t pleural effusion (open
arrow). In pneumonia,
effusion may be either
parapneumonic or
empyema. (Right) Axial
CECT shows the lingular
pneumonia with air
bronchogram (arrow) and
the pleural effusion (open
arrows) with adjacent
passive atelectasis lefl/ower
lobe. Diagnostic tap
demonstrated an exudative
parapneumonic effusion.

Variant
(Leh) Fron/al radiograph
shows focal consolidation in
the axillary subsegmen/ of
the right upper lobe
consistent with pneumonia
in teenager with fever and
cough. (Right) Frontal
radiograph 1 week later
demonstrates the
development of cavitation or
pneumatoceles in the area of
pneumonia. Discoid
atelectasis (arrow). Culture
proven S. aureus.

Other
(Left) Fron/al radiograph
shows dense homogeneous
consolidation in the right
middle lobe. Right heart
border silhouelled. No
pleural effusion, cavitation,
or adenopathy. (Right)
Lateral radiograph shows
expanded right middle lobe
with bulging fissures
(arrows). Klebsiella is the
typical organism for this
pattern. With complete lobar
opacification, the possibility
of an obstructing mass
should be considered.
 STAPHYLOCOCCUS PNEUMONIA
1
6

Frontal radiograph shows bilateral lower lobe dense Axial CECT shows dense consolidation in the right lower
opaciUes (arrows) from this patient with bilateral lobe and multiple small patchy areas of opacity in the
methicillin-sensitive Staphylococcus pneumonia. No right upper lobe, as a result of methicillin-sensitive
effusions evident. Staphylococcus pneumonia.

o Parapneumonic pleural effusion is very common, up

I TERMI NOlOGY to 2/3 of patients
Abbreviations and Synonyms Empyema due to methicillin-resistant organisms
becoming more common, especially in children
Staphylococcus aureus pneumonia
Location: Patchy bronchopneumonia with a
Nosocomial pneumonia
multisegmental distribution, frequently bilateral
Definitions Size: Ranging from small non-descript areas, to lobar
Lung infection caused by gram positive organism pneumonia, to diffuse alveolar damage and adult
Staphylococcus, usually S. aureus respiratory distress syndrome (ARDS)
Imaging Recommendations
I IMAGING FINDINGS Best imaging tool
o Chest radiographs for initial diagnosis and
General Features evaluation of severity usually suffices
Best diagnostic clue o CT scanning for detection of abscesses and
o Rapid onset patchy or lobar consolidation, marked empyema
by widespread, rapid, severe lung destruction with
abscess formation
Abscess can occur with a variety of different I DIFFERENTIAL DIAGNOSIS
bacterial pneumonias
Other Bacterial Pneumonias
Imaging shows thick-walled cavities, 30-40%
Abscesses can heal with pneumatocele formation Gram-positive organisms such as Streptococcus
(thin walled air-cyst in -2%), and can last for years pneumoniae and Streptococcus pyogenes
Gram-negative organisms such as Pseudomonas,
Klebsiella, Enterobacter, Serratia

DDx: Pneumonia

Pneumococcal Pneumonia Aspiration Laceration

 STAPHYLOCOCCUS PNEUMONIA

Key Facts
1
Imaging Findings Pathology 7
Rapid onset patchy or lobar consolidation, marked by Most common bronchopneumonia
widespread, rapid, severe lung destruction with Common cause of death during outbreaks of
abscess formation influenza
Location: Patchy bronchopneumonia with a
multi segmental distribution, frequently bilateral
Clinical Issues
Age: Children and elderly more susceptible, especially
Top Differential Diagnoses as sequelae to influenza
Other Bacterial Pneumonias

Aspiration ICLINICALISSUES
Can be bland with rapidly clearing perihilar opacities
Presentation
or polymicrobial pneumonia and lung abscess
Most common signs/symptoms: Acute, sudden febrile
Blunt Pulmonary Injury respiratory illness, with productive cough
Contusion and hemorrhage Other signs/symptoms: Viral prodrome during
Pulmonary lacerations infl uenza season
Demographics
Age: Children and elderly more susceptible, especially
I PATHOLOGY
as sequelae to influenza
General Features Gender: Women may have a higher colonization of S.
Genetics: Staphylococcus aureus strains carrying the aureus
gene for the Panton-Valentine leukocidin (PVL) cause Natural History & Prognosis
rapidly progressive, hemorrhagic, necrotizing
Mortality varies widely depending upon the strain of
pneumonia, typically in otherwise healthy children
organism, expression of virulence, and host factors
and young adults
Imaging follow-up depends upon underlying
Epidemiology co-morbidities and complications
o Most common bronchopneumonia
o Vast majority are hospital acquired Treatment
o Common cause of death during outbreaks of Methicillin-resistant Staphylococcus aureus (MRSA)
influenza pneumonia becoming more common
Community acquired in these instances
Increased morbidity and mortality in elderly
Microscopic Features I SELECTED REFERENCES
Gram-positive cocci that occur in clusters 1. Hodina M et al: Imaging of cavitary necrosis in
S. aureus commonly colonizes the nasal passages compiicated childhood pneumonia. Eur Radiol.
12(2):391-6,2002
2. Macfarlane J et al: Radiographic features of staphylococcal
pneumonia in adults and children. Thorax. 51(5):539-40,
1996

I IMAGE GALLERY

(Left) Frontal radiograph shows a left lower lobe focal opacity as a result of methicillin-resistant Staphylococcus pneumonia. (Center) Frontal
radiograph shows diffuse lung opacities from this patient with methicillin-resistant Staph pneumonia induced ARDS. (Right) Axial CECT obtained
within a few days of initial hospitalization shows multiple cavitary lesions scallered throughout the lungs secondary to methicillin-resistant Staph
pneumonia abscesses.
 MYCOBACTERIAL PNEUMONIA

Coronal graphic shows reactivation tuberculosis with Frontalradiograph shows distortion of lung markings in
cavitary disease in the apical lung segments. the right upper lobe with cavitation (arrows) consistent
Bronchogenic spread to the dependent right lower lobe with fibrosis and bronchiectasis from reactivation
and apicalpleural thickening are also seen. tuberculosis.

Miliary: Diffuse tiny relatively well-defined

I TERMINOlOGY nodules without adenopathy or effusion
Abbreviations and Synonyms Reactivation: Apical fibrosis, cavitation,
Mycobacterial pneumonia (MTB) calcification
Tuberculosis (TB), consumption o Most infected patients have a + purified protein
derivative (PPO) and a normal chest radiograph
Definitions Location
Indolent bacterial (mycobacterium tuberculosis) o Primary: Any lobe
infection, often relapsing course, associated with o Miliary: Diffuse, nodules often larger in upper lung
fibrosis, calcification and adenopathy zones with chronic disease
o Varying appearance depending on time course o Reactivation: Apical and apical posterior segments of
Primary tuberculosis, initial infection upper lobes
Miliary tuberculosis, overwhelming infection a May also involve breast (tuberculous mastitis)
Reactivation tuberculosis, recurrent infection a May also involve spine (Pott disease)
Size
a Primary: May be lobar or segmental
IIMAGING FINDINGS a Miliary: Multilobar involvement
a Reactivation: Generally limited to apical or apical
General Features posterior segments
Best diagnostic clue Morphology
o Depends on the type of disease present: Primary, a Primary: Airspace opacity with or without
miliary or reactivation adenopathy
Primary: Airspace consolidation with adenopathy, a Miliary: Nodular disease, uniform 3-4 mm in
effusion common diameter

DDx: Chronic Lung Opacities

Lung Adenocarcinoma Pulmonary Lymphoma Progressive Massive Fibrosis

 MYCOBACTERIAL PNEUMONIA
Key Facts
1
Terminology Top Differential Diagnoses 9
Indolent bacterial (mycobacterium tuberculosis) Chronic Fungal Infection
infection, often relapsing course, associated with Ankylosing Spondylitis
fibrosis, calcification and adenopathy Progressive Massive Fibrosis (PMF)
Varying appearance depending on time course Sarcoidosis
Bronchogenic Carcinoma
Imaging Findings Pulmonary Lymphoma
Primary: Airspace consolidation with adenopathy,
effusion common Pathology
Miliary: Diffuse tiny relatively well-defined nodules Increased susceptibility in patients with impaired
without adenopathy or effusion cellular immunity
Reactivation: Apical fibrosis, cavitation, calcification Incidence world-wide has increased in part due to
Most infected patients have a + purified protein Human immunodeficiency virus (HIV) infection
derivative (PPD) and a normal chest radiograph Acid-fast bacilli located in macro phages, obligate
May also involve spine (Pott disease) aerobe
May present with adenopathy alone

o Reactivation: Fibrosis and scarring, cavitation, o Miliary disease: Profuse uniform distribution of 2-3
bronchiectasis, retraction, calcification mm nodules
Complications o Lymph nodes: Low density center and peripheral
o Fibrosing mediastinitis rim-enhancement
o Empyema, may burrow into chest wall (empyema
necessitatis) Imaging Recommendations
o Bronchopleural fistula Best imaging tool
o Pericardial involvement may give rise to constrictive o Chest radiography for initial detection
pericarditis Usually sufficient for monitoring therapy
o Hemoptysis may be due to Rasmussen aneurysm,
mycetomas, bronchiectasis, or broncholith
o Fibrosis and retraction can cause secondary I DIFFERENTIAL DIAGNOSIS
bronchial obstruction Chronic Fungal Infection
o Mycetomas-saprophytic aspergillus colonization in
Histoplasmosis, coccidiomycosis, sporotrichosis
cavitary areas
Resembles post-primary TB
Radiographic Findings
Ankylosing Spondylitis
Radiography
Associated spine changes, TB must be excluded by
o Primary: Airspace opacities in one lobe
culture
Unilateral hilar or mediastinal adenopathy
May present with adenopathy alone Progressive Massive Fibrosis (PMF)
Can spread if untreated to other PMF masses may cavitate, usually located in upper
lobes-(bronchogenic spread) lobes
Effusions may be small or large Appropriate occupational exposure history
Cavitation uncommon Increased incidence of tuberculosis, TB must be
o Miliary: Diffuse nodules excluded by culture
Discrete, uniform, small
Evenly distributed, but may appear more Sarcoidosis
numerous in upper lobes chronically Early stage may mimic primary TB with chronic lung
o Reactivation: Upper lobe fibrosis opacities and adenopathy
Cavitation, distortion Late stage may mimic reactivation TB with cavitation,
Calcification fibrosis (adenopathy absent)
No adenopathy
Bronchogenic Carcinoma
CT Findings Adenopathy and chronic lung opacities, most often in
NECT: Can show calcifications in lung, hila, or bronchoalveolar cell type
mediastinum that may be missed with contrast Cavitation most common in squamous cell carcinoma
May demonstrate findings not appreciated with
radiography Pulmonary Lymphoma
o Bronchogenic spread Adenopathy and chronic lung opacities
Peribronchial patchy opacities or centrilobular Cavitation rare
rosettes May have associated low grade fever, weight loss
Branching nodulation: Tree-in-bud appearance
 MYCOBACTERIAL PNEUMONIA
1 Other Calcified Chest Abnormalities
o Variable: Primary pneumonia often asymptomatic,
miliary disease with nonspecific malaise and weight
10 Chondroid tumors, chondrosarcoma, osteochondroma loss
Pleurodesis with pleural calcifications o Infection often subacute
Calcified fungal disease May not have high fevers
Other Chest Infections with Adenopathy May be advanced at time of diagnosis
o Chronic symptoms
Plague
Brucellosis Cough
Tularemia Low grade fever
Infectious mononucleosis Malaise
Weight loss
Measles
o Pneumonia unresponsive to standard antibiotics
Fungal infections
Demographics
Age: Any age
I PATHOLOGY
Natural History & Prognosis
General Features Primary disease self-limited, many years later may
General path comments develop reactivation
o Caseating granuloma due to mycobacterial infection Variable depending on drug resistance and health of
o Primary TB host
Delayed hypersensitivity 4-10 weeks after initial
exposure, then +PPD Treatment
Pneumonia with caseous necrosis and regional Respiratory isolation for cavitary disease or grossly
lymphadenitis positive sputum smear until antibiotics instituted
Pulmonary focus may evolve into tuberculomas Anti-tuberculous drugs depending on sensitivity
o Reactivation TB o Public health issues with drug resistance
Immediate hypersensitivity Inadequate treatment increases resistance
Pneumonia, cavity formation In many third-world countries, complex
Scarring, distortion, bronchiectasis, multi-drug treatment regimens needed
bronchostenosis, cysts, bullae o Poor response to treatment, consider AIDS or
Etiology drug-resistant TB
o Increased susceptibility in patients with impaired Pleural effusion (pleurisy) does not require chest tube
cellular immunity drainage, will resolve with antibiotics
HIV positive, elderly, prisoners, indigent and o Tuberculous empyema requires chest tube drainage
homeless Bronchial artery embolization or surgery for
Epidemiology hemoptysis
o Incidence world-wide has increased in part due to
Human immunodeficiency virus (HIV) infection
May have different presentation in HIV-infected I DIAGNOSTIC CHECKLIST
patients
HIV-infected patients may not respond normally Consider
to skin testing Reactivation tuberculosis with upper lobe nodular or
HIV-infected patients may not respond normally tubular opacities
to standard TB therapy Image Interpretation Pearls
o Kills 4 people per minute worldwide
Acutely, look for adenopathy
o Second only to acquired immune deficiency
Chronically, look for calcification and cavitation
syndrome (AIDS) in terms of infectious mortality
Consider other organ involvement (breast, spine,
Gross Pathologic & Surgical Features kidneys)
In cases of chronic infection, surgical resection may
become necessary
I SElECTED REFERENCES
Microscopic Features 1. Lee CH et al: Response to empirical anti-tuberculosis
Acid-fast bacilli located in macrophages, obligate treatment in patients with sputum smear-negative
aerobe presumptive pulmonary tuberculosis. Respiration.
72(4):369-74,2005
2. Okeke IN et al: Antimicrobial resistance in developing
IClINICAllSSUES countries. Part II: Strategies for containment. Lancet Infect
Dis. 5(9):568-80, 2005
Presentation 3. Nuermberger Eet al: Latent tuberculosis infection. Semin
Most common signs/symptoms Respir Crit Care Med. 2S(3):317-36, 2004
4. Goo JM et ai: cr of tuberculosis and non tuberculous
mycobacterial infections. Radiol Clin North Am. 40(1):
73-87,2002
 MYCOBACTERIAL PNEUMONIA

I IMAGE GALLERY 1
11
Typical
(Left) Frontal radiograph
shows extensive airspace
opacity throughout the right
lung and focal airspace
opacity (arrows) in the left
lung from bronchogenic
spread. (Right) Frontal
radiograph shows diffuse left
pleural thickening with
dense calcification
producing a fibrothorax as a
long term sequela of
tuberculous empyema.

(Left) Frontal radiograph

shows a calcified right hilar
granuloma (arrow) and an
adjacent calcification in the
right mid lung (curved
arrow). (Right) Axial NEeT
shows calcified right hilar
node (arrow) and calcified
right middle lobe granuloma
(curved arrow).

Variant
(Left) Frontal radiograph
shows lobular mediastinal
widening without obvious
lung abnormalities. (Right)
Axial NEeT shows paraspinal
mass (cold abscess) with
erosion of adjacent spine
(arrow) on bone window
images. There is also a
calcified right paratracheal
node (curved arrow).
 LUNG ABSCESS
1
12

Frontal radiograph shows airspace opacification in the Lateral radiograph shows an abscess in an
right lower lung (arrow) with a large cavity and an air over-expanded right middle lobe (curved arrows). The
fluid level (open arrow). location suggests aspiration. Cultures showed mixed
aerobic and anaerobic bacteria.

Location
I TERMINOLOGY o Posterior segments of upper lobes or superior
Abbreviations and Synonyms segments of lower lobes, in supine patients
Subacute lung infection, necrotizing pneumonia, o Lower lobes and right middle lobe, in upright
pulmonary gangrene, phlegmon patients
o Unilateral or bilateral; right side more common than
Definitions left
Cavitary parenchymal process secondary to an o Usually one focus of disease, but multilobar also
infectious process common
Cavity refers to an air containing lesion with a Size: < 1 cm to > 10 cm
relatively thick wall (> 4 mm) or within an area of a Morphology: Spherical thick-walled cavity with
surrounding opacity or mass irregular inner and outer margin
Radiographic Findings
I IMAGING FINDINGS Radiography
o Initially, fluid filled cavity may appear mass-like
General Features o Thick-walled spherical cavity with irregular inner
Best diagnostic clue wall, often containing air fluid level
o Irregular thick walled cavity, often containing air o To distinguish abscess from empyema
fluid level Cavity: Spherical, equal air fluid levels on frontal
o May begin as a focus of consolidation and evolve and lateral views, acute angles with chest wall
into an abscess over days or weeks Empyema: Lenticular, unequal air fluid levels on
o Often in dependent lung because a common cause is frontal and lateral views, obtuse angles with chest
aspiration wall

DDx: Cavitary Lesions

MAl Pneumonia Lung Cancer Wegener

 LUNG ABSCESS

Key Facts
1
Imaging Findings Bronchopleural fistula: Development of 13
Irregular thick walled cavity, often containing air hydropneumothorax, empyema
fluid level Top Differential Diagnoses
May begin as a focus of consolidation and evolve into Pneumatocele Formation
an abscess over days or weeks Other infections: Tuberculosis (TB), fungal infections,
Often in dependent lung because a common cause is Pneumocystis pneumonia (PCP)
aspiration Neoplasm: Primary lung or metastases (squamous cell
Posterior segments of upper lobes or superior carcinoma, adenocarcinoma), lymphoma
segments of lower lobes, in supine patients
Initially, fluid filled cavity may appear mass-like Clinical Issues
Cavity: Spherical, equal air fluid levels on frontal and Good prognosis with early diagnosis and treatment
lateral views, acute angles with chest wall Bronchoscopy to assess for an endobronchial lesion
Abscess: Thick, irregular wall, spherical, small contact or foreign body if medical treatment has failed
with chest wall, bronchovascular markings extend Percutaneous drainage, controversial
toward the abscess

o Lower lobe abscesses are usually larger than upper

lobe abscesses
o Extensive peri cavitary consolidation suggests abscess
rather than neoplasm
o Airspace nodules and confluent opacities in
dependent lung (bronchogenous spread)
CT Findings
Initially abscess may appear mass-like with central low
attenuation, Le., liquified central necrotic process
Air fluid level or central air collection indicates
bronchial communication
Cavity wall thickness: Variable, 4 mm to < IS mm;
thick wall is more common
o Luminal interior wall is usually shaggy
o Thin-walled cavity seen with coccidioidomycosis,
paragonimiasis, hydatid, Pneumocystis,
pneumatocele, bulla
Pericavitary opacification (airspace or ground-glass)
may show air-bronchograms or tiny air bubbles
CT to distinguish abscess from empyema: Can occur
together
o Abscess: Thick, irregular wall, spherical, small
contact with chest wall, bronchovascular markings
extend toward the abscess
o Empyema: Thin uniform wall, lenticular shape,
broad contact with chest wall, split pleura sign,
adjacent compressed lung
Reactive hilar and mediastinal lymphadenopathy
occur
Bronchopleural fistula: Development of
hydropneumothorax, empyema
Necrotic debris: Suggests pulmonary gangrene
Air crescent: Suggests invasive aspergillosis or
mycetoma in a pre-existing cavity
Imaging Recommendations
Best imaging tool: CT to distinguish pulmonary
abscess from empyema, and to evaluate for associated
lung cancer
Protocol advice: Standard CECT
I DIFFERENTIAL DIAGNOSIS
Pneumatocele Formation
Difficult to distinguish from abscess, especially in
Staph pneumonia
Other Infections
Other infections: Tuberculosis (TB), fungal infections,
Pneumocystis pneumonia (PCP)
o Usually patients do not have abundant foul smelling
expectorate
o Bilateral upper lobe location raises possibility of TB
Neoplasm
Neoplasm: Primary lung or metastases (squamous cell
carcinoma, adenocarcinoma), lymphoma
o Cavity wall> IS mm suggests tumor
Septic Embolism
CT demonstration of a feeding vessel to the cavity
Endocarditis, extrathoracic site of infection or infected
catheter
Wegener Granulomatosis
Nodules or masses, with or without cavitation, air
fluid levels rare
History of sinus disease, acute renal disease
Necrobiotic Rheumatoid Nodules, Caplan
may
Syndrome
Nodules or masses, with or without cavitation, air
fluid levels rare
History of rheumatoid arthritis, and/or dust inhalation
Pulmonary Infarct
Usually does not cavitate, no air fluid levels
Symptoms of pulmonary embolism, sudden onset of
dyspnea, hypoxia and chest pain
Infected Congenital Bronchogenic lung
Cyst, or Intralobar Sequestration
Recurrent pneumonias at the same site
 LUNG ABSCESS
1 !PATHOLOGY
14 General Features
Etiology
o ]n patients with aspiration often due to mixed
aerobic and anaerobic polymicrobial bacterial
infection
Anaerobes such as Peptostreptococcus,
Bacteroides, Fusobacterium, Microaerophilic strep
Pathogens such as Staphylococcus, Klebsiella,
Strep viridans, Pseudomonas, Melioidosis, E. coli,
Proteus, Nocardia, Actinomyces, Rhodococcus,
Pasteurellae
o May develop in patients with
inappropriate/inadequate antibiotic treatment of
pneumonia
o In patients with aspiration from decreased level of
consciousness, seizure disorder, alcohol abuse,
swallowing disorders, receiving mechanical
ventilation
o Predisposition in patients with immune deficiency,
bronchiectasis, malignancy, emphysema, steroid
treatment
o In patients with pulmonary gangrene
Pathogens such as S. pneumoniae, Klebsiella, TB,
anaerobic infection, Aspergillus, Mucormycosis
Epidemiology
o 70-80% are smokers; 12% have associated lung
cancer, (infected lung cancer rare)
o Up to 50% of patients develop effusions that
inevitably represent empyema
Associated abnormalities: May progress to empyema
and bronchopleural fistula
Gross Pathologic & Surgical Features
Vary in size from microscopic to large
Contain semi-solid or liquid pus
Parenchymal destruction that heals with scarring,
bronchiectasis, cyst formation
Uncommon complication, pulmonary gangrene with
necrotic lung fragments in abscess cavity (pulmonary
sequestrum)
Microscopic Features
Half the cases have anaerobic organisms alone that
must be cultured with anaerobic techniques
Gram stain of sputum classically polymicrobial with
many neutrophils
TB or Nocardia detected with acid fast stain; fungi
detected with silver stain
Fine needle aspiration biopsy, transbronchial biopsy or
open lung biopsy for actinomycosis
I CLINICAL ISSUES
Presentation
Most common signs/symptoms
o Often subacute illness
o Fever, leukocytosis in 90% of patients
o Expectoration of large amount of fluid; cough,
abundant foul smelling expectoration may be
variable
o Hemoptysis can occur, may be fatal
Other signs/symptoms
o Consider anaerobic infection in patients with
periodontal disease or foul smelling sputum
o Consider lung cancer in edentulous patient
Demographics
Age: Childhood to elderly, mainly 4-6th decades
Gender: M:F = 4:1
Natural History & Prognosis
Resolution is slower than non-cavitary pneumonia
Heals with scarring, bronchiectasis, cystic change
Good prognosis with early diagnosis and treatment
Mortality higher in elderly debilitated
immunocompromised patients with large abscesses
Mortality, 15-20%
Treatment
Usually responds to antibiotics, in contrast to
abscesses elsewhere that usually require tube drainage
Diagnosis can be obtained with CT or ultrasound
guided fine needle aspiration biopsy
No bronchoscopy in acute phase for abscess> 4 em
because of potential spillover of contents to normal
lung
Bronchoscopy to assess for an endobronchial lesion or
foreign body if medical treatment has failed
Less than 10% require surgery for non-resolving
abscess
Percutaneous drainage, controversial
o Reserved for non-resolving abscess or empyema that
abuts the chest wall
I DIAGNOSTIC CHECKLIST
Consider
CT to confirm abscess versus normal lung,
pneumatocele or emphysematous spaces interspersed
in areas of consolidation
CT to evaluate for complications such as empyema
and bronchopleural fistula
CT or ultrasound guided fine needle aspiration biopsy
for diagnosis of actinomycosis, fungal infection and
cancer
I SELECTED REFERENCES
1. Ryu JH et al: Cystic and cavitary lung diseases: focal and
diffuse. Mayo Clin Proc. 78(6):744-52, 2003
2. Mueller PR et al: Complications of lung abscess aspiration
and drainage. A)R Am) Roentgenol. 178(5): 1083-6,2002
3. Franquet T et al: Aspiration diseases: findings, pitfalls, and
differential diagnosis. Radiographies. 20(3):673-85, 2000
4. EM Marom et al: The many faces of pulmonary aspiration
Am.). Roentgenol. 172: 121-128, 1999
5. Woodring)H et al: Solitary cavities of the lung: diagnostic
implications of cavity wall thickness. A)R Am) Roentgenol.
135:1269-1271, 1980
 LUNG ABSCESS

I IMAGE GALLERY 1
15

(Left) Lateral radiograph

shows an abscess in the
superior segment of the left
lower lobe (curved arrow), a
typicallocalion for aspiration
in the supine position.
(Right) Axial CECT shows a
spherical abscess with air
fluid levels (arrow) and
acute (open arrow) and
obtuse angles to chest wall.
Lung markings extend 10 the
margins. Diagnosis:
Pseudomonas abscess

Variant
(Left) Frontal radiograph
shows left lower lobe
necrotizing pneumonia
(arrow). Sputum smears and
culture revealed
Staphylococcus aureus
infection. (Right) Frontal
radiograph shows a
pneumothorax (open
arrows) indicating
development of a
bronchopleural fistula.
Cavities are evident within
the consolidated lung
(curved arrows).

Variant
(Left) Axial CECT shows a
lenticular-shaped empyema
with air bubbles and smooth
enhancing pleura (split
pleura sign). The angle to
chest wall is obtuse (arrow)
and adjacent lung is
compressed. (RighI) Frontal
radiograph shows same
patient following chest tube
placement. Empyemas
require percutaneous
drainage. Abscesses
ordinarily resolve with
appropriate antibiotic
therapy.
 HISTOPLASMOSIS
1
16

Axial CECT shows a small nodule (arrow) with a halo of Axial CECT in same patient shows ipsilateral right hilar
ground-glass opacification, representing histoplasma lymphadenopathy (arrow). Typical appearance for
pneumonitis. acute histoplasma infection.

I TERMINOlOGY Radiographic Findings

Acute histoplasma pneumonia: Airspace opacities any
Abbreviations and Synonyms lobe, solitary or multiple; usually lower lungs
Acute, massive inhalational, or acute disseminated o Ipsilateral hilar mediastinal lymphadenopathy,
histoplasmosis, chronic pulmonary or mediastinal common
histoplasmosis, histoplasmoma o Pleural or pericardial effusions and cavitation,
Definitions uncommon
Infection with histoplasma capsulatum Massive inhalational histoplasmosis
o Multilobar pneumonitis; hilar lymphadenopathy
o Complete resolution, or evolution to tiny calcified
I IMAGING FINDINGS or non-calcified nodules
Disseminated histoplasmosis: Miliary or diffuse
General Features airspace opacities; cavities
Best diagnostic clue: Central, lamellated or diffuse o May have normal radiograph
calcification of a nodule < 3 cm virtually diagnostic of Hilar mediastinal lymphadenopathy without lung
histoplasmoma opacification
Location: Pulmonary, mediastinal, most common o Right middle lobe syndrome: Chronic right middle
Size: In endemic areas, > 90% of nodules < 2 cm are lobe collapse due to bronchial compression
granulomas Mediastinal involvement: Mediastinal granuloma or
Morphology: Variable fibrosing mediastinitis
o Partially calcified mediastinal mass, especially
superior mediastinum, unilateral or bilateral
o Encased airways, systemic veins, pulmonary arteries

DDx: Variable Patterns Of Histoplasmosis

Lung Cancer Blastomycosis Lymphoma

 HISTOPLASMOSIS
Key Facts
Imaging Findings
Best diagnostic clue: Central, lamellated or diffuse
calcification of a nodule < 3 cm is virtually diagnostic
of histoplasmoma
Size: In endemic areas, > 90% of nodules < 2 cm are
granulomas
Acute histoplasma pneumonia: Airspace opacities any
lobe, solitary or multiple; usually lower lungs
Ipsilateral hilar mediastinal lymphadenopathy,
common
Thin-section CT to characterize nodule
Fibrosing mediastinitis: Increased attenuation of
mediastinal fat with soft tissue encasing, narrowing
and obliterating mediastinal airways, veins, arteries,
esophagus
Broncholith: Calcified lymph node that has eroded
into a bronchus
a Post-obstructive pneumonia, interstitial opacities,
atelectasis, oligemia, effusion
CT Findings
NECT
a Thin-section CT to characterize nodule
a Thin-section CT and multi planar reformation to
show endobronchial location of broncholith
a Satellite nodules: Benign characteristic of solitary
pulmonary nodule
a Liver and spleen calcified granulomas
CECT
a Enlarged lymph nodes show central low attenuation
from caseous necrosis
a Mediastinal granuloma
Enlarged mediastinal lymph nodes impinge on
adjacent mediastinal structures
Does not progress to fibrosing mediastinitis
a Fibrosing mediastinitis
Fibrosing mediastinitis: Increased attenuation of
mediastinal fat with soft tissue encasing,
narrowing and obliterating mediastinal airways,
veins, arteries, esophagus
Superior vena cava (SVC) syndrome: Narrowed or
obstructed veins and collateral venous pathways
a Right middle lobe syndrome: Lymphadenopathy
compressing and obstructing bronchus with chronic
collapse
HRCT: Disseminated disease: Miliary pattern, }-3 mm
diameter nodules randomly distributed
MR Findings
Lymphadenopathy shows decreased signal; may
represent calcification
Fluoroscopic Findings
Barium swallow to show esophageal stenosis, fistula
formation, diverticula, downhill varices
1
HRCT: Disseminated disease: Miliary pattern, 1-3 mm 17
diameter nodules randomly distributed
Top Differential Diagnoses
Other Pneumonias with Lymphadenopathy
Fibrosing Mediastinitis, Other Etiologies
Solitary Pulmonary Nodule
Clinical Issues
Chronic histoplasma pneumonia: Progressive upper
lobe patchy opacities, fibrosis, bullae, honeycombing
(20%)
Broncholithiasis: Calcified lymph nodes eroding into
airways, mainstem, lobar, segmental bronchi
Imaging Recommendations
Best imaging tool: Radiography usually sufficient to
show the variety of manifestations of intrathoracic
histoplasmosis
Protocol advice: NECT, 1-3 mm thick cuts to show
benign pattern calcification in pulmonary nodules
that are < 3 cm in diameter
I DIFFERENTIAL DIAGNOSIS
Other Pneumonias with Lymphadenopathy
Primary tuberculosis, infectious mononucleosis,
children with bacterial pneumonia
Fungal infections
Plague
Brucellosis
Tularemia
Infectious mononucleosis
Measles
Lymphadenopathy, Other Causes
Benign: Sarcoidosis, tuberculous mediastinitis, fungal
disease in AIDS
Malignant: Lymphoma, bronchogenic carcinoma,
metastases
o Central low attenuation lymph nodes: Tuberculosis,
squamous cell carcinoma
Upper Lobe Fibrocystic Changes
Post-primary tuberculosis, atypical tuberculosis,
sporotrichosis, blastomycosis
o Sputum cultures to distinguish
Fibrosing Mediastinitis, Other Etiologies
Tuberculosis, autoimmune disease, syphilis, drug
reaction (methysergide), radiation therapy, idiopathic
Solitary Pulmonary Nodule
Malignant: Bronchogenic carcinoma, carcinoid,
solitary metastasis
Benign: Fungal (blastomycosis, coccidioidoma),
nocardia, hamartoma, intrapulmonary lymph node
 HISTOPLASMOSIS
Calcified nodules: Eccentric calcification may be seen
in malignant nodules; popcorn calcifications in
18 hamartomas
o Calcified metastases: Osteosarcoma,
chondrosarcoma, carcinoid, mucin producing
adenocarcinomas (gastrointestinal, ovary)
I PATHOLOGY
General Features
Etiology
o Septate mycelium in soil; temperate zones
o Inhalation of airborne spores from soil infected by
excreta and feathers of birds; infected bats
o Yeast form in human tissues
o Progressive disseminated form: Abnormal T-cell
immunity, AIDS, chemotherapy, steroids, organ
transplants, lymphoma
Epidemiology
o Central and Eastern United States (Ohio, Mississippi
river valleys); Central and South America; Africa
o > 80% in endemic areas infected
Gross Pathologic & Surgical Features
Benign extra pulmonary spread in most infections,
liver spleen calcified granulomas
Microscopic Features
Yeast forms in tissues; granulomas with caseous
necrosis; fibrous capsule, calcification
Progressive disseminated disease: Bone marrow
involvement
Usually not isolated from mediastinal fibrosis, pleural
or pericardia I effusions
ICLINICALISSUES
Presentation
Most common signs/symptoms
o Immunocompetent: Asymptomatic or minimal
symptoms; disease self-limited
o Immunosuppressed, patients with emphysema:
Symptomatic
o Malaise, fever, headache, muscle pains,
non-productive cough, wheezing, dysphagia,
oropharyngeal ulcers, hemoptysis, or chest pains
o Adult respiratory distress syndrome: In
immunosuppressed or those with large inoculum
o Lymphadenopathy, hepatosplenomegaly, erythema
nodosum, erythema multiforme, pericarditis
o Progressive disseminated disease: Reduced T-cell
immunity, infants, steroids, chemotherapy, AIDS
o Progressive disseminated form: Adrenal insufficiency
Other signs/symptoms
o Diagnosis: Smears and culture of specimens from
bronchoscopy, lymph node, bone marrow
Radioimmunoassay of antigen in urine or serum
Demographics
Age: Any age; clinical symptoms more common in
infants, elderly
Gender: Disseminated disease, M:F = 4:1
Natural History & Prognosis
Late sequelae
o Pulmonary and nodal findings may resolve
completely
o Histoplasmoma: Well-defined nodule, usually < 2
cm (range, 0.5-3 cm)
Single or multiple; smaller satellite lesions
surrounding dominant nodule
Margin, smooth or lobulated; sometimes irregular;
cavitation uncommon
Calcification (50%): Central nidus, lamellated,
diffuse
Calcified granuloma(s) of healed disease; 3
months to years for foci to calcify
Ipsilateral hilar/mediastinallymph node mulberry
calcification, common; mimics primary complex
of tuberculosis (Ghon complex)
Histoplasmomas may enlarge slowly, 2 mm/year
o Chronic histoplasma pneumonia: Progressive upper
lobe patchy opacities, fibrosis, bullae,
honeycombing (20%)
In patients with emphysema; unilateral or
bilateral
Opacities outline emphysematous spaces;
simulates cavities with thin or thick walls
Mycetomas may develop in upper lobe cavities
Apical pleural thickening; no pleural effusion or
lymphadenopathy
o Chronic hilar and mediastinal lymphadenopathy
o Calcific or constrictive pericarditis: Residua from
histoplasma pericarditis
o Broncholithiasis: Calcified lymph nodes eroding
into airways, mainstem, lobar, segmental bronchi
Airway obstruction: Atelectasis, post-obstructive
pneumonia, mucoid impaction, bronchiectasis,
expiratory air trapping
Death from respiratory failure, rare; cor pulmonale
with fibrosing mediastinitis
Treatment
Immunocompetent: Usually resolves without
treatment (99%)
Immunosuppressed or large inoculum exposure:
Ketoconazole, itraconazole therapy; amphotericin B
for overwhelming infection
Mediastinal granuloma: Surgery usually not indicated
Fibrosing mediastinitis: Does not respond to drug
treatment, surgery often dangerous and unsuccessful
I SELECTED REFERENCES
1. Franquet T et al: Imaging of opportunistic fungal infections
in immunocompromised patient. Eur J Radiol. 51(2):130-8,
2004
2. Seo JB et al. Broncholithiasis: review of the causes with
radiologic-pathologic correlation. Radiographies. 22 Spec
No:S199-213,2002
3. Gurney JW et al: Pulmonary histoplasmosis. Radiology .
199(2):297-306, 1996
 HISTOPLASMOSIS
I IMAGE GALLERY 1
19
Typical
(Left) Frontal radiograph
shows right upper lobe
vague parenchymal
opacification (arrow) and
right hilar lymphadenopathy
(curved arrow). Diagnosis:
Acute histoplasma
pneumonia. (Right) Frontal
radiograph in a patient who
cut down an old tree that
was populated by many
birds shows bilateral
muftifocal airspace opacities
(arrows). Diagnosis: Massive
inhalational histoplasmosis.

(Left) Frontal radiograph in

an elderly man with
emphysema shows bilateral
upper lobe fibrocystic
change with air-fluid level
(curved arrows), apical
pleural thickening (arrows)
from chronic active
histoplasma pneumonia.
(Right) Axial HRCT in a
patient with AIDS shows
irregular airspace opacity in
the lingula (arrow) and
diffuse bilateral miliary
nodules (curved arrows).
Bone marrow biopsy showed
disseminated histoplasmosis.

Typical
(Left) Frontal radiograph
shows widened superior
mediastinal contours (white
arrows). Curved arrow
shows narrowed left
mainstem bronchus. Black
arrow shows a left pleural
effusion. (Right) Axial CECT
in same patient shows
fibrosing mediastinitis
obliterating SVc, right
pulmonary artery and
narrowing right mainslem
bronchus (arrow). Note
calcified lymph nodes and
left upper lobe granuloma
(curved arrows).
 ASPERGillOSIS
1
20

Coronal graphic shows multiple foci of invasive Frontal radiograph shows bilateral foci of invasive
aspergillosis with central necrotic lung balls, surrounding aspergillosis with surrounding air crescents (arrows) in a
air crescents & halos of peripheral hemorrhage. 33 year old woman after bone marrow transplant.

Location: Aspergillomas usually develop in

I TERMINOlOGY pre-existing cavities, which occur most frequently in
Abbreviations and Synonyms upper lobes
Semi-invasive aspergillosis = chronic necrotizing Size: Foci of aspergillus infection vary from miliary
aspergillosis nodules to widespread bilateral consolidation
Most mycetomas are aspergillomas & terms are often Radiographic Findings
used interchangeably Invasive aspergillosis
Definitions o Initially, chest radiograph can be normal
Aspergillosis: A fungal infection caused by genus o Lung nodules or areas of consolidation progress
aspergillus rapidly
o Air crescent sign
Crescent-shaped gas collection within a
I IMAGING FINDINGS pulmonary nodule or consolidation
Consistent with development of invasive
General Features aspergillosis in setting of immune-compromise
Best diagnostic clue Indicates recovery of white blood cell function &
o Invasive aspergillosis: CT halo sign is associated with favorable outcome
Ground-glass opacity, usually related to o Can progress to extensive cavitation & necrosis
hemorrhage, surrounding a nodule, mass or focus o Can invade pleural space, causing empyema or
of consolidation pneumothorax
o Aspergilloma: Dependent, rounded or oval nodule Semi-invasive aspergillosis
developing in cavity or cyst o Varied appearance, may present as slowly growing
nodule or focus of consolidation at lung apex

DDx: Cavitary lung lesions

Lung Carcinoma Staph Abscess Wegener

 ASPERGillOSIS

Key Facts
1
Imaging Findings Pathology 21
Invasive aspergillosis: CT halo sign Etiology: Most aspergillus infections are caused by
Ground-glass opacity, usually related to hemorrhage, aspergillus fumigatus
surrounding a nodule, mass or focus of consolidation
Aspergilloma: Dependent, rounded or oval nodule
Clinical Issues
developing in cavity or cyst Patients with mycetoma are at risk for hemoptysis,
which can be massive
Top Differential Diagnoses
Diagnostic Checklist
Other Fungi
Mycobacterial Pneumonia Sentinel sign for mycetoma
Bacterial Pneumonia Development of pleural thickening adjacent to a
Pulmonary Emboli pre-existing cavity
Wegener Granulomatosis, Pulmonary Such a cavity should be examined for a mycetoma &
Non-Small Cell Lung Cancer observed closely on subsequent exams
Development of a low density rim around an area of
invasive aspergillosis can indicate improvement in
neutropenia

a An aspergilloma can be present a Prone & supine CT scans can demonstrate mobility
a Associated with pre-existing pulmonary scarring or of mycetoma within a cavity
other lung disease
Aspergilloma
a Dependent, rounded or oval nodule with I DIFFERENTIAL DIAGNOSIS
surrounding gas developing in cavity or cyst
Other Fungi
CT Findings Mucormycosis, candidiasis & coccidioidomycosis are
Invasive aspergillosis angioinvasive & can simulate aspergillosis
a Can appear as lobar or peribronchial consolidation,
centrilobular nodules or ground-glass opacity Mycobacterial Pneumonia
a Another appearance is pleural-based wedge-shaped Typical & atypical tuberculosis can consolidate,
consolidation similar to pulmonary infarct exhibit a halo sign & cavitate
a CT halo sign is very suggestive of invasive Cavitating tuberculosis can simulate a mycetoma
aspergillosis in an immune-compromised patient
Bacterial Pneumonia
Warrants starting antifungal therapy before
Pulmonary abscess can cavitate & simulate
confirmation by other tests
angioinvasive aspergillosis
a Other causes of CT halo sign
Other fungal infections Pulmonary Emboli
Hemorrhagic pulmonary metastases Wedge-like consolidation or pulmonary infarcts
Wegener granulomatosis a Seen with bland or septic pulmonary emboli &
Kaposi sarcoma angioinvasive aspergillosis
a CT correlate of air crescent sign suggests invasive
aspergillosis Wegener Granulomatosis, Pulmonary
Air crescent has limited utility Can cause foci of consolidation with a CT halo sign &
Seen in less than half of patients, often appears infarcts that can cavitate
late or after therapy started
a Invasive tracheobronchial aspergillosis Non-Small Cell Lung Cancer
Ulcerations of trachea & central bronchi Cavitating lung carcinoma can simulate mycetoma
Can be associated with atelectasis & consolidation Tumor can cause angioinvasion & infarcted lung,
Sometimes seen in lung transplant simulating aspergillosis
Semi-invasive aspergillosis
a Nodule, mass or consolidation
Aspergilloma I PATHOLOGY
a Fungus ball or sponge-like mass of mycelia filling
General Features
entire cavity
Genetics: Virulence of aspergillus fumigatus is
Imaging Recommendations probably polygenic
Best imaging tool: High-resolution CT Etiology: Most aspergillus infections are caused by
Protocol advice aspergillus fumigatus
a Decubitus radiographs Epidemiology
Can show mobility of mycetoma within a cavity a Aspergillomas occur in pre-existing cavities caused
or show air crescent within invasive aspergillosis by
 ASPERGillOSIS
1 Typical & atypical tuberculosis
Fungal disease
Natural History & Prognosis
22 Sarcoidosis Invasive aspergillosis progresses over days to weeks
a Semi-invasive aspergillosis occurs in mildly a Prognosis is guarded
immune-compromised patients with Semi-invasive aspergillosis progresses over weeks to
Steroid use years
Malignancy a Prognosis is often good, some patients require no
Diabetes mellitus therapy
Alcoholism Aspergilloma can remain stable for many years
Sarcoidosis a Prognosis is generally good, unless hemoptysis is
a Invasive aspergillosis occurs in present
immune-compromised patients with Treatment
Bone marrow, lung & liver transplants
Options, risks, complications
Acute leukemia
a Drug therapy: Amphotericin B or itraconazole
Chemotherapy-induced immune deficiency
a Caspofungin: Newly developed drug that acts
a No increased risk for aspergillosis with acquired
against fungal wall
immune deficiency syndrome without additional
a Patients with mycetoma are at risk for hemoptysis,
pre-disposing factor
which can be massive
Neutropenia & steroid use raise risk
a Surgery may be indicated in some patients,
Gross Pathologic & Surgical Features particularly for treatment of massive hemoptysis
Hyphae invade pulmonary arteries in invasive Post-operative risks include bleeding,
aspergillosis bronchopleural fistula & empyema
a Invasion causes occlusion, hemorrhage & infarction a Hemoptysis can sometimes be treated with
bronchial artery embolization
Microscopic Features Collateral vessels limit this option
Aspergillus is a dimorphic fungus, which exists in two a Aspergillomas are sometimes treated by direct
states, conidia & hyphae intracavitary instillation of drugs via a catheter
Conidia are inhaled & transform into hyphal forms
Aspergilloma is a rounded mass of hyphae
Hyphal form invades lung in invasive aspergillosis I DIAGNOSTIC CHECKLIST
Necrotic lung within an air crescent of invasive
aspergillosis is not an aspergilloma Image Interpretation Pearls
Sentinel sign for mycetoma
a Development of pleural thickening adjacent to a
IClINICAllSSUES pre-existing cavity
Such a cavity should be examined for a mycetoma &
Presentation observed closely on subsequent exams
Most common signs/symptoms: Cough, fever, chills, Development of a low density rim around an area of
dyspnea & chest pain invasive aspergillosis can indicate improvement in
Other signs/symptoms: Weight loss & hemoptysis neutropenia
Clinical Profile Low density rim presages development of an air
a Invasive aspergillosis crescent around area of consolidation
Severely immune-compromised, especially with
neutropenia
No pre-existing lung damage I SElECTED REFERENCES
a Semi-invasive aspergillosis 1. Rementeria A et al: Genes and molecules involved in
Mildly immune-compromised Aspergillus fumigatus virulence. Rev Iberoam Mical.
Pre-existing lung damage 22(1):1-23, 2005
a Mycetoma 2. Pinto PS:The CT halo sign. Radiology. 230(1):109-10, 2004
No serious immune problems 3. Kartsonis NA et al: Caspofungin: the first in a new class of
Pre-existing lung cavity or cyst antifungal agents. Drug Resist Updat. 6(4):197-218, 2003
a Diagnostic options 4. Abramson 5: The air crescent sign. Radiology. 218(1):230-2,
2001
Sputum culture, bronchoalveolar lavage,
S. Franquet T et al: Spectrum of pulmonary aspergillosis:
transthoracic biopsy & open lung biopsy histologic, clinical, and radiologic findings. Radiographics.
Serum aspergillus precipitin test 21(4):825-37,2001
6. Kim SYet al: Semiinvasive pulmonary aspergillosis: CT and
Demographics pathologic findings in six patients. AJRAm J Roentgenol.
Age 174(3):795-8,2000
a Older, debilitated patients are more susceptible, but 7. Gefter WB: The spectrum of pulmonary aspergillosis. J
aspergillosis can affect any age Thorac Imaging. 7(4):56-74, 1992
a Most patients with semi-invasive aspergillosis are 8. Libshitz HI et al: Pleural thickening as a manifestation of
middle-aged aspergillus superinfection. Am J Roentgenol Radium Ther
Nucl Med. 120(4):883-6, 1974
 ASPERGillOSIS
I IMAGE GALLERY 1
23

(Left) Coronal CECT shows

central consolidation from
invasive aspergillosis (curved
arrows) & a surrounding halo
o( ground-glass opacity
(arrows), consistent with
hemorrhage from
angioinvasion. (Right)
Coronal CECT from same
palient later in hospital
course shows gas (arrows)
surrounding a ball of
necrolic lung (open arrows)
within a dense focus of
consolidation (crescent sign)
related to angioinvasive
aspergillosis.

(Leh) Frontal radiograph in a

debilitated elderfy man with
severe bilateral emphysema,
multiple medical problems &
proved aspergillosis shows
dense right upper lobe
consolidation (arrows).
(Right) Axial CECT in same
patient with severe
emphysema shows a dense
focus of consolidation in
right upper lobe (arrows),
from semi-invasive
aspergillosis.

(Left) Axial CECT in a 56

year old man with a history
of right upper lobe abscess
shows a dependent
aspergilloma (open arrow),
which developed in an
abscess cavity (arrows) of
right upper lobe. (Right)
Coronal CECT of same
patient again shows the
aspergilloma (open arrow)
that developed in a
pre-existing cavity (arrows)
in right upper lobe.
 BLASTOMYCOSIS
1
24

Frontal radiograph shows mass-like consolidation in left Axial CECT in same patient shows an oval, mass-like
lower lobe (arrow), caused by proven blastomycosis. A consolidation in left lower lobe (arrows), caused by
mass that is typical of blastomycosis is often confused blastomycosis infection.
with lung carcinoma.

o Mass: More often central, mean 8 cm in diameter

ITERMINOlOGY o Consolidation: Lobar, segmental &. nonsegmental
Abbreviations and Synonyms Unilateral or bilateral (cavitation 15%)
Blastomycosis = blasto, Chicago disease, Gilchrist o Lung nodules: Varying sizes, can be miliary
disease, North American blastomycosis o Hilar &. mediastinal lymphadenopathy: Uncommon
o Pleural effusion in 20%, pleural thickening common
Definitions
Imaging Recommendations
Blastomycosis: Fungal infection caused by Blastomyces
dermatitidis; pulmonary and disseminated forms Best imaging tool: Chest radiographs usually suffice
for detection &. follow-up

I IMAGING FINDINGS
I DIFFERENTIALDIAGNOSIS
General Features
Bronchogenic Carcinoma
Best diagnostic clue: Airspace disease or mass in an
outdoorsman from an endemic area Masses of lung carcinoma &. blastomycosis often
Location: Upper lobes more commonly involved indistinguishable
Size: Ranges from tiny nodules to lobar consolidation Lytic metastases simulate fungal osteomyelitis
Morphology Pneumonia
o Presents as consolidation or lung nodule(s) Bacterial, mycobacterial &. fungal
o Cavitation in 15-20% o Varied presentations including airspace disease, lung
Radiographic Findings nodules &. masses
Radiography

DDx: Mass-Like Consolidation

 BLASTOMYCOSIS

Key Facts
1
Imaging Findings Pathology 25
Best diagnostic clue: Airspace disease or mass in an Etiology: Inhalation of conidia of Blastomyces
outdoors man from an endemic area dermatitidis
Cavitation in 15-20% Epidemiology: Endemic to Southeastern US, Great
Lakes region, Central & South America, Africa
Top Differential Diagnoses
Bronchogenic Carcinoma Diagnostic Checklist
Pneumonia CultureJor blastomycosis in needle biopsy of lung
Round Atelectasis mass from patient living or traveling in endemic area

Clinical Profile: Young to middle-aged male,

Round Atelectasis outdoorsman or outdoor worker
Like blastomycosis, round atelectasis can have
mass-like appearance & contain air bronchograms Demographics
Age: Most patients are adults, uncommon in children
Gender: Strong male predominance
I PATHOLOGY Natural History & Prognosis
General Features Mortality for untreated blastomycosis nearly 60%
Etiology: Inhalation of conidia of Blastomyces If untreated, tends to recur within 3 years
dermatitidis Some develop progressive or disseminated disease
Epidemiology: Endemic to Southeastern US, Great Treatment
Lakes region, Central & South America, Africa
Most patients should be treated, particularly if disease
Associated abnormalities: Verrucous or ulcerative skin
is extrapulmonary or disseminated
lesions, osteomyelitis, genitourinary infection (25%)
ltraconazole: Usual drug of choice
Gross Pathologic & Surgical Features
Consolidating pneumonia at presentation evolves into
noncaseating granulomas with central microabscesses I DIAGNOSTIC CHECKLIST
Microscopic Features Consider
Dimorphic fungus Culture for blastomycosis in needle biopsy of lung
o Source: Probably soil - mycelial form mass from patient living or traveling in endemic area
o Host: Thick-walled yeast with single broad-based
bud
I SElECTED REFERENCES
1. Bradsher RW et al: Blastomycosis. Infect Dis Clin North
IClINICAllSSUES Am. 17(1):21-40, vii, 2003
2. Wheat LJ et al: State-of-the-art review of pulmonary fungal
Presentation infections. Semin Respir Infect. 17(2):158-81,2002
Most common signs/symptoms: Most commonly 3. Winer-Muram HT et al: Blastomycosis of the lung: CT
asymptomatic, but can have flu-like illness features. Radiology. 182(3):829-32, 1992

I IMAGE GALLERY

(Left) Frontal radiograph in a 26 year old man shows right upper lobe consolidation (arrows) from blastomycotic pneumonia. (Courtesy j.
Shepherd III, MO). (Center) Frontal radiograph shows multiple smal/lung nodules caused by disseminated blaslOmycosis. Pleural effusion causes
hazy opacity over lower right chest. (Right) Axial CECT in same patient demonstrates fine bilateral miliary nodules from disseminated
blastomycosis. Moderate bilaleral pleural effusions.
 COCCIDIOIDOMYCOSIS
1
26

Frontal radiograph shows extensive consolidation in left Frontal radiograph from same patient obtained 74
lower lobe related to proven coccidioidomycosis months fater demonstrates evo/utjon of lobar
pneumonia at presentation in first of a series of five consolidation into a focus of multifocal nodularity
radiographs. (arrows).

I TERMINOlOGY Radiographic Findings

Solitary or multifocal segmental or lobar consolidation
Abbreviations and Synonyms
Solitary or multiple lung nodules
Coccidioidomycosis = valley fever, cocci o Nodules can cavitate with thick or thin walls
Definitions Hilar lymph node enlargement occurs in 20%
Coccidioidomycosis: Infection from fungi Mediastinal lymph node enlargement usually seen
Coccidioides immitis only in disseminated disease
Pleural effusion in 20%
Imaging Recommendations
IIMAGING FINDINGS Best imaging tool: Chest radiographs usually suffice
General Features for detection & follow-up
Protocol advice: Conventional enhanced chest CT for
Best diagnostic clue: Cavitating segmental or lobar
evaluation of lung & mediastinal pathology
consolidation in an endemic area
Location
o Primary form limited to lungs & chest lymph nodes
o Disseminated form can spread to nearly any tissue
I DIFFERENTIALDIAGNOSIS
Size: Pulmonary manifestation ranges from solitary Bacterial Pneumonia
lung nodule to disseminated lung disease Identical radiographic appearance, usually
Morphology: Foci of consolidation that can evolve symptomatic with fever, productive cough
into nodule(s) or thin-walled cyst

DDx: Cavitary lesions

Bronchoalveolar Carcinoma Lung Infarct Staphylococcus Abscess

 COCCIDIOIDOMYCOSIS

Key Facts 1
Imaging Findings Other Fungal Pneumonia 27
Best diagnostic clue: Cavitating segmental or lobar Pathology
consolidation in an endemic area
Etiology: Inhalation of arthroconidia of Coccidioides
Solitary or multifocal segmental or lobar immitis
consolidation
Endemic to arid regions of desert Southwest
Solitary or multiple lung nodules
Clinical Issues
Top Differential Diagnoses
Most common signs/symptoms: Asymptomatic
Bacterial Pneumonia
Amphotericin B is drug of choice
Mycobacterial Pneumonia

Mycobacterial Pneumonia I CLINICAL ISSUES

Postprimary tuberculosis can simulate
coccidioidomycosis
Presentation
Most common signs/symptoms: Asymptomatic
Other Fungal Pneumonia Other signs/sym ptoms: Flu-like illness, erythematous
Other fungal pneumonias indistinguishable rash, erythema nodosum &: erythema multiforme
radiographically
Demographics
Age: Incidence increases with increasing age
I PATHOLOGY Gender: More common in males
Ethnicity: African-Americans &: Hispanics: High risk
General Features Natural History & Prognosis
Genetics: HLA class II DRBl*1301 allele predisposes to
Most infections resolve; some turn into chronic
severe disseminated disease
granulomas, called coccidiomas
Etiology: Inhalation of arthroconidia of Coccidioides
Chronic lung disease SOlo; disseminated disease < 1%
immitis
Cyst develops in SOlo
Epidemiology
o Endemic to arid regions of desert Southwest Treatment
o Especially California, Arizona, New Mexico &: Texas Amphotericin B is drug of choice
o Patients with a high dust exposure at risk Treat severe disease &: patients with risk factors
Gross Pathologic & Surgical Features
Initial presentation often a segmental or lobar
pneumonia
I SElECTED REFERENCES
Inflammation typically resolves or evolves into a 1. Feldman BS et al: Primary pulmonary coccidioidomycosis.
non-calcified granuloma Semin Respir Infect. 16(4):231-7,2001
2. Louie Let al: Influence of host genetics on the severity of
Microscopic Features coccidioidomycosis. Emerg Infect Dis. 5(5):672-80, 1999
3. McAdams HP et al: Thoracic mycoses from endemic fungi:
Dimorphic fungus
radiologic-pathologic correlation. Radiographics.
o Soil: Mycelia; host: Endosporulating spherules
15(2):255-70, 1995

I IMAGE GALLERY

(Left) Frontal radiograph obtained eight months later reveals a developing cavity (open arrow) & surrounding nodularity (arrows). (Center)
Frontal radiograph seven months/ater shows enlargement of cavity (open arrow). Surrounding nodularity has decreased, but some mural
nodularity remains (arrow). (Right) Frontal radiograph four months later demonstrates slightly thinner walls & a slight decrease in size of cavity
(open arrow).
 PARASITIC PNEUMONIA
1
28

Frontal radiograph shows patchy, predominantly upper Frontal radiograph showing patchy bilateral
zone opacities (arrows) in a patient with poorly-defined parenchymal opaciUes (arrows) in a
paragonimiasis. patient with paragonimiasis.

o Trypanosomiasis
I TERMI NOLOGY Features reflecting cardiac dysfunction:
Definitions Cardiomegaly, pulmonary edema, pleural
Pulmonary infection secondary to parasitic infestation effusions, septal lines; features of achalasia; rarely
bronchiectasis and tracheomegaly
o Schistosomiasis
IIMAGING FINDINGS Early: Ill-defined intrapulmonary nodules; diffuse
ground-glass opacification; reticulo-nodular
General Features pattern
Best diagnostic clue Late: Pulmonary artery enlargement from cor
o Nonspecific imaging findings, but dependent on the pulmonale
infecting agent o Paragonimiasis
o Malaria: Features of acute respiratory distress Fleeting patchy consolidation with or without
syndrome cavitation (cysts), cysts (up to 5 cm in diameter)
Diffuse bilateral ground-glass opacification in may contain a mural nodule (the worm), cysts
non-dependent lung; dense parenchymal opacities may communicate with bronchus
(represent atelectatic lung) in dependent regions Pleural effusions common, may be massive
o Amebiasis o Echinococcosis
Elevated hemidiaphragm and pleural effusion Cyst (single or multiple/unilateral or bilateral)
Parenchymal abnormalities primarily in right usually in lower lobe
lower lobe due to extension from liver Liver cysts may calcify (not in lung)
o Ascariasis Meniscus or crescent sign: Air entering space
Migratory, fleeting perihilar air space opacities or between peri cyst and exocyst
nodules mimics eosinophilic pneumonia

DDx: Parasitic Pneumonia

Angioinvasive Aspergillosis Pulmonary Hemorrhage Pulmonary Edema

 PARASITIC PNEUMONIA

Key Facts
1
Terminology Pathology 29
Pulmonary infection secondary to parasitic Worldwide distribution of parasitic infections
infestation Individual parasitic infection more commonly a
problem in subjects living in and traveling to
Imaging Findings endemic regions
Nonspecific imaging findings, but dependent on the Variations in prevalence dependent on infective
infecting agent organism and geographical location
Best imaging tool: Computed tomography best
demonstrates parenchymal abnormalities and Clinical Issues
associated findings (e.g., liver disease) In general symptoms are nonspecific but dependent
on parasite and common non-pulmonary system
Top Differential Diagnoses involvement
Non-Parasitic Infections
Non-Cardiogenic Pulmonary Edema Diagnostic Checklist
Diffuse Alveolar Hemorrhage Consider diagnosis in any patient with nonspecific
Eosinophilic Pneumonia symptoms living in or traveling through endemic
Cryptogenic Organizing Pneumonia regions

Water-lily or sign of camalote: Membranes

floating on fluid within cyst produce an air-fluid
I PATHOLOGY
level with irregular interface General Features
o Strongyloidiasis Etiology
Patchy, bilateral patchy migratory consolidation; o Most common human parasitic diseases worldwide
miliary pattern include
Imaging Recommendations o Malaria: Plasmodium species (P. falciparum, P. ovale,
P. malariae, P. viva x)
Best imaging tool: Computed tomography best
demonstrates parenchymal abnormalities and Transmitted by bite of Anopheles mosquito
associated findings (e.g., liver disease) o Amebiasis: Entameba histolytica
Infection acquired through oral route (ingestion
of cysts); pulmonary infection usually a
consequence of direct contiguous spread from
I DIFFERENTIAL DIAGNOSIS liver disease
Non-Parasitic Infections o Ascariasis: Ascaris lumbricoides
Widespread air space opacities Infection acquired via oral route by ingestion of
Identical radiographic findings, culture and travel food/fluid contaminated with infected fecal
history important material
o Trypanosomiasis: Trypanosoma cruzi
Non-Cardiogenic Pulmonary Edema Transmitted by insect bite of the Reduviidae
Diffuse symmetric ground-glass opacification in family
non-dependent lung o Schistosomiasis: Schistosoma species (S.
Dense parenchymal opacification in dependent lung hematobium, S. mansoni, S. japonicum)
Relevant history of pulmonary or extra pulmonary Direct infection through skin exposed to water
lung injury contaminated by cercariae excreted by snails or
less commonly through drinking of infected water
Diffuse Alveolar Hemorrhage o Paragonimiasis: Paragonimus species (P. westermani)
Diffuse ground-glass opacification Infection acquired via oral route by ingestion of
Thickened interlobular septa poorly cooked and infected crayfish or crab
History of hemoptysis, anemia, or known underlying o Echinococcosis: Echinococcus species (E. granulosus,
cause of pulmonary hemorrhage E. multilocularis, E. vogeli)
Eosinophilic Pneumonia Infection acquired via oral route
o Strongyloidiasis: Strongyloides stercora lis
Fleeting patchy regions of ground-glass opacification
Infective larvae in soil penetrate skin of host to
Peripheral upper lobe predominance (in chronic
reach lungs and small intestine
eosinophilic pneumonia)
Epidemiology
Cryptogenic Organizing Pneumonia o Worldwide distribution of parasitic infections
Patchy bilateral areas of consolidation/ground-glass o Individual parasitic infection more commonly a
opacification problem in subjects living in and traveling to
Typically in mid and lower zones endemic regions
Occasionally features include subpleural or radial o Variations in prevalence dependent on infective
linear pattern, perilobular distribution and mass lesion organism and geographical location
 PARASITIC PNEUMONIA
1 Malaria: Estimates suggest between 400-490
million infected subjects worldwide and over 2
30 million deaths per annum worldwide; endemic in
Southern Asia, Africa and South America
Amebiasis: Affecting approximately 1% of world
population and associated with> 100,000 deaths
per annum worldwide; most common in tropical
and subtropical climates
Ascariasis: Affecting over 1 billion worldwide but
only leading to approximately 1,500 deaths per
year
Trypanosomiasis: Over 20 million people infected
worldwide with approximately 60,000 deaths per
annum; endemic in Central/South America
Schistosomiasis: Affects up to 200 million people
worldwide and results in half a million deaths per
annum; endemic in the Middle East (especially
Egypt and Saudi Arabia), Central/Southern Africa
and South America
Paragonimiasis: 20 million subjects infected in
endemic regions (including Asia, Africa, United
States and Latin America)
Echinococcosis: Around 60 million subjects
infected in endemic regions which include Africa,
South America, Australia, New Zealand, the
Mediterranean and Northern Europe
Strongyloidiasis: Around 35 million subject
infected worldwide; endemic in the United States
Gross Pathologic & Surgical Features
Malaria
o Acute respiratory distress (ARDS) syndrome typical
pulmonary manifestation of malaria
Amebiasis
o Direct spread from subdiaphragmatic (intrahepatic)
focus results in pleural effusions (common); lobar
consolidation (with or without cavitation)
Ascariasis
o Parasitic migration from small bowel to pulmonary
circulation; subsequent maturation leads to capillary
and alveolar wall damage results in edema;
hemorrhage; epithelial desquamation; neutrophilic
and eosinophilic infiltration
Trypanosomiasis
o Initial multiplication within macrophages causing
eventual rupture and hematogenous dissemination;
invasion of heart and gastrointestinal tract result in
Acute myocarditis
Destruction of myenteric plexus in
gastrointestinal tract (may result in achalasia)
Schistosomiasis
o Eggs impacted in small arteries and extrude into
perivascular interstitium which produces intense
inflammatory reaction and fibrosis leading to
pulmonary hypertension
Paragonimiasis
o Single or multiple cystic spaces in lungs; cysts in
close proximity to airway (development of
bronchopneumonia if erosion into adjacent airway);
eosinophilic cellular infiltration
Echinococcosis
o Intrapulmonary cysts: Cysts comprised of two layers:
The exocyst (a laminated outer membrane) and the
endocyst (an inner layer producing fluid and
daughter cysts); surrounding fibrous tissue and
chronic inflammation represents the pericyst
I CLINICAL ISSUES
Presentation
Most common signs/symptoms
o In general symptoms are nonspecific but dependent
on parasite and common non-pulmonary system
involvement
Malaria: Fever (malignant tertian fever in P.
falciparum, quartan fever in P. malariae and
benign tertian fever with P. vivax and ovale);
respiratory distress
Amebiasis: Right upper quadrant pain, fever
(intrahepatic abscess); diarrhea (colonic
involvement); dry cough (in early pulmonary
involvement, later productive), hemoptysis,
biloptysis
Ascariasis: Non-productive cough, "burning" chest
pain, hemoptysis, breathlessness; fever
Trypanosomiasis: Fever; symptoms due to cardiac
involvement
Schistosomiasis: Fever, cough, malaise,
breathlessness; hematuria, dysuria (due to bladder
urinary tract involvement)
Paragonimiasis: Hemoptysis, breathlessness, fever,
weight loss, pleuritic chest pain
Echinococcosis: May be asymptomatic if
pulmonary cysts are intact; cough (dry or
productive if rupture of cysts into airway),
hemoptysis, fever
I DIAGNOSTIC CHECKLIST
Consider
Consider diagnosis in any patient with nonspecific
symptoms living in or traveling through endemic
regions
I SELECTED REFERENCES
1. Kim TSet al: Pleuropulmonary paragonimiasis: CT findings
in 31 patients. AJRAmJ Roentgenol. 185(3):616-21,2005
2. Benhur Junior A et al: Pulmonary strongyloidiasis. RevSoc
BrasMed Trop. 37(4):359-60, 2004
3. Betharia SM et al: Disseminated hydatid disease involving
orbit, spleen, lung and liver. Ophthalmologica.
216(4):300-4,2002
4. Schwartz E: Pulmonary schistosomiasis. Clin Chest Med.
23(2):433-43, 2002
5. Shamsuzzaman SM et al: Thoracic amebiasis. Clin Chest
Med. 23(2):479-92, 2002
 PARASITIC PNEUMONIA

I IMAGE GALLERY 1
31

(Left) Frontal radiograph

shows thin-walled hydatid
cyst in the left upper lobe
(arrows). The walls o(
internal endocysls are just
visualized (open arrows).
(Right) Axial CECT in the
same patient demonstrates a
well defined thin-walled cyst
in the left upper lobe
(arrows). The "collapsed"
walls of endocysts are clearly
visualized within (open
arrow).

Variant
(Left) Anteroposterior
radiograph shows collapse of
the right upper lobe.
Diagnosis of strongyloidiasis
confirmed following
examination of mucus plugs
aspirated at bronchoscopy.
(Right) Anteroposterior
radiograph in same patient 2
weeks later with combined
collapse of middle and lower
lobes (arrows) presumably
related 10 passage of
parasites through the
airways.

(Left) Axial CECT shows

marked dilatation of the
main trunk (arrows) and
right main pulmonary artery
(open arrow), reflecting
pulmonary arterial
hypertension, in a patient
with schistosomiasis. (Right)
Frontal radiograph showing a
widespread reticulo~nodular
pal/ern (reflecting a pal/ern
of U interstitial pneumonia")
in leishmaniasis. There is
enlargement of right hilar
lymph nodes.
 CARDIOGENIC PULMONARY EDEMA
1
32

Frontal radiograph demonstrates bilateral perihilar Frontal radiograph of same paUent only 4 hours later
alveolar edema, pulmonary venous hypertension, small shows increase of bilateral pleural effusions & rapid
bilateral pleural effusions & cardiomegaly in paUent with progression of confluent alveolar disease from
congestive heart failure. worsening cardiac pulmonary edema.

I TERMI NOLOGY Radiographic Findings

Abbreviations and Synonyms
Congestive heart failure (CHF)
Pulmonary venous hypertension (PVHTN)
Definitions
Increased fluid in extravascular compartment
from hemodynamic dysfunction
I IMAGING FINDINGS
General Features
Best diagnostic clue: Cardiomegaly, pulmonary venous
hypertension (PVHTN) & pleural effusions
Location: Worse in gravity dependent locations
Size: Ranges from subtle thickening of interlobular
septa to diffuse airspace process
Morphology
o Edema thickens interlobular septa & central
interstitium in interstitial phase
o Airspace consolidation typical manifestation of
alveolar edema
Radiography
o Radiographic precursor of edema: PVHTN
Upper lobe vessels ~ diameter of lower lobe
vessels
Increased pulmonary artery/bronchus ratio in
of lung upper lobes
Ill-defined lower lung vessels
o Stepwise progression from PVHTN to interstitial
edema to alveolar edema
o Interstitial edema - peripheral & central
Thickening of interlobular septa
Kerley A: Long lines in upper lobes radiating
towards h,ilum (rare)
Kerley B: Short, peripheral, perpendicular lines
generally in lower lobes (common)
Lower zonal & perihilar haze
Subpleural edema thickens interlobar fissures
Peribronchial cuffing
o Alveolar edema
Rapid appearance & disappearance, coalescence, &
fluffy margins

DDx: Bilateral Airspace Disease

Negative Pressure Edema Pneumocystis Pulmonary Hemorrhage

 CARDIOGENIC PULMONARY EDEMA

Key Facts
1
Terminology Pathology 33
Increased fluid in extravascular compartment of lung Imbalance in Starling forces
from hemodynamic dysfunction Lungs: Reddish-blue, heavy, boggy & fluid-filled
Edema, frequently bloody, can be squeezed from cut
Imaging Findings surfaces
Best diagnostic clue: Cardiomegaly, pulmonary
venous hypertension (PVHTN) & pleural effusions Clinical Issues
Location: Worse in gravity dependent locations Most common signs/symptoms: Paroxysmal
nocturnal dyspnea, dyspnea on exertion, orthopnea
Top Differential Diagnoses Serum B-type natriuretic peptide: High positive &
Pneumonia negative predictive value for CHF
Lymphangitic carcinomatosis Standard treatment: Oxygen, diuretics, morphine,
Non-cardiogenic edema nitroglycerin, afterload reduction & inotropic agents
Pulmonary hemorrhage
Alveolar proteinosis Diagnostic Checklist
Acute eosinophilic pneumonia Appearance of cardiogenic pulmonary edema can be
modified by noncardiogenic factors

Shifts gradually with position (gravitational shift o Heart normal size

test) o Usually no pleural effusion
Batwing (butterfly, perihilar) edema: Uncommon Lymphangitic carcinomatosis
Acute mitral valve insufficiency: Classic cause for o Normal heart size
batwing edema o Known history of malignancy
o Pleural effusions: Often bilateral, usually larger on o Usually not diffuse like pulmonary edema
right, rarely unilateral on left o Lymphadenopathy
o Cardiomegaly: Chronic (normal heart size with
acute myocardial ischemia or arrhythmia) Alveolar Edema
Cardiac size often small in chronic obstructive Non-cardiogenic edema
pulmonary disease due to hyperinflation o More often acute respiratory distress syndrome
Subsequent increases in heart size may not be (ARDS)
beyond range of normal o Pleural effusions unusual with ARDS
o Widened vascular pedicle - from azygos vein & o ARDS patients usually require intubation to support
superior vena cava distention ventilation
Temporal relationship of pressure & volume Pneumonia
o Acute pressure (capillary wedge pressure) elevation o Radiographic findings can be identical
Initially normal, edema accumulates over 12 hour o Usually more heterogeneous & asymmetrical
period o Heart usually normal in size
o Pressure decreases with treatment o Pneumonia will not shift with gravity (gravitational
Edema resolves hours to days; radiograph lags shift test)
clinical course Pulmonary hemorrhage
o Normal heart size with no pleural effusions
CT Findings o Patients usually anemic
HRCT o Hemorrhage will not shift with gravity (gravitational
o Smooth thickening of interlobular septa shift test)
o Bronchovascular bundle thickening Alveolar proteinosis
o Gravity-dependent ground-glass &/or airspace o Batwing pattern identical to CHF, patients
opacities asym ptoma tic
o Mildly enlarged mediastinal lymph nodes o Heart size normal with no pleural effusions
Acute eosinophilic pneumonia
Imaging Recommendations o Heart size normal with no pleural effusions
Best imaging tool: Chest radiography usually suffices o Patients usually younger & have fever
for diagnosis & treatment
Protocol advice: Suspend ventilation during CT Interstitial Edema, Cardiomegaly, Pleural
scanning Effusions
Erdheim-Chester disease (rare, non-Langerhan cell
granulomatosis)
I DIFFERENTIAL DIAGNOSIS o Will not respond to diuretics
Interstitial Edema o Sclerotic bone lesions
Pneumonia
o Febrile, usually viral or mycoplasma etiology
 CARDIOGENIC
1 I PATHOLOGY
34 General Features
General path comments
o Imbalance in Starling forces
o Increase in microvascular pressure increases
endothelial gaps
o Transvascular (low protein content) fluid moves into
interstitial spaces
o Alveolar edema permeates across alveolar-capillary
membrane
o Lymphatic flow increases in chronic edema
(lO-fold), but not acute edema
Etiology
o Multifactorial
Myocardial infarction, ischemic cardiomyopathy
Mitral valve disease, left atrial myxoma
Fluid overload, renal failure
Veno-occlusive disease, fibrosing mediastinitis
o Pulmonary venous hypertension usually due to left
heart failure, which elevates microvascular pressure
o Fluid flows into interstitial spaces
o Rate depends on hydrostatic & osmotic pressures in
vessels, interstitium & lymphatics
o Upper zone vascular distention with wedge pressures
of 12-18 mm Hg
o Kerley lines develop when wedge pressures reach
20-25 mm Hg
o Alveolar edema develops with wedge pressures of
25-30 mm Hg
Epidemiology: Atherosclerotic heart disease is most
common, so it dominates epidemiology
Associated abnormalities: Pericardial effusion, ascites
& anasarca
Gross Pathologic & Surgical Features
Lungs: Reddish-blue, heavy, boggy & fluid-filled
Thickened interlobular septa visible on lung surface
Edema, frequently bloody, can be squeezed from cut
surfaces
Microscopic Features
With elevated hydrostatic pressure, transudate
accumulates in loose connective tissue of interstitium
Red blood cells also seep into interstitium
This edema extends into peribronchovascular
interstitium & into interlobular septa
Lymphatics dilate in response to edema
As more fluid accumulates, alveolar spaces are flooded
Fibrin & red blood cells accumulate in alveoli
Hemosiderin-containing macrophages called heart
failure cells commonly seen in long-standing CHF
I CLINICAL ISSUES
Presentation
Most common signs/symptoms: Paroxysmal nocturnal
dyspnea, dyspnea on exertion, orthopnea
Other signs/symptoms: Frothy, blood-tinged sputum
Pertinent historical data
o History of CHF
o Myocardial infarction
PULMONARY EDEMA
o Coronary artery disease
o History of cardiac surgery
Physical examination
o Third heart sound (ventricular filling gallop) has
high diagnostic value
o Jugular venous distension
o Pulmonary rales & wheezes
o Lower extremity edema
o Hepatic congestion
Electrocardiography very helpful in diagnosis of
arrhythmias or myocardial ischemia/infarction
Elevated values of serum B-type natriuretic peptide
present in CHF
Serum B-type natriuretic peptide: High positive &
negative predictive value for CHF
Pulmonary function tests show decreased lung
compliance
Superimposed pulmonary embolus more likely to
result in infarction
Demographics
Age
o Can occur at any age, but incidence & prevalence of
cardiac problems increases wi th age
o Atherosclerotic disease generally affects men at a
younger age
Gender: M = F
Natural History & Prognosis
Onset can be acute or insidious
Prognosis depends on severity & reversibility of
underlying hemodynamic dysfunction
Initial episode of CHF often readily treatable
Treatment
Standard treatment: Oxygen, diuretics, morphine,
nitroglycerin, afterload reduction & inotropic agents
Edema can clear rapidly with treatment
I DIAGNOSTIC CHECKLIST
Consider
Appearance of cardiogenic pulmonary edema can be
modified by noncardiogenic factors
Image Interpretation Pearls
Review of prior radiographs can reveal previous
episodes of cardiac decompensation
I SElECTED REFERENCES
1. Wang CS et al: Does this dyspneic patient in the
emergency department have congestive heart failure?
JAMA. 294(15):1944-56, 2005
2. Gehlbach BKet al: The pulmonary manifestations of left
heart failure. Chest. 125(2):669-82, 2004
3. Scillia P et al: Computed tomography assessment of lung
structure and function in pulmonary edema. Crit Rev
Comput Tomogr. 45(5-6):293-307, 2004
4. Gluecker T et al: Clinical and radiologic features of
pulmonary edema. Radiographies 19:1507-31,1999
5. Pistolesi M et al: The chest roentgenogram in pulmonary
edema. Clin Chest Med. 6(3):315-44,1985
 CARDIOGENIC PULMONARY EDEMA

I IMAGE GALLERY 1
35

(Left) Axial CECT shows

moderate bilateral effusions.
Bilateral parahilar
ground-glass opacities from
lung edema are visible (open
arrows). Note characteristic
graded density better seen
on left. (Right) Coronal CECT
shows subpleural edema
(white open arrow),
interlobular septal thickening
(black arrow), &
peribronchial cuffing (white
arrows) in a young woman
with postpartum
cardiomyopathy.

Typical
(Left) Frontal radiograph in
patient with congestive heart
failure shows multiple
horizontally oriented
interlobular septa/lines in left
lung base, called Kerley B
lines (arrows). (Right)
Frontal radiograph in patient
with congestive heart Failure
shows subpleural edema of
minor fissure (curved arrow).
Kerley A line is visible as thin
white line with same
orientation (arrow).

Variant
(Left) Axial CECT in chronic
heart failure demonstrates
interlobular septal thickening
(arrows), peribronchial
thickening (open arrows) &
increased lung density in
lower lobes from pulmonary
edema. (Right) Coronal
CECT in same patient
demonstrates CT correlate of
Kerley B lines (open arrows).
Left upper lobe interlobu/ar
septal thickening (arrows)
forms CT correlate of Kerley
A lines.
 NONCARDIAC PULMONARY EDEMA

36

Graphic shows increased capillary permeability with Radiograph in a victim of a road fraffic accident with
proteinaceous hemorrhagic fluid filling alveoli in ARDS. ARDS. There is symmefric airspace opacification in
Other features include hyaline membrane formation, both. The changes are most marked in the periphery of
alveolar atelectasis & small vesselmicrothrombosis. the lungs.

a Reasonably good correlation between radiographic

I TERMINOLOGY patterns and serial histopathologic changes
Abbreviations and Synonyms a Diffuse and bilateral airspace opacification
Noncardiogenic pulmonary edema; increased Favors the lung periphery
permeability edema Septal (Kerley B) lines less common than in
cardiogenic edema
Definitions Peribronchial cuffing less common than in
Extravascular lung water due to increased permeability cardiogenic edema
of the alveolar-capillary barrier Normal heart size
No pulmonary vascular redistribution
a May have small pleural effusions but less common
I IMAGING FINDINGS than in cardiogenic edema
a Initial use of positive end-expiratory pressure (PEEP)
General Features may increase lung volume giving apparent
Best diagnostic clue: Diffuse bilateral air space radiographic "improvement"
opacification on plain chest radiography and CT a Barotrauma common with PEEP
Location: Tends to be more peripheral than a Superimposed (ventilator-related/nosocomial)
cardiogenic edema (as compared to cardiogenic edema pneumonia common
which tends to be more central with a "bat's wing"
distribution) CT Findings
HRCT
Radiographic Findings a Acute respiratory distress syndrome (ARDS) best
Radiography "model" for noncardiac pulmonary edema

DDx: Noncardiac Pulmonary Edema

Cardiogenic Edema Diffuse Hemorrhage Pneumocystis jiroveci Pneumonia

 NONCARDIAC PULMONARY
Key Facts
Terminology
Extravascular lung water due to increased
permeability of the alveolar-capillary barrier
Imaging Findings
Best diagnostic clue: Diffuse bilateral air space
opacification on plain chest radiography and CT
Favors the lung periphery
Septal (Kerley B) lines less common than in
cardiogenic edema
Acute respiratory distress syndrome (ARDS) best
"model" for non cardiac pulmonary edema
In secondary (extrapulmonary) ARDS, roughly
symmetric ("typical") changes seen more often than
in primary (pulmonary ARDS)
o In contrast to radiographic appearances, strikingly
inhomogeneous distribution on CT
o In secondary (extrapulmonary) ARDS, roughly
symmetric ("typical") changes seen more often than
in primary (pulmonary ARDS)
o Typical pattern characterized by
Ground-glass opacification (representing edema
and hemorrhagic fluid in the interstitium and
alveoli) admixed with apparently normally
aerated lung in the non-dependent lung
Dense parenchymal opacification in the
dependent lung (representing atelectatic lung)
o Primary (pulmonary) ARDS, more often associated
with an "atypical" pattern
Foci of dense parenchymal opacification in
non dependent lung more common than in
extra pulmonary ARDS
Foci of dense parenchymal opacification in
dependent lung less common than in
extrapulmonary ARDS
Diffuse ground-glass opacification and apparently
normally lung (as with extrapulmonary ARDS)
Multiple thin-walled cysts more common than in
extrapulmonary ARDS
o In survivors of ARDS signs of fibrosis (reticular
pattern and ground-glass opacification) in
non-dependent lung, presumably because
dependent atelectatic lung in acute phase protected
from high airway pressure
Imaging Recommendations
Best imaging tool
o Plain chest radiography ideal for "serial" monitoring
of progress
o CT ideal for more accurate evaluation of
morphologic changes
o CT also of significant value in "problem-solving" of
apparently complex plain radiographic appearances
in critically-ill patients
Protocol advice
o Portable chest radiograph: Notation of position and
ventilator settings by technicians helpful in
interpretation
EDEMA
1
Top Differential Diagnoses 37
Cardiogenic Edema
Diffuse Pulmonary Hemorrhage
Widespread (Opportunistic) Pulmonary Infection
Pathology
Increased alveolar-capillary permeability;
inflammatory mediators damage capillary membrane
Exudative
Proliferative
Chronic
Clinical Issues
Onset of symptoms/signs may be insidious (over a
few days) or relatively rapid (over a few hours) after
an inciting pulmonary or extrapulmonary "event"
o Digital radiography superior to "conventional"
film-screen combinations
o Chest radiography
To evaluate extent of parenchymal opacification
Monitor progress of parenchymal opacification
Location of support and monitoring apparatus
To detect complications of barotrauma
o CT used as problem solving tool, extremely helpful
for pleural space collections
I DIFFERENTIAL DIAGNOSIS
Cardiogenic Edema
Separation from cardiogenic pulmonary edema not
always possible based on radiographic features
Elements of cardiogenic and noncardiogenic
pulmonary edema may coexist-exist in the same
patient
Signs more frequently associated with cardiac
pulmonary edema include
o Increased heart size
o Central ("bat's wing") distribution
o Thickened septal (Kerley B) lines
o Peribronchial cuffing
o Pleural effusions
Diffuse Pulmonary Hemorrhage
May have identical radiographic findings, patient
often anemic with history of hemoptysis
Normal heart size
Diffuse distribution
Not usually associated with pleural effusions
Widespread (Opportunistic) Pulmonary
Infection
History of immunocompromise
No pulmonary vascular redistribution
Normal heart size
Normal vascular pedicle width
May result in ARDS
 NONCARDIAC PULMONARY
[PATHOLOGY
38 General Features
General path comments
o Increased alveolar-capillary permeability;
inflammatory mediators damage capillary
membrane
o May be associated with or without obvious
alveolar-capillary epithelial damage
o 3 relatively distinct but overlapping histopathologic
phases
Exudative
Proliferative
Chronic
Etiology
o ARDS is best example of increased permeability
edema associated with alveolar-capillary damage
ARDS may be due to indirect injury to lungs
(called secondary or extrapulmonary ARDS) as
seen secondary to systemic sepsis, massive
transfusion, following (non-thoracic) surgery,
eclampsia
ARDS may be due to direct injury to lungs (called
primary or pulmonary ARDS) typically secondary
to severe pulmonary infection, massive aspiration
but also toxic fume inhalation and 0, toxicity
o While increased microvascular permeability is an
important component of ARDS, likely that there is a
generalized systemic inflammatory disorder [termed
"systemic inflammatory response syndrome" (SIRS)]
o Causes of increased permeability edema not
obviously associated with alveolar-capillary
epithelial damage include
Rapid pulmonary re-expansion
Neurogenic
Severe upper (extrathoracic) airway obstruction
Hantavirus infection
High altitude
Exudative: Heavy, airless, deep purple lung
Hepatization of lung, fibrosis, cysts: May eventually
return to normal
Microscopic Features
Histopathologic features are essentially those of diffuse
alveolar damage
Three recognizable but interlinked stages
o Exudative phase: Vascular congestion, exudation of
proteinaceous fluid into interstitium and airspaces,
microatelectasis, hyaline membrane formation
o Proliferative: Proliferation of myofibroblasts in
airspace and interstitium, deposition of
proteoglycans (and eventually collagen) in
interstitium
o Chronic: Hyperplasia type II pneumocyte,
fibroblastic infiltration and fibrosis
Staging, Grading or Classification Criteria
Stage 1: Exudative (first 24 hours)
Stage 2: Proliferative (1-7 days)
Stage 3: Chronic (> 1 week)
EDEMA
ICLINICAl ISSUES
Presentation
Most common signs/symptoms
o Onset of symptoms/signs may be insidious (over a
few days) or relatively rapid (over a few hours) after
an inciting pulmonary or extra pulmonary "event"
o Typical symptoms/signs include
Breathlessness
Tachypnea
Dry cough
Cyanosis
Agitation
Coarse crackles on auscultation
Bronchial breathing on auscultation
Other signs/symptoms
o May have no chest radiographic abnormalities in
first 12 hours (during acute exudative phase)
o Normal wedge pressure: Decreased lung compliance
o Decreased pulmonary compliance (hence "stiff
lung")
o ARDS defined by ratio of PaO,/FiO" <200 mmHg =
ARDS; <300 mmHg = acute lung injury
Natural History & Prognosis
In general, high mortality rate but improved survival
in specialist units with advance in intensive care
Survivors may have either restrictive or obstructive
functional deficits
Treatment
Supportive treatment in the intensive care is mainstay
Steroids or extra corporeal membrane oxygenation
(ECMO) not shown to be beneficial; supportive,
mechanical ventilation: PEEP
I DIAGNOSTIC CHECKLIST
Consider
Usually patients with ARDS rapidly intubated to
support oxygenation even when severity of
consolidation mild
Image Interpretation Pearls
Rather than radiographic differentiation, clinical
management based on Swan-Ganz catheter and
pulmonary capillary wedge pressure (PCWP)
measurements
I SELECTED REFERENCES
1. Bernard GR: Acute respiratory distress syndrome: a
historical perspective. AmJ Respir Crit Care Med.
] 72(7):798-806,2005
2. Desai SRet al: Acute respiratory distress syndrome caused
by pulmonary and extrapulmonary injury: a comparative
CT study. Radiology. 2]8(3):689-93, 200]
3. Gattinoni Let al: What has computed tomography taught
us about the acute respiratory distress syndrome? Am J
Respir Crit Care Med. ]64(9):]701-11,2001
4. Desai SRet al: Acute respiratory distress syndrome: CT
abnormalities at long-term follow-up. Radiology.
210(1):29-35, 1999
 NONCARDIAC PULMONARY EDEMA

I IMAGE GALLERY 1
39
Typical
(Left) Axial HRCT showing
"typical" changes o( AROS:
There is symmetric
ground-glass opacification in
the non-dependent lung but
dense parenchymal
opacification in the
dependent lung (arrows).
(Right) Anteroposterior
radiograph shows diffuse
symmetric and
homogeneous air space
opacification in AROS.

Typical
(Left) Axial HRCT symmetric
changes in both lungs
comprising dependent
regions of dense
parenchymal opacification
and ground-glass in the
non-dependent lung. (Right)
Axial HRCT shows diffuse
but non symmetric airspace
opacification in a patient
with "primary" AROS. A
shallow pneumothorax is
a/50 seen anteriorly on the
right farrow).

Typical
(Left) Axial radiograph shows
subtle diffuse ground-glass
opacification in both lungs in
a patient following
non-thoracic surgery. (Right)
Axial HRCT shows a
characteristic coarse reticular
pattern in the anterior
non-dependent lung in an
ARDS survivor, changes
thought to be a related to
barotrauma in the acute
stage.
 FAT PULMONARY EMBOLISM

40

Anteroposterior radiograph obtained shortly after this Anteroposterior radiograph obtained for sudden
young man suffered a femur fracture shows normal dyspnea, 24 hours after femur fracture and subsequent
lungs. repair shows new bilateral diffuse lung opacification
consistent with pulmonary edema and FES.

Location: Widespread through lungs

ITERMINOlOGY
Radiographic Findings
Abbreviations and Synonyms Radiography
Fat embolism o Nonspecific, diffuse parenchymal opacities, without
Fat embolism syndrome (FES) zonal predilection, that are typical of pulmonary
Definitions edema or diffuse pneumonia of any etiology
o Extent of lung opacification generally reflects
Fat embolism
o Release of fat globules or bone marrow into the severity of disease
venous system o Can be identical to features of adult respiratory
o Often incidental and benign distress syndrome (ARDS)
o Pleural effusions uncommon
Fat embolism syndrome
o Respiratory distress and multi-organ dysfunction CT Findings
related to fat embolism
NECT
o Classical clinical triad of hypoxia and respiratory o In mild fat embolism
failure, petechial rash, and altered mental status Bilateral ground-glass opacities and thickening of
Petechiae occur over the upper half of the body the interlobular septa
Small (3-5 mm) nodular opacities tend to be
located predominantly in the centrilobular and
I IMAGING FINDINGS subpleural regions
Nodules presumably represent alveolar edema or
General Features
hemorrhage secondary to the fat embolism
Best diagnostic clue: Diffuse heterogeneous air-space
syndrome
opacities in appropriate clinical setting

DDx: Diffuse Lung Opacification

Acute Renal Failure Heroin Overdose Pneumocystis Pneumonia

 FAT PULMONARY EMBOLISM
Key Facts
1
Terminology Top Differential Diagnoses 41
Classical clinical triad of hypoxia and respiratory Acute Respiratory Distress Syndrome
failure, petechial rash, and altered mental status Hydrostatic Pulmonary Edema
Infection
Imaging Findings Acute Venous Thromboembolic Disease
Nonspecific, diffuse parenchymal opacities, without
zonal predilection, that are typical of pulmonary Pathology
edema or diffuse pneumonia of any etiology Free fatty acids cause endothelial damage and
Bilateral ground-glass opacities and thickening of the permeability edema
interlobular septa
Small (3-5 mm) nodular opacities tend to be located Clinical Issues
predominantly in the centrilobular and subpleural Diagnosis requires at least one sign from Gurd's major
regions criteria and at least four signs from Gurd minor
Resolution of the CT abnormalities occurs in about 2 criteria category
weeks, with a range of from 1-3 weeks The prognosis is worse in older patients and in those
with more severe injuries

Resolution of the CT abnormalities occurs in

about 2 weeks, with a range of from 1-3 weeks Pulmonary Hemorrhage
CECT: Endoluminal filling defect in pulmonary artery Iron deficiency anemia, hemoptysis (80%)
containing fat, rare Diffuse consolidation evolves over several days into
interstitial thickening and resolves over 10-15 days
Nuclear Medicine Findings
v /Q Scan: Perfusion scan shows multiple peripheral
Acute Venous Thromboembolic Disease
sub-segmental defects CTA findings of large pulmonary artery embolic
disease should distinguish
Echocardiographic Findings Usually develops later in clinical course following
Echocardiogram: Fat emboli can be identified in traumatic injury
real-time during orthopedic procedures
Pulmonary Contusion
Imaging Recommendations Unlike FES, radiographic abnormality nearly always
Best imaging tool: Chest radiograph usually adequate present immediately or within a few hours after blunt
for detection of lung disease and monitoring course chest trauma
Aspiration
I DIFFERENTIAL DIAGNOSIS Typically present immediately, perihilar and bibasilar
distribution, variable time to clearing
Acute Respiratory Distress Syndrome
Sepsis
Multi-organ failure I PATHOLOGY
Radiographic features and timing of disease are often
coincident General Features
Distinction can be difficult without following Gurd Etiology
criteria for a clinical diagnosis o No specific pathologic features diagnostic of fat
embolism
Hydrostatic Pulmonary Edema o Release of fat globules or fatty bone marrow into
More commonly shows Kerley B lines, small bilateral venous system
pleural effusions, enlarged heart, and batwing Usually due to long bone fracture
distribution of edema Occurs during placement of intra-medullary rods
o Fat embolism syndrome
Neurogenic Pulmonary Edema Fat droplets act as emboli within the pulmonary
Usually upper lung zones and develops immediately microvasculature and other microvascular beds,
following central nervous system (CNS) injury such as the skin and brain
Infection Initial symptoms are considered due to a
mechanical occlusion from fat globules that are
May have similar radiographic findings, usually less
too large to pass through the capillaries
homogeneous
Because of their fluid nature, fat globules do not
Develops later in clinical course
completely obstruct capillary blood flow
Fever, chills, productive cough and leukocytosis
Hydrolysis of intravascular fat into more irritating
common
free fatty acids by intra-pulmonary lipase
 FAT PULMONARY EMBOLISM
Free fatty acids cause endothelial damage and
permeability edema
Demographics
Epidemiology Age
o Fat embolism occurs almost universally following o 15-35
long bone fracture or intra-medullary rod placement Uncommon over age of 40
o Fat embolism syndrome occurs in about 2% of Gender: Incidence and outcome not directly affected
patients with fat embolism by gender
o Closed fractures produce more emboli than open Natural History & Prognosis
fractures The prognosis is worse in older patients and in those
o Long bones, pelvis and rib fractures cause more fat with more severe injuries
emboli o Fat embolism-universally good outcome
o Multiple fractures generate more emboli o Fat embolism syndrome: 5-15% mortality
o Sternum and clavicle fractures produce less fat
emboli Treatment
Associated abnormalities Supportive care
o Fat globules in sputum o Good arterial oxygenation
o Lipuria Rapid surgical stabilization of long bones fractures
o Retinal emboli reduces the risk of FES
Microscopic Features
Diffuse alveolar damage I DIAGNOSTIC CHECKLIST
Consider
ICLINICAllSSUES FES when a new pulmonary edema pattern develops
radiologically, within the 24-72 hour window after
Presentation
multiple long bone fractures
Most common signs/symptoms Can be difficult or impossible to radiologically
o Diagnosis requires at least one sign from Gurd's distinguish from ARDS from any other cause
major criteria and at least four signs from Gurd
minor criteria category Image Interpretation Pearls
o Gurd major criteria Radiographic and CT features are nonspecific, but
Axillary or subconjunctival petechia: Occurs usually adequate for detection of lung disease and
transiently (4-6 hours) in 50-60 % of the cases monitoring course
Hypoxemia (Pa02 < 60 mm Hg; Fi02, < = 0.4)
Central nervous system depression
disproportionate to hypoxemia I SElECTED REFERENCES
Pulmonary edema 1. Riding G et al: Paradoxical cerebral embolisation. An
o Gurd's minor criteria explanation for fat embolism syndrome. J Bone Joint Surg
Tachycardia (more than 110 beats per minute) Br. 86(1):95-8, 2004
Pyrexia (temperature higher than 38.5 degrees) 2. Bokhari SI et al: Probable acute coronary syndrome
Emboli present in retina on funduscopic secondary to fat embolism. Cardiol Rev. 11(3):156-9,2003
examination 3. Malagari K et al: High-resolution CT findings in mild
Fat present in urine pulmonary fat embolism. Chest. 123(4):1196-201,2003
Sudden unexplainable drop in hematocrit or 4. Choi JA et al: Nontraumatic pulmonary fat embolism
platelet values syndrome: radiologic and pathologic correlations. J Thorac
Imaging. 17(2):167-9,2002
Fat globules present in sputum 5. Parisi OM et al: Fat embolism syndrome. Am J Orthop.
Increasing sedimentation rate 31(9):507-12,2002
Other signs/symptoms: Occurring within 72 hours of 6. Mellor A et al: Fat embolism. Anaesthesia. 56(2):145-54,
skeletal trauma: Shortness of breath; altered mental 2001
status; occasional long tract signs and posturing; 7. Robinson CM: Current concepts of respiratory
urinary incontinence insufficiency syndromes after fracture. J Bone Joint Surg BI.
Non-traumatic etiologies for FES 83(6):781-91,2001
o Fatty liver; prolonged corticosteroid therapy; acute 8. Arakawa H et al: Pulmonary fat embolism syndrome: CT
findings in six patients. J Com put AssistTomogr.
pancreatitis; osteomyelitis; conditions causing bone 24(1):24-9, 2000
infarcts, such as sickle cell disease 9. Heyneman LEet al: Pulmonary nodules in early fat
Other rare traumatic etiologies for FES embolism syndrome: a case report. J Thorac Imaging.
o Liposuction; severe burns; massive soft tissue injury; 15(1):71-4,2000
and bone marrow biopsy 10. Stoeger A et al: MRI findings in cerebral fat embolism. Eur
Neurologic signs are typically nonspecific with features Radial. 8(9):1590-3,1998
of diffuse encephalopathy 11. Bulger EM et al: Fat embolism syndrome. A lO-year review.
o Acute confusion, stupor, coma, rigidity, or Arch Surg. 132(4):435-9, 1997
12. ten Duis HJ: The fat embolism syndrome. Injury.
convulsions 28(2):77-85, 1997
 FAT PULMONARY EMBOLISM

I IMAGE GAllERY 1
43
Typical
(Leh) Anteroposterior
radiograph from a patient
before meeting clinical
criteria for fat embolism
syndrome shows low lung
volumes but no definite
abnormality. (Right)
Anteroposterior radiograph
from the same patient as
previous image, after
meeting Curd criteria for fat
embolism syndrome, shows
diffuse perihilar, somewhat
nodular opacities.

Typical
(Leh) Anteroposterior
radiograph from the same
patient as the exemplary
case, but taken on day 75 of
hospitalization, shows
complete clearing of diffuse
lung opacities, typical for
uncomplicated FES. (Right)
Anteroposterior radiograph
from a patient who suffered
multiple long bone and
spinal fractures shows
typical, but nonspecific,
diffuse, somewhat nodular,
opacities consistent with
clinical diagnosis of FES.

Variant
(Leh) Axial CECT shows
endoluminal filling defect
with Hounsfield unit value
less than -40 (arrow). This is
a rare observation. (Right)
Anteroposterior radiograph
from a patient with mild fat
embolism syndrome shows
widespread peripheral
nodular opacities.
 DIFFUSE ALVEOLAR HEMORRHAGE
1
44

Graphic demonstrates blood within the alveoli, which is Frontal radiograph in a 21 yo male who is 76 days from
eventually cleared away by the pulmonary an allogeneic BMT who developed an acute onset of
macrophages. Repeated bouts of pulmonary dyspnea and hemoptysis. Bronchoscopy confirmed
hemorrhage will result in septal fibrosis. DAH.

ITERMINOlOGY Radiographic Findings

Acute bilateral and often basilar consolidation
Definitions o With persistent bieeding or recurrent episodes,
Variable syndromes that have diffuse alveolar reticular opacities and fibrosis tend to develop
hemorrhage (DAH) as a common manifestation Idiopathic pulmonary hemosiderosis, Goodpasture
o Can classify based on the patient's immune status, syndrome and microscopic angitis have significant
size of vessels involved, immune complexes or types imaging and clinical overlap
of inflammatory cells present o Presence of antibasement membrane antibodies,
Immunocompetence: Goodpasture syndrome renal involvement and patient's age can be help to
[antibasement membrane antibody disease distinguish among these diseases
(ABMABD)], Wegner granulomatosis, systemic lupus Wegner Granulomatosis most often manifests with
erythematosus (SLE) vasculitis, microscopic angitis and multifocal peripheral consolidations and/or cavitary
idiopathic pulmonary hemosiderosis (IPH) nodules
Immunocompromised: Bone marrow transplantation o Capillaritis form (5-10%) predisposed to diffuse
(BMT) and leukemia alveolar hemorrhage
o Pleural effusions and enlarged lymph nodes
uncommon with this latter manifestation
I IMAGING FINDINGS SLE vasculitis
o Triad of anemia, acute bilateral consolidation and
General Features
hemoptysis supports DAH, seen in 2% of all SLE
Best diagnostic clue: Acute onset of bilateral, diffuse or
patients
predominantly basilar consolidation in an anemic
patient

DDx: Diffuse Alveolar Hemorrhage

Pneumocystis jiroveci Pneumonia Bacterial Pneumonia

 DIFFUSE ALVEOLAR HEMORRHAGE
Key Facts
Terminology
Variable syndromes that have diffuse alveolar
hemorrhage (DAH) as a common manifestation
Can classify based on the patient's immune status,
size of vessels involved, immune complexes or types
of inflammatory cells present
Imaging Findings
Best diagnostic clue: Acute onset of bilateral, diffuse
or predominantly basilar consolidation in an anemic
patient
IPH, ABMABD and microscopic angitis demonstrate
extensive bilateral areas of ground glass and
consolidation in the acute setting
Term acute "lupus pneumonitis" commonly used,
but this term also applied to diffuse alveolar
damage, infection and cellular nonspecific
interstitial pneumonitis (NSIP)
Bone marrow transplant: 20% develop DAH
o Time course very helpful since the majority develop
around the time of engraftment, usually between
10-25 days post transplantation
o Onset rapid with diffuse bilateral consolidation
Bronchoscopy yields progressively increased blood
return on lavage
Leukemia
o Extensive bilateral consolidation most often
secondary to hemorrhage
Infection, edema and leukemic involvement
found in decreasing order
CT Findings
HRCT
o IPH, ABMABD and microscopic angitis demonstrate
extensive bilateral areas of ground glass and
consolidation in the acute setting
Sparing of the lung periphery and costophrenic
angles characteristic
o Over the ensuing 48 hours, intralobular and smooth
interlobular thickening often develop within the
areas of ground glass, yielding a "crazy-paving"
pattern
o Imaging features resolve over 7-14 days as the
hemorrhage removed by macrophages
o Repeated episodes of hemorrhage lead to fibrosis,
1-3 mm scattered nodules, and a persistence of
reticular and ground glass opacities
o Wegner granulomatosis often has cavitary nodules
and multi focal areas of consolidation, which
extends to the lung periphery
Capillaritis form demonstrates similar imaging as
lPH, ABMABD and microscopic angitis with
sparing of lung periphery and eventual fibrosis
with recurrent bleeding episodes
o BMT associated DAH often not imaged with CT
given the severity of their clinical status
Bilateral extensive consolidations
1
Over the ensuing 48 hours, intralobular and smooth 45
interlobular thickening often develop within the
areas of ground glass, yielding a "crazy-paving"
pattern
Imaging features resolve over 7-14 days as the
hemorrhage removed by macrophages
Repeated episodes of hemorrhage lead to fibrosis, 1-3
mm scattered nodules, and a persistence of reticular
and ground glass opacities
Diagnostic Checklist
Although thin section CT can be helpful, it is clinical
history, tissue sampling and laboratory investigation
that are required in order to differentiate among the
DAH diseases
o Leukemia demonstrates bilateral multifocal or
diffuse ground glass and consolidation
Persistent septal lines over days to weeks should
also suggest cardiac decompensation or leukemic
pulmonary involvement
MR Findings
Has no important role in the evaluation of DAH
Reports demonstrate intermediate signal on Tl
sequences and low signal on T2 (iron susceptibility
effect)
o Pulmonary edema and pneumonia often
demonstrate high signal on T2
Imaging Recommendations
Best imaging tool
o Chest radiograph usually sufficient to document
extent of pathology in the acute setting
o Thin section CT: More sensitive in detecting DAH
and following its evolution from
consolidation/ground-glass to septal thickening,
small nodules and potentially fibrosis
I DIFFERENTIAL DIAGNOSIS
Pulmonary Edema
Cardiogenic: Cardiomegaly, bilateral symmetric
consolidative opacities, vascular indistinctness, septal
thickening and pleural effusions
o Resolves rapidly with therapy
Non-cardiogenic edema: Diffuse distribution of
grou nd -glass/ consolidati 0n
o Small bilateral effusions are commonly seen on CT
Hemorrhage will not shift with gravity (gravitational
shift test) as opposed to edema
Pulmonary Infection
Fever, chills, productive cough and elevated WBC are
common
Consolidation tends to be multifocal and asymmetric
Most infections will not evolve from consolidation to
a reticular pattern
 DIFFUSE ALVEOLAR HEMORRHAGE
1 less Common Etiologies for Diffuse Alveolar
46 Hemorrhage
Henoch-Sch6nlein purpura, severe uremia or bleeding
diathesis (disseminated intravascular coagulation
(DIC) and anticoagulation overdose)
I PATHOLOGY
General Features
General path comments
o Pathophysiology
Hemorrhage into alveolar spaces (consolidation)
Blood removed from alveoli by macrophages (2-3
days)
Macrophages migrate into interstitium (septal Demographics
thickening)
Macrophages removed by lymphatics (7-14 days)
o Chronic disease: Moderate to severe fibrosis
Associated abnormalities: Hemosiderin-laden
macrophages are a common finding with
bronchoalveolar lavage (BAL)
Microscopic Features
Goodpasture syndrome/ABMABD
o Extensive intra-alveolar blood and accumulation of
hemosiderin filled macrophages
o Mild neutrophilic capillaritis, but not a dominant
feature
o Immunofluorescence: Linear deposition of IgG and
complement along the basement membranes of the
glomeruli and alveoli
Idiopathic pulmonary hemosiderosis
o Intra-alveolar hemorrhage and hemosiderin filled
macrophages
o Mild neutrophilic capillaritis, but not a dominant
feature
o Chronic disease will demonstrate pulmonary fibrosis
Microscopic polyangiitis
o Extensive, but patchy areas of neutrophilic
capillaritis is predominately seen among the
background of pulmonary hemorrhage
Inflammation involving the arterioles and/or
venules may be present
Necrotizing glomerulonephritis (97%) and I DIAGNOSTIC
leukocytoclastic vasculitis of the skin are common
o Concurrent areas of hyaline membranes are
occasionally present, making this diagnosis difficult
at times to distinguish from diffuse alveolar damage
Wegner granulomatosis
o Parenchymal necrosis, granulomatous inflammation
and vasculitis major criteria for diagnosis
Vasculitis can affect arteries, arterioles, capillaries
and venules
SLE 1.
o Vasculitis of the arterioles or capillaritis can be seen
Immune complexes are demonstrated with
immunofluorescence
o Renal involvement in 60-90% of patients
ICLINICAL ISSUES
Presentation
Most common signs/symptoms: Cough, hemoptysis
(80%), and dyspnea
Other signs/symptoms
o Iron deficiency anemia
o P-ANCA for microscopic polyangiitis (80%)
o C-ANCA positive in 85-98% of patients with active
Wegner granulomatosis
Goodpasture syndrome/ABMABD
o May follow influenza-illness
BMT
o Usually occurs during the marrow engraftment
period (10-21 days following transplant)
Age: IPH usually < 15 yo, Goodpasture syndrome often
young adults, microscopic polyangiitis mean age is 55
yo and Wegner is most common between 30-55 yo
Gender
o Goodpasture syndrome has a male predominance,
up to 9:1
o SLE has a female predominance (70%) as does
microscopic polyangiitis of 1.5: 1
Natural History & Prognosis
IPH survival is about 50% after 5 years
SLE with DAH has a mortality rate of 40-60%
Microscopic polyangiitis 5 year survival is about 70%
Wegner granulomatosis has a 90% mortality if
untreated, but up to 75% will experience complete
remission following therapy
o Worse prognosis in patients> 60
Treatment
Immune complex diseases and inflammatory vasculitis
o Immunosuppression: Especially cytotoxic drugs
o Corticosteroid therapy
o Plasmapheresis to remove circulating antibodies
Bone marrow transplantation
o Early institution of high dose corticosteroids
improves survival
CHECKLIST
Image Interpretation Pearls
Although thin section CT can be helpful, it is clinical
history, tissue sampling and laboratory investigation
that are required in order to differentiate among the
DAH diseases
I SELECTED REFERENCES
Marten et ale Pattern-Based differential diagnosis in
pulmonary vasculitis using volumetric CT. AJR
184:720-733,2005
2. Hansell D: Small-vessel diseases of the lung: CT-pathologic
correlates. Radiology. 225:639-653, 2002
3. Travis et al: Non-neoplastic disorders of the lower
respiratory tract. 1st ed. Washington, DC, AFIP. 176-]87,
2002
 DIFFUSE ALVEOLAR HEMORRHAGE
I IMAGE GALLERY 1
47

(Left) Axial CECT in a 27 yo

male with Goodpasture
syndrome/ABMABD
demonstrates bilateral diffuse
ground-glass and ill-defined
centrilobular nodules. Note
sparing of the subpleural
region. (Right) Axial HRCT in
a 43 yo with SLE, 2 days
after presenting with DAH. It
demonstrates ground-glass
opacities with intralobu/ar
and smooth interlobular
septal thickening
("crazy-paving" ).

Typical
(Left) Axial HRCT in a 29 yo
female with IPH (biopsy
proven) and intermittent
hemoptysis. Ground-glass,
centrilobular nodules and
reticular opacities ref/ect
persistent bleeding episodes.
(Right) Axial HRCT in a 3 yo
with glomerulonephritis
demonstrates bilateral
ground-glass and short
reticular opacities, consistent
with DAH. Biopsies showed
microscopic polyangiitis, rare
in children.

Typical
(Left) Axial CECT in a 21 yo
male with AML, acute
dyspnea and hemoptysis.
Bilateral extensive patchy
lobular consolidation.
Bronchoscopy found only
blood and hemosiderin-filled
macrophages. (Right) Frontal
radiograph in a 27 yo male
with respiratory failure and
hematuria shows bilateral
peripheral consolidations
(DAH). C-ANCA was
positive and biopsies
confirmed Wegner
granulomatosis.
 GOODPASTURE SYNDROME

Diffuse consolidaUon with relaUve sparing of the Frontal radiograph in a 22 year old male with acute
periphery and costophrenic angles is characteristic of hemoptysis and hematuria, who had a viral illness 5
Goodpasture syndrome. weeks ago. Renal biopsy demonstrated IgG glomeruli
deposition (Goodpasture syndromeIABMABD).

ITERMINOlOGY I IMAGING FINDINGS

Abbreviations and Synonyms General Features
Antibasement membrane antibody disease (ABMABD) Best diagnostic clue: Acute diffuse ground-glass and
consolidation in a young adult patient with
Definitions hemoptysis and evidence of renal disease
First reported in 1919 by Goodpasture
o Patient with hemoptysis Radiographic Findings
o Autopsy: Diffuse alveolar hemorrhage (DAH) and Radiographic findings vary depending on length of
glomerulonephritis disease and the number of hemorrhagic episodes
o Patient was 6 weeks out from an influenza infection Early: Acute onset 24 hours) of a bilateral, but often
Goodpasture syndrome named in 1958 to describe the asymmetric ground-glass and consolidative opacities
combination of DAH and glomerulonephritis o Lung periphery and costophrenic angles are usually
Most patients found to have antibodies directed spared
against their glomerular basement membranes Involvement of the periphery or costophrenic
o Combination of circulating antiglomerular angles should suggest another diagnosis
basement membrane antibodies, glomerulonephritis o Unilateral involvement rare, but well-described
and DAH termed ABMABD o Radiograph improves over 5-10 days once bleeding
Terms vary has ended
o Most use Goodpasture syndrome to describe both Pleural effusions rare and should suggest another
DAH and glomerulonephritis diagnosis or concurrent cardiac failure
o ABMABD used when circulating anti basement Late manifestations relate to the underlying
antibodies present hemosiderin deposition and pulmonary fibrosis

DDx: Goodpasture Syndrome (ABMABD)

Capillary Leak Vasculitis Pneumonia

 GOODPASTURE SYNDROME
Key Facts
Terminology
Antibasement membrane antibody disease (ABMABD)
Combination of circulating anti glomerular basement
membrane antibodies, glomerulonephritis and DAH
termed ABMABD
Most use Goodpasture syndrome to describe both
DAH and glomerulonephritis
Imaging Findings
Best diagnostic clue: Acute diffuse ground-glass and
consolidation in a young adult patient with
hemoptysis and evidence of renal disease
Radiographic findings vary depending on length of
disease and the number of hemorrhagic episodes
Lurig periphery and costophrenic angles are usually
spared
o Fibrosis can become quite severe and even lead to
pulmonary hypertension and cor pulmonale
CT Findings
HRCT
o Bilateral patchy or diffuse areas of ground-glass
opacities and consolidation common in acute
episodes
Sparing of the subpleural regions and
costophrenic angles characteristic
o Late: Asymmetric pulmonary fibrosis, reticular
opacities and traction bronchiectasis
MR Findings
MRI's role in imaging patients with Goodpasture
limited
o T2 weighted images: Decreased signal within the
lungs secondary to the paramagnetic properties of
hemosiderin
Pulmonary edema and pneumonia demonstrate
high signal intensity on T2 weighted images
Imaging Recommendations
Best imaging tool
o Thin-section CT to characterize the nature and
extent of disease
Most patients quite ill at presentation and chest
radiograph may be the only imaging option
I DIFFERENTIAL DIAGNOSIS
Idiopathic Pulmonary Hemosiderosis
Diffuse pulmonary hemorrhage syndrome without an
identifiable cause
Recurrent episodes of diffuse pulmonary hemorrhage,
usually in young patients 10 years old)
o Although 20% may occur in adults
Hemoptysis very common and can be quite severe
No renal involvement and ANCA's or anti basement
membrane antibodies absent
1
Involvement of the periphery or costophrenic angles 49
should suggest another diagnosis
Late: Asymmetric pulmonary fibrosis, reticular
opacities and traction bronchiectasis
Clinical Issues
Acute shortness of br,eath, cough with mild
hemoptysis and anemia
Hemoptysis reported in 80-95%
Recurrent episodes of pulmonary hemorrhage
common
Untreated Goodpasture syndrome often has
fulminant course leading to death
Early therapy results in both renal and lung disease
remission
Combination of plasmapheresis and
immunosuppressive therapy
SLE Vasculitis
Systemic immune disorder in which autoantibodies
are directed against nuclear antigens
2% of all patients with SLE develop DAH
May be the presenting problem, closely resembling
ABMABD or idiopathic pulmonary hemosiderosis
o Renal involvement seen in 60-90%
Immune complexes seen on immunofluorescence
Microscopic Polyangiitis
Necrotizing vasculitis involving small vessels
(arterioles, venules and capillaries) and no immune
deposits
Most common cause of pulmonary-renal syndrome
o Involvement of the kidneys> 95% and lung about
50%
o Greater than 80% have a positive ANCA, usually
P-ANCA
Onset may be rapid, which includes fever, myalgia,
arthralgia and ear, nose or throat symptoms
o Extensive bilateral consolidations common, usually
with a lower lobe distribution
Wegner Granulomatosis
Systemic vasculitis, typically affects the kidneys, upper
and lower respiratory tract
Peripheral wedge-shaped areas of consolidation and/or
cavitary nodules
o Less common: Acute diffuse bilateral consolidation,
often seen early or in younger patients
o DAH likely represents a capillaritis form of Wegner
C-ANCA positive in 85-98% of patients with active
disease
Systemic symptoms of fever, weight loss, arthralgias
and peripheral neuropathy
o Involves multiple organ systems
Churg-Strauss Syndrome
Multisystem disorder: Asthma/history of allergy,
peripheral blood eosinophils and systemic vasculitis
o Diagnosis based on clinical criteria
 GOODPASTURE SYNDROME
1 Multifocal and evolving areas of consolidation one of
six criteria
Renal involvement alone in 20-40% and lung as
the only site in < 10%
50 o Consolidation not diffuse and tends to involve o Pulmonary hemorrhage may sometimes precede
periphery of lung renal disease, even up to several months
Symptoms more indolent Other signs/symptoms
o Hematuria, proteinuria and elevated serum creatine
Pneumocystis Jiroveci Pneumonia
may be found at presentation
Seen in patients with cell-mediated Glomerulonephritis usually rapidly progressive
immunodeficiency, especially AIDS o History of recent viral illness common
More subacute onset of dyspnea, fever and hypoxia
Common: Perihilar or diffuse ground glass and Demographics
consolidative opacities Age
o No renal involvement, hemoptysis and onset more o Bimodal distribution
insidious Most young men with lung and renal disease,
usually more than 15 years old
Non-Cardiogenic Edema
Less common: Older women with predominantly
Common: Acute onset of diffuse ground-glass and renal involvement
consolidative opacities Gender: M:F at least 2:1, but maybe as high as 9:1 in
Hemoptysis rare younger patients
Renal involvement not characteristic, unless etiology
for the edema renal failure Natural History & Prognosis
Recurrent episodes of pulmonary hemorrhage
common
I PATHOLOGY o May lead to progressive pulmonary insufficiency
and fibrosis
General Features o Progressive renal insufficiency common
Genetics Untreated Goodpasture syndrome often has fulminant
o ABMABD reported in siblings and identical twins, course leading to death
supporting genetic predisposition Early therapy results in both renal and lung disease
HLA-DR2 seen in majority of patients remission
Etiology
o Anti-basement membrane antibodies detected by Treatment
radioimmunoassay in the blood in > 90% Combination of plasmapheresis and
Antibodies directed at type IV collagen's alpha 3 immunosuppressive therapy
chain o Plasmapheresis removes circulating antibodies
Epidemiology: Associated with influenza A and various o Immunosuppression with corti co steroids and
inhalation exposures such as hydrocarbons cytotoxic drugs
Associated abnormalities: 30% have positive serum C- Consider transplantation for renal failure
ar P-ANCA's
Microscopic Features I SELECTED REFERENCES
Renal biopsy demonstrates linear deposition of IgG
1. Collard HR et al: Diffuse alveolar hemorrhage. Clin Chest
along the glomerular basement membranes by
Med. 25(3):583-92, vii, 2004
immunofluorescence 2. Jara LJ et al: Pulmonary-renal vasculitic disorders:
o Renal biopsy the most common method to establish differential diagnosis and management. Curr Rheumatol
diagnosis Rep. 5(2):107-15, 2003
Lung biopsy not common, but similar findings of 3. Travis et al: Non-neoplastic disorders of the lower
linear IgG found along alveolar basement membranes respiratory tract. 1st ed. Washington, DC, AFIP. 184-185,
o Distribution more patchy and more difficult to 2002
interpret than renal biopsy specimens 4. Specks U: Diffuse alveolar hemorrhage syndromes. Curr
Opin Rheumatol. 13(1):12-7, 2001
o Intraalveolar hemorrhage and hemosiderin 5. Savage CO et al: ABC of arterial and vascular disease:
o Occasional neutrophilic capillaritis present, but vasculitis. Bmj. 320(7245):1325-8, 2000
extensive vasculitis not seen 6. Primack, S: Pulmonary hemorrhage. Pulmonary and
cardiac Imaging. New York, Marcel Dekker, 219-43,1997
7. Primack SL et al: Diffuse pulmonary hemorrhage: clinical,
IClINICALISSUES pathologic, and imaging features. AJR Am J Roentgenol.
164(2):295-300, 1995
Presentation 8. Conlon PJ et al: Antiglomerular basement membrane
Most common signs/symptoms disease: the long-term pulmonary outcome. Am J Kidney
Dis. 23(6):794-6,1994
o Acute shortness of breath, cough with mild
9. Mulier NL et al: Diffuse pulmonary hemorrhage. Radial
hemoptysis and anemia CIin North Am. 29(5):965-71, 1991
Hemoptysis reported in 80-95% 10. Young KR et al: Pulmonary-renal syndromes. Clin Chest
Anemia and pallor common Med. 10(4):655-75, 1989
o Renal and lung involvement in 60-80%
 GOODPASTURE SYNDROME
I IMAGE GALLERY 1
51

(Left) Frontal radiograph in a

27 year old male with acute
shortness of breath. Subtle
ground-glass opacities are
seen bilaterally. The patient
had hemoptysis and
hematuria. (Right) Frontal
radiograph 8 hours later after
multiple episodes of
hemoptysis. The portable
exam demonstrates diffuse
worsening ground-glass
opacities.

Typical
(Left) Axial HRCT
demonstrates a diffu?e
distribution of these
predominantly ill-defined
ground-glass nodular
opacities. (Right) Axial HRCT
does not show any pleural
effusions, common with
edema. Bronchoscopy
demonstrated blood in the
lavage. A renal biopsy
confirmed the diagnosis of
ABMABO.

(Left) Frontal radiograph in a

S2 year old male with
ABMABO. Renal biopsy
demonstrated IgC deposits
along the glomeruli. Similar
histology was found in the
lung at autopsy. (Courtesy
Steve Primack, MOJ. (Right)
Axial HRCT in a 47 year old
female demonstrates bilateral
asymmetric ground glass
opacities. This scan was 2
days after her hemoptysis
event. ABMABO was
confirmed by renal biopsy.
 EOSINOPHiliC PNEUMONIA
1
52

Radiograph shows bilateral, peripheral areas of Axial NECT shows bilateral, lower-lobe predominant,
consolidation. This pattern has been referred to as the confluent areas of ground-glass opacity and patchy foci
"photographic negative of pulmonary edema" and is of consolidation due to AEP.Also note small, dependent
characteristic of CEP. pleural effusions.

Location
I TERMINOLOGY o AEP: Lower lung zone predominant
Abbreviations and Synonyms o CEP: Upper lung zone predominant
Acute eosinophilic pneumonia (AEP) Radiographic Findings
Chronic eosinophilic pneumonia (CEP) Radiography
Pulmonary infiltration with eosinophilia (PIE), LOffler o AEP
syndrome
Combined alveolar and interstitial pattern with
Definitions lower lung zone predominance
Acute and chronic pneumonias due to eosinophilic Rapid progression over hours to days
infiltration with or without blood eosinophilia Small pleural effusions common
Eosinophils cause damage by recruitment, activation, Rapid response to corticosteroid therapy
and interaction with other inflammatory and immune o CEP
cells Bilateral, nonsegmental, homogeneous
consolidation with peripheral distribution and
upper lung zone predominance (66%)
I IMAGING FINDINGS May involve entirety of one lung
Usually persistent over time in absence of
General Features treatment, but sometimes transient/fleeting
Best diagnostic clue When recurrent, often in same location
o AEP: Mimics pulmonary edema (bilateral alveolar Pleural effusions rare
and interstitial opacities) Rapid response to corticosteroid therapy
o CEP: Photographic negative of pulmonary edema In response to therapy, the most peripheral areas
(peripheral consolidation) of consolidation are typically the first to clear

DDx: Peripheral Lung Opacities

Churg-Strauss Syndrome Cryptogenic Organizing Pneumonia

 EOSINOPHiliC PNEUMONIA

Key Facts
1
Terminology Aspiration Pneumonia 53
Acute eosinophilic pneumonia (AEP) Pathology
Chronic eosinophilic pneumonia (CEP) AEP: Diffuse alveolar damage (acute or organizing)
Acute and chronic pneumonias due to eosinophilic associated with large number of interstitial and
infiltration with or without blood eosinophilia alveolar eosinophils
Imaging Findings CEP: Filling of alveolar air spaces by inflammatory
AEP: Mimics pulmonary edema (bilateral alveolar and infiltrate with a high proportion of eosinophils
interstitial opacities) Clinical Issues
CEP: Photographic negative of pulmonary edema Both AEP and CEP show rapid response to
(peripheral consolidation) corticosteroid therapy
Chest radiograph usually suffices for diagnosis and Relapse unusual in AEP but common in CEP
follow-up
Diagnostic Checklist
Top Differential Diagnoses Peripheral band-like opacities paralleling chest wall
Cryptogenic Organizing Pneumonia (COP) subtle clue to the diagnosis
Pulmonary Infarcts

As the peripheral areas of consolidation clear, Eosinophilic lung disease due to specific causes may
residual band-like opacities may be visualized mimic AEP
coursing parallel to pleural surface o Drugs
Antibiotics
CT Findings Nonsteroidal anti-inflammatory agents
NECT Agents used for treatment of inflammatory bowel
o AEP disease
Bilateral, lower-lobe predominant ground-glass Inhaled non-therapeutic drugs including cocaine
opacities and heroin
Smooth interlobular septal thickening and o Parasitic infestation
thickening of bronchovascular bundles Ascaris lumbricoides
Occasional localized areas of consolidation or Strongyloides stercoralis
small nodules Ancylostoma duodenale
Small pleural effusions common o Fungal infection
Band-like opacities paralleling chest wall that may Allergic bronchopulmonary aspergillosis
even cross pleural fissures nearly pathognomonic Coccidiomycosis
o CEP Hypereosinophilic syndrome
Peripheral distribution of consolidation more o Persistent eosinophilia> 6 months
frequently detected with CT than with chest o Multiorgan system involvement
radiographs o Transient consolidation on chest radiographs
Ground-glass opacities often seen in association Churg-Strauss syndrome
with consolidation, may give rise to halo sign o May be indistinguishable from CEP on imaging
HRCT: Ground-glass opacities admixed with septal studies
thickening (crazy paving) o Presence of systemic disease helps to distinguish
Imaging Recommendations from CEP
GI: Abdominal pain, diarrhea, bleeding
Best imaging tool
Cardiac: Heart failure, pericarditis
o Chest radiograph usually suffices for diagnosis and
Renal insufficiency
follow-up
o In CEP, CT may be helpful for cases that lack a Arthralgias
classic peripheral distribution on chest radiograph Cryptogenic Organizing Pneumonia (COP)
Characteristic peripheral distribution of CEP is Peripheral distribution of consolidation mimics CEP,
more frequently detected with CT (95%) than but lower lung zones more commonly affected in COP
with chest radiographs (65%) COP may also demonstrate bronchovascular
distribution in minority of cases

I DIFFERENTIAL DIAGNOSIS Pulmonary Infarcts

Peripheral distribution may mimic CEP, but infarcts
Other Causes of Eosinophilic Lung Disease are usually more discrete and less confluent than CEP
Simple pulmonary eosinophilia (Loffler syndrome) Lower lobe predominant and often wedge-shaped
o Peripheral consolidation mimics CEP, but opacities
are characteristically fleeting rather than persistent
o Spontaneous resolution within 1 month
 EOSINOPHiliC PNEUMONIA
1 Aspiration Pneumonia
CEP: Peripheral eosinophilia present> 90%;
increase in eosinophils on bronchoalveolar lavage
54 Dependent location (especially posterior aspect upper (> 25% eosinophils)
lobes and superior segment lower lobes)
Associated small airways disease (tree-in-bud pattern) Demographics
common Age: CEP peak incidence during 4th decade; AEP all
ages
Diffuse Pulmonary Hemorrhage
Gender: CEP women affected twice as frequently as
Diffuse pulmonary consolidation, evolves into men; AEP no gender predominance
reticular intersitial pattern during resolution
Anemia and hemoptysis (80%) common Natural History & Prognosis
History renal disease common Often misdiagnosed as pneumonia with apparent
"response" to antibiotics; this tends to delay the
diagnosis for months or years
I PATHOLOGY Rapid clearing with steroids over a period of days
(complete resolution in a week)
General Features AEP may be life-threatening
General path comments
o AEP: Diffuse alveolar damage (acute or organizing) Treatment
associated with large number of interstitial and Both AEP and CEP show rapid response to
alveolar eosinophils corticosteroid therapy
o CEP: Filling of alveolar air spaces by inflammatory Relapse unusual in AEP but common in CEP
infiltrate with a high proportion of eosinophils
Etiology: Pathogenesis unknown, but speculated to
represent a hypersensitivity reaction to an unknown I DIAGNOSTIC CHECKLIST
antigen
Epidemiology Consider
o Prevalence: 1 case per 1,000,000 population per year Always consider specific causes of eosinophilic lung
AEP may be increased in military deployments disease such as drugs, parasitic infestation and fungal
Associated abnormalities: Asthma in 50% of patients infection
with CEP
Image Interpretation Pearls
Microscopic Features AEP mimics pulmonary edema
Alveoli flooded with eosinophils, macrophages, and CEP demonstrates photographic negative of
mononuclear cells pulmonary edema
Review of multiple old films often suggestive of the
Staging, Grading or Classification Criteria diagnosis
Diagnosis made by satisfying one of 3 criteria Peripheral band-like opacities paralleling chest wall
o Peripheral eosinophilia and chest radiographic subtle clue to the diagnosis
abnormalities
o Tissue eosinophilia confirmed by biopsy
o Increased eosinophils in bronchoalveolar lavage I SElECTED REFERENCES
1. Silva Cl et al: Asthma and associated conditions:
high-resolution CT and pathologic findings. AJR Am J
I CLINICAL ISSUES Roentgenol. 183(3):817-24,2004
2. Johkoh T et al: Eosinophilic lung diseases: diagnostic
Presentation accuracy of thin-section CT in III patients. Radiology.
Most common signs/symptoms 216(3):773-80,2000
o AEP 3. Fraser RS et al: Eosinophilic lung disease. In: Fraser and
Acute onset of fever, shortness of breath, myalgias Pare's Diagnosis of Diseases of the Chest, 4th Ed. W.B.
and pleuritic chest pain Saunders Co., Philadelphia. 1743-1756, 1999
4. Allen IN et al: Eosinophilic lung diseases. Am J Respir Crit
Hypoxemic respiratory failure
Care Med. 150:1423-38, 1994
May be more common in smokers 5. Mayo JR et al: Chronic eosinophilic pneumonia: CT
o CEP findings in six cases. AJR. 153:727-30, 1989
Insidious onset fever (often at night), malaise, 6. Gaensler EA et al: Peripheral opacities in chronic
weight loss, dyspnea and cough eosinophilic pneumonia: The photographic negative of
Nearly 50% have asthmatic symptoms pulmonary edema. AJR. 128:1-13, 1977
90% nonsmokers
Other signs/symptoms
o Laboratory data
AEP: Peripheral eosinophilia absent but marked
increase in eosinophils on bronchoalveolar lavage
(> 30-40% eosinophils)
 EOSINOPHILIC PNEUMONIA

I IMAGE GALLERY 1
55

(Left) Axial H RCT shows

bilateral peripheral areas of
consolidation and
ground-glass opacity in
patient with CEP (Right)
Radiograph shows bilateral
peripheral areas of
consolidation (photographic
negative of pulmonary
edema) due to CEP

Typical
(Left) Frontal radiograph
shows extensive
consolidation in left perihilar
region and throughout the
periphery of the left lung due
to CEP (Right) Frontal
radiograph obtained 3 days
following initiation of
corticosteroid therapy shows
rapid improvement in
consolidation.

(Left) Axial NECT shows a

band-like area of
consolidation in the right
upper lobe which parallels
the chest wall and spares the
extreme lung periphery. This
is characteristic of resolving
CEP (Right) Axial NECT of
the same patient shows an
additional band-like area of
opacity in the right lower
lobe.
 ACUTE INTERSTITIAL PNEUMONIA
1
56

Axial HRCT in the proliferative phase of AlP shows Axial HRCT showing diffuse symmetrical ground-glass
diffuse but patchy ground-glass opacification in both opacification. A few lobular areas of sparing are noted
lungs. There is evidence of traction bronchiectasis (arrow) and there are bilateral pleural effusions (open
(arrows) in both lungs. arrows).

o Diffuse bilateral and symmetrical air space

I TERMI NOLOGY opacification
Abbreviations and Synonyms Normal in first 24 hours
Acute interstitial pneumonia (AlP), Hamman-Rich No particular zonal predilection
syndrome, traumatic wet lung, non-cardiogenic Pleural effusions/septal lines less common than in
pulmonary edema, Da Nang lung cardiogenic edema

Definitions CT Findings
Rapidly progressive disorder of unknown etiology HRCT
characterized by diffuse alveolar damage on biopsy o More sensitive than plain radiography in evaluating
Formerly called Hamman-Rich syndrome diffuse lung disease
o Bilateral abnormalities most common in lower zone
and dependent lung
IIMAGING FINDINGS o More often symmetrical than acute respiratory
distress syndrome (ARDS)
General Features o Ground-glass opacification
Best diagnostic clue: Diffuse apparently symmetrical May be seen in all phases of AlP
air space opacification Likely reflects differing histopathologic processes
Location in different phases
o Bilateral More extensive ground-glass opacification
o Lower lung zones (without traction bronchiectasis/bronchiolectasis)
associated with better outcome
Radiographic Findings o Dense parenchymal opacification
Radiography Also may be seen in all phases of AlP

DDx: Acute Interstitial Pneumonia

ARDS Diffuse Hemorrhage

 ACUTE INTERSTITIAL PNEUMONIA
Key Facts
Terminology
Rapidly progressive disorder of unknown etiology
characterized by diffuse alveolar damage on biopsy
Imaging Findings
Best diagnostic clue: Diffuse apparently symmetrical
air space opacification
Diffuse bilateral and symmetrical air space
opacification
Bilateral abnormalities most common in lower zone
and dependent lung
Ground-glass opacification
Dense parenchymal opacification
Architectural distortion
Honeycombing
Traction bronchiectasis/bronchiolectasis
More extensive air space opacification (without
traction bronchiectasis/bronchiolectasis)
associated with better outcome
o Architectural distortion
May be seen in the proliferative/fibrotic phases of
AlP
o Honeycombing
More common than in ARDS
o Traction bronchiectasis/bronchiolectasis
May be seen in the proliferative/fibrotic phases of
AlP
Associated with poorer outcome
May persist in survivors
o Less common CT findings include
Interlobular septal thickening
"Crazy paving" appearance
Nodular opacities
Thickening of bronchovascular bundles
Imaging Recommendations
Best imaging tool: CT or HRCT for characterization of
diffuse pulmonary disease
Protocol advice: HRCT (1 mm collimation at 10 mm
intervals, supine, ventilation suspended in inspiratory
phase if possible) or multidetector-row CT acquisition
I DIFFERENTIAL DIAGNOSIS
Acute Respiratory Distress Syndrome (ARDS)
Associated with a known cause (direct or indirect lung
insult)
Diffuse bilateral air space opacification (ground-glass
opacification and dense parenchymal opacification
admixed with regions of apparently normally-aerated
lung)
Honeycombing less common in ARDS
Lower zone and symmetric distribution less common
in ARDS
Hydrostatic Pulmonary Edema
Bilateral air space opacification
Enlarged heart
1
Top Differential Diagnoses 57
Acute Respiratory Distress Syndrome (ARDS)
Hydrostatic Pulmonary Edema
Diffuse Intraalveolar Hemorrhage
Alveolar Proteinosis (and Potentially Other Causes of
a "Crazy-Paving" Pattern)
Disseminated Infection (e.g., Pneumocystis Jiroveci
Pneumonia)
Accelerated Idiopathic Pulmonary Fibrosis (IPF)
Adenocarcinoma/Bronchoalveolar Cell Carcinoma
Pathology
Diffuse alveolar damage is the histopathologic
hallmark and evolves through three (overlapping)
stages
Pleural effusion
History of cardiac disease
Diffuse Intraalveolar Hemorrhage
Diffuse ground-glass opacification, often evolves into
reticular pattern
Features of pulmonary fibrosis may be seen but
generally only with repeated episodes
Anemia and hemoptysis (80%) common
Alveolar Proteinosis (and Potentially Other
Causes of a "Crazy-Paving" Pattern)
Geographical areas of ground-glass opacification and
thickened smooth interlobular septa
No particular zonal distribution
Recognized association between alveolar proteinosis
and hematologic malignancies in adults
Recognized association between alveolar proteinosis
and immunodeficiency states in children
Disseminated Infection (e.g., Pneumocystis
Jiroveci Pneumonia)
Variable appearances but typically bilateral
ground-glass opacification
Known history of immunocompromise
Accelerated Idiopathic Pulmonary Fibrosis
(IPF)
Rare complication of IPF
Diffuse but patchy ground-glass opacification on
background of characteristic changes of IPF
Poor prognosis
Adenocarcinoma/Bronchoalveolar Cell
Carcinoma
Diffuse bilateral ground-glass opacification
No signs of fibrosis (parenchymal distortion, traction
bron chiecta sis/b ron chio Iectasi s)
Insidious onset and progressive course
 ACUTE INTERSTITIAL PNEUMONIA
1 I PATHOLOGY Recurrent episodes and end-stage interstitial fibrosis
with honeycombing may occur in survivors
58 General Features Treatment
General path comments
No known treatment effective
o Diffuse alveolar damage is the histopathologic
Supportive care is mainstay
hallmark and evolves through three (overlapping)
stages
Acute exudative phase
Proliferative phase
I DIAGNOSTIC CHECKLIST
Fibrotic phase Image Interpretation Pearls
Epidemiology Consider diagnosis in patients with rapidly progressive
o Rare fulminant form of idiopathic lung injury breathlessness, diffuse opacification on radiologic
o Occurring over wide age range (mean = SO yes) examination but no obvious precipitating factor
o No gender predominance

Gross Pathologic & Surgical Features

Three recognizable but overlapping phases
I SELECTED REFERENCES
o Acute exudative 1. Lynch DA et al: Idiopathic Interstitial Pneumonias: cr
o Proliferative Features. Radiology. 236(1):10-21, 2005
o Fibrotic 2. Pipavath 5 et al: Imaging of the chest: idiopathic interstitial
pneumonia. Clin Chest Med. 25(4):651-6, v-vi, 2004
Microscopic Features 3. Wittram C: The idiopathic interstitial pneumonias. Curr
Probl Diagn Radiol. 33(5):189-99, 2004
Acute exudative phase
4. Willram C et al: CT-histologic correlation of the AT5/ER5
oEdema
2002 classification of idiopathic interstitial pneumonias.
o Hemorrhagic fluid in air spaces Radiographies. 23(5):1057-71, 2003
o Type I pneumocyte necrosis 5. Ichikado K et al: Acute interstitial pneumonia: comparison
o Hyaline membranes of high-resolution computed tomography findings
Proliferative phase between survivors and nonsurvivors. Am J Respir Crit Care
o Type II pneumocyte proliferation Med. 165(11):1551-6,2002
o Collagen deposition 6. Tomiyama N et al: Acute respiratory distress syndrome and
o Myofibroblast proliferation acute interstitial pneumonia: comparison of thin-section
CT findings. J Comput Assist Tomogr. 25(1):28-33, 2001
Fibrotic phase 7. Mihara N et al: Can acute interstitial pneumonia be
o Fibrosis within alveoli and interstitium (may be differentiated from bronchiolitis obliterans organizing
severe) pneumonia by high-resolution CT? Radiat Med.
18(5):299-304, 2000
8. Tomiyama N et al: Acute parenchymal lung disease in
I CLINICAL ISSUES immunocompetent patients: diagnostic accuracy of
high-resolution cr. AJR Am J Roentgenol. 174(6):1745-50,
Presentation 2000
9. Vourlekis J5 et al: Acute interstitial pneumonitis. Case
Most common signs/symptoms
series and review of the literature. Medicine (Baltimore).
o Acute onset (over a period of 1-3 weeks)
79(6):369-78,2000
o Similar presentation to ARDS except no etiologic 10. Johkoh T et al: Acute interstitial pneumonia: thin-section
factor identifiable CT findings in 36 patients. Radiology. 211(3):859-63, 1999
Breathlessness 11. Johkoh T et al: Crazy-paving appearance at thin-section
Cough cr: spectrum of disease and pathologic findings.
Rapid progression to respiratory failure requiring Radiology. 211(1):155-60,1999
mechanical ventilation 12. JOhkoh T et al: Idiopathic interstitial pneumonias:
Majority of patients fulfill American-European diagnostic accuracy of thin-section CT in 129 patients.
Radiology. 211(2):555-60,1999
Consensus Conference Criteria for diagnosis of
13. Murayama 5 et al: "Crazy paving appearance" on high
ARDS resolution cr in various diseases. J Com put Assist Tomogr.
Pyrexia and "viral-like" illness in nearly 50% at 23(5):749-52, 1999
presentation 14. Howling 5J et al: The significance of bronchial dilatation
o Widespread diffuse crackles on auscultation on cr in patients with adult respiratory distress syndrome.
Other signs/symptoms Clin Radial. 53(2):105-9, 1998
o Myalgia 15. Akira M et al: CT findings during phase of accelerated
a Arthralgia deterioration in patients with idiopathic pulmonary
fibrosis. AJR Am J Roentgenol. 168(1):79-83, 1997
Demographics 16. Ichikado K et al: Acute interstitial pneumonia:
high-resolution CT findings correlated with pathology. AJR
Age: Wide age range
Am J Roentgenol. 168(2):333-8, 1997
Gender: M = F 17. Primack 5L et al: Acute interstitial pneumonia:
Natural History & Prognosis radiographic and CT findings in nine patients. Radiology.
188(3):817-20, 1993
Poor prognosis (mortality rate usually;:>: 50%; most
deaths within 2 months of onset)
 ACUTE INTERSTITIAL PNEUMONIA
I IMAGE GALLERY 1
59

(Left) Frontal radiograph

with subtle but diffuse
ground-glass opacification of
AlP in the acute exudative
phase. (Right) Axial HRCT
shows diffuse symmetric
ground-glass opacification in
AlP. Bilateral small volume
pleural effusions.

(Left) Axial HRCT at the level

of the aortic arch showing
widespread ground-glass
opacification, severe traction
bronchiectasis (arrows) and
honeycombing (open
arrow). (Right) Axial H RCT
at the level of the venous
confluence. There is
ground-glass opacification
and traction bronchiectasis.

Typical
(Left) Axial HRCT shows
diffuse ground-glass
opacification and traction
bronchiectasis/bronchia/ectasis.
Also evidence of peripheral
honeycombing (arrows)
bilaterally. (Right) Axial
HRCT at the level of the
aortic arch shows diffuse but
asymmetric abnormality in
AlP. Left upper lobe is spared
(arrows).
 METASTATIC PULMONARY CALCIFICATION
1
60

Frontal radiograph shows diffuse nodularity more Axial H RCT shows clusters of poorly defined
profuse in the upper lung zones. Mild cardiomegaly and centrilobular nodules in the dorsal aspect of the upper
bipolar pacemaker. Long history of renal dialysis. lobes. Note the well defined emphysema.

Normal high kVp technique not optimal for

ITERMINOLOGY detection of calcification
Abbreviations and Synonyms Dual-energy digital radiography more sensitive
Pulmonary calcinosis than conventional
o Diffuse or focal, ill-defined, nodular and linear
Definitions opacities
Calcium deposition in normal tissue, predominantly Often mistaken for pneumonia, aspiration, or
affects lung, stomach, kidney, and heart edema
o Upper lobes most commonly involved
o Focal lobar or segmental consolidation due to an
IIMAGING FINDINGS infarct
o Cardiovascular
General Features May have cardiomegaly (from renal failure)
Best diagnostic clue: High density (or calcific) focal Often have pacemakers or internal defibrillators
opacities in the upper lung zones due to conduction abnormalities
Location Indwelling hemodialysis catheters
o Tropism for tissues with relative alkaline pH o Lytic bone lesions (brown tumors) from
Upper lung zones hyperparathyroidism or spinal changes from renal
Gastric wall osteodystrophy
Kidney medulla
CT Findings
Radiographic Findings CECT
Radiography o Focal calcification may be due to vascular occlusion
o Rarely detected unless severe which may be identified with CT angiography

DDx: Metastatic Pulmonary Calcification

Microlithiasis
 METASTATIC PULMONARY CALCIFICATION
Key Facts
1
Terminology Talcosis 61
Calcium deposition in normal tissue, predominantly Amyloidosis
affects lung, stomach, kidney, and heart Alveolar Microlithiasis
Idiopathic Ossification
Imaging Findings
Pathology
Mulberry-shaped or miniature cotton balls,
amorphous calcifications 3-10 mm diameter Physiology: Normally high V/Q ratio in upper lobes
leads to alkaline pH (7.51)
Centrilobular location
Best imaging tool: CT or bone scanning sensitive for Calcium is less soluble in alkaline environment
detection of calcium, CT useful to characterize Clinical Issues
distribution Gradual onset dyspnea, however, some have sudden
Top Differential Diagnoses onset of symptoms and rapid fulminant course
Tuberculosis Diagnostic Checklist
Silicosis Suspect in chronic hemodialysis patients with
Sarcoidosis chronic ill-defined opacities in the upper lung zones
Mitral Stenosis

a Associated findings Cavitation not seen in metastatic calcification but may

Parathyroid masses in parathyroid adenomas
Multiple thyroid nodules (medullary thyroid
carcinoma) or adrenal masses
(Pheochromocytoma) suggests multiple endocrine
neoplasia 2 (MEN 2): 20% develop
hyperparathyroidism
Pancreatic mass (Islet cell), thymic or bronchial
carcinoids suggests MEN 1: 80% develop
hyperparathyroidism
Lytic bone lesions from hyperparathyroidism
HRCT
a More sensitive than chest radiography for calcium
a Mulberry-shaped or miniature cotton balls,
amorphous calcifications 3-10 mm diameter
Centrilobular location
Edge of nodule may be slightly denser than the
center producing faint "rings" on mediastinal
windows
a Non-specific ground-glass opacities or areas of
consolidation
a Small vessel calcification in the chest wall, heart, or
pulmonary vasculature
a Emphysema may be admixed with areas of
ground-glass opacities or consolidation
Other Modality Findings
Bone Scanning
a Uptake with bone seeking radionuclides
Imaging Recommendations
Best imaging tool: CT or bone scanning sensitive for
detection of calcium, CT useful to characterize
distribution
I DIFFERENTIAL DIAGNOSIS
Tuberculosis
Upper lobes also primarily involved but does not have
extensive calcification unless healed
have emphysema admixed with the areas of
parenchymal involvement
Prior granulomatous disease more likely to result in
traction bronchiectasis and lung scarring
Silicosis
Silicotic nodules may calcify, upper lobes also
primarily involved
Subpleural nodules rare with metastatic calcification
Occupational history important
Mediastinal and hilar adenopathy not seen with
metastatic calcification
Sarcoidosis
Nodules may calcify although rare, upper lobes also
primarily involved
Peribronchial and subpleural distribution not seen
with metastatic calcification
Adenopathy (which typically regresses with worsening
of lung disease) not seen with metastatic calcification
Sarcoidosis associated with hypercalcemia (due to
increased production of calcitriol) and thus are at risk
to develop metastatic pulmonary calcification
a Hypercalcemia in sarcoidosis seasonal due to UV
light sensitivity
Mitral Stenosis
Left atrial enlargement and vascular redistribution
(pulmonary venous hypertension)
a Generalized cardiomegaly and chronic edema also
common with metastatic calcification
Ossification primarily lower lobes
Takosis
History of drug abuse
Upper lobe micronodules 1 mm) smaller than those
in metastatic calcification and tend to aggregate into
perihilar fibrotic masses
Basilar panacinar emphysema not seen with metastatic
calcification
Amyloidosis
Nodules larger, small nodules generally do not calcify
 METASTATIC PULMONARY
1 Often have interlobular septal thickening
o Even though the alveolar septa calcify pathologically
62 there is usually no signs of septal thickening
Confluent irregular consolidation
Osseous metaplasia
Alveolar Microlithiasis
Calcification smaller, on the order of 1 mm
Diffuse disease more severe in lower lobes
Paraseptal emphysema
Idiopathic Ossification
Dendritic calcification in the lower lobes
o Maybe isolated or in conjunction with other
interstitial lung fibrosis
Generally seen as incidental finding in old men
I PATHOLOGY
General Features
General path comments: Calcium deposition in
otherwise normal tissue in contrast to dystrophic
calcification which is calcium deposition in abnormal
tissue
Etiology
o Hypercalcemic conditions (high calcium phosphate
product> 70, normal 40)
Chronic renal failure
Steroid and phosphate therapy
Chronic immobilization
Skeletal metastases
Hyperparathyroidism
Hypervitaminosis D
Milk-alkali syndrome
Sarcoidosis
o Physiology: Normally high v /Q ratio in upper lobes
leads to alkaline pH (7.51)
Calcium is less soluble in alkaline environment
Alkaline pH also in gastric wall, renal medulla
o Focal lobar or segmental calcification suggests
vascular occlusion to the supplied area
High V/Q ratio from vascular occlusion
predisposes to calcium deposition
Associated abnormalities: In order, lung, stomach,
kidney and heart most frequently involved
Gross Pathologic & Surgical Features
Rigid and gritty on cut section with retention of lung
architecture
Involved lung does not collapse in contrast to
noninvolved areas
Involvement of various body sites parallel each other
Microscopic Features
Alveolar septal and vascular deposition (SOx over
normal)
o In contrast, interstitial findings rare at HRCT
o Alkaline tissues (stomach, kidney) also preferentially
affected
o Vascular elastica often affected early
Organization and calcification of intra-alveolar
exudate
Tropism for elastic tissues
CALCIFICATION
Calcium stain positive with Alizarin red and Von
Kossa stains
Fibrosis develops in more severe or long standing cases
ICLINICAL ISSUES
Presentation
Most common signs/symptoms
o Asymptomatic to slow progressive respiratory failure
o Gradual onset dyspnea, however, some have sudden
onset of symptoms and rapid fulminant course
Other signs/symptoms
o With severe disease, restrictive pulmonary function
and decreased carbon monoxide diffusion in the
lung (DLCO)
Function inversely correlated with hypercalcemia
Natural History & Prognosis
75% of patients with chronic renal failure have
calcification at microscopic examination
Variable from incidental finding that remains
unchanged for years to fulminant life-threatening
course in a matter of days
Death usually due to cardiac involvement (conducting
pathways)
May be reversible with correction of hypercalcemia
o With fibrosis will not return to normal
Treatment
Correct hypercalcemia and treat the underlying cause
I DIAGNOSTIC CHECKLIST
Consider
Hypercalcemia also common in sarcoidosis
MEN 1 or 2 in those with parathyroid adenomas,
pancreatic islet cell tumors, adrenal
pheochromocytomas, or thymic or bronchial
carcinoids
Image Interpretation Pearls
Suspect in chronic hemodialysis patients with chronic
ill-defined opacities in the upper lung zones
Confirm with HRCT or bone-scanning agents
I SELECTED REFERENCES
1. Lingam RK et al: Case report. Metastatic pulmonary
calcification in renal failure: a new HRCT pattern. Br J
Radiol. 75(889):74-7,2002
2. Hartman TE et al: Metastatic pulmonary calcification in
patients with hypercalcemia: findings on chest radiographs
and CT scans. AJR AmJ Roentgenol. 162(4):799-802, 1994
3. Johkoh T et al: Metastatic pulmonary calcification: early
detection by high-resolution CT. J Com put Assist Tomogr.
17(3):471-3,1993
4. Sanders C et al: Metastatic calcification of the heart and
lungs in end-stage renal disease: detection and
quantification by dual-energy digital chest radiography.
AJR Am J Roentgenol. 149(5):881-7, 1987
5. Conger JD et al: Pulmonary calcification in chronic dialysis
patients. Clinical and pathologic studies. Ann Intern Med.
83(3):330-6, 1975
 METASTATIC PULMONARY CALCIFICATION
I IMAGE GAllERY 1
63
Typical
(Left) Frontal radiograph
shows multiple calcified
nodules concentrated at the
lung apex. Mild
cardiomegaly and
hemodialysis catheter in the
right atrium. (Right) Frontal
radiograph shows laint
ground-glass opacities in
both upper lobes (arrows)
superimposed on both
clavicles. Left upper lobe
opacity is slightly denser with
pinpoint nodules.

Typical
(Left) Axial HRCT shows
calcific densities in the upper
lobes. Note the partial
calcific rings in the left upper
lobe (arrow). (Right) Axial
CECT at lung window
demonstrates the more
extensive nature of the
nodular process in the lung.
Note the admixture with
emphysema.

Typical
(Left) Frontal radiograph of
autopsy specimen. Discrete
calcifications predominantly
, , .~ffi;"'t==J..--..., in the upper lobes. Note the
segmental calcification in the

I'
right lower lobe (arrows)
from vascular occlusion.
~ (Right) Axial CECT shows a

o cluster of cotton-ball shaped

calcifications in the anterior
right upper lobe. Multiple
~ similar nodules were
~ scaltered throughout the

\\~ ~,~ - -,,:

fi;:.
~-'.:..-
.. .. - - - -- ,--
..
~
.........
---
~
upper lobes (not shown).
 ALVEOLAR MICROLITHIASIS
1
64

. ~

-...

?7
-.,
Graphic shows laminated calcospherites (arrows) in the
alveolar spaces. Calcospherites stay confined to the
alveolar space and do not aggregate together.
Frontal radiograph coned view left upper lobe shows
sandstorm pattern from micronodu/ar calcifications.
Cardiac border is obscured (open arrow) and black
pleural sign also present (arrow).

I TERMINOlOGY Radiographic Findings

Radiography
Abbreviations and Synonyms
o Usually an unexpected discovery
Pulmonary alveolar microlithiasis o Lungs extremely dense, obscuring heart borders and
Definitions diaphragm
Unknown etiology and rare disorder characterized by o Normal high kVp technique not optimal for
diffuse bilateral deposition of intra-alveolar microliths detection of calcification
(calcospherites) Low kVp useful to demonstrate calcification
Overpenetrated films also useful to demonstrate
calcification
IIMAGING FINDINGS o Diffuse miliary calcifications "sandstorm" due to
micronodular microliths
General Features Relatively symmetric
Best diagnostic clue: Dense lungs generally out of Increased density in dorsal aspect of the lung
proportion to clinical symptoms Increased density toward the lung bases, often
Location: Diffuse but gradient of calcification obscures the diaphragmatic, mediastinal, and
increasing in the lung bases and more pronounced in cardiac borders
dorsal aspect of the lung o "Black" pleura due to small subpleural cysts (5-10
Size: 0.2-0.3 mm in diameter, may measure up to 3 mm diameter)
mm in diameter Previously thought to be a visual illusion created
Morphology: Sand-like grains within the alveolus by relatively increased contrast in lung and ribs
Pose risk for spontaneous pneumothorax
o Small apical bullae occasionally present

DDx: Alveolar Microlithiasis

Tuberculosis Metastatic Calcification

 ALVEOLAR MICROLITHIASIS

Key Facts
1
Terminology Silicosis 65
Unknown etiology and rare disorder characterized by Sarcoidosis
diffuse bilateral deposition of intra-alveolar Mitral Stenosis
microliths (calcospherites) Amyloidosis
Tuberculosis
Imaging Findings Pulmonary Alveolar Protein os is
Usually an unexpected discovery
Diffuse miliary calcifications "sandstorm" due to
Pathology
micronodular microliths Genetics: Familial autosomal recessive (50% of cases)
"Black" pleura due to small subpleural cysts (5-10 mm Calcospherites round or slightly lobulated with
diameter) concentric lamination
Pose risk for spontaneous pneumothorax Clinical Issues
Top Differential Diagnoses Often asymptomatic (70%) in spite of the gross
Metastatic Pulmonary Calcification radiographic abnormalities
Talcosis Normal serum calcium and phosphorus
Idiopathic Ossification No known treatment

CT Findings I DIFFERENTIAL DIAGNOSIS

NECT: More sensitive than chest radiography for
calcium
HRCT
o Ground-glass opacities early
o Micronodular discrete calcifications superimposed
on ground-glass opacities
o Preferential distribution: 2 patterns
Micronodular calcification preferentially
peripheral and basilar
Anterolateral aspect lingula and middle lobe and
anterior aspect upper lobes
o Secondary pulmonary lobule
Periphery secondary pulmonary lobule
preferentially affected producing
polygonal-shaped calcified densities
Calcifications more numerous along the pleura,
interlobular septa and the bronchovascular
bundles
o Small cysts scattered in random pattern in severe
cases due to emphysema
o "Black" pleura represents subpleural cysts, subpleural
cysts also extend along the pleura
Other Modality Findings
Bone scanning
o Uptake with bone seeking radionuclides
o Absent uptake in some cases
Imaging Recommendations
Best imaging tool: Chest radiography usually suffice,
CT useful in those whose calcifications not evident on
the chest radiograph
Protocol advice: CT or bone scanning sensitive for
detection of calcium, CT useful to characterize
distribution and to differentiate from other diseases
associated with diffuse calcification
Metastatic Pulmonary Calcification
Seen in those with disorders of calcium homeostasis
Clustered calcifications larger and less sharply defined
than microlithiasis
Preferentially involves the upper lung zones
Talcosis
History of drug abuse
Upper lobe micronodules 1 mm) tend to aggregate
into perihilar fibrotic masses
Progressive massive fibrosis not seen with alveolar
microlithiasis
Idiopathic Ossification
Seen in elderly asymptomatic men
Dendritic calcification in the lower lobes
Radiographic density not as striking as microlithiasis
Silicosis
Occupational history important
Silicotic nodules may calcify
Nodules more profuse in upper lung zones, may lead
to progressive massive fibrosis
Adenopathy absent in microlithiasis
Sarcoidosis
Nodules rarely calcify
Nodules more profuse in upper lung zones, may lead
to peribronchial fibrosis
Adenopathy absent in microlithiasis
Mitral Stenosis
Left atrial enlargement and vascular redistribution
from pulmonary venous hypertension
Ossification primarily septal in lower lung zones
Amyloidosis
Nodules larger, small nodules generally do not calcify
Tuberculosis
Nodules usually larger and not as diffuse
Calcification seen with healed disease
 ALVEOLAR MICROLITHIASIS
1 Primarily affects the dorsal aspect of the upper lung
zones
66
Pulmonary Alveolar Proteinosis
May also be relatively asymptomatic even with
marked disease
Perihilar chronic alveolar process
Crazy-paving pattern may also be seen with
microlithiasis
Does not calcify
I PATHOLOGY
General Features
General path comments: Unique disorder with diffuse
intra-alveolar deposition of laminated calcospherites
Genetics: Familial autosomal recessive (50% of cases)
Etiology: Unknown stimulus may cause increased
alkalinity in intra-alveolar secretions promoting
precipitation of calcium phosphates and carbonates
Epidemiology: More prevalent in Turkey (33% of
world's cases)
Associated abnormalities
o Microliths have also been found in lumbar
sympathetic chain and testes
o Not associated with nephrocalcinosis or
cholelithiasis
Gross Pathologic & Surgical Features
Lung are heavy and cut with great difficulty,
(sometimes need to use a saw)
Sandpaper appearance on cut section
o Microliths fall out of lung tissue easily
Microscopic Features
up to 80% alveoli contain calcospherites
Calcospherites round or slightly lobulated with
concentric lamination
o Associated with interstitial fibrosis and pleural
thickening
Several microliths may occupy an alveolus or a large
one may fill an entire alveolus
o Microlith mean diameter 190 um
Mean alveolar size is 200-250 um
Microliths do not aggregate together
Chemically composed of calcium phosphate
concretions in hydroxyapatite matrix
I CLINICAL ISSUES
Presentation
Most common signs/symptoms
o Often asymptomatic (70%) in spite of the gross
radiographic abnormalities
Cough and dyspnea late
o Normal serum calcium and phospllOTUS
Other signs/symptoms
o Microliths may be recovered from bronchoalveolar
lavage fluid or transbronchial biopsy and is
diagnostic
o Pulmonary function tests
Usually normal or mild restriction early
Severe disease will show decrease in diffusing
capacity
Demographics
Age
o Average age 35, most between 30 and 50
o Occurs in children and infants and elderly
Gender
o Slight female predominance in familial cases
o Sporadic cases slightly more common in males
Natural History & Prognosis
Slow progression may eventually result in respiratory
or cardiac failure
Treatment
No known treatment
o Bronchoalveolar lavage ineffective
o Steroids and chelating agents ineffective
Lung transplant for end stage disease
I DIAGNOSTIC CHECKLIST
Consider
Microlithiasis in those with dense lungs
Most often confused with metastatic pulmonary
calcification or pulmonary sarcoidosis
Image Interpretation Pearls
Low kVp films may be useful to detect calcification
Progressive massive fibrosis or peribronchial fibrosis
absent, commonly seen in other diseases that may
calcify
I SELECTED REFERENCES
1. Gasparetto EL et al: Pulmonary alveolar microlithiasis
presenting with crazy-paving pattern on high resolution
CT. Br J Radiol. 77(923):974-6, 2004
2. Mariotta 5 et al: Pulmonary alveolar microlithiasis: report
on 576 cases published in the literature. Sarcoidosis Vase
Diffuse Lung Dis. 21(3):173-81, 2004
3. Castellana G et al: Pulmonary alveolar microlithiasis.
World cases and review of the literature. Respiration.
70(5):549-55,2003
4. Chan ED et al: Calcium deposition with or without bone
formation in the lung. Am J Respir Crit Care Med.
165(12):1654-69,2002
5. Chang YC et al: High-resolution computed tomography of
pulmonary alveolar microlithiasis. J Formos Med Assoc.
98(6):440-3, 1999
6. Brown K et al: Intrathoracic calcifications: Radiographic
features and differential diagnoses. Radiographies.
14:1247-61, 1994
7. Korn MA et al: Pulmonary alveolar microlithiasis: findings
on high-resolution cr. AJR Am J Roentgenol. 158(5):981-2,
1992
8. Cluzel Pet al: Pulmonary alveolar microlithiasis: CT
findings. J Comput Assist Tomogr. 15(6):938-42, 1991
9. Sosman MC et al: The familial occurrence of pulmonary
alveolar microlithiasis. AJR Am J Roentgenol. 77:947-1012,
1957
 ALVEOLAR MICROLITHIASIS
I IMAGE GALLERY 1
67
Typical
(Leh) Frontal radiograph
shows dense lungs
predominantly centrally and
in the lower lungs. Less
involved areas have
sandstorm micronodufar
pattern. Black pleural sign
(arrows). (Right) Axial NECT
shows subpleural cysts along
the chest wall and
mediastinal pleura.
Ground-glass densities follow
the bronchovascular
bundles. Note the lucency
around the larger arteries
(arrow).

Typical
(Left) Axial HRCT at bone
window shows subpleural
cysts extending along fissures
(arrow). Di(fuse tiny
calcifications superimposed
on diffuse ground-glass
opacities. (Right) Axial HRCT
shows polygonal
accentuation (arrow).
Calcifications superimposed
on ground-glass opacities
with slight accentuation
peripherally adjacent to the
subpleural cysts.

Typical
(Leh) Anteroposterior
radiograph shows relatively
symmetric miliary densities,
predominantly in the
periphery of the lower lobes.
Calcification difficult to
discern on high kVp
technique. (Right) Axial
HRCT shows discrete
calcifications predominantly
in the subpleural lung more
profuse in the anterior
lingula. Note the absence of
aggregation or architectural
distortion.
 LIPOID PNEUMONIA
1
68

Frontalradiograph shows extensive involvement of the Axial HRCT shows extensive unilateral ground-glass
right lovverand middle lobes. The right heart border is attenuation with superimposed septal thickening
partiallyobscured (arrow). ("crazy-paving"). Focal ground-glass opacity is also
seen in the left lower lobe (arrows).

o Incidental radiographic finding in asymptomatic

ITERMINOlOGY patients
Abbreviations and Synonyms o Radiographic appearance nonspecific
Endogenous lipid pneumonia ("golden pneumonia") o Vary with the quantity of aspiration
and exogenous lipid pneumonia o Acute aspiration
Diffuse airspace consolidation
Definitions Uni or multifocal segmental areas of
Endogenous lipid pneumonia: Accumulation of consolidation, predominantly in lower lobes
intraalveolar macrophages secondary to airway Bilateral or unilateral
obstruction or impaired mucociliary clearance In debilitated patients - in posterior segments of
Exogenous lipid pneumonia: Aspiration or inhalation upper lobes and superior segments of lower lobes
of fatty or oily substances: Animal or vegetable oils, o Chronic aspiration
oral laxatives, oil-based nose drops, and liquid paraffin Mass-like or nodular lesion with irregular margins
simulating carcinoma
Findings of fibrosis: Architectural distortion,
I IMAGING FINDINGS volume loss

General Features CT Findings

Best diagnostic clue: Low CT attenuation
and -]50 HU) in consolidated lung
Radiographic Findings
Radiography
areas (-30 Homogeneous segmental consolidation
Lower lung predominance
Low attenuation (fat density) focal consolidation
Areas of low or fat attenuation within mass-like areas
of consolidation
Fat may shift to dependent lung with postural change

DDx: Disorders Mimicking Exogenous lipoid Pneumonia

A/veo/ar Proteinosis Bronchi%a/veo/ar Cell Carcinoma Adenocarcinoma

 LIPOID PNEUMONIA

Key Facts 1
Terminology Mixed diffuse ground-glass with interlobular septal 69
Endogenous lipid pneumonia: Accumulation of thickening and intralobular lines ("crazy-paving")
intraalveolar macrophages secondary to airway Top Differential Diagnoses
obstruction or impaired mucociliary clearance
Bacterial pneumonia
Exogenous lipid pneumonia: Aspiration or inhalation Organizing pneumonia
of fatty or oily substances: Animal or vegetable oils, Bronchogenic carcinoma
oral laxatives, oil-based nose drops, and liquid
paraffin Pathology
Epidemiology: Patients at particular risk are:
Imaging Findings
Neonates, older patients, and those with any
Best diagnostic clue: Low CT attenuation areas (-30 underlying swallowing dysfunction
and -150 HU) in consolidated lung Chronically, lipid is fibrogenic, affected lung will
Uni or multifocal segmental areas of consolidation, eventually become distorted and shrunken
predominantly in lower lobes
Mass-like or nodular lesion with irregular margins Clinical Issues
simulating carcinoma History of lipid use may be difficult to elicit from
patient

Fat density is not always visible on CT Extramedullary hematopoiesis

Mixed diffuse ground-glass with interlobular septal Liposarcoma
thickening and intralobular lines ("crazy-paving")
Subpleural pulmonary fibrosis
MR Findings
I PATHOLOGY
May show fat: High T1 and T2 signal or chemical shift General Features
General path comments: Mixed inflammatory cells
Imaging Recommendations
containing numerous lipid-laden macrophages
Best imaging tool: CT the best imaging tool to
Etiology
characterize lesions for presence of fatty component o Mineral oil is most common agent, but may occur
with animal or vegetable oils
o Initial reaction is a bronchopneumonia;
I DIFFERENTIAL DIAGNOSIS macrophages ingest the lipid
Consolidation o Clearing occurs by mucociliary transport or
macrophage migration via the interstitium and
Bacterial pneumonia
lymphatics to mediastinal lymph nodes
Organizing pneumonia
o Giant cell or granuloma formation may occur
o Uni or multifocal peripheral pulmonary
consolidation o With mineral oil aspiration, there are oil droplets
o No fat attenuation within multinucleated giant cells, lymphocytes and
fibrous tissue
Nodule or Mass-like Consolidation Epidemiology: Patients at particular risk are: Neonates,
Bronchogenic carcinoma older patients, and those with any underlying
o Identical radiographic findings for solitary mass swallowing dysfunction
o Spiculated margins
Gross Pathologic & Surgical Features
o Cavitated carcinoma may have low attenuation
Chronically, lipid is fibrogenic, affected lung will
material but not of fat density
eventually become distorted and shrunken
"Crazy-Paving" Appearance
Microscopic Features
Alveolar proteinosis
Bronchioloalveolar cell carcinoma (BAe) Cytology: Large foamy cells with small vesicular nuclei
Pneumocystis jiroveci infection Positive Congo red stain
Pulmonary hemorrhage Alveolar lipid-iaden macrophages
Interstitial accumulation of lipid material,
Adult respiratory distress syndrome
inflammatory cellular infiltration, and variable
Nonspecific interstitial pneumonia
amount of fibrosis
Other Intrathoracic lesions Containing Fat Chronic granulomatous lesions
Hamartoma Paraffinoma: Term applied to iocalized tumor-like
Diaphragmatic hernias lesion caused by the aspiration of liquid paraffin
Lipomas, pleural
Mediastinal lipomatosis
Thymolipoma
Germ cell tumors
 LIPOID PNEUMONIA
1 I CLINICAL ISSUES I DIAGNOSTIC CHECKLIST
70 Presentation Consider
Most common signs/symptoms High index of clinical suspicion
o Difficult to diagnose because it mimics various Diagnosis requires a constellation of clinical,
diseases radiographic, and pathologic criteria
o History important: Chronic use of oily-nose drops, Bronchoscopy with BAL may provide useful
mineral oil laxative information
History of lipid use may be difficult to elicit from Positive lipid-laden alveolar macrophages in BAL
patient Histopathological diagnosis of lipid pneumonia to rule
o Repeated subclinical episodes of aspiration out bronchogenic carcinoma
Usually asymptomatic
Lipid material not irritant, aspiration often silent Image Interpretation Pearls
Usually during sleeping CT
Neurological or esophageal disease may promote o Areas of low or fat attenuation within the lesion
aspiration o "Crazy-paving" pattern in acute exogenous lipoid
o Chronic non-productive cough pneumonia
o Pleuritic chest pain MR
Other signs/symptoms o High T1 and T2 signal or chemical shift
o Lipid-laden macrophages may be seen in
bronchioalveolar lavage (BAL) fluid
o Transthoracic needle biopsy can provide definitive I SElECTED REFERENCES
diagnosis 1. Chung, MJ et al: Metabolic lung disease: imaging and
histopathologic findings. Eur J Radiol. 54(2): 233-245, 2005
Demographics 2. Baron SE et al: Radiological and clinical findings in acute
Age and chronic lipoid pneumonia J Thorac Imaging.
o Any age 18:217-224,2003
o Debilitated and patients with impaired swallowing 3. Rossi SE et al: "Crazy-paving" pattern at thin-section CT of
mechanisms or esophageal abnormalities at the lungs: radiologic-pathologic overview. Radiographies.
23: 1509-1519,2003
increased risk
4. Johkoh, T et al. Crazy-paving appearance at thin-section
o Aspiration of oil used as a lubricant in infants with CT: spectrum of disease and pathologic findings.
feeding problems Radiology. 211: 155-160, 1999
o Aspiration of mineral oil used for constipation in 5. Laurent F et al: Exogenous lipoid pneumonia: HRCT, MR,
elderly and pathologic findings. Eur Radiol. 9: 1190-1196, 1999
o Head & neck cancers post radiation therapy and 6. Lee JY et al: Squalene-induced extrinsic lipoid pneumonia:
surgery: Oily-nose drops often used to reduce serial radiologic findings in nine patients.) Com put Assist
mucositis side effects Tomogr. 23: 730-735, 1999
7. Lynch DA et al: Pediatric diffuse lung disease: diagnosis
Natural History & Prognosis and classification using high-resolution CT. A)R Am J
Roentgenol.l73: 713-718,1999
Frequency unknown
8. Seo JB et al: Shark liver oil-induced lipoid pneumonia in
Prognosis directly related to the type and extent of pigs: Correlation of thin-section CT and histopathologic
aspiration findings. Radiology. 212:88-96,1999
Depends on underlying clinical condition: Hiatal 9. Franquet T et al: The crazy-paving pattern in exogenous
hernia, Zenker diverticulum, gastroesophageal reflux, lipoid pneumonia: CT-pathologic correlation. AJR.
esophageal carcinoma 170:315-317,1998
Good when diagnosis is made in early phase 10. Lee KSet al: Lipoid pneumonia: CT findings. J Com put
Recurrent aspiration can result in permanent damage Assist Tomogr. 19:48-51,1995
11. Annobil SH et al: Chest radiographic findings in childhood
Unnecessary lobectomy in cases mimicking lung
lipoid pneumonia following aspiration of animal fat. Eur J
cancer Radiol. 16: 217-220, 1993
May increase risk for bronchogenic carcinoma and 12. Chang HY et al: Successful treatment of diffuse lipoid
nontuberculous mycobacterial infection pneumonitis with whole lung lavage. Thorax.
48:947-948,1993
Treatment 13. Brechot)M et al: Computed tomography and magnetic
Depends largely on avoiding the source of aspiration resonance findings in lipoid pneumonia. Thorax. 46:
o Discontinuation of use of lipoid agent 738-739, 1991
Small amount aspirated: Little impairment 14. Hugosson CO et al: Lipoid pneumonia in infants: a
Large amounts: Restrictive lung disease (fibrosis) or cor radiological-pathological study. Pediatr Radiol. 21:
pulmonale 193-197,1991
15. Van den Plas 0 et al: Gravity-dependent infiltrates in a
Surgery for esophageal abnormalities patient with lipoid pneumonia. Chest. 98:1253-1254,1990
16. Beerman Bet al. Lipoid pneumonia: an occupational
hazard of fire eaters. BM). 289:1728-1729,1984
17. Felson Bet al. Carcinoma of the lung complicating lipoid
pneumonia. A)R. 141:901-907,1983
 LIPOID PNEUMONIA

I IMAGE GALLERY 1
71
Typical
(Left) Anteroposterior
radiograph shows bifateral
areas of consolidation in a
predominantly basal
distribution. (Right! Axial
HRCT shows bilateral areas
of ground-glass attenuation
with superimposed septal
thickening ("crazy-paving").
Note the sharp demarcation
between normal and
abnormal lung (arrows).

Typical
(Left) Axial HRCT shows a
loculated pleural coffection
and bilateral ground-glass
opacities in the lower lobes.
(Right! Axial CECT shows a
segmental consolidation with
fat attenuation values (-40
HU) in the right lower lobe
(arrows). (Courtesy j. Mata,
MD).

Typical
(Left) Axial H RCT shows a
solitary pulmonary nodule in
the right lower lobe (arrow)
with spiculated margins.
(Right! Micropathology, low
power photomicrograph
(original magnification, x 10;
H-E stain) reveals fatty
vacuoles (open arrows)
surrounded by organized
pneumonitis (arrows).
 PULMONARY ALVEOLAR PROTEINOSIS
1
72

Graphic shows pulmonary alveolar proteinosis. Alveolar Frontal radiograph shows multifexal areas of airspace
filling with lipid-rich proteinaceous material that opacity in a symmetric distribution, with slight
resembles surfactant. Filling results in chronic airspace predilection for the bases.
disease.

o May be focal and nodular

I TERMI NOLOGY o More often segmental or lobar in distribution
Abbreviations and Synonyms Morphology
Pulmonary alveolar proteinosis (PAP), alveolar o Airspace opacities
proteinosis o Air bronchograms uncommon
o May have interstitial or ground-glass pattern
Definitions
Radiographic Findings
Rare diffuse lung disease characterized by the
accumulation of abundant protein-rich and lipid-rich Radiography: Chest radiograph may look much worse
surfactant material in alveoli than the patient's clinical complaints
o Diagnosed and treated with bronchioloalveolar Alveolar consolidation
lavage (BAL) and irrigation o Chronic and progressive
o Air bronchograms uncommon
o Bilateral (not unilateral)
IIMAGING FINDINGS o Rarely nodular
o Classically central "bat-wing" pattern similar to
General Features pulmonary edema
Best diagnostic clue o Focal mass or asymmetry suggests superimposed
o Chronic airspace disease on chest radiographs infection
o "Crazy-paving" pattern at HRCT Rarely, interstitial reticular pattern
Location No pleural effusions or mediastinal adenopathy or
o Central "bat-wing" pattern chest radiographs cardiac enlargement
o Geographic pattern at HRCT Post whole lung lavage treatment
Size

DDx: Chronic Airspace Opacities

Bronchoa/veoJar Carcinoma Cryptogenic Organizing Pneumonia Hemorrhage

 PULMONARY ALVEOLAR PROTEINOSIS

Key Facts
1
Terminology Diffuse Pulmonary Hemorrhage 73
Rare diffuse lung disease characterized by the Bronchioloalveolar Cell Carcinoma
accumulation of abundant protein-rich and lipid-rich Pathology
surfactant material in alveoli Primary: Abnormality of surfactant production,
Imaging Findings metabolism, or clearance
Chronic airspace disease on chest radiographs Associated abnormalities: Often superinfected with
"Crazy-paving" pattern at HRCT Nocardia, Aspergillus, Cryptococcus and other
Central "bat-wing" pattern chest radiographs organisms
Geographic pattern at HRCT Clinical Issues
Air bronchograms uncommon Disease specific survival exceeds 80% at 5 years
Pleural effusions absent: Presence suggests Whole lung lavage
superinfection Experimental treatment includes plasmapheresis for
Top Differential Diagnoses removal of autoantibodies
Pulmonary Edema Diagnostic Checklist
Pneumonia "Crazy-paving" is not specific for PAP

o Immediate complications: Pneumothorax or

pneumomediastinum
I DIFFERENTIAL DIAGNOSIS
o Acute increase in lung opacification due to retained Pulmonary Edema
lavage fluid "Bat-wing" pattern in patient with cardiomegaly and
o Gradual improvement 1st week pulmonary venous hypertension
o Marked improvement by 6 weeks, however Pleural effusions uncommon with PAP
persistent abnormalities common Airspace opacities generally more acute than PAP
CT Findings Pneumonia
HRCT Febrile, not seen in PAP
o Geographic areas of ground-glass opacity with Positive cultures
superimposed thickened interstitial lines clearly Pneumocystic jiroveci in particular may give
demarcated from adjacent normal lung "crazy-paving" pattern
o "Crazy-paving" Airspace opacities generally more acute than PAP
Thickening of interlobular septa
Produces polygonal linear opacities throughout Diffuse Pulmonary Hemorrhage
involved lung Patients usually have anemia and may have
Thickened septa superimposed on ground-glass hemoptysis
opacity Radiographic findings may be identical
Ground-glass due to microscopic alveolar filling Findings may be more acute than PAP
o Severity correlated with decrements in pulmonary
function Cryptogenic Organizing Pneumonia (COP)
o Pleural effusions absent: Presence suggests Focal airspace consolidation or ground-glass opacities
superinfection Typically periphery of lower lobes
o Mild mediastinal adenopathy
Bronchioloalveolar Cell Carcinoma
] -2 nodes> ] cm short axis diameter
May have identical radiographic pattern
More numerous nodes suggest superinfection or
Look for other constitutional symptoms, weight loss,
predisposing hematopoietic disorder
marked hypoxia
Nuclear Medicine Findings May have associated adenopathy, not seen with PAP
Ga-67 Scintigraphy "Crazy-Paving" Pattern
o Uptake on gallium scanning may not correlate with
Also consider Pneumocystis, sarcoidosis, hemorrhage,
CT findings
adult respiratory distress syndrome (ARDS), and acute
o May show marked uptake in regions with relatively
exogenous lipoid pneumonia
normal CT
o May show persistent findings after lavage despite
clinical and CT improvement
I PATHOLOGY
Imaging Recommendations
General Features
Best imaging tool
o HRCT for characterizing diffuse lung disease Etiology
o CECT: Best for detection of complications such as o Primary: Abnormality of surfactant production,
opportunistic infection metabolism, or clearance
 PULMONARY ALVEOLAR PROTEINOSIS
1 May represent an autoimmune disease
Patients have antibody to granulocyte
o Marked increase in survival over recent decades due
to better treatment
74 macrophage colony stimulating factor (GM-CSF) o Lavage techniques have markedly improved
GM-CSF important in macrophage clearance of prognosis
surfactant Significant pulmonary fibrosis rare
Detection of antibodies to GM-CSF may allow less
invasive diagnostic test Treatment
May also have abnormal secretion of surfactant Whole lung lavage
and surfactant precursors o 25-40 liters of saline, both lungs usually done
Balance of abnormal secretion (by type 2 sequentially
pneumocytes) and abnormal clearance (by o May be repeated several times
macrophages) o Most patients gain significant improvement from a
o Secondary single thorough lavage
Lysinuric protein intolerance (rare genetic o After lavage, up to 70% of patients remain symptom
disorder) in children free at 7 years
Acute silica exposure (also titanium and other o Few patients require annual or biannual therapeutic
dusts) BAL
Immunodeficiency states: Severe combined Immunomodulation
immunodeficiency, immunoglobulin A deficiency, o Experimental treatment includes plasmapheresis for
solid organ transplantation, hematopoietic removal of autoantibodies
malignancies (myeloid leukemia and o Subcutaneous administration of GM-CSF may also
myelodysplastic syndromes) improve symptoms
Epidemiology: Prevalence 3 cases per million
population
Associated abnormalities: Often superinfected with I DIAGNOSTIC CHECKLIST
Nocardia, Aspergillus, Cryptococcus and other
Image Interpretation Pearls
organisms
"Crazy-paving" is not specific for PAP
Microscopic Features "Crazy-paving" is classic for pulmonary alveolar
Accumulation of abundant protein rich and lipid rich proteinosis but this disease is rare
surfactant material that stains pink with periodic-acid Consider PAP in chronic multifocal airspace or mixed
Schiff (PAS) stain airspace-interstitial processes
Even though septa thickened at HRCT, septal
thickening histologically uncommon
o Radiographic thickening may represent aggregation I SELECTED REFERENCES
of PAS-lipoprotein in periphery of secondary 1. Bonfield TL et al: Multiplexed particle-based
pulmonary lobule anti-granulocyte macrophage colony stimulating factor
assay used as a pulmonary diagnostic test. Clin Diagn Lab
Immuno\. 12:821-4,2005
I CLINICAL ISSUES 2. Hashimoto M et al: Mismatch between galIium-67 uptake
and CT findings in a case of pulmonary alveolar
Presentation proteinosis. Ann Nucl Med. 19:47-50,2005
3. Tazawa R et al: Granulocyte-macrophage
Most common signs/symptoms colony-stimulating factor and lung immunity in
o 33% are symptomatic pulmonary alveolar proteinosis. Am] Respir Crit Care Med.
o Smoking history 70% 171:1142-9,2005
o Gradual onset dyspnea and cough 4. Beccaria M et al: Long-term durable benefit after whole
o Clubbing of fingers and toes lung lavage in pulmonary alveolar proteinosis. Eur Respir].
Other signs/symptoms 23:526-31,2004
o Pulmonary function tests 5. Brasch F et al: Surfactant proteins in pulmonary alveolar
Decreased diffusion capacity (DLCO) proteinosis in adults. Eur Respir]. 24:426-35, 2004
6. Presneilll] et al: Pulmonary alveolar proteinosis. Clin
Decreased lung volumes Chest Med. 25:593-613, 2004
Decreased compliance 7. Venkateschiah et al: Pulmonary alveolar proteinosis.
Demographics Clinical manifestations and optimal treatment strategies.
Treat Respir Med. 3:217-27, 2004
Age 8. RossiSEet al: "Crazy-paving" pattern at thin-section CT of
o Most common in adults 20-50 years old (median age the lungs: radiologic-pathologic overview. Radiographies.
40) 23:1509-19,2003
o Can occur in young children 9. Kjeldsberg KMet al: Radiographic approach to multifocal
Gender: M:F = 2:1 consolidation. Semin Ultrasound CT MR. 23:288-301, 2002
10. Holbert]M et al: CT features of puimonary alveolar
Natural History & Prognosis proteinosis. A]R. 176:1287-94,2001
Good prognosis 11. Murch CR et al: Computed tomography appearances of
o Disease specific survival exceeds 80% at 5 years pulmonary alveolar proteinosis. Clin Radio\. 40:240-43,
1989
 PULMONARY ALVEOLAR PROTEINOSIS

I IMAGE GALLERY 1
75
Typical
(Left) Axial NECT shows
classic findings of
"crazy-paving", with
thickening of interstitium
producing polygonal
opacities filled with
ground-glass opacity. (Right)
Axial NECT shows intersitial
thickening with minimal
superimposed ground-glass
opacity in the left lower lobe.

Variant
aeft)Ax~INECTshows
more dense consolidation
with air bronchograms
(arrows) representing
superinfection with
Nocardia. (Right) Axial
NECT shows multifocal
geographic areas of
ground-glass opacity.

Variant
(Left) Frontal radiograph
shows very vague nodular
density (arrow) overlying the
anterior left 3rd rib. (Right)
Axial NECT shows a
localized nodule (arrow)
with possible air
bronchogram as well as
smaller areas of ground-glass
opacity (curved arrows).
 DESQUAMATIVE INTERSTITIAL PNEUMONIA
1
76

Graphic shows vague diffuse peripheral subpleural Axial HRCT in 38 year old heavy smoker shows
basilar ground-glass opacities (arrows) that represent the ill-defined patches of ground-glass opacification
most common pattern of opacification seen in DIP. (arrows) in the right upper lobe. Diagnosis: DfP

o Lower lung predominance 70%

!TERMINOlOGY o Peripheral subpleural distribution 60%
Abbreviations and Synonyms Size: Variable extent of opacification
Desquamative interstitial pneumonia (DIP), alveolar Morphology
macrophage pneumonia o HRCT: Ground-glass attenuation
o Reticular interstitial changes minimal, primarily
Definitions limited to lung bases
Chronic idiopathic interstitial pneumonia
Radiographic Findings
characterized by macrophage filling of alveolar spaces,
probably related to cigarette smoking Radiography
o Term "desquamative" is a misnomer: Cells filling o Variable and nonspecific appearance
alveoli initially thought to represent desquamated Normal in 20%
alveolar lining cells Widespread vague opacification
Continuum of smoking related lung injury: Bibasilar irregular linear opacities, usually of mild
Respiratory bronchiolitis ~ respiratory bronchiolitis severity
associated interstitial lung disease (RB-ILD) ~ DIP Lung volumes variable
o Mildly reduced due to DIP, but may be increased
with coexistent emphysema
IIMAGING FINDINGS Bilateral basilar irregular linear opacities
o Admixed with consolidated lung 50%
General Features Honeycombing 10%
Best diagnostic clue: Smoker with HRCT showing CT Findings
diffuse ground-glass opacities
HRCT: Nonspecific appearance, may be normal in
Location mild (or early) disease

DDx: Diffuse Ground-Glass Opacities

Pneumocystis jiroveci Pneumonia Drug Reaction Hypersensitivity Pneumonitis

 DESQUAMATIVE INTERSTITIAL PNEUMONIA
Key Facts
1
Terminology Top Differential Diagnoses 77
Chronic idiopathic interstitial pneumonia RB-ILD
characterized by macrophage filling of alveolar Cryptogenic Organizing Pneumonia (COP)
spaces, probably related to cigarette smoking Drug Reaction
Term "desquamative" is a misnomer: Cells filling Hypersensitivity Pneumonitis
alveoli initially thought to represent desquamated Nonspecific Interstitial Pneumonia
alveolar lining cells Lymphoid Interstitial Pneumonia
Sarcoidosis
Imaging Findings
Best diagnostic clue: Smoker with HRCT showing Pathology
diffuse ground-glass opacities Association with smoking, 90%
Variable and nonspecific appearance Concept that DIP evolves to UIP now discredited
Ground-glass pattern (80%) Intraalveolar space and alveolar duct accumulation of
Lower lung zones predominance 70% pigmented macrophages
Peripheral predominance 60%
Reticular pattern (60%) Clinical Issues
Small well-defined cysts Insidious onset dyspnea, dry cough

Ground-glass pattern (80%) Steroid responsive

o Predominant abnormality
Lower lung zones predominance 70% Drug Reaction
Peripheral predominance 60% Identical radiographic findings (bleomycin or
Random distribution, 25% nitrofurantoin)
Diffuse (20%) Steroid responsive
Mid and upper lungs may be affected Hypersensitivity Pneumonitis
preferentially (15%)
Uncommon in smokers
Reticular pattern (60%)
Diffuse ground-glass opacities
o Irregular linear opacities predominately in lower
Ill-defined centrilobular nodules
lung zones
No basilar predominance
o Honeycombing, unusual and if present usually mild
Mosaic pattern; air trapping, common
Small well-defined cysts
Steroid responsive
o Round, thin-walled, < 2 cm in diameter
o Superimposed emphysema common in older Nonspecific Interstitial Pneumonia
patients Identical radiologic appearance
o Honeycomb lung, infrequent
Imaging Recommendations
Two forms, cellular and fibrosing
Best imaging tool: HRCT higher sensitivity than chest
o Histopathology: Temporal homogeneity of lung
radiography
insult
o Cellular form has a good prognosis
I DIFFERENTIAL DIAGNOSIS Lymphoid Interstitial Pneumonia
Associated with collagen vascular disease,
RB-ILD immunodeficiency, and Sjogren syndrome
Smoking-related disease Identical radiologic appearance
Findings centered on the respiratory bronchiole Interstitial infiltrate of lymphocytes
Centrilobular ground-glass opacities Steroid responsive
Ground-glass opacities, less diffuse, more patchy,
poorly defined Idiopathic Pulmonary Fibrosis (IPF)
Bronchiolocentric accumulation of pigmented alveolar Relationship to smoking, controversial
macrophages May show ground-glass opacities, as seen with DIP
Mild bronchiolar fibrosis and chronic inflammation Fibrosis, architectural distortion, honeycombing, more
Responsive to smoking cessation common, especially in basilar subpleural lung
Histopathology: Temporal heterogeneity of lung insult
Cryptogenic Organizing Pneumonia (COP) Prognosis, poor; 3 year median length of survival from
Not related to smoking time of diagnosis
Subpleural ground-glass opacities or consolidation
Bronchovascular bundle thickening Asbestosis
No honeycombing Subpleural interlobular septal fibrosis, honeycombing
Histopathology: Lymphocytes and plasma cells within Ground-glass opacities, uncommon
fibrous tuft in airway Pleural plaques
o Uniform temporal appearance Occupational history important
 DESQUAMATIVE INTERSTITIAL PNEUMONIA
1 Pneumocystis Jiroveci Pneumonia IClINICAllSSUES
78 History of immunosuppression
Presentation
Diffuse ground-glass opacities
Pneumatoceles Most common signs/symptoms
o History of smoking (90%)
Sarcoidosis o Insidious onset dyspnea, dry cough
Ground-glass opacities o Digital clubbing, 40%
Micronodules, nodules in lymphatic distribution Other signs/symptoms
Bronchovascular bundle thickening o Pulmonary function tests
Beaded vessels, septa, fissures Decreased diffusion capacity (DLCO)
Transbronchial biopsy: Noncaseating granulomas Restrictive abnormality
Demographics
I PATHOLOGY Age
o 30-40 years
General Features o One of the more common forms of interstitial lung
Genetics disease in children (however very rare disease in
o DIP-like illness in infants children)
Mutations in gene encoding surfactant protein C Gender: M:F = 2:1
Etiology Natural History & Prognosis
o Association with smoking, 90%
DIP worse prognosis than RB-ILD
o Association with other diseases: Connective tissue
Good prognosis with smoking cessation and steroid
disease, drug induced lung disease, Langerhans cell
treatment
histiocytosis, asbestosis and hard-metal
o Even when pulmonary abnormalities persist
pneumoconiosis, leukemia
o Fluctuating persistent opacities, months to years
o Concept that DIP evolves to UIP now discredited
after smoking cessation
Epidemiology
HRCT useful for prognosis
o Lung diseases, in decreasing order of certainty for
o Ground-glass, favorable
smoking etiology
o Honeycombing and traction bronchiectasis, survival
Lung cancer
decreases
Emphysema
Evolution
Chronic bronchitis
o May spontaneously remit
Respiratory bronchiolitis
o May progress with corticosteroid therapy
RB-ILD o Late relapse described
DIP o May recur in transplanted lung
Langerhans cell histiocytosis
o No progression to usual interstitial pneumonia
Usual interstitial pneumonia, relationship with
Mortality 30%, 10 years after diagnosis due to
smoking controversial
respiratory failure
Associated abnormalities
o RB-ILD and DIP Treatment
DIP may represent end of a spectrum of similar Transbronchiallung biopsies generally not diagnostic
disease o Bronchoalveolar lavage: Nonspecific increased
DIP, increased severity of involvement number of pigmented alveolar macrophages
Similar histopathologic appearance o Transbronchial biopsy useful to exclude sarcoidosis,
o DIP does not progress to usual interstitial infections, neoplasia
pneumonia Surgical biopsy when HRCT not typical for IPF
Microscopic Features Smoking cessation
o Corticosteroid and cytotoxic agents, limited success
Intraalveolar space and alveolar duct accumulation of
pigmented macrophages
Mild interstitial chronic inflammation
o Lymphoid aggregates, occasional eosinophils
I SElECTED REFERENCES
Fibrosis, less likely than usual interstitial pneumonia 1. Lynch DAet al: Idiopathic interstitial pneumonias: CT
o Mild to moderate fibrotic thickening of alveolar features. Radiology, 2005
septa 2. RyuJH et al: Desquamative interstitial pneumonia and
respiratory bronchiolitis-associated interstitial lung disease.
o Honeycombing, minimal Chest. 127(1):178-84,2005
Distinction of DIP from RB-ILD 3. Desai SRet al: Smoking-related interstitial lung diseases:
o RB-ILD: Macrophage accumulation and fibrosis histopathological and imaging perspectives. Clin Radiol.
Centered on small airways with some extension 58(4):259-68,2003
into alveoli 4. Wittram C et al: CT-histologic correlation of the ATS/ERS
o DIP: More uniform and widespread involvement 2002 classification of idiopathic interstitial pneumonias.
Radiographies. 23(5):1057-71, 2003
 DESQUAMATIVE INTERSTITIAL PNEUMONIA
I IMAGE GAllERY 1
79

(Left) Frontal radiograph in a

smoker shows bilateral large
foci of vague "ground-glass"
opaci{;cation (open arrows)
in a patient with biopsy
proven DIP. Curved arrows
show linear reticular
shadowing. (Right) Lateral
radiograph in same patient
shows to better advantage
the linear/reticular opacities
(curved arrows) in this
patient with DIP.

Typical
(Left) Axial HRCT in a 50
year old male smoker shows
peripheral distribution of
ground-glass opacities
(arrows). Fine intralobular
septal opacities are evident
within the ground-glass
opacities. (Right) Axial HRCT
in same patient shows more
extensive and diffuse
involvement in the right
middle and lower lobes
(arrows). Diagnosis: DIP

Variant
(Left) Axial NECT in a 52
year old smoker with
progressive dyspnea shows
little opacification in left
upper lobe (arrow). Diffuse
ground-glass opacities were
present in the lower lungs
(not shown) and open lung
biopsy showed DIP. (Right)
Axial HRCT in same patient
10 months later after
treatment with steroids
shows progression of disease
with new diffuse
ground-glass opacities
(arrows) in both upper
lobes.
 CRYPTOGENIC ORGANIZING PNEUMONIA
1
80

Radiograph showing diffuse but predominantly mid- Axial HRCT shows typical bilateral patchy areas of
and lower zone air space opacities in a patient with consolidaUon in the lower lobes in a patient with
cryptogenic organizing pneumonia. cryptogenic organizing pneumonia.

May be unilateral in minority

I TERMI NOLOGY o Usually patchy distribution but may be subpleural
Abbreviations and Synonyms o Preserved lung volumes
o Rare findings
Cryptogenic organizing pneumonia (COP),
proliferative bronchiolitis, idiopathic bronchiolitis Small nodules
obliterans organizing pneumonia (BOOP) Large nodules (sometimes simulating malignant
disease)
Definitions Reticulo-nodular pattern
Clinicopathological entity characterized by polypoid Solitary pulmonary nodule (simulating primary
plugs of loose granulation tissue within air spaces bronchogenic neoplasm)
CT Findings
HRCT
I IMAGING FINDINGS o Consolidation
General Features Alone or as part of mixed pattern seen in majority
Best diagnostic clue: Bilateral, peripheral and basal, of patients
patches of consolidation Predominantly subpleural and/or
Location: Typically in mid and lower zones peribronchovascular
Typically in mid and lower zones
Radiographic Findings More common in immunocompetent compared
Radiography to immunocompromised patients
o Bilateral areas of consolidation ground-glass Presence of consolidation associated with greater
opacification likelihood of partial or complete response to
Opacities may be migratory treatment

Pulmonary NHL Alveolar Sarcoid Fungal Infection

 CRYPTOGENIC ORGANIZING PNEUMONIA

Key Facts
1
Terminology Aspiration 81
Clinicopathological entity characterized by polypoid Lipoid Pneumonia
plugs of loose granulation tissue within air spaces Pulmonary Embolism

Imaging Findings Pathology

Bilateral areas of consolidation ground-glass Need to exclude other causes of an organizing
pneumonia pattern
opacification
Opacities may be migratory Clinical Issues
May be unilateral in minority Symptoms generally develop over a period of few
Usually patchy distribution but may be subpleural weeks
Preserved lung volumes Response to steroids generally striking
Top Differential Diagnoses Diagnostic Checklist
Lymphoma COP effectively a diagnosis of exclusion and other
Bronchioloalveolar Cell Carcinoma (BAe) potential causes of chronic multifocal air space
Chronic Eosinophilic Pneumonia opacification need to be considered
Lung Cancer (Solitary Mass)

o Ground-glass opacities
Usually randomly distributed in lungs
Sarcoidosis
o Nodules No peripheral predominance, follows bronchovascular
Usually randomly distributed in lungs bundles
o Reticular pattern Alveolar sarcoid: Few large airspace masses with air
Associated with increased risk of persistent or bronchograms
progressive disease Preferentially involves the upper lung zones
Not as prevalent as consolidation or ground-glass May be associated with symmetric hilar adenopathy
opacification Chronic Eosinophilic Pneumonia
o Less common CT patterns include Eosinophilic pneumonia usually upper lung zone
Bronchial wall thickening (eosinophilia absent in COP)
Bronchial dilatation Nodules, non-septal linear pattern, reticulation and
Solitary pulmonary masses (with spiculated or peri-bronchiolar distribution more common in COP
irregular margins) simulating malignant lesions Septal lines more common in chronic eosinophilic
Peri-lobular pattern pneumonia
"Reverse halo" sign (foci of ground-glass
opacification surrounded by a halo of Lung Cancer (Solitary Mass)
consolidation) No distinguishing features, diagnosis by fine needle
Honeycombing biopsy
Occasional small effusion Relatively uncommon pattern of COP
Imaging Recommendations Aspiration
Best imaging tool: HRCT to detect and characterize Opacities not as chronic or peripheral as COP
airspace disease Predominately dependent lung segments
Protocol advice Typical predisposing conditions: Esophageal motility
o Standard HRCT technique disorder, obtundation, alcoholism
o Chest radiographs usually sufficient for follow-up
o CT may be useful to characterize pulmonary disease Lipoid Pneumonia
and to exclude pulmonary embolism Lipoid pneumonia may have fat density in areas of
consolidated lung at CT
May present with "crazy-paving" appearance on CT
I DIFFERENTIAL DIAGNOSIS History of lipoid ingestion: Oily-nose drops, mineral
oil
Lymphoma
Pulmonary lymphoma usually secondary to known Pulmonary Embolism
disease Multiple infarcts peripherally located in bases
Adenopathy in other lymph node groups (identical to COP)
No peripheral predominance, often centered on Usually associated with pleural effusions
bronchi with air bronchograms Known risk factors for thromboembolism

Bronchioloalveolar Cell Carcinoma (BAC)

BAC not predominately subpleural
Foci usually ground-glass opacities
 CRYPTOGENIC ORGANIZING PNEUMONIA
1 I PATHOLOGY Chest pain
Arthralgia
82 General Features Night sweats
General path comments: Contrary to name, primary Bronchorrhea
pathology located in alveolus and secondarily extends o Treatment and prognosis
into small airways Steroids, less dramatic response than eosinophilic
Etiology pneumonia
o Idiopathic (by definition) Resolves over period of weeks
o Need to exclude other causes of an organizing Good, but may relapse on discontinuation of
pneumonia pattern steroids
Infection (bacteria, fungi, viruses and parasites) Demographics
Drugs (amiodarone, bleomycin, busulphan, gold Age: Typically aged 50-60 years (but wide range)
salts, sulfasalazine, tacrolimus, cocaine)
Gender: M = F
Connective tissue disease (rheumatoid arthritis,
Sjogren, polymyalgia rheumatica) Natural History & Prognosis
Transplant (lung, bone marrow, liver) Usually waxes and wanes, often treated for months for
Inflammatory bowel diseases (ulcerative colitis, "recurrent" pneumonia
Crohn disease)
Hematologic disorders (myelodysplastic Treatment
syndrome, leukemia) Corticosteroids are the mainstay of treatment
Immunologic/inflammatory disorders (Behcet o Response to steroids generally striking
disease, common variable immunodeficiency) o Symptomatic improvement usually in 24-48 hours
Radiation therapy o Complete radiographic resolution can take a few
Aspiration weeks
Epidemiology Relapses (despite treatment) in over 50%
o Equal gender predominance o Relapse associated with delay in initiation of
o Patients typically aged 50-60 years (but wide range treatment, presence of mild cholestasis and rapid
from 20-80 years) withdrawal of therapy
o Most patients are non-smokers or ex-smokers o Prolonged treatment not needed to suppress relapses
o Very rare seasonal cases (associated with o Prognosis not influenced by relapse
biochemical cholestasis)
Gross Pathologic & Surgical Features I DIAGNOSTIC CHECKLIST
Lung architecture preserved (no fibrosis)
Granulation tissue extends into airway lumen Image Interpretation Pearls
(bronchiolitis component) COP effectively a diagnosis of exclusion and other
potential causes of chronic multifocal air space
Microscopic Features opacification need to be considered
Buds of loosely organized granulation tissue extend
through pores of Kohn to next alveolus ("butterfly"
pattern) I SElECTED REFERENCES
Mononuclear cell interstitial infiltration admixed with
1. Lynch DA et al: Idiopathic interstitial pneumonias: CT
other inflammatory cells, no specific microscopic
features. Radiology. 236(1):10-21, 2005
feature 2. Oymak FSet al: Bronchiolitis obliterans organizing
pneumonia. Clinical and roentgenological features in 26
cases. Respiration. 72(3):254-62, 2005
I CLINICAL ISSUES 3. Takada H et al: Bronchiolitis obliterans organizing
pneumonia as an initial manifestation in systemic lupus
Presentation erythematosus. Pediatr Pulmonol. 2005
Most common signs/symptoms 4. Cordier JF: Cryptogenic organizing pneumonia. Clin Chest
o Symptoms generally develop over a period of few Med. 25(4):727-38, vi-vii, 2004
weeks 5. Epler GR: Drug-induced bronchiolitis obliterans organizing
pneumonia. C1in Chest Med. 25(1):89-94, 2004
o Typical symptoms include 6. Pipavath S et al: Imaging of the chest: idiopathic interstitial
Non-productive cough pneumonia. Clin Chest Med. 25(4):651-6, v-vi, 2004
Malaise 7. Tanaka N et al: Rheumatoid arthritis-related lung diseases:
Weight loss CT findings. Radiology. 232(1):81-91, 2004
Anorexia 8. Vjita M et al: Organizing pneumonia: perilobular pattern at
Mild exertional dyspnea thin-section CT. Radiology. 232(3):757-61, 2004
o Pulmonary function test usually restrictive, may be 9. Vlubas B et al: Bronchiolitis obliterans organizing
mixed restrictive and obstructive pneumonia associated with sulfasalazine in a patient with
rheumatoid arthritis. Clin Rheumatol. 23(3):249-51, 2004
Other signs/symptoms 10. Kim 5J et al: Reversed halo sign on high-resolution CT of
o Less common clinical features include cryptogenic organizing pneumonia: diagnostic
Hemoptysis implications. AJRAm J Roentgenol. 180(5):1251-4,2003
 CRYPTOGENIC ORGANIZING PNEUMONIA

I IMAGE GAllERY 1
83

(Leh) Axial HRCT shows

patchy bronchocentric air
space opacification at both
lung bases in a patient with
COP. (Right) Axial HRCT
below the carina shows
patchy bilateral air space
opacities in the periphery of
both lungs.

(Left) Axial HRCT shows

typical peripheral areas of
consolidation in an 18 year
old patient with COP. The
opacification is strikingly
bronchocentric in the left
upper lobe (curved arrow).
(Right) Axial HRCT showing
a predominant nodular
pattern with ground-glass
opacification in a patient
with COP.

Variant
(Leh) Axial HRCT shows that
the outlines of many
secondary pulmonary
lobules are highlighted by
peri-lobular curvilinear
opacities (arrows) giving rise
to the so-called peri-lobular
pattern of COP. (Right) Axial
HRCT shows COP
manifesting as coarse linear
bands which appear radially
distributed (arrows) within
both lungs.
 BRONCHIOLOALVEOLAR CELL CARCINOMA
1
84

Graphic shows BAC spreading along the airway Axial CECT shows a right lower lobe airspace opacity
framework of the lung without invasion of interstitium. (arrow) from bronchioloa/vealar cell carcinoma.
Patent small bronchioles and small cystic spaces within Centrilobular right middle lobe nodules suggest
the tumor are often seen as air bronchiolograms. bronchogenic spread (curved arrow).

I TERMINOlOGY Radiographic Findings

Focal (80%)
Abbreviations and Synonyms
o Small peripheral nodule(s) most common
Bronchioloalveolar cell carcinoma (BAC), alveolar cell radiographic abnormality, solitary or multiple
carcinoma, bronchiolar carcinoma o Mass> 3 cm
Definitions o Unifocal, multifocal or diffuse ill-defined airspace
Lung cancer: Subtype of adenocarcinoma opacities, that simulate pneumonia, 30%
Often with air bronchograms
Peripheral, central or both
I IMAGING FINDINGS Segmental spread via airways
o Lobar consolidation may cause lobar expansion with
General Features bulging fissures
Best diagnostic clue: Chronic progressive lobar or o Lobar atelectasis without air bronchogram: Rare
multilobar consolidation o Elongated opacity simulating mucoid impaction:
Location Rare
o Unilateral, bilateral; lobar or multilobar Diffuse (20%)
o Nodules tend to be peripheral o Diffuse nodules, miliary and larger in size
o Consolidation can be peripheral, central or both o Diffuse pulmonary consolidation simulating
Size: Variable, centrilobular to widespread pulmonary edema
consolidation in advanced disease CT Findings
Morphology: Ill-defined ground-glass or airspace
nodule or opacity NECT
o Ill-defined, spiculated, round, oval or lobulated
peripheral nodule(s)

DDx: Focal/Multifocal Airspace Opacity

Pneumonia COP (BOOP) Lymphoma

 BRONCHIOLOALVEOLAR CELL CARCINOMA

Key Facts
1
Imaging Findings Top Differential Diagnoses 85
Small peripheral nodule(s) most common Lung cancer (non-BAC), granuloma, metastasis, focus
radiographic abnormality, solitary or multiple of scarring, sarcoid, Wegener, rheumatoid nodule,
Unifocal, multifocal or diffuse ill-defined airspace amyloid
opacities, that simulate pneumonia, 30% Pneumonia
Spectrum: Pure ground-glass opacity, mixed
ground-glass and solid opacities; pure solid opacity Pathology
Centrilobular or brof\chocentric nodules, usually due No definite relationship to cigarette smoking
to tumor spread along airways (bronchogenic) Tends to develop in a focus of scarring
Air bronchograms, bronchiolograms or bubble-like Clinical Issues
lucencies, 50% When resected, 75% 5 year survival
Narrowing, stretching, spreading, distortion of
bronchi due to desmoplastic reaction Diagnostic Checklist
High false negative rate for focal tumors Pneumonia that is not resolving with treatment
should raise possibility of bronchioloalveolar cell
carcinoma

o Focal or multifocal airspace opacities, unilateral or

bilateral
o Lobar consolidation with volume loss or increased
volume with bulging fissures
o Pleural effusion 33%; lymphadenopathy 20%
CECT
o CT angiogram sign
Contrast-enhanced vessels through low
attenuation consolidation
Caused by low density mucus in the neoplasm
Also seen in postobstructive consolidation,
pneumonia, passive atelectasis, lymphoma, lipoid
pneumonia
HRCT
o Ground-glass ill-defined opacification, focal or
multifocal
Spectrum: Pure ground-glass opacity, mixed
ground-glass and solid opacities; pure solid
opacity
Nodule with peripheral halo
Crazy paving appearance: Mixed ground-glass
opacities with septal thickening
Centrilobular or bronchocentric nodules, usually
due to tumor spread along airways (bronchogenic)
o Air bronchograms, bronchiolograms or bubble-like
lucencies, 50%
Narrowing, stretching, spreading, distortion of
bronchi due to desmoplastic reaction
o Cavitation, rare
o Calcification from psammoma bodies (rare)
o Central scar or pleural indentation representing
desmoplastic reaction in tumor
May show additional areas of involvement, lung,
pleura, mediastinum
MR Findings
T2WI: High signal lung, related to high mucin content
Nuclear Medicine Findings
PET
o High false negative rate for focal tumors
o Highly sensitive for multifocal disease
Imaging Recommendations
Best imaging tool: Serial CT to demonstrate chronicity,
extent, and progression of opacities
Protocol advice: Include HRCT without contrast
supine and prone to characterize
I DIFFERENTIAL DIAGNOSIS
Solitary Pulmonary Nodule
Lung cancer (non-BAC), granuloma, metastasis, focus
of scarring, sarcoid, Wegener, rheumatoid nodule,
amyloid
o Clinical presentation and fine needle biopsy for final
diagnosis
Multiple Pulmonary Nodules
Sarcoid: Symmetric hilar/mediastinal
lymphadenopathy
Cryptogenic organizing pneumonia (COP or BOOP):
Tends to wax and wane, responds to steroid treatment
(no effect with BAC)
Metastases, vascular tumors, choriocarcinoma: Known
primary tumor, rapid growth
Lymphoma: Usually associated with bulky
lymphadenopathy
Wegener granulomatosis: Renal failure, sinus disease
Rheumatoid nodule: Arthritis, exposure to silica/coal
dust
Amyloid: Secondary form associated with arthritis,
osteomyelitis, malignant neoplasms, multiple
myeloma
Pneumonia
Will respond and regress with appropriate antibiotics
(BAC will not improve with antibiotics)
Aspiration
Gravity-dependent location
Resolves, time dependent on quality of aspirate
 BRONCHIOLOALVEOLAR CELL CARCINOMA
1 Alveolar Proteinosis Demographics
86 Usually diffuse bilateral distribution with few Age: > 40 years old
symptoms Gender: M = F
Septal lines uncommon with BAC
Natural History & Prognosis
Resembles other consolidative processes but progresses
I PATHOLOGY over time
May wax and wane; this may be due to shifting
General Features locations of mucin filling airways
General path comments Peripheral nodule: Better prognosis than other lung
o Subtype of adenocarcinoma ca n cer types
o May arise from type 11pneumocytes and bronchiolar o More likely to be detected as stage I
epithelium o May show little or no growth during 2 year
o Lipidic growth: Tumor cells spread using the observation CT scans
underlying pulmonary architecture as scaffolding o Prognosis best for nodules < 2 em with pure
without distortion of the surrounding lung ground-glass opacity
Lipidic growth may cause bronchial narrowing, o Slower growing nodules with longer doubling times
stretching and spreading have improved survival
o Bronchogenic spread: Tumor cells may spread to o When resected, 75% 5 year survival
other lobes or contralateral lung via the Noguchi types D, E, F have worse prognosis than A, B
tracheobronchial tree or C
o Lymphatic spread suggested by septal lines Diffuse form of disease: Consolidative, multifocal and
o Pleural tail represents desmoplastic reaction in diffuse disease
peripheral nodule o Worse prognosis, poorest prognosis for mucinous
Etiology tumors
o No definite relationship to cigarette smoking Despite 5 year "cure" may recur up to 20 years later
o Tends to develop in a focus of scarring
o May arise from bronchogenic cyst or congenital
Treatment
cystic adenomatoid malformation Diagnosis by sputum cytology, fine needle aspiration
biopsy or transbronchial biopsy
Epidemiology
o 2-5% of lung cancers Surgical resection for localized disease
o Increasing in frequency Radiation therapy and chemotherapy for disseminated
disease
Staging
o TNM classification same as nons mall cell carcinoma
Gross Pathologic & Surgical Features I DIAGNOSTIC CHECKLIST
Mucinous and non-mucinous forms
Consider
Microscopic Features CT and HRCT to characterize, define extent of disease
Malignant cells lining the alveoli and small airways and for staging
(lipidic growth)
Image Interpretation Pearls
Staging, Grading or Classification Criteria Pneumonia that is not resolving with treatment
Noguchi classification should raise possibility of bronchioloalveolar cell
o Types A, B, C are adenocarcinomas showing growth carcinoma
with replacement of alveolar lining cells, primarily
pure ground-glass opacities with HRCT
o Type C foci of active fibroblastic proliferation, I SElECTED REFERENCES
mixed ground-glass and solid component with 1. Heyneman LE et al: PET imaging in patients with
HRCT bronchioloalveolar cell carcinoma. Lung Cancer.
o Types D, E, F are non-replacement types of 38(3):261-6,2002
adenocarcinoma, primarily solid with HRCT 2. Aoki T et al: Peripheral lung adenocarcinoma: correlation
of thin-section CT findings with histologic prognostic
factors and survival. Radiology. 220(3):803-9, 2001
I CLINICAL ISSUES 3. Shah RM et al: CT angiogram sign: incidence and
significance in lobar consolidations evaluated by
Presentation contrast-enhanced CT. AJR Am J Roentgenol.
170(3):719-21,1998
Most common signs/symptoms 4. Lee KS et al: Bronchioloalveolar carcinoma: Clinical,
o With mucinous type: Cough and bronchorrhea may histopathologic, and radiologic findings. Radiographies
be severe 17:1345-57, 1997
o Peripheral nodules usually found incidentally with 5. Noguchi M et al: Small adenocarcinoma of the lung.
chest radiography Histologic characteristics and prognosis. Cancer.
15;75(12):2844-52,1995
 BRONCHIOLOALVEOLAR CELL CARCINOMA

I IMAGE GAllERY 1
87
Typical
(Left) Axial CECT shows
peripheral airspace opacity
with subtle air bronchograms
in the right lower lobe
(arrow). The patient was
treated with antibiotics (or
presumed pneumonia,
without resolution. (Right)
Axial CECT performed 3
months later shows
increased size and density of
the right lower lobe opacity
(arrow) despite treatment.
Final diagnosis
bronchioloalveolar cell
carcinoma.

(Left) Axial NECT shows a

left upper lobe ill defined
nodular ground-glass opacity
with a pleural tag (arrow).
Air bronchograms are noted
within the nodule. (Right)
Axial NECT performed 4
years later shows increased
size and density of the mass
(arrow). Patient declined
treatment. This slow-growing
BAC had a measured
doubling time of /375 days.

Typical
(Left) Frontal radiograph
shows right lower lobe
airspace opacification
(arrow) and stranding in the
left lower lobe (curved
arrow). Bronchoalveolar
lavage specimens showed
BAC (RighI) Frontal
radiograph 4 months later
shows increasing opacities in
the left upper (curved arrow)
and lower lobes (arrow)
indicating bronchogenic
spread of tumor. This BAC is
fast growing.
 Infectious
Viral Pneumonia 1-2-2
Pneumocystis Pneumonia 1-2-6

Inflammatory - Degenerative
Sarcoidosis, Pulmonary 1-2-10
Idiopathic Pulmonary Fibrosis 1-2-14
Hypersensitivity Pneumonitis 1-2-18
Rheumatoid Arthritis 1-2-22
Scleroderma, Pulmonary 1-2-26
Polymyositis - Dermatomyositis, Pulmonary 1-2-30
Nonspecific Interstitial Pneumonitis 1-2-34

Toxic - Metabolic
Asbestosis 1-2-38
Silicosis - Coal Worker Pneumoconiosis 1-2-42
Berylliosis 1-2-46
Lung Ossification 1-2-50

Neoplastic
Lymphangitic Carcinomatosis 1-2-52
Lymphocytic Interstitial Pneumonia 1-2-56
Lymphangiomyomatosis 1-2-60

Congenital
Diffuse Pulmonary Lymphangiomatosis 1-2-64
 VIRAL PNEUMONIA

2
2

Frontal radiograph in a 2 year old boy shows increased lAteral radiograph shows linear opacities that represent
perihi/ar and basi/ar interstitial markings representing segmentallsubsegmental atelectasis in the right middle
viral pneumonia (arrows). He had cold and flu-like lobe, lingula and lower lobes (curved arrows), most
symptoms, dry cough and fever for a few days. likely due to mucous plugging.

ITERMINOlOGY Radiographic Findings

Radiography
Abbreviations and Synonyms
o Chest radiography usually sufficient for
Atypical pneumonia, cytomegalovirus (CMV), severe documenting pattern and extent of disease
acute respiratory syndrome (SARS, coronavirus) o Variable and overlapping appearance
Hemorrhagic fever with renal syndrome (Hantavirus), o Bronchiolitis: Hyperinflation, vague small nodular
Epstein-Barr (EB) virus
opacities
Definitions o Small airway involvement: Bronchial wall
Pulmonary infection with a viral pathogen thickening
o Atelectasis: Segmental/subsegmental; lobar
atelectasis, especially in children
I IMAGING FINDINGS o Pneumonia
Peribronchial, patchy and/or diffuse
General Features Ill-defined nodules> 1 em in measles
Best diagnostic clue: Diffuse interstitial thickening in Diffuse ill-defined nodular opacities, 5-10 mm, in
febrile patient varicella-zoster
Location Reticulonodular interstitial opacities
o Unilateral or bilateral, peribronchial, perihilar or Vague hazy or dense airspace opacities
diffuse Noncardiogenic edema (ARDS), Hantavirus, SARS
o Focal disease in SARS o Bacterial superinfection: Lobar/multilobar
Size: Variable consolidation, cavitation, pleural effusion
Morphology: Usually interstitial or o Hilar/mediastinal adenopathy: Measles (in children),
bronchopneumonic EB virus (infectious mononucleosis)

DDx: Diffuse Mixed Interstitial and Airspace Opacities

Pulmonary Edema Hemorrhage Alveolar Proteinosis

 VIRAL PNEUMONIA

Key Facts
Imaging Findings CT/HRCT: More sensitive, important in
Best diagnostic clue: Diffuse interstitial thickening in immunocompromised patients to document disease
febrile patient and begin early treatment
Variable and overlapping appearance Top Differential Diagnoses
Atelectasis: Segmental/subsegmental; lobar atelectasis,
especially in children
Edema
Hemorrhage
2
Bacterial superinfection: Lobar/multilobar Aspiration
consolidation, cavitation, pleural effusion 3
Ground-glass, airspace, interstitial opacities and/or Pathology
centrilobular nodules Influenza A and B most common viral pneumonia in
Centrilobular nodules: Varicella-zoster, CMV and healthy adults
influenza Respiratory syncytial virus, most common in infants
Chest radiography: Usually sufficient for and children < age 4
documenting pattern, extent of disease and to Higher prevalence of influenza, varicella-zoster,
monitor therapy measles in pregnancy

o Effusions, rare except for adenovirus, measles, Interstitial thickening will change with position
Hantavirus, Herpes simplex type 1 (gravitational shift test)
o Pericardial effusion in Hantavirus
Hemorrhage
CT Findings Anemia with hemorrhage, often hemoptysis
NECT Identical radiographic findings
o Variable and overlapping appearance Rapid evolution from consolidation to interstitial
o Ground-glass, airspace, interstitial opacities and/or thickening, approximately 3 days
centrilobular nodules
Peribronchial, segmental, patchy, or diffuse Aspiration
Normal radiograph and abnormal HRCT with Identical radiographic findings
ground-glass opacities, in SARS Often recurrent, viral pneumonias tend not to be
Thickened interlobular septa: CMV, Hantavirus recurrent
Ground-glass and/or airspace consolidation Cryptogenic Organizing Pneumonia (COP or
o Ground-glass with lobular distribution: Herpes
simplex, influenza BOOP)
o Diffuse ground-glass or airspace opacities: Multifocal areas of peripheral pulmonary
Hantavirus, CMV consolidation
o Consolidation and ground glass opacities: SARS Often waxes and wanes, unusual evolution with viral
o Segmental consolidation: Adenovirus, herpes pneumonia
simplex Farmer's lung
Centrilobular nodules: Varicella-zoster, CMV and
Farmer's lung often mistaken as pneumonia: Tends to
influenza
be recurrent with repeated exposure to offending
o Varicella-zoster nodules
antigen
Diffuse 1-10 mm, well and ill-defined; peri nodular
ground-glass halo; coalescing Alveolar Proteinosis
Imaging Recommendations "Bat's wing" central consolidation
Patients often asymptomatic in contrast to patients
Best imaging tool
with viral pneumonia
o Chest radiography: Usually sufficient for
documenting pattern, extent of disease and to
monitor therapy
o CT/HRCT: More sensitive, important in I PATHOLOGY
immunocompromised patients to document disease General Features
and begin early treatment General path comments
Protocol advice: HRCT 1-2 mm at 1 cm intervals o Tracheobronchitis, bronchiolitis
without contrast, supine and prone if possible o Pneumonia
Mixed inflammatory cells, predominately
lymphocytic in epithelium or interstitium
I DIFFERENTIAL DIAGNOSIS Intra-alveolar hemorrhage
Edema Diffuse alveolar damage with fulminant infection
Edema will evolve quickly and resolve with diuretics
 VIRAL PNEUMONIA
o Influenza pneumonia, secondarily infected with
Strep or Staph
o Measles pneumonia
Secondarily infected with Haemophilus and
Neisseria meningitidis
Lymphocytic infiltration interstitium:
Multinucleated giant cells highly specific for
2 measles
Etiology
4 o Portal of entry: Inhalation
o Coronavirus pneumonia (SARS), inhalation from
other infected humans
o Hantavirus pneumonia, inhalation of dust from
infected rodents and deer mice
Epidemiology
o Influenza A and B most common viral pneumonia
in healthy adults
Epidemics, late winter most common
o Respiratory syncytial virus, most common in infants
and children < age 4
Almost all children infected by age 3
Winter most common
o Hantavirus, Southwest, arid climate
o Higher prevalence of influenza, varicella-zoster,
measles in pregnancy
o Viral reactivation of CMV in patients with bone
marrow and solid organ transplants, and acquired
immunodeficiency syndrome
o Herpes simplex type 1 in immunosuppressed
Gross Pathologic & Surgical Features
Airway
o Tracheobronchitis and bronchiolitis
Microscopic Features
Offending organism rarely cultured
Tracheobronchitis, bronchiolitis
o Bronchial wall thickening and edema, sloughing
ciliated cells
o Mononuclear infiltration bronchial walls
Pneumonia
o Interstitial lymphocytic infiltration, hemorrhage,
edema, diffuse alveolar damage
COP or BOOP
Bronchiolitis obliterans
I CLINICAL ISSUES
Presentation
Most common signs/symptoms: Fever, rhinitis,
pharyngitis, headache, dry cough, myalgias, arthralgia,
dyspnea
Other signs/symptoms
o Respiratory physical exam may be normal
o Common cause of confusion in adults
o Fulminant disease in elderly and immunosuppressed
Specific virus
o Influenza in elderly with cardiopulmonary disease,
severe hemorrhagic pneumonia
o Hantavirus, hypotension, renal failure
o Varicella-zoster (chicken pox) severe pneumonia in
patients with lymphoma, immunosuppressed or
pregnant
o Herpes simplex type 1 associated with oral ulcers
and airway irritation
o Epstein-Barr infection, primarily lymphadenopathy
and splenomegaly without pneumonia
Demographics
Age: Infant to elderly
Gender: Male = female
Natural History & Prognosis
Resolution usually complete in immunocompetent
Variable prognosis with increased virulence of virus
and decreased host response
Sequelae
o Bronchiectasis or bronchiolitis obliterans (Swyer
James syndrome): Adenovirus
o COP (BOOP): Influenza, adenovirus, measles
o Interstitial fibrosis in patients who survive ARDS:
Hantavirus, SARS
o Innumerable small calcified nodules (2-3 mm):
Healed varicella-zoster
Varicella-zoster, mortality 9-50%; SARS, mortality 11%
Hantavirus with ARDS, mortality approximately 50%
Treatment
Preventive: Influenza vaccine, measles vaccine,
varicella vaccine, adenovirus vaccine for recruits
Supportive
Acyclovir for varicella or herpes: Ganciclovir for CMV
I DIAGNOSTIC CHECKLIST
Consider
HRCT in high-risk patients
o In elderly, pregnant, with hematologic malignancy,
AIDS, bone marrow and solid organ transplantation
Image Interpretation Pearls
Much overlap with other entities: Remember to
include in differential diagnosis when opacities are
peribronchial or segmental
I SELECTED REFERENCES
I. Muller NL et al: High-resolution CT findings of severe
acute respiratory syndrome at presentation and after
admission. AJRAm J Roentgenol. Jan;182(1):39-44, 2004
2. Franquet T et al: Thin-section CT findings in 32
immunocompromised patients with cytomegalovirus
pneumonia who do not have AIDS.AJRAmJ Roentgenol.
181(4):1059-63,2003
3. Oikonomou A et al: Radiographic and high-resolution CT
findings of influenza virus pneumonia in patients with
hematologic malignancies. AJRAmJ Roentgenol.
181(2):507-11,2003
4. Wong KTet al: Severe acute respiratory syndrome:
radiographic appearances and pattern of progression in 138
patients. Radiology. 228:401-406, 2003
5. Kim EAet al: Viral pneumonias in adults: radiologic and
pathologic findings. Radiographies. 22 Spec No:S137-49,
2002
 VIRAL PNEUMONIA
I IMAGE GALLERY
Typical
(Leh) Frontal radiograph in a
pregnant woman shows
diffuse bilateral small nodular
opacities. Her sick child was
2
home recovering from
chicken pox. She required 5
intubation for hypoxia.
(Right) Lateral radiograph
coned down view in same
patient shows the ill-defined
nodular opacities (arrow)
that are seen with varicella
pneumonia.

Typical
(Leh) Frontal radiograph in
an elderly male shows
diffuse bilateral hazy
opacities. The heart size is
slightly enlarged. He
presented with dyspnea and
confusion. (Right) Axial
CECT shows diffuse
ground-glass opacities and
few septal lines. Geographic
sparing seen at anterior
segmentofnghtupperand
left upper lobes (arrows).
Ox: Fulminant viral
pneumonia

(Leh) Frontal radiograph In a

patient with AIDS shows
diffuse bilateral small
nodules. Cytomegalovirus
was isolated from protected
BAt specimens. (Right)
Frontal radiograph close-up
in same patient shows the
diffuse small nodules (arrow)
typical of CMV pneumonia.
 PNEUMOCYSTIS PNEUMONIA

2
6

PneumocysUs pneumonia often presents with perihilar Frontal radiograph in a 35 year old male with fever and
or diffuse ground-glass opaciUes. Upper lobe cysts may dyspnea. A predominantly upper and mid lung
be seen in patients with AIDS. ground-glass opaciUes with concurrent large cysts.
PneumocysUs was isolated on sputum.

o If untreated, evolves into a consolidative appearance

ITERMINOlOGY o Slight upper lobe distribution in some, may be
Abbreviations and Synonyms associated with aerosolized pentamidine prophylaxis
o Unusual distribution seen in patients with prior
Pneumocystis pneumonia (PCP)
lung radiation therapy with lung involvement only
Acquired immune deficiency syndrome (AIDS)
outside radiation port
Definitions Upper lobe cysts (10%) in AIDS patients
Opportunistic fungal infection often affecting Less common
individuals with T-cell immunodeficiency o Asymmetric consolidation
o Two major forms: Trophozoites and cysts o Multiple nodules (rarely miliary)
o Predominant reticular opacities
Pleural effusions and enlarged lymph nodes
I IMAGING FINDINGS uncommon
o Presence of either: Consider a different or
General Features concurrent disease process
Best diagnostic clue: Ground-glass (hazy increased
opacity) on radiograph or HRCT in a hypoxic CT Findings
immunocompromised patient HRCT
Location: Perihilar, diffuse or less commonly, with a o Ground-glass often the dominant finding,
slight upper lobe distribution particularly when patchy and bilateral
Concurrent upper lobe cysts, usually located in
Radiographic Findings the periphery in 30% of AIDS patients
Most common manifestation: Ground-glass opacities
that are perihilar or diffuse

DDx: Pneumocystis Pneumonia

CMV Pneumonitis Pulmonary Edema Hypersensitivity Pneumonitis

 PNEUMOCYSTIS
Key Facts
Terminology
Opportunistic fungal infection often affecting
individuals with T-cell immunodeficiency
Imaging Findings
Most common manifestation: Ground-glass opacities
that are perihilar or diffuse
If untreated, evolves into a consolidative appearance
Concurrent upper lobe cysts, usually located in the
periphery in 30% of AIDS patients
Clinical Issues
Presentation variable with a significant difference
between patients with and without AIDS
Presenting symptoms: Non-productive cough, fever
and hypoxia
Cysts predispose to pneumothorax, often difficult
to treat since persistent bronchopleural fistulas
common
o Superimposed intralobular and smooth interlobular
septal thickening within the ground-glass -
"crazy-paving" pattern
o 5-10% atypical patterns such as multiple nodules
(some with cavitation), asymmetric consolidations
or rarely, dominant reticular opacities
o "Tree-in-bud" pattern (filling of the terminal
bronchioles) not present
Consider bacterial pneumonia, aspiration, or
endobronchial tuberculosis
o Non-HlV: Similar findings, with the exception of
cysts
Patchy or peribronchial areas of consolidation
slightly more common
Nuclear Medicine Findings
Historically, gallium scan used for questionable cases
o Widespread lung activity is present with PCP
Imaging Recommendations
Best imaging tool
o HRCT useful for detection and characterization of
diffuse pulmonary abnormalities
Radiograph often suggestive, but subtle cases of
ground-glass may not be apparent
o Extremely rare to have PCP with a normal HRCT
examination
Protocol advice
o Each CT should have at least some thin collimation
images
Contrast enhanced CT may be helpful to search
for other diseases if effusions or enlarged lymph
nodes are present on the radiograph
I DIFFERENTIAL DIAGNOSIS
Non-Cardiogenic Edema
Diffuse ground-glass to consolidative appearance
o Onset usually quite rapid, usually < 24 hours
PNEUMONIA
AIDS patients: Symptoms often subacute, with a
prodrome of malaise, fever and dyspnea gradually
worsening over 2-6 weeks
Non-HlV patients: Symptoms often more rapid,
usually presenting over 4-10 days
Hypoxia on room air very common and important 2
clinical feature, especially seen during minimal
exercise 7
Patients with PCP at risk for other
immunosuppressed-related infections or neoplasms
Commonly some worsening of the radiograph during
the early course of therapy since these medications
require tremendous amount of IV fluid for
administration
o Fever not a characteristic feature
o Small bilateral pleural effusions common
Cytomegalovirus Pneumonitis
Similar predisposition (cell-mediated
immunodeficiency): Most common associated
infection with PCP
o Bilateral ground-glass opacities and multiple small
nodules the most frequent finding
o Concurrent reticular opacities may be seen on CT
Patchy consolidation a less common feature
Diffuse Pulmonary Hemorrhage Syndromes
Wegner granulomatosis, Goodpasture syndrome,
Churg-Strauss syndrome, connective tissue vasculitis,
microscopic polyangiitis, bone marrow
transplantation and Idiopathic pulmonary
hemosiderosis
Diffuse or extensive bilateral ground-glass and
consolidative opacities
o These syndromes usually have an acute onset of
dyspnea, often < 24 hours
Hypersensitivity Pneumonitis
Commonly from medications or extrinsic allergic
alveolitis
Diffuse ground-glass most common imaging
manifestation
o Onset of dyspnea and non-productive cough tends
to be more subacute or chronic
o Hypoxia is often more mild and fever is less
common
HRCT may demonstrate characteristic ill-defined
centrilobular nodules
o Cysts are very uncommon
o Air-trapping common at expiratory CT
Pulmonary Alveolar Proteinosis
Ground-glass with concurrent intralobular and
interlobular septal thickening ("crazy paving" pattern)
o Symptoms much indolent (often over months),
except rarely in some patients with hematological
malignancy
 PNEUMOCYSTIS PNEUMONIA
o Fever and severe hypoxia uncommon
I PATHOLOGY
General Features
Etiology
2 o Patients with impaired cell-mediated immunity
predisposed to PCP
8 AIDS patients, especially with CD4 counts below
200
Long term corticosteroid therapy, particularly
during tapering phase
Organ transplantation, bone marrow
transplantation (BMT) and chemotherapy
Congential immunodeficiency such as thymic
aplasia, bare T-cell disease and combined
immunodeficiency syndrome
o Premature infants and malnutrition
Epidemiology: Organism can be found in normal
lungs
Gross Pathologic & Surgical Features
Patchy and often extensive areas of consolidated lung
tissue
Cysts common in AIDS patients and usually occur in
subpleural lung
Microscopic Features
Gomori methenamine silver (GMS) stain excellent for
detecting cysts
Giemsa stain useful to demonstrate trophozoites
Intra-alveolar foamy exudate with fungus seen as tiny
"bubble-like" areas
o Mild-moderate interstitial pneumonitis along with
areas of chronic diffuse alveolar damage common
Cysts (AIDS patients), necrotizing granulomas and
subpleural emphysematous blebs common
Non-HIV patients: Foamy exudates absent in 50%
ICLlNICAL ISSUES
Presentation
Most common signs/symptoms
o Presentation variable with a significant difference
between patients with and without AIDS
o Presenting symptoms: Non-productive cough, fever
and hypoxia
AIDS patients: Symptoms often subacute, with a
prodrome of malaise, fever and dyspnea gradually
worsening over 2-6 weeks
Non-HIV patients: Symptoms often more rapid,
usually presenting over 4-10 days
Less common, in non-HIV patients is an indolent
course with minimal symptoms
Other signs/symptoms
o Hypoxia on room air very common and important
clinical feature, especially seen during minimal
exercise
Absence of hypoxia should place other potential
disease processes above PCP
o Crackles often heard on auscultation
o AIDS patients usually develop the infection when
CD4 count drops < 200
90% have an elevated LDH
LDH prognostic; a rising level despite therapy
predicts a poor outcome
Demographics
Age: Any age, depends on risk factors
Natural History & Prognosis
AIDS: Very common infection, although incidence
appears to have decreased over past decade
o Thin-walled cysts may develop and predispose to
pneumothorax
o Diagnosis often straightforward since the fungal
load large and inflammatory component minimal
o Sputum and/or BAL often positive
Non-HIV: PCP more difficult to diagnose
o Fungal load much less and inflammatory
component greater
o Delayed diagnosis: Negative sputum and/or BAL
may lead the physician to consider another
diagnosis
High clinical suspicion required
Transbronchial biopsy may be needed for
diagnosis
Patients with PCP at risk for other
immunosuppressed-related infections or neoplasms
o Cytomegalovirus
o Mycobacterium tuberculosis
o Disseminated mycobacteria avium complex (MAC)
o Lymphoma
o Kaposi sarcoma
Treatment
Appropriately treated PCP very good prognosis: Up to
90%
o Trimethoprim-sulfamethoxazole or intravenous (IV)
pentamidine effective in most
Commonly some worsening of the radiograph
during the early course of therapy since these
medications require tremendous amount of IV
fluid for administration
o Patients with PCP and severe hypoxia, early
adjunctive treatment with corticosteroids has
significantly decreased rate of respiratory failure
Prophylactic therapy for both AIDS and patients on
prolonged corticosteroid therapy recommended
o Oral Trimethoprim-sulfamethoxazole or, if allergic,
oral dapsone main prophylactic medications
I SELECTED REFERENCES
1. Feldman C: Pneumonia associated with HIV infection.
Curr Opin Infect Dis. 18(2):165-70,2005
2. Gosselin M: Diffuse lung disease in the
immunocompromised non-HIV patient. Seminars in
Roentgenology Vol. 37: 37-53, 2002
3. Travis et al: Non-neoplastic disorders of the lower
respiratory tract. 1st ed. Washington DC, AFlP.668-680,
2002
 PNEUMOCYSTIS PNEUMONIA

I IMAGE GAllERY

(Left) Frontal radiograph In

this 39 year old AIDS palient
with 3 weeks of worsening
fever and dyspnea. Bilateral 2
perihilar ground-glass
opacities were round to be 9
Pneumocystis pneumonia.
(Right) Frontal radiograph in
a 40 year old AIDS patient
with proven PCP. There is
bilateral ground-glass
opacilies with an upper lobe
distribution. Patient is a/so
taking aerosolized
pentamidine.

Variant
(Left) Axial HRCT in a 43
year old female with SLE on
prolonged corticosteroid
therapy presents with fever
and dyspnea over 7 days.
Bilateral "crazy paving"
ground-glass opacities
proved to be PCP (Right)
Axial CECT in a 60 year old
with lymphoma and recent
chemotherapy. He
developed progressive
dyspnea and (evers over 3
weeks. Bilateral asymmetric
ground-glass and
consolidations were PCP.

Variant
(Left) Axial CECT in a 24 yo
AIDS patient shows mulliple
bilateral cavitary nodules
and complex cysts (arrows).
Open lung biopsy removed
3 nodules, all demonstrated
only Pneumocystis jiroveci.
(Right) Frontal radiograph in
a patient with a large
lymphomatous mass and
recent radiation therapy.
Ground-glass opacities (PCP)
have developed outside the
left radialion portal (arrows).
 SARCOIDOSIS, PULMONARY

2
10

Graphic shows upper and mid lung reticulonodular Frontal radiograph shows symmetric hilar and right
opacities. Symmetrical para tracheal, hilar and subcarinal paratracheallymphadenopathy (arrows). The lungs are
adenopathy, bronchovascular bundle thickening and normal. The appearance is typical for sarcoidosis.
absence of pleural disease.

Morphology: Radiograph shows 1, 2, 3 nodes = right

ITERMINOLOGY paratracheal, right and left hilar
Abbreviations and Synonyms Radiographic Findings
Lofgren syndrome: Fever, bilateral hilar adenopathy, Radiography: 5-15% have normal chest radiograph at
erythema nodosum, arthralgia onset; abnormal chest radiograph: 95%
Lupus pernio: Chronic sarcoid, cutaneous lesions, Lymphadenopathy
bone cysts, pulmonary fibrosis o (80%) most common finding: Bilateral
Definitions hilar/paratracheal
Common systemic granulomatous disease of unknown Pulmonary involvement, 20%
etiology o Reticulonodular opacities (90%) predominately
upper lung zones
o Large airspace nodules with air bronchograms
I IMAGING FINDINGS (alveolar sarcoid)
o Pulmonary fibrosis
General Features Upper lobe and superior segment lower lobe
Best diagnostic clue: Symmetric hilar and mediastinal predominance
lymphadenopathy; without or with pulmonary Upper lobe cyst formation (honeycombing),
opacities traction bronchiectasis with severe disease
Location: Upper lung predilection Atypical appearances
Size o Atypical lymphadenopathy: Unilateral hilar,
o Subtle enlargement to bulky "potato" lymph nodes posterior mediastinal
o Miliary to moderate sized nodules o Unilateral lung disease, cavitary lung lesions, or
o 3 cm to large conglomerate masses pleural effusion

DDx: Hilar and Mediastinal Lymphadenopathy

Histoplasmosis Primary Tuberculosis Hodgkin Lymphoma

 SARCOIDOSIS, PULMONARY

Key Facts
Terminology Progressive massive fibrosis, architectural distortion,
Common systemic granulomatous disease of honeycombing, cysts, bullae
unknown etiology Top Differential Diagnoses
Imaging Findings Berylliosis
Best diagnostic clue: Symmetric hilar and mediastinal

Silicosis
Tuberculosis (TB)
2
lymphadenopathy; without or with pulmonary
opacities Histoplasmosis, coccidioidomycosis, cryptococcus 11
Micronodules (1-5 mm) Clinical Issues
Centrilobular, perivascular, perilymphatic, Major complications include respiratory failure from
bronchovascular bundles, subpleural, septal fibrosis, mycetomas, hemorrhage, cor pulmonale
Often extends in a swath from the hilum to lung Cardiac disease: Myocardial infarction in 5%,
periphery arrhythmias, heart block, sudden death
Predilection for posterior (sub)segment upper lobes Variable, worse in African-Americans (more
and superior segments lower lobes extra pulmonary involvement); better in children
Alveolar sarcoid: Airspace nodules and consolidation
with air bronchograms

Protocol advice: HRCT supine and prone without

CT Findings contrast
NECT
a Lymphadenopathy, bilateral hilar/mediastinal
a Nodal calcification: Amorphous, punctate, eggshell I DIFFERENTIAL DIAGNOSIS
in chronic disease
CECT: For nodes in left paratracheal, aortopulmonary Lung Disease and Enlarged Lymph Nodes
window, anterior mediastinum, retroperitoneal lymph Berylliosis
node groups, and liver and spleen involvement a Identical findings, need occupational history
HRCT Silicosis
a Micronodules (1-5 mm) a Occupational history otherwise identical
Centrilobular, perivascular, perilymphatic, radiographic findings
bronchovascular bundles, subpleural, septal Tuberculosis (TB)
Often extends in a swath from the hilum to lung a Primary TB: Lymphadenopathy asymmetric and
periphery ipsilateral with the consolidation, pleural effusion
Predilection for posterior (sub)segment upper a Miliary TB: Random nodule distribution, none or
lobes and superior segments lower lobes little lymphadenopathy
a Thickened interlobular septa Histoplasmosis, coccidioidomycosis, cryptococcus
a Ground-glass opacities, nodular or lobular in size a Asymmetric lymphadenopathy
may precede or coexist with nodules Lymphoma, mediastinal nodal metastases from intra
a Alveolar sarcoid: Airspace nodules and consolidation or extrathoracic primary
with air bronchograms a Asymmetric nodal enlargement
a Cysts and cavitation in necrotizing sarcoidal angiitis
a Bronchial wall thickening, large and small airway Lung Disease
stenoses Langerhans cell histiocytosis
a Progressive massive fibrosis, architectural distortion, a Minimal adenopathy, lacks peribronchial
honeycombing, cysts, bullae distribution, cysts more common
Due to aggregation of small nodules Hypersensitivity pneumonitis
Traction bronchiectasis a No adenopathy, lacks peribronchial distribution,
Secondary mycetomas in cavities and cysts mosaic attenuation more common from small
a Uncommon manifestations airways disease
Pleural disease: Effusion, thickening, calcification,
chylothorax
Cavity formation, pneumothorax (more common I PATHOLOGY
with alveolar sarcoid)
General Features
Imaging Recommendations General path comments
Best imaging tool a Widespread noncaseating granulomas that resolve
a Radiography usually sufficient for diagnosis and or cause fibrosis
follow-up a Exclusion of an alternative etiology
a CT or HRCT: Useful to characterize interstitial lung Etiology: Unknown
disease and adenopathy Epidemiology
Findings can be pathognomonic for sarcoidosis a Geographic predilection: Swedish, Danish, Japanese
 SARCOIDOSIS, PULMONARY
o Constitutional symptoms more frequent in Cardiac disease: Myocardial infarction in 5%,
African-Americans and Asian-Indians arrhythmias, heart block, sudden death
Staging
o Stage 0: Normal chest radiograph (50%, at Demographics
presentation) Age
o Stage 1: Lymphadenopathy (45-65%) o Children to age 65; onset: Usually age 20-40
o Stage 2: Lymphadenopathy and lung opacities o Onset after age 65: Rare
2 (30-40%) Gender: Females> males
Ethnicity: Predilection in African-American females
o Stage 3: Lung opacities (10-15%)
12 o Stage 4: Fibrosis with or without lymphadenopathy Natural History & Prognosis
(5-25%)
Lymphadenopathy: Usually not visible at 2 years;
Gross Pathologic & Surgical Features however may persist for many years
Symmetrically enlarged lymph nodes Lymph nodes may calcify, sometimes eggshell
Lungs affected in > 90% calcification
Honeycombing usually more severe in upper lung Lung disease 50%), often worsens with nodal
zones regression
80% of cases resolve completely; fibrosis develops in
Microscopic Features 20%
Tight, well-formed granulomas, with rim of Sequelae of pulmonary fibrosis: Cysts, honeycombing,
lymphocytes and fibroblasts traction bronchiectasis, mycetomas, pulmonary artery
Perilymphatic interstitial distribution of granulomas hypertension
Differential diagnosis Prognosis
o Sarcoid-like reaction in hypersensitivity lung disease o Best with erythema nodosum and asymptomatic
o Sarcoid-like reaction in lymphoma and solid organ lymphadenopathy
malignancies (incidence, 5-15%) o Most patients have a good prognosis with resolution
in < 2 years
o Variable, worse in African-Americans (more
ICLINICAllSSUES extrapulmonary involvement); better in children
o Mortality: 1-5%; death respiratory failure,
Presentation myocardial involvement, cor pulmonale,
Most common signs/symptoms hemorrhage (mycetomas)
o Fatigue, malaise, weight loss, fever, night sweats,
dyspnea, dry cough Treatment
o Chest pain, erythema nodosum, uveitis, skin lesions, up to 50% are treated with steroids;
arthropathy immunosuppression for nonresponder
o Asymptomatic, 50%
Other signs/symptoms: Multiorgan disorder:
Symptoms from skin, muscle, bone, joint, neurologic, I DIAGNOSTIC CHECKLIST
eye, cardiac, renal, genital, gastrointestinal
involvement Consider
Tendency to wax and wane CT or HRCT
Symptoms may exacerbate after parturition o Atypical clinical and radiographic findings
May follow interferon therapy 1%), or treatment for o For complications: Bronchiectasis, aspergilloma,
lymphoma fibrosis, cysts/honeycombing, superimposed
Recurrence in transplanted lung has been reported infection, malignancy
Human immunodeficiency virus infected patients: Image Interpretation Pearls
New onset sarcoidosis with restoration of immune
Upper lobe small nodules: Differential diagnosis
system
includes all granulomatous diseases
In < 2% TB precedes sarcoidosis or develops later
Cutaneous anergy, anemia, leukopenia, elevated
sedimentation rate, hypercalcemia (2-10%),
nephrolithiasis
I SElECTED REFERENCES
Raised angiotensin-converting enzyme level, not l. Koyama T et al: Radiologic manifestations of sarcoidosis in
specific various organs. Radiographies. 24(1):87-104, 2004
2. Haramati LB et al: Newly diagnosed pulmonary sarcoidosis
Diagnosis from lung, lymph nodes, and liver biopsy in HIV-infected patients. Radiology. 218(1):242-6, 2001
Transbronchial biopsy: Positive in 90% of cases, even 3. Ravenel jG et al: Sarcoidosis induced by interferon therapy.
if chest radiograph normal AjRAmj Roentgenol. 177(1):199-201,2001
Pulmonary function tests: Variable typically restrictive 4. Traill ZC et al: High-resolution CT findings of pulmonary
with interstitial lung disease but may be obstructive in sarcoidosis. AjR 168:1557-60,1997
those with bronchiectasis 5. Miller BH et al: Thoracic sarcoidosis: Radiologic-pathologic
Major complications include respiratory failure from correlation. Radiographies 15:421-37, 1995
fibrosis, mycetomas, hemorrhage, cor pulmonale
 SARCOIDOSIS, PULMONARY
IIMAGE GALLERY

Typical
(Left) Frontal radiograph
shows bulky hilar
lymphadenopathy (curved
arrow) and multiple bilateral 2
nodules (arrows). (Right)
Axial CECT in same patient 13
shows right upper lobe
nodules indicating alveolar
sarcoid (open arrows) and
left hilar (white curved
arrow) and mediastinal
(black curved arrow)
lymphadenopathy

Typical
(Left) Axial H RCT shows
micronodules, small nodules,
vague ground-glass opacities
in both upper lobes
(arrows). The nodules are
subpleural, centrilobular,
perilobular. (Right) Axial
H ReT in same patient with
sarcoidosis shows
micronodules, small and
inlermediate~sized nodules
(curved arrow).
Micronodu/ar beading seen
along the left major fissure
(open arrow).

Variant
(Left) Frontal radiograph
shows bilateral mid lung
confluent and interstitial
opacities that represent
pulmonary fibrosis (arrows).
The patient was treated with
steroids. (RighI) Frontal
radiograph in the same
patient 2 years later shows
new large cavities in the right
lung (arrows). Bronchoscopy
showed tuberculosis.
Diagnosis: Sarcoidosis (stage
4) and active TB.
 IDIOPATHIC PULMONARY FIBROSIS

2
14

Graphic illustrating the classical subpleural and basal Axial HRCT showing a characteristic bilatera/subp/eural
distribution of fibrosis in fPF. reticular pattern with honeycombing (arrow). There is
evidence of traction bronchieclasis (open arrow).

I TERMINOLOGY Radiographic Findings

Radiography
Abbreviations and Synonyms
o Reticular nodular pattern
Idiopathic pulmonary fibrosis (lPF) o Mid and lower zone predominance
Cryptogenic fibrosing alveolitis (CFA) o Lower zone volume loss
Usual interstitial pneumonia (UIP) Note spurious preservation of lung volume with
Definitions coexistent emphysema
Distinct form of fibrosing idiopathic interstitial CT Findings
pneumonia associated with a histologic pattern of HRCT
usual interstitial pneumonia on surgical biopsy o Predominant reticular pattern with honeycombing
Honeycomb cysts may vary in size dependent on
phase of respiratory cycle
I IMAGING FINDINGS Extent and severity of honeycombing may change
General Features over time
o Basal and peripheral predominance
Best diagnostic clue: Subpleural and basal reticular
o Traction bronchiectasis or bronchiolectasis
pattern with honeycombing (minimal ground-glass
o Ground-glass opacification
opacification) on HRCT
Seldom dominant pattern
Location: Subpleural lung in mid and lower zones
When associated with traction bronchiectasis or
Morphology: Predominant reticular, traction
bronchiolectasis reflects fine fibrosis below
bronchiolectasis, and honeycombing
resolution limits of HRCT
o Volume loss in advanced disease

DDx: Idiopathic Pulmonary Fibrosis

Asbestosis Hypersensitivity Pneumonitis Sarcoidosis

 IDIOPATHIC PULMONARY FIBROSIS

Key Facts
Terminology Pathology
Distinct form of fibrosing idiopathic interstitial Spatial and temporal heterogeneity (a key finding)
pneumonia associated with a histologic pattern of Characteristic fibroblastic foci
usual interstitial pneumonia on surgical biopsy
Clinical Issues
Imaging Findings Insidious onset of breathlessness 2
Best diagnostic clue: Subpleural and basal reticular Inexorable progression with poor prognosis
pattern with honeycombing (minimal ground-glass To date no treatment regimen of proven benefit in 15
opacification) on HRCT improving survival in IPF
Top Differential Diagnoses Diagnostic Checklist
Asbestosis Drug reaction in any patient with IPF pattern
Chronic Hypersensitivity Pneumonitis Subpleural, basal reticular pattern with
Rheumatoid Arthritis honeycombing in the absence of any known cause of
Systemic Sclerosis pulmonary fibrosis should enable confident HRCT
Drug Reaction diagnosis of IPF
Sarcoidosis

o Coexistent centrilobular or paraseptal emphysema

in about 30%
o Mediastinal lymph node enlargement commonly
present
o Supervening (squamous) lung cancer in some
patients
General
o CT more sensitive and accurate than chest
radiography
CT may be normal in mild (or early) disease
o Pattern and distribution
Crucial in making a confident (and accurate)
radiologic diagnosis
Atypical morphologic pattern or distribution in
about 30%
Useful for mapping areas to biopsy
o HRCT may be prognostically useful
Ground-glass opacification without associated
traction bronchiectasis may identify "treatable"
inflammation
More extensive CT reticular pattern associated
with poorer outcome
o Valuable for detection of associated lung cancer
Imaging Recommendations
Best imaging tool: HRCT to detect and characterize
interstitial lung disease
Protocol advice
o Standard high-resolution images
1-1.5 mm collimation
10-20 mm interslice spacing
High-frequency reconstruction algorithm
Chronic Hypersensitivity Pneumonitis
Relatively sparing of extreme bases
o Usually the most severely involved in IPF
Lobular areas of apparent sparing (due to associated
bronchiolar obstructive component)
o When combined with signs of parenchymal scarring
or distortion, gives rise to so-called "head-cheese"
sign
Appearance may be indistinguishable from IPF
Rheumatoid Arthritis
Pulmonary fibrosis indistinguishable from IPF
Erosive arthritis not seen with IPF
More frequently associated with nonspecific
interstitial pneumonia (NSIP) pattern
Systemic Sclerosis
Pulmonary fibrosis indistinguishable from IPF
Dilated esophagus not seen with IPF
More frequently associated with NSIP pattern
Drug Reaction
Can cause similar radiographic abnormalities
Typical drugs: Macrodantin or chemotherapy drugs
Fibrosis will not progress off drug therapy
Sarcoidosis
Typically causes bronchocentric fibrosis in the upper
lung zones
Small proportion have pattern indistinguishable from
IPF

I PATHOLOGY
I DIFFERENTIAL DIAGNOSIS General Features
Asbestosis General path comments: Key features: Patchy fibrosis
Subpleural reticular pattern with honeycombing and architectural distortion
o NB Subpleural lines not pathognomonic of Genetics
asbestosis o Familial cases of IPF reported (probably autosomal
Fibrosis in asbestosis may be coarser than in IPF dominant inheritance)
Associated (calcified) pleural plaques o No genetic markers yet identified
o No definite association with HLA subtypes
 IDIOPATHIC PULMONARY FIBROSIS
o Putative link with aI-antitrypsin inhibition alleles
on chromosome 14 Natural History & Prognosis
Etiology Inexorable progression with poor prognosis
o Unknown etiology Median survival following diagnosis about 3Yz years
Suspected but unproven association with cigarette Rarely, rapid decline and death after period of
smoking relatively slower progression
Desquamative interstitial pneumonia (DIP) o Diffuse alveolar damage on histologic examination
2 previously thought to be "early" cellular phase of Treatment
usual interstitial pneumonia (UIP) but no longer
16 To date no treatment regimen of proven benefit in
felt to be the case
improving survival in IPF
o Unproven associations
Drugs conventionally used in IPF include
Chronic aspiration o Corticosteroids
Infections (e.g., Epstein-Barr, influenza &
o Cytotoxic agents
cytomegalovirus)
o Antifibrotic drugs
Inorganic dusts and solvents
Single lung transplantation for progressive functional
Epidemiology decline
o True incidence and prevalence difficult to estimate
Incidence: 7-10 cases/l00,000 per year
Prevalence: 3-6/100,000
o No geographical predisposition
I DIAGNOSTIC CHECKLIST
Gross Pathologic & Surgical Features Consider
Usual interstitial pneumonia Drug reaction in any patient with IPF pattern
o Spatial and temporal heterogeneity (a key finding) Image Interpretation Pearls
Varying proportions of fibrosis, inflammation and Subpleural, basal reticular pattern with honeycombing
honeycombing interspersed with normal lung in the absence of any known cause of pulmonary
parenchyma fibrosis should enable confident HRCT diagnosis of IPF
Microscopic Features
Fibrosis
o Characteristic fibroblastic foci I SELECTED REFERENCES
Not a feature of other idiopathic interstitial 1. Lee HLet al: Familial idiopathic pulmonary fibrosis:
pneumonia clinical features and outcome. Chest. 127(6):2034-41,2005
o Dense acellular collagen 2. Martinez FJet al: The clinical course of patients with
Mild to moderate interstitial inflammation idiopathic pulmonary fibrosis. Ann Intern Med. 142(12 Pt
1):963-7,2005
o Histiocytes 3. Nishiyama 0 et al: Familial idiopathic pulmonary fibrosis:
o Plasma cells serial high-resolution computed tomography findings in 9
o Lymphocytes patients. J Comput AssistTomogr. 28(4):443-8, 2004
o Type II pneumocyte hyperplasia 4. American Thoracic 50ciety; European Respiratory Society:
Honeycombing American Thoracic Society/European Respiratory Society
o Honeycomb cysts lined by bronchiolar epithelium International Multidisciplinary Consensus Classification of
Regions of normal lung the' Idiopathic Interstitial Pneumonias. This joint
statement of the American Thoracic Society (ATS),and the
European Respiratory Society (ERS)was adopted by the ATS
board of directors, June 2001 and by the ERSExecutive
ICLINICALISSUES Committee, June 2001. AmJ Respir Crit Care Med.
165(2):277-304,2002
Presentation 5. Johkoh T et al: Respiratory change in size of
Most common signs/symptoms honeycombing: inspiratory and expiratory spiral
o Insidious onset of breathlessness volumetric CT analysis of 97 cases. J Comput Assist
Usually present for some months prior to clinical Tomogr. 23(2):174-80,1999
presentation 6. Chan- Yeung M et al: Cryptogenic fibrosing alveolitis.
o Non-productive cough Lancet. 350(9078):651-6, 1997
7. Niimi H et al: CT of chronic infiltrative lung disease:
Other signs/symptoms prevalence of mediastinal lymphadenopathy. J Comput
o Digital clubbing AssistTomogr. 20(2):305-8, 1996
o Fine inspiratory ("velcro") crackles 8. Lynch DAet al: Can CT distinguish hypersensitivity
o Signs of right heart failure pneumonitis from idiopathic pulmonary fibrosis? AJRAmJ
o Pulmonary function tests Roentgenol. 165(4):807-11, 1995
Restrictive with decreased diffusion capacity 9. Mathieson JR et al: Chronic diffuse infiltrative lung disease:
(OLCO) comparison of diagnostic accuracy of CT and chest
radiography. Radiology. 171(1):111-6, 1989
Demographics 10. Staples CA et al: Usual interstitial pneumonia: correlation
Age: 55-70 years of CT with clinical, functional, and radiologic findings.
Radiology. 162(2):377-81, 1987
Gender: M > F
 IDIOPATHIC PULMONARY FIBROSIS
I IMAGE GALLERY
Typical
(Left) Axial H RCT shows
relatively asymmetric
subpleural reticular pattern
with honeycombing (arrows)
in right lung. There is
2
associated centrilobu/ar
17
emphysema. (Right) Axial
HRCT image at same level
on mediastinal windows
showing enlarged
peri-tracheal lymph nodes, a
reasonably common finding
in IPF

Variant
(Left) Axial HRCT shows 2.5
em diameter biopsy-proven
squamous carcinoma in the
posterior segment of the right
upper lobe in a smoker with
IfF. There is extensive
coexistent centri/obular
emphysema. (Right) Axial
HRCT shows mixed reticular
pattern with honeycombing
but also ground-glass
opacification in both lung
bases in a patient with
biopsy-proven IPF

Typical
(Left) Axial HRCT shows a
reticular pattern with gross
honeycombing in IPF The
changes are most
pronounced at the lung
bases. Note the significant
retraction of extra-pleural fat
bilaterally (arrows). (Right)
Axial H RCT at the level of
the carina shows a reticular
pattern (anteriorly) and
extensive coexistent
centrilobu/ar emphysema in
a heavy smoker with IPF
 HYPERSENSITIVITY PNEUMONITIS

2
18

Axial graphic shows typical features of hypersensitivity Axial HRCT shows multiple low density ill-defined
pneumonitis. Centrilobular ground-glass nodules centrilobularnodules.
uniformly distributed throughout the lung Lobular
air-trappingalso frequently present.

ITERMINOLOGY Radiographic Findings

Radiography
Abbreviations and Synonyms o Vary with the stage of the disease: Acute, subacute
Extrinsic allergic alveolitis or chronic
Definitions Chest usually normal in acute, subacute disease
Group of allergic lung diseases caused by the o Acute stage
inhalation of a variety of organic and chemical Chest radiography abnormal in only about 10%
antigens Fine nodular or reticulonodular pattern
o Farmer's lung and bird fancier's lung the most Bilateral airspace consolidation in the lower lobes
common forms Often misdiagnosed as "pneumonia"
o "Hot tub" lung latest source o Subacute stage
Chest radiograph usually abnormal (90%)
Poorly-defined small nodules
I IMAGING FINDINGS Diffuse or middle and lower increased lung
density
General Features Obscuration of vascular margins
Best diagnostic clue o Chronic stage
o Midlung miliary or interstitial disease, sparing Findings of fibrosis: Architectural distortion,
costophrenic angles volume loss
o Mosaic perfusion: "Head-cheese" sign Midlung fibrosis
Even more accurate when lung cysts are present No pleural disease or adenopathy
Usually spares or less severe in costophrenic angles

DDx: Bilateral Ground-Glass Opacities

RB-ILD, Emphysema NSIP Idiopathic Pulmonary Fibrosis

 HYPERSENSITIVITY PNEUMONITIS
Key Facts
Terminology Top Differential Diagnoses
Group of allergic lung diseases caused by the Nonspecific Interstitial Pneumonia (NSIP)
inhalation of a variety of organic and chemical Respiratory Bronchiolitis-Interstitial Lung Disease
antigens (RB-ILD)
Farmer's lung and bird fancier's lung the most Silicosis
common forms Scleroderma 2
Imaging Findings Pathology 19
Vary with the stage of the disease: Acute, subacute or More than 200 different organic antigens from a
chronic variety of sources
Ground-glass opacities (patchy distribution)
Ill-defined centrilobular nodules Diagnostic Checklist
Mosaic perfusion: "Head-cheese" sign Diagnosis requires a constellation of clinical,
Middle and lower lung predominance radiographic, physiologic, pathologic, and
Lung cysts immunologic criteria
Fibrosis: Honeycombing, traction bronchiectasis, and
architectural distortion

HRCT: Honeycombing, bibasilar reticular opacities,

CT Findings and traction bronchiectasis
NECT Typical anatomic distribution: Peripheral, subpleural,
a More sensitive but may be normal and bibasilar
a Most prominent mid to lower lungs Lack of peribronchovascular distribution
a Spares costophrenic angles Does not spare costophrenic angles, in fact, usually
a Acute stage severely involved
Small, ill-defined centrilobular nodules Air-trapping not a feature
Bilateral airspace consolidation
a Subacute stage Nonspecific Interstitial Pneumonia (NSIP)
Ground-glass opacities (patchy distribution) Younger age than lPF (40-50 years)
Ill-defined centrilobular nodules Two histologic patterns: Cellular and fibrotic
Mosaic perfusion: "Head-cheese" sign HRCT: Ground-glass opacities, irregular reticulation,
Middle and lower lung predominance and patchy consolidation
Lung cysts Honeycombing absent or minimal
a Chronic stage Peripheral and/or peribronchovascular distribution
Fibrosis: Honeycombing, traction bronchiectasis, Good prognosis
and architectural distortion Air-trapping not a feature
Reticular opacities
Superimposed subacute findings: Ground-glass Respiratory Bronchiolitis-Interstitial Lung
opacities and small ill-defined centrilobular Disease (RB-ILD)
nodules Smokers
Random distribution: Peribronchial and Predominantly upper lung zones
subpleural Associated centrilobular emphysema
Middle and upper lung predominate
Costophrenic angles less severely involved Sarcoid
Peribronchovascular distribution, subpleural nodules,
Imaging Recommendations adenopathy
Best imaging tool Predominantly upper lung zones
a Chest radiograph usually sufficient to document
extent of pathology in the acute form Silicosis
a Thin section CT: More sensitive in detecting Occupational history
hypersensitivity pneumonitis (HP) and following its May have adenopathy
evolution from consolidation/ground-glass to Subpleural lymphatic deposits rare in HP
centrilobular nodules and potentially fibrosis Air-trapping not a feature
Scleroderma
Dilated esophagus, basilar fibrosis and honeycombing
I DIFFERENTIAL DIAGNOSIS
Idiopathic Pulmonary Fibrosis (IPF)
Age> 50 years
Exclusion of known causes of infiltrative lung diseases:
Exposures, drugs, and connective tissue diseases
 HYPERSENSITIVITY PNEUMONITIS
I PATHOLOGY Natural History & Prognosis
Frequency unknown
General Features Personal habits alter the appearance and course of
General path comments disease
o Allergic reaction to airborne organic particles (1-5 Variable, complete recovery with removal of antigen
um) to end stage fibrosis
o More than 200 different organic antigens from a
2 variety of sources
Good when diagnosis is made in early stages
Clinical improvement before first 6 months
o 95% of cases occurs in nonsmokers Acute form rare in smokers
20
Etiology
o Antigens from a variety of different sources Treatment
o Small particles deposit in bronchioles, incite allergic Depends largely on avoiding the antigen
granulomatous reaction Removal from environment
o Thermophilic actinomycetes common antigen Steroids
Gross Pathologic & Surgical Features
Honeycomb lung in chronic HP I DIAGNOSTIC CHECKLIST
Distribution mid to upper lung
Costophrenic angles less involved Consider
Microscopic Features Diagnosis requires a constellation of clinical,
radiographic, physiologic, pathologic, and
Scattered small interstitial noncaseating granulomas,
immunologic criteria
pleomorphic diffuse interstitial infiltrate of
High index of clinical suspicion: Exposure to a known
lymphocytes, and cellular bronchiolitis
inciting antigen is considered the strongest single
Granulomas may be few and difficult to find
predictor of hypersensitivity pneumonitis
Bronchiolitis obliterans organizing pneumonia (BOOP)
Histopathological diagnosis of NSIP should be
pattern
evaluated for possible hypersensitivity pneumonitis
Image Interpretation Pearls
I CLINICAL ISSUES Centrilobular nodules adn air trapping in
acute-subacute disease
Presentation Subpleural basilar honeycoming with less involvment
Most common signs/symptoms of costophrenic angles characteristic of chronic
o Acute, subacute, chronic forms, considerable overlap hypersensitivity pneumonitis
o Nonspecific symptoms
o Sudden onset of a flu-like syndrome (fever, chills,
and malaise) indicates acute illness I SELECTED REFERENCES
Pulmonary symptoms: Severe dyspnea, chest
tightness, and dry or mildly productive cough 1. Franquet et al: Lung cysts in subacute hypersensitivity
pneumonitis. J Comput Assist Tomogr. 27(4):475-8, 2003
Peak intensity of symptoms: 3-6 hours after initial 2. Hartman TE:The HRCTFeatures of Extrinsic Allergic
exposure Alveolitis.Semin Respir Crit Care Med. 24:419-426, 2003
Signs/symptoms gradually clear over 24-48 hours 3. Herraez I et al: Hypersensitivity pneumonitis producing a
Often mistaken as pneumonia BOOP-likereaction: HRCT/pathologic correlation. J Thorac
o Insidious onset of symptoms indicates Imaging 17:81-83, 2002
subacute/chronic illness 4. Matar LOet al: Hypersensitivity pneumonitis. AJR.
Some hemoptysis in up to one-fourth of patients 174:1061-6,2000
Clinically better after avoiding exposure 5. Patel RAet al: Hypersensitivity pneumonitis: patterns on
high-resolution CT.J Comput AssistTomogr. 24:965-970,
o Chronic dyspnea indicates prolonged exposure and
2000
chronic illness 6. Hansell, OM et al: Hypersensitivity pneumonitis:
Indistinguishable from other chronic correlation of individual CT patterns with functional
inflammatory lung diseases abnormalities. Radiology. 199:123-128, 1996
Outcome similar to idiopathic pulmonary fibrosis 7. Lynch OAet al: Can CT distinguish hypersensitivity
Other signs/symptoms pneumonitis from idiopathic pulmonary fibrosis? AJR.
o Typical exposure 165:807-11,1995
Wet hay: Farmer's lung 8. Remy-Jardin M et al: Subacute and chronic bird breeder
hypersensitivity pneumonitis: sequential evaluation with
Birds: Pigeon-breeder's lung CT and correlation with lung function tests and
Office: Humidifier lung bronchoalveolar lavage. Radiology. 189: 111-118, 1993
Numerous other organic antigens identified (Le., 9. Gurney JW: Hypersensitivity pneumonitis. Radiol Clin
mushrooms, etc.) North Am. 30:1219-1230, 1992
"Hot tub" lung 10. McLoud TC: Occupational lung disease. Radiol Clin North
o Individual must be susceptible (allergic response), Am. 29:931-941, 1991
most dust-exposed individuals (90%) have no 11. Silver SFet al: Hypersensitivity pneumonitis: evaluation
response with CT. Radiology. 173:441-445,1989
 HYPERSENSITIVITY PNEUMONITIS
I IMAGE GALLERY
Typical
(Left) Frontal radiograph
shows bilateral ill-defined
areas of consolidation.
(Right) Axial '-IRCT shows 2
extensive areas of
ground-glass attenuation and 21
focal (lobular) areas of low
attenuation and decreased
perfusion (arrows)
representing associated
air-trapping.

(Left) Axial HRCT shows

patchy bilateral ground-glass
attenuation and centrilobu/ar
ill-defined nodules. A solitary
thin-walled cyst is visible in
the left lower lobe (arrow).
(Right) Axial HRCT
performed at maximal
expiration (same level)
shows a lobu/ar area of
decreased attenuation
(air-trapping) (arrow), not
visible in previous series. The
size of the cyst has not
changed.

(Left) Axial HRCT performed

at end-inspiration shows a
typical mosaic perfusion
pattern. Note the
geographical areas of
decreased attenuation and
vascularity in both lower
lobes (arrows). (RighI) Axia'
HRCT performed at maximal
expiration shows that areas
of decreased attenuation
visible on inspiratory CT
scans, have not changed in
volume due to air-trapping
(arrows).
 RHEUMATOID ARTHRITIS

2
22

Frontal radiograph shows basilar reticular interstitial lung Lateral radiograph again shows the basilar
disease with a nodular opacity at the lelt lung base predominance 01 the interstitial changes typical lor RA.
larrows) in this patient with a history 01 long standing In the absence 01 other findings 01 RA, the interstitial
rheumatoid arthritis. changes a/one are nonspecific.

!TERMINOLOGY Radiographic Findings

Pleural disease
Abbreviations and Synonyms o Pleural thickening (20%)
Rheumatoid arthritis (RA) o Pleural effusion
Definitions More common in males
Subacute or chronic inflammatory polyarthropathy of Small to large, usually unilateral, can be bilateral
unknown cause Transient, persistent or relapsing
o Associated lung findings: Pleural disease, interstitial Fibrothorax
fibrosis with honeycombing, micronodules, small Susceptible to empyema
and large nodules, and airway disease o Pneumothorax: Rare
o Complications include pneumonia, empyema, drug Associated with rheumatoid nodules
reaction, amyloid, cor pulmonale Parenchymal disease
o Reticulonodular and irregular linear opacities, lower
lung zones
I IMAGING FINDINGS o Distortion, honeycombing, progressive loss of
volume
General Features o Upper lobe fibrobullous disease, rare
Best diagnostic clue: Diffuse interstitial thickening o Rheumatoid nodules (seen in < 5%)
with erosion of distal clavicles Solitary or multiple, 5 mm to 7 cm
Location: Interstitial lung disease: Subpleural lower Peripheral (subpleural)
lobes Wax and wane
May cavitate (50%), thick smooth wall
May calcify: Rarely

DDx: Interstitial/Inflammatory lung Disease

Scleroderma
 RHEUMATOID ARTHRITIS
Key Facts
Terminology
Subacute or chronic inflammatory polyarthropathy of
unknown cause
Associated lung findings: Pleural disease, interstitial Top Differential
fibrosis with honeycombing, micronodules, small
and large nodules, and airway disease
Imaging Findings
Best diagnostic clue: Diffuse interstitial thickening Clinical Issues
with erosion of distal clavicles
Rheumatoid nodules (seen in < 5%)
Caplan syndrome: Rare
Hyperinflation (bronchiolitis obliterans) or
cryptogenic organizing pneumonia (COP) pattern
Bronchiectasis (20%)
Pulmonary fibrosis often indistinguishable from usual
interstitial pneumonia (VIP)
Identical to subcutaneous nodules
More common in males, especially smokers
May have uptake on PET
o Caplan syndrome: Rare
Hypersensitivity reaction to dust
Associated with coal miners pneumoconiosis
Large rounded nodules (0.5-5 em)
Nodules have a peripheral distribution
Redefined to include: Silica, asbestos, dolomite,
carbon
Serologic but not clinical rheumatoid arthritis
Airway disease
o Hyperinflation (bronchiolitis obliterans) or
cryptogenic organizing pneumonia (COP) pattern
o Diffuse reticulonodular pattern: Follicular
bronchiolitis
o Bronchiectasis (20%)
Isolated or traction bronchiectasis
Peribronchovascular micronodules give
"tree-in-bud" appearance
CT Findings
HRCT Imaging Recommendations
o HRCT useful to investigate pleuropulmonary and
airway disease
o Abnormal in 50%, more sensitive than pulmonary
function tests (PFTs)
o Pleural disease: Most common abnormality in RA
May be associated with pericarditis, interstitial
fibrosis, interstitial pneumonia or lung nodules
o Parenchymal disease
Pulmonary fibrosis often indistinguishable from
usual interstitial pneumonia (VIP)
Interstitial fibrosis seen in 5% by chest
radiography and 30-40% by HRCT
Honeycombing (10%), subpleural in lower lobes
Consolidation (5%)
Ground-glass opacities (GGO) (15%)
Micronodules (20%) (centrilobular, peribronchial,
subpleural)
COP pattern: GGO, nodular opacities,
peribronchial or peribronchiolar distribution
o Nodules or masses
Pleural abnormalities and pulmonary nodules, if
present, help distinguish RA related interstitial lung
disease (ILD) from VIP
Diagnoses
Hand films or findings of distal clavicle erosions
useful to differentiate RA from other interstitial lung
2
disease 23
Most have arthritis; positive rheumatoid factor (RF)
(80%), and cutaneous nodules
Pleural fluid: High protein, low glucose, low pH, high
LDH, high RF,low complement
Death from infection, respiratory failure, cor
pulmonale, amyloidosis
Drugs used to treat RA may cause lLD
Resemble neoplasm, discrete, rounded or
lobulated, subpleural
Pleural abnormalities and pulmonary nodules, if
present, help distinguish RA related interstitial
lung disease (ILD) from VIP
o Airway disease
COP pattern, bronchiectasis
Obliterative bronchiolitis pattern rare; mosaic
pattern with hyperinflation, air trapping on
expiratory exam
Micronodules < 1 em; centrilobular, subpleural,
peribronchial; centrilobular branching pattern in
follicular bronchiolitis
Bronchocentric granulomatosis: Bronchocentric
nodules, similar to rheumatoid nodules
Follicular bronchiolitis rare; centrilobular nodules
and peribronchial thickening, caused by lymphoid
follicular hyperplasia along the airways
o Other findings
Cor pulmonale, lymphadenopathy, sclerosing
mediastinitis, pericarditis
Best imaging tool: HRCT useful to characterize pattern
and extent of disease
I DIFFERENTIAL DIAGNOSIS
General
Hand films or findings of distal clavicle erosions useful
to differentiate RA from other interstitial lung disease
Lung
Interstitial lung disease
o VIP/IPF
Identical radiographic findings: HRCT shows
peripheral, basilar ILD
No bony erosive changes
o Scleroderma
Identical radiographic findings
Dilated esophagus: Relaxation lower esophageal
sphincter
 RHEUMATOID ARTHRITIS
No joint erosive changes as in RA: Hallmark is
acroosteolysis (resorption distal phalanx)
o Asbestosis
Identical radiographic findings
May have pleural plaques (often calcify)
Occupational history
No osseous erosions
2 o Cryptogenic organizing pneumonia (COP)
Bilateral or unilateral, patchy consolidation, GGO,
24 often subpleural, peribronchial
Basilar irregular linear opacities
I PATHOLOGY
General Features
General path comments
o Subacute or chronic inflammatory polyarthropathy
of unknown cause
o Interstitial lung disease
Usual interstitial pneumonia
Nonspecific intersitial pneumonia (NSIP)
Etiology: Possible inflammatory, immunologic,
hormonal, and genetic factors
Epidemiology: Thoracic involvement more common
in males
Microscopic Features
Pulmonary fibrosis, either UIP or NSIP pattern
o Other: Interstitial pneumonitis, COP, lymphoid
follicles, rheumatoid nodules (pathognomonic)
Pleural biopsy: May show rheumatoid nodules
Pleural fluid: Lymphocytes, acutely neutrophils and
eosinophils, low in glucose
ICLINICAllSSUES
Presentation
Most common signs/symptoms
o Primary sites of inflammation: Synovial membranes
and articular structures
o Onset is usually between 25 and 50 years
o Insidious onset, with relapses and remissions
Other signs/symptoms: Extraarticular RA: More
common in males, age 50-60 years
Thoracic symptoms
o Thoracic disease may develop before, at onset or
after onset of arthritis
o Asymptomatic, or dyspnea, cough, pleuritic pain,
finger clubbing, hemoptysis, infection,
bronchopleural fistula, pneumothorax
o Most have arthritis; positive rheumatoid factor (RF)
(80%), and cutaneous nodules
o Pleural fluid: High protein, low glucose, low pH,
high LDH, high RF, low complement
o PITs: Restrictive defect, reduced diffusing capacity,
sometimes obstructive defect if predominant airways
disease
Demographics
Age: Any age but more common in middle-aged adults
Gender: RA is 3x more common in females
Natural History & Prognosis
5 year survival 40%
Death from infection, respiratory failure, cor
pulmonale, amyloidosis
o Infection most common cause of death
o Pleural disease common; 40-75% in postmortem
studies
Treatment
Treatment: Steroids, immunosuppressant drugs
Drugs used to treat RA may cause ILD
o Methotrexate
o Gold
o D-penicillamine
I DIAGNOSTIC CHECKLIST
Consider
Evaluate clavicles for changes of RA in any patient
with IPF pattern
I SElECTED REFERENCES
1. Lee HK et al: Histopathologic pattern and clinical features
of rheumatoid arthritis-associated interstitial lung disease.
Chest. 127(6):2019-27, 2005
2. Lynch DA et al. Idiopathic Interstitial Pneumonias: CT
Features Radiology. 236:10-21, 2005
3. Zrour SH et al: Correlations between high-resolution
computed tomography of the chest and clinical function
in patients with rheumatoid arthritis. Prospective study in
75 patients. Joint Bone Spine. 72(1):41-7, 2005
4. Biederer J et al: Correlation between HRCT findings,
pulmonary function tests and bronchoalveolar lavage
cytology in interstitial lung disease associated with
rheumatoid arthritis. Eur Radiol. 14(2):272-80,2004
5. Fischer T et al. The idiopathic interstitial pneumonias: a
beginner's guide. Imaging. 16,37-49,2004
6. Tanaka N et al: Collagen vascular disease-related lung
disease: high-resolution computed tomography findings
based on the pathologic classification. J Com put Assist
Tomogr. 28(3):351-60, 2004
7. Terasaki H et al: Respiratory symptoms in rheumatoid
arthritis: relation between high resolution CT findings and
functional impairment. Radiat Med. 22(3):179-85, 2004
8. Yoshinouchi T et al: Nonspecific interstitial pneumonia
pattern as pulmonary involvement of rheumatoid arthritis.
Rheumatollnt. 2004
9. Dawson JK et al: Predictors of progression of HRCT
diagnosed fibrosing alveoli tis in patients with rheumatoid
arthritis. Ann Rheum Dis. 61(6):517-21, 2002
10. Dawson JK et al: Fibrosing alveoli tis in patients with
rheumatoid arthritis as assessed by high resolution
computed tomography, chest radiography, and pulmonary
function tests. Thorax. 56(8):622-7, 2001
11. Flaherty KR et al. Histopathologic Variability in Usual and
Nonspecific Interstitial Pneumonias. Am. J. Respir. Crit.
Care Med. 164(9): 1722-1727, 2001
12. Rockall AG et al. Imaging of the pulmonary manifestations
of systemic disease. Postgrad Med. 77:621-638, 2001
13. Demir R et al: High resolution computed tomography of
the lungs in patients with rheumatoid arthritis. Rheumatol
Int. 19(1-2):19-22, 1999
14. Perez T et al: Airways involvement in rheumatoid arthritis:
clinical, functional, and HRCT findings. Am J Respir Crit
Care Med. 157(5 Pt 1):1658-65, 1998
 RHEUMATOID ARTHRITIS
I IMAGE GAllERY

(Left) Axial HRCT shows

honeycombing and septal
thickening at the lung base.
While the findings are
2
nonspecific, they are typical
for that of RA. (Right) Axial 25
CECT shows a small centrally
calcified nodule in the RLL
(arrow). The patient was a
smoker with this spiculated
growing nodule which was
removed and found to be a
rheumatoid nodule.

(Left) Frontal radiograph

shows necrobiotic nodules of
RA (arrows). They may be
multiple and may cavitate.
Pulmonary nodules may
precede the systemic
findings of RA. (Courtesy I.
Speckman, MO). (Right)
Radiograph of the hand
shows severe erosive
changes of the distal ulna
and radius and carpal bones.
Rheumatoid arthritis is the
most common purely erosive
inflammatory arthropathy.

Typical
(Left) Anteroposterior
radiograph of the hand
shows early marginal erosive
changes of the proximal
interphalangeal (PIP) joints
(arrows), with joint space
narrowing. (Courtesy of C
Bush, MO). (Right)
Anteroposterior radiograph
of the shoulder shows
chondrolysis, oSleopenia,
and erosions of the humeral
head and clavicle. Rotator
cuff degeneration results in a
high-riding humeral head.
 SCLERODERMA, PULMONARY

2
26

Frontal radiograph shows fine "lace-like" diffuse Axial HRCT shows fine intralobular interstitial thickening
intersUlial thickening from scleroderma. Heart is mildly farrow), ground-glass opacification, extending along
enlarged and central pulmonary arteries (arrow) are bronchovascular pathways fopen arrows) in NSIP
enlarged from pulmonary arterial hypertension. pattern.

!TERMINOLOGY I IMAGING FINDINGS

Abbreviations and Synonyms General Features
Systemic sclerosis Best diagnostic clue: Basilar interstitial thickening with
dilated esophagus
Definitions
Location: Basilar subpleural lung
Generalized connective tissue disorder affecting Morphology: Fine "lace-like" reticular pattern at HRCT
multiple organ systems including the skin, lungs,
heart and kidneys Radiographic Findings
Limited cutaneous systemic sclerosis (60%) Radiography
o Skin involvement hands, forearms, feet and face o Abnormal in 20-65%
o Long-standing Raynaud phenomenon o Lung
o CREST syndrome: Calcinosis, Raynaud, esophageal Widespread symmetric basal reticulonodular
dysmotility, sclerodactly, telangiectasias pattern with cysts (1-30 mm) and/or
Diffuse cutaneous systemic sclerosis (40%) honeycombing
o Acute onset: Raynaud, acral and truncal skin Progression of fine basilar reticulation ("lace-like")
involvement to coarse fibrosis
o High frequency interstitial lung disease Decreased lung volumes, sometimes out of
Scleroderma sine scleroderma (rare) proportion to lung disease
o Interstitial lung disease but no skin manifestations Elevated diaphragms may also be due to
diaphragmatic muscle atrophy and fibrosis
o Associated findings
Dilated, air-filled esophagus without an air-fluid
level best seen on lateral

DDx: Scleroderma

Nonspecific Interstitial Pneumonitis Idiopathic Pulmonary Fibrosis Asbestosis

 SCLERODERMA, PULMONARY

Key Facts
Terminology Top Differential Diagnoses
Systemic sclerosis Idiopathic Pulmonary Fibrosis
Generalized connective tissue disorder affecting Aspiration Pneumonia
multiple organ systems including the skin, lungs, Nonspecific Interstitial Pneumonitis
heart and kidneys

Asbestosis
Rheumatoid Arthritis 2
Imaging Findings Drug Reaction
Best diagnostic clue: Basilar interstitial thickening 27
with dilated esophagus Clinical Issues
Esophageal dilatation (80%) air-filled Most common presentation is Raynaud phenomenon
Pulmonary artery enlargement from pulmonary (up to 90%), tendonitis, arthralgia, arthritis
artery hypertension, 500/0)may be separate from Poor; 70% 5 year survival; cause of death usually
interstitial lung disease (ILD) (10%) aspiration pneumonia
1/3rd have pattern similar to idiopathic pulmonary
fibrosis (IPF)
Diagnostic Checklist
Lung carcinoma in patient with dominant nodule or
focal ground-glass opacity

Pleural thickening and effusions, rare 15%) o Peripheral posterior basilar distribution
Musculoskeletal: Superior and posterolateral rib Tends to have a bronchovascular distribution
erosion 20%) rather than a subpleural distribution
Absorption distal phalanges, tuft calcification
Other Modality Findings
Secondary lung cancer, often bronchioloalveolar
cell carcinoma or adenocarcinoma Esophagram
o Cardiomegaly may be due to pericardial effusion, o Dilated, aperistaltic esophagus (50-90%)
secondary to pulmonary artery hypertension, o Gastroesophageal reflux, patent gastroesophageal
myocardial ischemia due to small vessel disease, or junction
infiltrative cardiomyopathy Imaging Recommendations
CT Findings Best imaging tool: HRCT more sensitive for pulmonary
CECT disease; esophagram for motility
o Esophageal dilatation (80%) air-filled
o Lymphadenopathy (60-70%)
Rarely identified on chest radiography I DIFFERENTIAL DIAGNOSIS
Usually seen in those with interstitial lung disease Idiopathic Pulmonary Fibrosis
o Pulmonary artery enlargement from pulmonary
Lacks esophageal dilatation or musculoskeletal
artery hypertension, 50%) may be separate from
changes
interstitial lung disease (ILD) (10%)
Interstitial lung disease more coarse, honeycombing
o Pleural thickening (pseudoplaques, 33%)
more common
Subpleural micronodules
Ground-glass opacification less common
Pseudoplaques (90%): Confluence of subpleural
Subpleural distribution prominent
micronodules < 7 mm in width
Diffuse pleural thickening (33%) Aspiration Pneumonia
HRCT Recurrent opacities and chronic fibrosis in dependent
o Abnormal in 60-90% (may have false negatives) lung
o Wide spectrum from ground-glass opacities and Known esophageal motility disorder
micronodules to honeycombing Scleroderma patients at risk
o 1/3rd have pattern similar to idiopathic pulmonary
fibrosis (IPF) Nonspecific Interstitial Pneumonitis
Subpleural distribution Identical radiographic pattern
Honeycombing (tends to be less coarse and Esophagus not dilated
"lace-like")
Minimal ground-glass opacification Asbestosis
o Nonspecific interstitial pneumonitis (NSIP) Pleural plaques (80%)
Ground-glass opacification Lacks esophageal dilatation
Honeycombing less common Rheumatoid Arthritis
Peripheral bronchovascular distribution
Lacks esophageal dilatation
o Cysts
Identical radiographic pattern
May have thin-walled subpleural cysts 10-30 mm
Symmetric articular erosive changes
in diameter
Predominately in mid and upper lobes
 SCLERODERMA, PULMONARY
o Tightening, induration and thickening of the skin,
Drug Reaction Raynaud phenomenon, vascular abnormalities,
Lacks esophageal dilatation musculoskeletal manifestations, visceral
Identical radiographic pattern involvement of lungs, heart, and kidneys
Sarcoidosis oEsophageal dysmotility, gastroesophageal reflux,
Lacks esophageal dilatation esophageal candidiasis, and stricture, weight loss
o Renal disease: Hypertension (renal crisis in 10%),
2 Nodular interstitial thickening of lymphatics
predominantly in mid-upper lung
found
renal failure
o Antinuclear antibodies (100%)
28 o Pulmonary function tests
I PATHOLOGY Restrictive or obstructive
Decreased diffusion capacity
General Features o Bronchoalveolar lavage varies from lymphocytic to
General path comments neutrophilic alveolitis (SO%)
o Overproduction and tissue deposition of collagen o Scleroderma features seen in
o Lung fourth most common organ involved after CREST, mixed connective tissue disease (MCTD),
skin, arteries, esophagus diffuse fasciitis and eosinophilia, carcinoid
Genetics: Suspect genetic susceptibility and/or syndrome, drug reactions, chronic graft versus
environmental factors (silica, industrial solvents) host disease
Etiology Demographics
o Reduced circulating T-suppressor cells and natural
Age: Usual onset age 30-50
killer cells which can suppress fibroblast
Gender: M:F = 1:3
proliferation
o Antitopoisomerase I (30%), anti-RNA polymerase [([ Natural History & Prognosis
and antihistone antibodies associated with Lung disease indolent and progressive
interstitial lung disease Poor; 70% 5 year survival; cause of death usually
o Anticentromere antibodies in CREST variant aspiration pneumonia
associated with absence of interstitial lung disease
Epidemiology Treatment
o Scleroderma uncommon 1.2/100,000 No specific treatment
o Pulmonary disease in 70-100% at autopsy Renal failure may actually improve musculoskeletal
disease
Microscopic Features
Pulmonary hypertension
o Most distinctive finding: Concentric laminar fibrosis I DIAGNOSTIC CHECKLIST
with few plexiform lesions
NSIP: Cellular or fibrotic (80%) Consider
Usual interstitial pneumonitis (UIP): Fibroblast Lung carcinoma in patient with dominant nodule or
proliferation, fibrosis and architectural distortion focal ground-glass opacity
(10-20%)
Image Interpretation Pearls
Staging, Grading or Classification Criteria In patients with chronic interstitial lung disease, look
American College of Rheumatology criteria: for dilated esophagus
Scleroderma requires 1 major or 2 minor
o Major criteria: Involvement of skin proximal to
metacarpophalangeal joints I SELECTED REFERENCES
o Minor criteria: Sclerodactyly, pitting scars, loss of 1. de Azevedo ABet al: Prevalence of pulmonary
finger tip tufts, bilateral pulmonary basal fibrosis hypertension in systemic sclerosis. Clin Exp Rheumatol.
23(4):447-54,2005
2. Galie N et al: Pulmonary arterial hypertension associated to
I CLINICAL ISSUES connective tissue diseases. Lupus. 14(9):713-7,2005
3. Highland KBet al: New Developments in Scleroderma
Presentation Interstitial Lung Disease. Curr Opin Rheumatol.
Most common signs/symptoms 17(6):737-45,2005
o Pulmonary disease usually follows skin 4. Desai SRet al: CT features of lung disease in patients with
manifestations systemic sclerosis: comparison with idiopathic pulmonary
fibrosis and nonspecific interstitial pneumonia. Radiology.
Most common presentation is Raynaud 232(2):560-7, 2004
phenomenon (up to 90%), tendonitis, arthralgia, 5. Kim EAet al: Interstitial pneumonia in progressive
arthritis systemic sclerosis: serial high-resolution CT findings with
Dyspnea (60%), cough, pleuritic pain, fever, functional correlation. J Comput AssistTomogr.
hemoptysis, dysphagia 25(5):757-63,2001
Other signs/symptoms 6. Diot Eet al: Relationship between abnormalities on
high-resolution CT and pulmonary function in systemic
sclerosis. Chest. 114(6):1623-9, 1998
 SCLERODERMA, PULMONARY
I IMAGE GALLERY

(Left) Frontal radiograph

shows diffuse fine reticular
interstitia/lung disease.
Dilated air-Wed esophagus 2
(arrows). Scleroderma.
(Right) Axial HRCT in same 29
patient shows fine reticular
pattern in the lower lungs
with decrease in volume of
the lower lobes (arrows).
Air-filled dilated esophagus
(open arrow).

Typical
(Left) Axial CECT shows
enlarged main and left
pulmonary artery from
pulmonary arterial
hypertension (open arrows).
Mild enlargement lymph
nodes (arrow) and mild
diffuse pleural thickening
(curved arrows). (Right)
Axial CECT shows severe
basilar honeycombing in
same patient. Cardiomegaly
and right heart dilatation
from cor pulmonale
(arrows).

Typical
(Left) Axial H RCT shows
predominant bronchiectasis
and volume loss in both
lower lobes (arrows). Dilated
lower esophagus with
air-fluid level (open arrow).
(Right) Axial H RCT shows
mild subpleural intralobular
interstitial thickening and
tiny cystic spaces (arrows). A
few subpleural nodules or
pseudoplaques (open
arrows).
 POLYMYOSITIS - DERMATOMYOSITIS, PULMONARY

2
30

Graphic shows characteristic early features of Transverse HRCT shows extensive bibasi!ar reticulation,
polymyositis-dermatomyositis. Diffuse subpleural patchy traction bronchiectasis (arrow) and honeycombing in a
ground-glass opacities. patient diagnosed with polymyositis.

ITERMINOlOGY Lungs
o Interstitial lung disease
Abbreviations and Synonyms Symmetric, basal reticular pattern of parenchymal
Polymyositis-dermatomyositis (PM-DM) involvement, may progress to honeycombing (less
Dermatomyositis-polymyositis (DM-PM) often)
Dermato-polymyositis (DPM) Interstitial thickening, predominantly lower lungs
Diffuse mixed alveolar-interstitial pattern
Definitions o Aspiration
Idiopathic, inflammatory, immune mediated Appearance depends on nature of aspirate
myopathic disorder with multiple systemic Lobar or segmental consolidation
manifestations Non-resolving opacities, focal or mass-like
Other
o Soft tissue calcifications
IIMAGING FINDINGS Often over bony prominences
More common in younger patients
General Features
Best diagnostic clue: Patchy subpleural consolidation CT Findings
in the setting of reduced lung volumes Lungs
o Ground-glass opacities
Radiographic Findings Early
10% have normal chest radiographs Characteristically bilateral, symmetric, basal fields
Lung volumes reduced Suggestive of active inflammation, potential for
o Elevated hemidiaphragms due to respiratory muscle reversibility with treatment
weakness, atelectasis o Consolidation

DDx: Bibasilar Interstitial Lung Diseases

Scleroderma Lupus Erythematosus

 POLYMYOSITIS - DERMATOMYOSITIS, PULMONARY

Key Facts
Terminology Hypersensitivity Pneumonitis
Idiopathic, inflammatory, immune mediated Asbestosis
myopathic disorder with multiple systemic Inhalational Injury
manifestations Rheumatoid Arthritis
Sjogren Syndrome
Imaging Findings Idiopathic Pulmonary Fibrosis 2
Best diagnostic clue: Patchy subpleural consolidation
Pathology 31
in the setting of reduced lung volumes
Elevated hemidiaphragms due to respiratory muscle Most likely precipitant appears to be a viral cause in a
weakness, atelectasis genetically susceptible individual
Interstitial thickening, predominantly lower lungs Clinical Issues
Soft tissue calcifications No established association between interstitial lung
Whole body turbo STIR to demonstrate soft tissue disease and extent of muscle or skin findings
inflammatory burden Aspiration pneumonia secondary to pharyngeal and
Top Differential Diagnoses esophageal myopathy in 15-20%
Drug Toxicity

Patchy, subpleural I DIFFERENTIAL DIAGNOSIS

Characteristically bilateral, symmetric, basilar lung
zones Drug Toxicity
May improve with treatment or progress to Review drug history
honeycombing (less often) Commonly chemotherapy drugs (Bleomycin,
o Reticular opacities Cyclophosphamide, Nitrosureas, etc.)
Parenchymal bands Can result in any pattern seen in PM-OM
Irregular bronchovascular thickening
Architectural distortion Hypersensitivity Pneumonitis
o Post treatment Exposure to known antigens
Residual bibasilar linear densities common Diffuse ground-glass opacities or centrilobular nodules
Often expiratory lobular air trapping
MR Findings
Whole body turbo STIR to demonstrate soft tissue Asbestosis
inflammatory burden Occupational history, insulation workers
Musculature Basilar reticular interstitial thickening
o Signal intensity abnormalities due to inflammation, Usually have pleural plaques (80%)
edema, scarring
o Symmetric involvement Inhalationallnjury
Proximal lower limb girdle, early Occupational history, anhydrous ammonia, silo fillers
Progression to proximal upper limb girdle, neck disease, etc.
flexors, pharyngeal muscles Cryptogenic organizing pneumonia pattern, early
Facial muscles typically spared Occasional constrictive bronchiolitis pattern, late
o Images may be used to guide muscle biopsy Rheumatoid Arthritis
Fluoroscopic Findings Erosive skeletal changes
Esophagram Basilar subpleural reticular interstitial lung disease
o Upper esophagus predominantly involved Sjogren Syndrome
o Similar to scleroderma, may result in reflux or
Often have sicca syndrome
aspiration
Lymphocytic interstitial pneumonia (LIP)
o Obstruction due to stricture or scarring, late
May have thin-walled cysts
Imaging Recommendations
Idiopathic Pulmonary Fibrosis
Best imaging tool
Subpleural honeycombing - basilar preferential
o CT: Affects patient prognosis by demonstrating
involvement
extent of pulmonary involvement, chronicity of
disease process, and response to treatment
o MR imaging useful to demonstrate areas of muscular
involvement
I PATHOLOGY
General Features
Etiology
o Presumed to be an auto-immune disease secondary
to defective cellular immunity
 POLYMYOSITIS - DERMATOMYOSITIS,
o Most likely precipitant appears to be a viral cause in
a genetically susceptible individual
Epidemiology: 5-10 new cases of
polymyositis-dermatomyositis per 100,000 people
yearly
Microscopic Features
2 Histology has prognostic significance and thought to
correlate with clinical presentation
32 Four commonly described histopathologic patterns
o Nonspecific interstitial pneumonia (NSIP), most
common
Cellular type often treatment-responsive,
consistent with acute-subacute presentation of
pulmonary disease
Fibrosing type less often treatment-responsive,
consistent with subacute to chronic presentation
o Cryptogenic organizing pneumonia (COP)
Often treatment-responsive, consistent with
acute-subacute onset of pulmonary manifestations
o Usual interstitial pneumonia (U1P)
Poor response to treatment, consistent with
chronic presentation
o Diffuse alveolar damage (DAD)
Very poor prognosis, consistent with acute
presentation
ICLINICALISSUES
Presentation
Most common signs/symptoms
o No established association between interstitial lung
disease and extent of muscle or skin findings
o Pulmonary manifestations in 5-50%
Precede muscle or skin disease in 33%
o Interstitial lung disease presents in one of three
patterns
Acute-subacute: Fever, rapidly progressive
dyspnea, lung infiltrates and hypoxemia within
one month of onset
Chronic: Insidious onset of dyspnea,
non-productive cough, weight loss
Asymptomatic: Abnormal chest radiograph or
reduced diffusion capacity of lungs for carbon
monoxide (DLCO) on pulmonary function testing
without respiratory symptoms
o Pulmonary manifestations due to myopathy
Inability to maximally inspire leading to
hypostatic pneumonia
Atelectasis secondary to bronchial mucous
plugging
Restrictive defect on pulmonary function testing
Hypercapnic respiratory failure occurs in less than
5%
Aspiration pneumonia secondary to pharyngeal
and esophageal myopathy in 15-20%
Other signs/symptoms
o Polymyositis
Subacute to acute onset proximal muscle weakness
and myalgias with general sparing of facial
muscles
Weakness is painless in 66%
PULMONARY
o Dermatomyositis
Violaceous heliotrope rash over anterior edge of
upper eyelids
Gottron sign: Bluish red plaques on posterior
knuckles
o Cardiac abnormalities in 70%, may precede skin and
muscle manifestations by up to 3 years
Dilated cardiomyopathy, congestive heart failure
Atrial and ventricular arrhythmias, conduction
disturbances
o Laboratory findings
Increased serum creatinine kinase
Serum Anti-lol antibodies (against cellular
amino-acyl t-RNA synthetase) in 25-35%
Serum ANA positive in 30%
Diagnostic considerations
o Association with other collagen vascular diseases
Scleroderma, lupus, rheumatoid arthritis, Sjogren
syndrome
o Beware of associated malignancy
Diagnosed concurrently or within one year in
10-20%
Cancers of the breast, lung, ovary and stomach
most common
Always examine breasts for occult cancer when
doing chest CT
Demographics
Age: Any age, 30-60 years of age most common
Gender: Women affected twice as often
Ethnicity: Three to five times more common in
African-Americans
Natural History & Prognosis
Pulmonary involvement adversely affects survival
Survival after diagnosis of interstitial lung disease
ranges from: 72-84% at one year, 34-77% at five years,
42-85% at 10 years
Factors predictive of more favorable prognosis
o Younger age (under 50 years) at presentation
o Relatively acute-subacute onset
o Histologic findings of COP or NSIP of
predominantly cellular type
Factors predictive of poor prognosis
o Acute deterioration in respiratory function
o Histologic finding of DAD
Most common cause of death respiratory failure
o Cardiovascular complications and cancer also
common causes of mortality
Treatment
Corticosteroids, variable response
I SELECTED REFERENCES
1. Bonnefoy 0 et al: Serial chest CT findings in interstitial
lung disease associated with polymyositis-dermatomyositis.
Eur J Radiol. 49(3):235-244, 2004
2. Arakawa H et al: Nonspecific interstitial pneumonia
associated with polymyositis and dermatomyositis: serial
high-resolution CT findings and functional correlation.
Chest 123(4):1096-1103, 2003
 POLYMYOSITIS DERMATOMYOSITIS, PULMONARY

I IMAGE GAllERY

(Left) Axial H RCT shows

extensive bibasilar traction
bronchiectasis (arrows) and 2
fibrosis in a patient with
polymyositis. NS/P paltern at
histology. (Right) Frontal 33
radiograph shows scattered
interstitial changes in a
patient diagnosed with
polymyositis.

(Left) Axial CECT

corresponding to previous
chest radiograph shows
scattered areas of subpleural
fibrosis (arrows) in the lower
lobes. (Right) Coronal NECT
reconstruction shows (ocal
areas of ground-glass
opacities and linear fibrosis
(arrows) in both mid to
lower lung zones in a patient
with dermatomyositis.

Typical
(Left) Axial H RCT shows
extensive bibasilar fibrosis
and early honeycombing
(arrows) in a
dermatomyositis patient.
(Right) Axial CECT shows a
spiculated left upper lobe
pulmonary nodule (arrow) in
a patient with
dermatomyositis, Patient was
subsequently diagnosed with
adenocarcinoma of the lung.
 NONSPECIFIC INTERSTITIAL PNEUMONITIS

2
34

Coronal maximum intensity projection (MIP) shows Axial H ReT shows bilateral patchy ground-glass
8round-glass opacities (arrows), bronchia/ectasis opacities (arrows). Open lung biopsy showed NSIP.
(curved arrow) and relicular opacities (open arrow) in a
patient with scleroderma and NSIP.

ITERMINOLOGY Radiographic Findings

Radiography
Abbreviations and Synonyms o Radiography: Nonspecific
Idiopathic interstitial pneumonia o Abnormal, 90%
Nonspecific interstitial pneumonia (NSIP) o Vague bilateral opacities
Definitions Vague airspace opacities
First described by Katzenstein in 1994 Interstitial opacities
For cases of idiopathic interstitial pneumonias that Mixed vague airspace and interstitial opacities
could not be classified o Mid and lower lungs
Debate regarding whether NSIP represents a "true" CT Findings
entity
NECT
o Bilateral patchy areas of ground-glass attenuation
(65%)
IIMAGING FINDINGS o Bilateral patchy areas of ground-glass attenuation
General Features with (35%)
Areas of consolidation, uncommon
Best diagnostic clue: HRCT: Bilateral patchy subpleural
Irregular lines
ground-glass opacity
Thickened bronchovascular bundles
Location
Bronchial dilation
o Basal predominance
Type 1 and 2
o Peribronchovascular or subpleural
o HRCT: Bilateral symmetrical ground-glass opacities
Size: Variable
Basal predominance
Morphology: Ground-glass opacities
Geographic distribution

DDx: Ground-Glass Opacities

Pulmonary Alveolar Proteinosis Desquamative Interstitial Pneumonia Idiopathic Interstitial Pneumonia

 NONSPECIFIC INTERSTITIAL PNEUMONITIS

Key Facts
Imaging Findings Pathology
Radiography: Nonspecific Type 1: Cellular interstitial pneumonia and relatively
HRCT: Bilateral symmetrical ground-glass opacities little fibrosis
Basal predominance Type 2: Cellular interstitial pneumonia and a
significant amount of admixed fibrosis

Crazy-paving appearance
Bronchiolectasis, out of proportion to adjacent lung Type 3: Predominant fibrosis 2
disease
Honeycombing, rare initially
Clinical Issues 35
Younger age than patients with VIP
Top Differential Diagnoses Fibrotic NSlp, worse prognosis than cellular NSIP
Desquamative Interstitial Pneumonia (DIP) Diagnosis: Open lung biopsy
Idiopathic Pulmonary Fibrosis (IPF) Steroids
Cryptogenic Organizing Pneumonia (COP)
Pulmonary Alveolar Proteinosis (PAP)
Diagnostic Checklist
Hypersensitivity Pneumonia More favorable prognosis with NSIP than with IPF
Sarcoidosis
.

Subpleural distribution, less common

o Crazy-paving appearance
Bilateral patchy areas of ground-glass attenuation
Intralobular lines in both the central and
peripheral lung
Interlobular septal thickening, minimal
o Irregular areas of consolidation
o Bronchiectasis, out of proportion to adjacent lung
disease
o Bronchiolectasis, out of proportion to adjacent lung
disease
o Bronchovascular bundles thickening
o Honeycombing, rare initially
Type 3: Predominantly fibrotic
o Lobar volume loss
o Architectural distortion
o Irregular linear opacities
o Traction bronchiectasis
o Honeycombing
Imaging Recommendations
Best imaging tool: CT to characterize and to assess
extent of disease
Protocol advice: HRCT, supine and prone; may show
changes to suggest type 3 fibrotic disease
Cryptogenic Organizing Pneumonia (COP)
Ground-glass opacities and/or consolidation
Bronchovascular bundle thickening
Reticular interstitial opacities, uncommon
Bronchiectasis but no bronchiolectasis
Steroid responsive
Pulmonary Alveolar Proteinosis (PAP)
Similar HRCT appearance
Crazy-paving appearance
Ground-glass opacities
Equal prominence of intra and inter-lobular septal
thickening
Hypersensitivity Pneumonia
Geographic ground-glass opacities
Mosaic pattern of perfusion
o Due to small airways disease
o Expiratory HRCT: Air trapping
Sarcoidosis
Ground-glass opacities
Micronodules, nodules, beaded vessels and fissures,
common
Bronchovascular bundle thickening, common
Transbronchial biopsy: Non-caseating granulomas

I DIFFERENTIAL DIAGNOSIS lipoid Pneumonia

Desquamative Interstitial Pneumonia (DIP)
Smokers
HRCT: Similar appearance
o DIP may have cysts, < 2 cm
Steroid responsive
Idiopathic Pulmonary Fibrosis (IPF)
HRCT: Appearance resembles fibrotic NSIP
o Subpleural location, more common
o No open lung biopsy required, in most cases
Histopathology: Temporal inhomogeneity of lung
injury
Poorer prognosis than NSIP
Inhalation or aspiration of lipid (e.g., mineral oil)
CT: Opacities may have fat attenuation
HRCT: Crazy-paving appearance
o Mass-like opacities
o Airspace opacities resembling pneumonia
Mucinous Bronchioloalveolar Cell
Carcinoma
May have bronchorrhea
Ground-glass, airspace opacities
Progressive over time
 NONSPECIFIC INTERSTITIAL PNEUMONITIS
Range: 1 week to 5 years
I PATHOLOGY Other signs/symptoms
General Features o Pulmonary function tests
Restrictive ventilatory defect
Etiology
o Idiopathic Mild reduction FEV,
o Collagen vascular diseases Reduced diffusion capacity (DLco)
Extent of the disease with HRCT correlates
2 Systemic sclerosis
Rheumatoid arthritis, usual interstitial significantly with pulmonary function
pneumonitis (VIP) more frequent than NSIP Demographics
36
o Hypersensitivity pneumonia
Age
o Drug-induced lung disease o 40-50 years, mean age 49 years
Amiodarone Younger age than patients with VIP
Nitrofurantoin VIP, age 40-70 years
Gold salts Gender: M ; F
Methotrexate
Vincristine Natural History & Prognosis
Fludarabine Potentially reversible features
o Radiation toxicity o Ground-glass opacities
o Healing diffuse alveolar damage o Reticular opaci ties
Associated abnormalities: Not associated with cigarette o Traction bronchiectasis
smoking The greater proportion of fibrosis the worse the
prognosis
Gross Pathologic & Surgical Features o Even with fibrosis, better prognosis than VIP
Lacks histologic features of specific interstitial o Fibrotic NSIP, worse prognosis than cellular NSIP
pneumonia
NSIP
Spatially homogeneous alveolar wall thickening o Recover completely, 45%
caused by inflammation and/or fibrosis o Stable or improved, 42%
Temporal uniformity of the lung injury o Mortality, 11%
Spatial and temporal homogeneity, important in o Median survival, 13.5 years
distinguishing NSIP from VIP Important to distinguish NSIP from VIP
Microscopic Features o Better prognosis with NSIP
Varying degrees of interstitial inflammation and/or o Median survival, NSIP > 13 years; VIP 2.8 years
fibrosis Treatment
Three types Transbronchial biopsy may provide incorrect
o Type 1: Cellular interstitial pneumonia and diagnosis, e.g., COP
relatively little fibrosis Bronchioloalveolar lavage: Increased lymphocytes
o Type 2: Cellular interstitial pneumonia and a Diagnosis: Open lung biopsy
significant amount of admixed fibrosis Steroids
o Type 3: Predominant fibrosis
Type 1 and 2
o Interstitial infiltrate: Lymphocytes, plasma cells I DIAGNOSTIC CHECKLIST
o Hyperplasia alveolar lining cells
o Cryptogenic organizing pneumonia Consider
o Foamy cell collections in alveolar spaces Open lung biopsy when HRCT appearance is
o Microscopic honeycombing with mucin stasis consistent with NSIP
Type 3 o More favorable prognosis with NSIP than with IPF
o Dense interstitial collagen deposition
o Marked alveolar septal thickening
o Derangement of lung architecture I SELECTED REFERENCES
o Difficult to distinguish from VIP
1. Lynch DAet al: Idiopathic interstitial pneumonias: CT
features. Radiology. 236(1):10-21, 2005
2. EllisSM et al: Idiopathic interstitial pneumonias:
I CLINICAL ISSUES imaging-pathology correlation. Eur Radiol. 12(3):610-26,
2002
Presentation 3. Coche E et al: Non-specific interstitial pneumonia showing
Most common signs/symptoms a "crazy paving" pattern on high resolution CT. BrJ Radial.
o Gradual onset 74(878):189-91,2001
o Dyspnea, cough, malaise, fatigue, crackles 4. Nishiyama 0 et al: Serial high resolution CT findings in
o Anorexia, weight loss, 50% nonspecific interstitial pneumonia/ fibrosis. J Comput
AssistTomogr. 24:41-6, 2000
o Finger clubbing < 35%
o Fever, uncommon
o Present for 6-18 months prior to presentation
 NONSPECIFIC INTERSTITIAL PNEUMONITIS
I IMAGE GALLERY

(Left) Axial NECT shows

peripheral ground-glass
opacities (arrow) with fine
reticular interstitial opacities
(open arrow) and
2
bronchia/ectasis (curved
37
arrow). Biopsy proven NSII'
(Right) Axial HRCT in same
patient shows ground-glass
opacities, bronchiectasis,
bronchia/ectasis and
architectural distortion that
follow bronchovascular
bundles (arrows). Diagnosis:
NSIP

fLeft) Frontal radiograph

shows bilateral diffuse vague
opacities. Prominent
pulmonary arteries (arrows)
represent pulmonary artery
hypertension related to
scleroderma. (Right) Axial
HRCT in same patient shows
bronchiectasis,
bronchiofectasis (arrow),
and fine linear opacities
(curved arrow), out of
proportion for surrounding
lung disease. NSIP.

Typical
(Left) Axial prone HRCT in
same patient shows traction
bronchiectasis and
honeycombing (arrow) that
likely represents type 3 or
fibrotic NSIP. fRight) Axial
HRCT in a patient with
scleroderma and NSIP shows
mixed ground-glass and
"/ace-like reticular opacities
/I

(open arrows). Traction

bronchia/ectasis (arrows) out
of proportion to lung disease.
NSII'
 ASBESTOSIS
.
~~~
. , ),
2
38
II
(."-~
I/"
\1[/.,
: ''J'
.

/'
,

: ... " .... '.

.'
~

)'
~

l
....

\~~'
Axial graphic shows abnormal interstitial thickening
predominantly involving the periphery or the lower
lobes. Pleural plaques may be absent in up to 20% or
patients with asbestosis.
..~
Coronal H RCT shows fine peripheral interstitial fibrosis
rcurved arrows) most marked in the basilar peripheral
lung. Multiple calcified and noncalcified diaphragmatic
plaques (arrows).

o Peripheral lower zone predominance

[TERMINOLOGY o Irregular reticular or small nodular opacities
Abbreviations and Synonyms o "Shaggy" cardiac silhouette in advanced disease
Pneumoconiosis o International labor office (ILO) classification
compared to standard radiographs "B" reading
Definitions Asbestosis generally s, t, or u opacities
Interstitial lung disease due to the inhalation of o Late: Endstage honeycombing
asbestos fibers o Pleural plaques (25%)
Asbestosis is not isolated pleural plaques, (asbestos o Lung cancer: Lower zone predominance in contrast
exposure proper term) to the upper zone predominance in the general
population of smokers
o Progressive massive fibrosis extremely rare
I IMAGING FINDINGS CT Findings
General Features CECT
Best diagnostic clue: Basilar interstitial fibrosis and o Useful to differentiate lung nodules from pleural
pleural plaques plaques, round atelectasis and lung fibrosis
Location: Posterobasilar subpleural lung 10% of asbestos exposed workers screened by CT
Morphology: Fibrosis centered on respiratory for asbestosis will have a lung mass
bronchioles HRCT
o More sensitive than chest radiograph
Radiographic Findings o Screening asbestos exposed workers
Radiography
o May be normal (10-20%)

DDx: Peripheral Basilar Fibrosis

Idiopathic Pulmonary Fibrosis Scleroderma

 ASBESTOSIS

Key Facts
Terminology Scleroderma
Interstitial lung disease due to the inhalation of Rheumatoid Arthritis
asbestos fibers Hypersensitivity Pneumonitis
Lymphangitic Tumor
Imaging Findings Cytotoxic Drug Reaction
Morphology: Fibrosis centered on respiratory
Pathology
2
bronchioles
Lung cancer: Lower zone predominance in contrast to Fibrosis + asbestos bodies = asbestosis 39
the upper zone predominance in the general Retention: Long thin fibers> short, thick fibers
population of smokers Fibrosis associated with> 1 million fibers/gm lung
Subpleural curvilinear lines early sign tissue
Protocol advice: Prone scans helps to differentiate Clinical Issues
true interstitial lung disease from gravity-related
Latent period 20-30 years
physiology Does not regress, slowly progresses
Top Differential Diagnoses High proportion die of lung cancer (1 in 4)
Idiopathic Pulmonary Fibrosis

Of those with clinical asbestosis: Chest Traction bronchiolectasis more common (less
radiographs abnormal in 80%; HRCT abnormal in common in asbestosis)
96% Band-like opacities less common
33% with neither clinical or chest radiographic Mosaic perfusion less common (no airways
evidence of asbestosis abnormal at HRCT obstruction)
False negatives for early asbestosis in 25%
o Peripheral posterobasilar interlobular septal Scleroderma
thickening (short lines) and centrilobular nodules or No plaques, however, pleural thickening and
branching opacities most common abnormality pseudoplaques common
o Subpleural curvilinear lines early sign Dilated esophagus
Parallel chest wall within 1 cm of the pleura, Fine reticular interstitial thickening similar
length 5-10 cm distribution
Represent peribronchial confluent fibrosis or Rheumatoid Arthritis
atelectasis associated with obstructed respiratory
No plaques
bronchioles
Joint erosions
o Parenchymal bands project from the pleura
Interstitial thickening similar
2-5 cm long
Fibrosis along interlobular septa or Hypersensitivity Pneumonitis
bronchovascular bundles No plaques
o Ground-glass opacities nonspecific Less severe in costophrenic angles, more severe mid
o Small airways obstruction and upper lungs
Mosaic perfusion Mosaic perfusion from air trapping more common
Traction bronchiolectasis uncommon
o Pleural plaques 80% lymphangitic Tumor
Asymmetric distribution
Nuclear Medicine Findings Nodular thickening septa and core bronchovascular
Ga-67 Scintigraphy: Usually positive in asbestosis, structures
rarely used today No plaques but pleural effusion common
Imaging Recommendations Cytotoxic Drug Reaction
Best imaging tool: HRCT to characterize lung and Prototypical drug: Methotrexate
pleural disease No plaques
Protocol advice: Prone scans helps to differentiate true Interstitial thickening similar
interstitial lung disease from gravity-related
physiology
I PATHOLOGY
I DIFFERENTIAL DIAGNOSIS General Features
General path comments
Idiopathic Pulmonary Fibrosis o Fibrous mineral properties: Heat resistant, high
No pleural plaques tensile strength, flexible, durable
Ground-glass opacities more common o Fibrosis + asbestos bodies = asbestosis
o 2 types of fibers: Serpentine and amphibole
 ASBESTOSIS
o Serpentine (chrysotile or white asbestos, 90%
commercial asbestos)
Curly, wavy fiber
Long (> 100 ~m)
Diameter (20-40 ~m) I CLINICAL ISSUES
o Amphibole
Crocidolite (blue asbestos), amosite (brown Presentation
2 asbestos), anthophyllite, tremolite, actinolite
Straight, rigid fiber
40 Aspect ratio (length/width) > 3: 1
o Retention: Long thin fibers> short, thick fibers
o Asbestos (ferruginous) bodies
Hemosiderin-coated fiber (mostly amphibole)
Incompletely phagocytized by macrophages
Not pathognomonic for asbestosis
Coated fibers fewer than uncoated fibers
Not correlated with fibrosis .
o Pathophysiology
Increased deposition of fibers in the lower lung
zones due to gravitational ventilatory gradient
Fibers deposit in the respiratory bronchioles
No lymphatic removal, largest and most harmful
asbestos fibers too large to be removed by
macrophages
Epidemiology
o Long term exposure to asbestos fibers: Asbestos
mills, insulation, shipyards, construction
o Dose-response relationship
Usually takes high dust concentrations
Typically 20 years following initial exposure but
could be as short as 3 years
1% risk of asbestosis after cumulative dose of 10
fiber-year/ml tissue
Associated abnormalities
o Asbestos related pleural disease
Benign exudative pleural effusions
Pleural plaques
Diffuse pleural thickening Treatment
o Round atelectasis
o Malignant mesothelioma
Gross Pathologic & Surgical Features
Coarse honeycombing and volume loss particularly of
lower lobes
Transbronchial biopsy yields poor and of little value
Microscopic Features
Early fibrosis: Centered on respiratory bronchioles and I DIAGNOSTIC
spreads centrifugally
o Important pathologic difference from idiopathic
interstitial fibrosis where the fibrosis generally
distorts these airways (traction bronchiolectasis)
Patchy distribution, severe honeycombing uncommon
Fibrosis associated with> 1 million fibers/gm lung
tissue
Asbestos or ferruginous bodies: Fibers coated with
ferritin
o May be retrieved with bronchoalveolar lavage (BAL)
Staging, Grading or Classification Criteria
American College of Pathology
o Grade 1: Fibrosis in wall respiratory bronchiole
o Grade 2 & 3: Extension into alveoli
o Grade 4: Alveolar and septal fibrosis with spaces
larger than alveoli (honeycombing)
Most common signs/symptoms
o Gradual onset dyspnea on exertion, nonproductive
cough
o Rales (end-inspiratory crackles)
o Clubbing in 1/3rd
o American Thoracic Society (ATS) general criteria for
diagnosis asbestosis (2003)
Evidence of structural pathology consistent with
asbestosis as documented by imaging or histology
Evidence of causation as documented by
occupational and exposure history (includes
pleural plaques and asbestos bodies)
Exclusion of alternative plausible causes for the
findings
Other signs/symptoms
o Pulmonary function tests
Restriction and decreased diffusion capacity
Decreased small airway flow rates
Demographics
Gender
o Men due to occupational exposure
Housewives (nonoccupational exposure) too
Natural History & Prognosis
Latent period 20-30 years
Does not regress, slowly progresses
Asbestos carcinogen: Multiplicative risk factor for lung
cancer
o High proportion die of lung cancer (1 in 4)
No treatment
Smoking cessation
Consider lung cancer screening
Control and regulation of asbestos in the workplace
Eligible for worker's compensation
o Pathologic tissue not required to gain compensation
CHECKLIST
Consider
May be reportable disease in some states
Image Interpretation Pearls
Be alert to pleural plaques in any patient with basilar
interstitial lung disease, conversely fibrosis without
plaques does not rule out asbestosis
I SELECTED REFERENCES
1. Akira M et al: High-resolution CT of asbestosis and
idiopathic pulmonary fibrosis. AJRAm J Roentgenol.
181(1):163-9,2003
2. Roach HD et al: Asbestos: when the dust settles an imaging
review of asbestos-related disease. Radiographies. 22 Spec
No(SI67-84,2002
 ASBESTOSIS

I IMAGE GALLERY

(Left) Frontal radiograph

shows mild basilar interstitial
thickening in the typical
railroad worker with
asbestosis. No pleural
2
plaques. (Right) Frontal
41
radiograph shows more
severe interstitial thickening
in asbestosis. "Shaggyll heart
borders. Calcified
diaphragmatic plaque
(arrow).

Typical
(Left) Axial NECT shows
ground-glass opacities and
reticular interstitial
thickening at the right base.
Parenchymal band anteriorly
(curved arrow). Tiny
calcified pleural plaque
(arrow). (Right) Axial HRCT
in same patient. Reticular
interstitial thickening better
demonstrated. Traction
bronchiectasis (arrow).
Parenchymal band (curved
arrow). Asymmetrical
involvement not unusual.

(Left) Axial HRCT shows

subpleural reticular lines in
asbestosis. Short lines
(curved arrow). Bilateral
calcified pleural plaques
(arrows). (Right) Axial HRCT
shows long parenchymal
bands (arrow) and
subpleural wedge-shaped
opacities (curved arrow) in
the base of the lung in
asbestosis.
 SiliCOSIS - COAL WORKER PNEUMOCONIOSIS

2
42

Frontal radiograph shows small nodules more profuse in Frontal radiograph 20 years later. Simple
the upper lobes. Occupational history of foundry work pneumoconiosis has evolved into progressive massive
for the last 20 years. fibrosis (PMF). Upper lobes volume loss. Number of
nodules has decreased with PMF.

I TERMINOlOGY I IMAGING FINDINGS

Abbreviations and Synonyms
Simple pneumoconiosis, complicated pneumoconiosis,
progressive massive fibrosis, anthracosis,
anthracosilicosis
Definitions
Lung disease due to the inhalation of inorganic
mineral dusts
Simple or chronic pneumoconiosis: Micronodules < 1
cm, more profuse in upper lung zones, often have
hilar and mediastinal lymphadenopathy, develops
more than 10 years after long term occupational
exposure
Complicated pneumoconiosis known as progressive
massive fibrosis (PM F): Aggregation of nodules into
large masses larger than 1 cm in diameter, evolves
from simple or chronic pneumoconiosis
Acute silicoproteinosis: Resembles alveolar proteinosis,
develops within weeks after heavy dust exposure
Caplan syndrome: Coal worker pneumoconiosis
(CWP) + rheumatoid arthritis + necrobiotic nodules
General Features
Best diagnostic clue: Micronodular interstitial
thickening in upper lung zones
Location
o Rounded dusts predominately affect the upper lung
zones
o Coal dust accumulates around respiratory
bronchioles (coal dust macule)
o Silica accumulates along lymphatics in the
centriacinar portion of the lobule and the lobule
periphery
Size: Nodules range from 1-3 mm in diameter
Radiographic Findings
Radiography
o Findings seen 10-20 years after exposure
o Silicosis and CWP similar, lung disease usually less
severe in CWP
o Simple pneumoconiosis
1-3 mm round nodules, nodules may calcify
(international labor office classification p, q, or r)

DDx: Silicosis - Coal Workers Pneumoconiosis

Sarcoid Langerhans Granulomatosis Farmer's Lung

 SiliCOSIS - COAL WORKER PNEUMOCONIOSIS

Key Facts
Terminology Top Differential Diagnoses
Simple or chronic pneumoconiosis: Micronodules < 1 Sarcoidosis
em, more profuse in upper lung zones, often have Tuberculosis (TB)
hilar and mediastinal lymphadenopathy, develops Langerhans Cell Histiocytosis
more than 10 years after long term occupational Hypersensitivity Pneumonitis
exposure Takosis 2
Complicated pneumoconiosis known as progressive
massive fibrosis (PMF): Aggregation of nodules into Pathology 43
large masses larger than 1 em in diameter, evolves Silica more fibrogenic than coal
from simple or chronic pneumoconiosis Primarily involves upper lung zones, PMF results in
Acute silicoproteinosis: Resembles alveolar end-stage lung
proteinosis, develops within weeks after heavy dust Clinical Issues
exposure
Typical occupations: Sandblasting, quarries, mining,
Caplan syndrome: Coal worker pneumoconiosis
glassblowing, pottery
(CWP) + rheumatoid arthritis + necrobiotic nodules

Predominantly affects the upper lung zones, o Micronodules < 7 mm in centrilobular and
particularly the dorsal aspects subpleural distribution
Hilar and mediastinal lymphadenopathy, eggshell More profuse in dorsal aspect of upper lobes, right
calcification (5%) > left
o Complicated pneumoconiosis or progressive massive Silicotic nodules tend to be more sharply defined
fibrosis (PMF) than CWP
Nodules coalesce and are> 1 em in diameter Calcification 3%
Usually bilateral, right> left, located in dorsal Chains of subpleural nodules produces
aspect of lung pseudoplaques
PMF may be lens shaped (wide PA and narrow o Intralobular or interlobular reticular thickening
lateral view) uncommon
Lateral margin coarsely parallels the chest wall o Aggregation of nodules into PMF more readily
and is sharply defined, medial inner edge less detected
well-defined Irregularly elliptical in shape with emphysema
Overall profusion of nodules decreases due to peripheral to mass
aggregation into PMF Masses larger than 4 em nearly all contains areas
May have foci of amorphous calcification of low attenuation due to necrosis
May cavitate
Migrates centrally with time Imaging Recommendations
Lung distal to PMF emphysematous: Risk for Best imaging tool: HRCT more sensitive for detection
pneumothorax lung disease and detection PMF
o Acute silicoproteinosis Protocol advice: ILO 12-point classification (B reading)
Central butterfiy alveolar pattern with air for standardizing the profusion and severity of
bronchograms pneumoconiosis
Hilar/mediastinallymphadenopathy common
Progresses rapidly over months
Later evolves into fibrosis with severe architectural I DIFFERENTIAL DIAGNOSIS
distortion, bullae, pneumothorax
o Caplan syndrome Sarcoidosis
Multiple large nodules usually less than 5 em in No occupational exposure, PMF less likely
diameter (may cavitate or calcify) Nodules tend to cluster (galaxy sign)
Nodules peripheral and subpleural in location, Tuberculosis (TB)
when cavitate may produce pneumothorax Nodules do not aggregate into mass, profusion
May evolve quickly, occasionally disappear nodules less
Nodules enlarge faster than silicotic PMF
Bone changes of rheumatoid arthritis: Humeral or Langerhans Cell Histiocytosis
clavicular erosions, lung changes may precede Less likely subpleural nodules, no PMF
bone disease Cysts, often irregular in shape common, not seen with
pneumoconiosis
CT Findings
HRCT Hypersensitivity Pneumonitis
o More sensitive than chest radiography for lung Less likely subpleural nodules, no PMF, primarily
disease midlung
 SiliCOSIS - COAL WORKER PNEUMOCONIOSIS
Small airway trapping common at HRCT, less likely o Caplan syndrome
with pneumoconiosis Clinical features of rheumatoid arthritis
o Pulmonary function tests
Takosis Simple pneumoconiosis: Usually normal
Nodules generally smaller, < 1 mm in diameter Complicated pneumoconiosis: Decreased diffusion
Pan acinar emphysema more common lower lobes capacity, decreased lung volumes, restrictive
defect
2 I PATHOLOGY
Often have mixed obstruction and restriction due
to the combined effects of smoking and interstitial
44 fibrosis
General Features Functional impairment more closely associated
General path comments with degree of emphysema (as determined by CT)
o Silica more fibrogenic than coal then profusion of nodules
o Increased risk of tuberculosis o Advanced disease develop cor pulmonale
Etiology Clinical Profile
o Inhalation of silica dust, silicon dioxide (Si02) or o Typical occupations: Sandblasting, quarries, mining,
coal, dust deposited in respiratory bronchioles, glassblowing, pottery
removed by macrophages and lymphatics o Coal mines usually contain silica (most common
o Removal slow process, half-time of single dust element, earth's crust)
burden on the order of 100 days o Acute silicoproteinosis
Epidemiology Massive exposure to silica dust, usually seen in
o Risk related to both dose (intensity of exposure) and sandblasters
time (length of exposure)
o Up to 15% of miners can progress to interstitial Demographics
fibrosis Age: Simple and complicated pneumoconiosis rare
under age 50
Gross Pathologic & Surgical Features Gender: More common in males due to occupational
Primarily involves upper lung zones, PMF results in risk
end-stage lung
Silicotic lung content generally 2-3% (up to 20%), Natural History & Prognosis
normal silica content 0.1% of dried lung Usually requires> 20 years exposure, silicosis
progressive even after removal of dust, CWP usually
Microscopic Features not progressive
Silica Simple pneumoconiosis, normal longevity
o Silica particles centered within concentric lamellae Complicated PMF, death from respiratory failure,
of collagen located along bronchioles, small vessels, pneumothorax, TB
and lymphatics Silicoproteinosis: Death within 2-3 years
o Birefringent silicate crystals (1-3 ~) in nodules by Debatable slight increased risk of lung cancer
polarized microscopy
o Silica-laden macrophages carry particles to hilar and Treatment
mediastinal nodes and form granulomas Prevention: Respirators in dusty environment, dust
o Silicoproteinosis contains high concentrations of control to reduce ambient dust concentrations
silica, alveolar filled by lipoproteinaceous material, Removal from work environment or transfer to less
similar to alveolar proteinosis dusty environment
Coal Smoking cessation
o Coal macule: Stellate collection of macro phages No specific treatment for pneumoconiosis available
containing black particles, (1-5 1') in terminal and At risk for TB; cavitation in PMF requires culture
respiratory bronchioles and pleural lymphatics, little o TB skin tests important
or no collagen
o Macule surrounded by focal emphysema
I DIAGNOSTIC CHECKLIST
ICLINICAL ISSUES Consider
Occupational history in any patient with upper lobe
Presentation nodular interstitial lung disease
Most common signs/symptoms
o Symptoms
None with simple silicosis I SELECTED REFERENCES
Miners commonly smoke and have bronchitis or 1. Remy-Jardin M et al: Coal worker's pneumoconiosis: CT
emphysema assessment in exposed workers and correlation with
Cough, dyspnea, increased sputum in complicated radiographic findings. Radiology. 177:363-71, 1990
disease 2. Bergin C) et al: CT in silicosis: Correlation with plain films
Black sputum in coal workers and pulmonary function tests. AJR. 146:477-83, 1986
Other signs/symptoms
 SiliCOSIS COAL WORKER PNEUMOCONIOSIS
I IMAGE GAllERY

(Left) Frontal radiograph

shows earfy PMF Lateral
margin parallels chest wall 2
and is sharply defined
(arrow). Background of
simple pneumoconiosis. As 45
PMF progresses, the number
of nodules will decrease.
(Right) Frontal radiograph
shows numerous 5 mm
nodules, primarily in upper
lobes with early aggregation
into PMF (arrows). Enlarged
hilar lymph nodes with
eggshell calcification (open
arrow).

Typical
(Left) Axial HRCT shows
clusters of 5 mm centriacinar
and subpleural nodules.
Nodules concentrated in the
dorsal aspect of the lung.
Foundry worker with
silicosis. (Right) Axial HRCT
shows nodules aggregating
into PMF (open arrows) in
the dorsal aspect o( the lung.
PMF masses elliptical in
shape. Numerous
pseudoplaques from clusters
o( nodules (arrows).

Variant
(Left) Axial HRCT shows
centriacinar micronodu/es
(arrow). Subpleural
pseudoplaque (open arrow)
from aggregation of nodules.
No PMF or architectural
distortion. Coal workers
pneumoconiosis. (Right)
Axial NECT shows several
variable sized subpleural
nodules, one of which is
cavitated (arrow). Large right
pneumothorax. Caplan
syndrome, rheumatoid
nodules.
 BERYLLIOSIS

2
46

Graphic shows typical features of berylliosis. Frontal radiograph shows enlarged hila (arrows) and
Symmetrically enlarged hilar and mediastinal lymph nodular interstitial thickening of the mid lungs. Forty
nodes. Diffuse nodular interstitial thickening, more years earlier patient had worked in light bulb factory.
pronounced in the upper lung zones. Diagnosis:Berylliosis.

Noncardiogenic pulmonary edema within 72 hr of

I TERMINOlOGY exposure
Abbreviations and Synonyms Slowly resolves within 1 to 4 weeks, 10% develop
Acute berylliosis, chronic berylliosis, Salem sarcoid chronic disease
o Subacute
Definitions Onset in weeks with lower exposures
Strong lightweight element with a high melting point, Findings overlap between acute onset (to a lesser
used in alloys in wide variety of industries, inhalation degree) and chronic disease
causes 2 pulmonary syndromes: Acute chemical o Chronic
pneumonitis and chronic granulomatous lung disease 50% normal
Symmetric bilateral hilar adenopathy associated
with lung disease, adenopathy not an isolated
IIMAGING FINDINGS finding (33%)
Diffuse nodular pattern [international labor office
General Features (ILO) category p or qJ in 75% profusion typically
Best diagnostic clue: Sarcoid pattern in patient with 111 to 2/2
exposure to beryllium Reticular pattern (ILO category sand t) in 25%
Location: Primarily midlung with tendency to upper profusion typically 1/1 to 2/2
lobe fibrosis with chronic fibrosis Lung nodules and lymph nodes may calcify
including egg-shell calcification
Radiographic Findings 10% develop large bullae
Radiography 10% upper zone pleural thickening (maybe due to
o Acute aggregation of subpleural nodules, seen in those
Requires overwhelming exposure with extensive lung disease

DDx: Berylliosis

Sarcoid Langerhans Granulomatosis

 BERYLLIOSIS

Key Facts
Terminology Top Differential Diagnoses
Strong lightweight element with a high melting Sarcoidosis
point, used in alloys in wide variety of industries, Tuberculosis
inhalation causes 2 pulmonary syndromes: Acute Langerhans Cell Granulomatosis
chemical pneumonitis and chronic granulomatous Silicosis
lung disease Hypersensitivity Pneumonitis 2
Idiopathic Pulmonary Fibrosis
Imaging Findings 47
Best diagnostic clue: Sarcoid pattern in patient with Clinical Issues
exposure to beryllium History of beryllium exposure, latent period 1 month
Symmetric bilateral hilar adenopathy associated with to 40 years (average 10-15 years)
lung disease, adenopathy not an isolated finding Positive blood or bronchoalveolar lavage beryllium
(33%) lymphocyte proliferation test
Nodules (65%) > ground-glass opacities (55%) > septal Noncaseating granulomas on lung biopsy
lines (50%) 10% of patients with acute disease go on to develop
Resolution ground-glass opacities replaced by chronic disease
microcysts or septal lines

May develop chronic fibrosis and volume loss Multiorgan potential

predominantly of upper lobes o May involve eyes (opthamologist), absent with
Spontaneous pneumothorax 10% beryllium
Enlarged pulmonary arteries from pulmonary o Cystic bone lesions not seen with beryllium
hypertension in end-stage disease
Tuberculosis
CT Findings Nodules and cavities in dorsal aspect upper lobes
HRCT Bronchogenic spread to axillary sub-segments upper
o Normal in 25% with proven disease lobes and superior segments lower lobes
o Identical pattern to sarcoid
o Nodules (65%) > ground-glass opacities (55%) > Langerhans Cell Granulomatosis
septal lines (50%) Nodules and or cysts primarily upper lobes in
o Nodules may aggregate into progressive massive centrilobular location
fibrosis (PMF) (5%) Also typically upper lobes but chronic disease tends to
o Thickened nodular bronchovascular bundles and have bizarre shaped cysts
nodular interlobular septal thickening (50%) Silicosis
o Hilar or mediastinal adenopathy (40%), always
Different occupational history
associated with lung disease
Nodules also centered on lymphatics with aggregation
Nodes: May have diffuse or egg-shell calcification
into progressive massive fibrosis
o Honeycombing in advanced disease (5%) typically
Also typically upper lobe
worse in upper lung zones
Nodules and nodes may also calcify
o Upper zonal pleural thickening due to
pseudoplaques (aggregation of subpleural nodules) Hypersensitivity Pneumonitis
o Treatment Mosaic attenuation from small airways obstruction
Ground-glass opacities resolve over 3 month May have upper lobe fibrosis with chronic disease
period No adenopathy
Resolution ground-glass opacities replaced by
microcysts or septal lines Idiopathic Pulmonary Fibrosis
Honeycombing lower lobe and subpleural
Imaging Recommendations predominant
Best imaging tool: HRCT more sensitive and better
characterizes lung disease
I PATHOLOGY
I DIFFERENTIAL DIAGNOSIS General Features
General path comments
Sarcoidosis o Pulmonary half-life weeks to 6 months, some
Hilar adenopathy may occur without lung disease beryllium may be retained for years
In sarcoidosis adenopathy typically regresses as lung > 80% have greater than 0.02 l-lgberyllium per
disease worsens; not seen with beryllium gram dried lung
Diffuse ground-glass opacities less common than o Beryllium also accumulates in bone, liver, and
berylliosis kidneys
May spontaneously resolve o Excreted by kidneys
 BERYLLIOSIS
Excretion more than 20 years after the last
exposure to beryllium
a Acute
Seen when respirable concentrations> 100 !Jm/m3
Pathology dose-dependent
Affect nasopharynx (septal perforation),
tracheobronchial tree (bronchitis), and lung
2 (diffuse alveolar damage)
a Chronic 2 subtypes
48 Moderate to marked lymphocytic infiltrate, poorly
to well formed granulomas, calcific inclusions
(80%)
Slight or absent lymphocytic infiltrate, well
formed granulomas, few or absent calcific
inclusions (20%): This pattern identical to sarcoid
Pathology dose-independent . 10% of patients with acute disease go on to develop
Genetics: Genotype HLA-DPb1 (Glu 69) marker for
susceptibility to disease
Etiology
a Delayed-type hypersensitivity reaction where
beryllium functions as a hapten leading to a
granulomatous reaction
a Generally inhaled but can be absorbed through the
skin
Epidemiology: 1-lS% of exposed persons develop
beryllium hypersensitivity and chronic disease
Gross Pathologic & Surgical Features
Distribution of infiltrate along lymphatics in
bronchovascular bundle, septa, and subpleural
locations
Microscopic Features
Noncaseating granulomas indistinguishable from
sarcoid granulomas in chronic disease
Diffuse alveolar damage in those with acute
pneumonitis, no granulomas
Gender: No preference
Natural History & Prognosis
Criteria for diagnosis
a History of beryllium exposure, latent period 1
month to 40 years (average 10-15 years)
o Positive blood or bronchoalveolar lavage beryllium
lymphocyte proliferation test
a Noncaseating granulomas on lung biopsy
High risk occupations
a Nuclear power, aerospace and electronics industries
a Used in X-ray tubes, rocket engines, ceramics,
computers, dental alloys
a Once major problem in manufacture of fluorescent
tubes, now no longer used
chronic disease
Progression relatively slow, survival of 15 to 20 years
common
Prognosis best for those with decreased diffusing
capacity, those with either restrictive or obstructive
patterns do worse
Treatment
Prevention
a Occupational Safety and Health Administration
standards: Peak level < 25 mcg/m3 and 8 hour
average maximum permissible level of 2 mcg/m3
a Neighborhood factory air not to exceed 0.01
mcg/m3
a Yearly chest radiographic surveillance
Removal from workplace environment, radiographic
abnormalities may improve if exposure ceases
Steroids and possibly methotrexate for symptomatic
disease, may relapse off therapy
Lung-transplantation for end-stage disease

I DIAGNOSTIC CHECKLIST
IClINICAL ISSUES
Consider
Presentation Occupational history in any patient with sarcoid-like
Most common signs/symptoms radiographic pattern
a Asymptomatic to respiratory failure
a Dyspnea most common symptom (95%)
a Cough, chest pain, arthralgia, fatigue, weight loss I SELECTED REFERENCES
Other signs/symptoms L Newman L5et al: Beryllium workers' health risks.J Occup
a Skin rash, poor wound healing, papular or vesicular Environ Hyg. 2(6):048-50, 2005
rash (itchy) 2. Infante PFet al: Beryllium exposure and chronic beryllium
a Lymphadenopathy, generalized disease. Lancet. 363(9407):415-6, 2004
a Hepatosplenomegaly (10%) 3. Fireman E et al: Misdiagnosis of sarcoidosis in patients with
a Uveitis, uveoparotid fever, cranial or peripheral chronic beryllium disease. Sarcoidosis Vase Oiffuse Lung
nerve involvement more common in sarcoidosis Ois. 20(2):144-8, 2003
4. Naccache JM et al: Ground-glass computed tomography
Clinical Profile pattern in chronic beryllium disease: pathologic
a Pulmonary function tests substratum and evolution. J Comput AssistTomogr.
Obstructive pattern in 40% 27(4):496-500, 2003
Restrictive pattern in 20% 5. Maier LA:Clinical approach to chronic beryllium disease
Diffusing capacity decreased in 15%, good marker and other nonpneumoconiotic interstitial lung diseases. J
for progression of disease Thorac Imaging. 17(4):273-84, 2002
a Lymphocytosis on bronchoalveolar lavage 6. Rossman MO: Chronic beryllium disease: a hypersensitivity
a 10% develop renal calculi from hypercalcemia disorder. Appl Occup Environ Hyg. 16(5):615-8,2001
7. Oaniloff EM et al: Observer variation and relationship of
Demographics computed tomography to severity of beryllium disease. Am
J Respir Crit Care Med. 155(6):2047-56, 1997
Age: Any age
 BERYLLIOSIS

I IMAGE GALLERY
Typical
(Left) Frontal radiograph
shows enlarged hilar lymph
nodes (arrows) and diffuse
nodular interstitial
2
thickening, larger nodules in
the upper lung zones. 49
Berylliosis identical pattern
to sarcoidosis. (Right) Frontal
radiograph shows enlarged
hilar lymph nodes (arrows),
diffuse nodular interstitial
pattern and focal
consolidation in the left
lower lobe (open arrow) in
patient with berylliosis.

Typical
(Left) Axial prone HRCT
shows diffuse ground-glass
opacities (arrows) in the
lower lobes. Chest
radiograph was normal.
Nonspecific finding in this
patient with chronic
beryllium disease. (Right)
Axial H ReT shows course
reticular interstitial
thickening (arrow),
bronchiolectasis (open
arrow), and subpleural
nodules (curved arrows) in
upper lobes in chronic
berylliosis.

Typical
(Left) Coronal multiplanar
reconstruction shows the
distribution of centri/obular
nodules predominantly in
the mid and lower lungs
(between arrows). Focal
aggregation of nodules in the
right mid lung (open arrow).
(Right) Axial HRCT shows
centrilobular nodules and
ground-glass opacities in a
perivascular distribution in
the mid-lungs bilaterally
(arrows). Imaging findings
identical to sarcoidosis.
Berylliosis.
 LUNG OSSIFICATION

2
50

Graphic shows diffuse peripheral dendritic calcifications Frontal radiograph shows very dense, coarse, reticular
within distorted inter/obular septa typical for diffuse opacities in the lung periphery (arrows) in this patient
pulmonary ossification. with diffuse pulmonary ossification.

I TERMI NOlOGY CT Findings

Dendritic or nodular 1-2 mm calcifications in
Abbreviations and Synonyms
periphery lower lobes
Diffuse pulmonary ossification (DPO) Typically associated with idiopathic pulmonary
Definitions fibrosis (JPF): Honeycomb pattern of basilar lung
fibrosiS--
Rare condition characterized by metaplastic mature
bone formation in lung parenchyma Nuclear Medicine Findings
Bone Scan: Because osseous metaplasia, will take up
bone scan agents
I IMAGING FINDINGS
General Features
Best diagnostic clue: Calcific interstitial thickening
I DIFFERENTIAL DIAGNOSIS
Location: DPO has similar distribution as IPF: Tuberculosis
Peripheral lung bases Upper lobe distribution, cavities
Radiographic Findings Silicosis
Radiography Diffuse nodules may calcify, occupational history
a Nodular pattern shows densely calcified 1-5 mm important
nodules that can coalesce or be trabeculated
a Dendriform pattern manifests as fine linear Sarcoidosis
branching opacities Nodules infrequently calcify, mid and upper lung
peribronchial distribution

DDx: Lung Ossification

Amyloidosis (Courtesy J. Caceres) Tuberculosis Silicosis

 LUNG OSSIFICATION

Key Facts
Terminology Top Differential Diagnoses
Rare condition characterized by metaplastic mature Sarcoidosis
bone formation in lung parenchyma Amyloidosis
Metastatic Pulmonary Calcification
Imaging Findings
Best diagnostic clue: Calcific interstitial thickening Clinical Issues 2
Location: OPO has similar distribution as IPF: No prognostic significance in pulmonary fibrosis;
Peripheral lung bases marker of chronicity or severity only; no specific 51
treatment

Other signs/symptoms: Relate to the inciting

Amyloidosis cardiopulmonary condition such as mitral valve
Nodules larger, small nodules generally do not calcify disease or idiopathic pulmonary fibrosis
Metastatic Pulmonary Calcification Demographics
Upper lobes most commonly affected (due to alkaline Age: Elderly
environment) Gender: Predominantly men
Natural History & Prognosis
I PATHOLOGY Can slowly progress or may remain stable; regression
does not occur
General Features
General path comments Treatment
a Nodular/granular pattern No prognostic significance in pulmonary fibrosis;
Characterized by 2-8 mm formations of mature marker of chronicity or severity only; no specific
lamellar bone, devoid of marrow treatment
a Dendriform/branching pattern
Characterized by linear branching deposits of
mature bone, typically containing marrow I DIAGNOSTIC CHECKLIST
Etiology: Considered true metaplasia of pulmonary
fibroblasts into osteoblasts in response to a chronic Image Interpretation Pearls
insult rather than transformation of metastatic Lower lobe dendritic or nodular calcifications 1-2 mm
calcification into bone in diameter

IClINICAllSSUES I SElECTED REFERENCES

Presentation
Most common signs/symptoms:
clinically or radiographically
1. Kim TS et al: Disseminated dendriform pulmonary
ossification associated with usual interstitial pneumonia:
Usually not diagnosed incidence and thin-section CT-pathologic correlation. Eur
Radial, 2005
2. Kanne JP et al: Diffuse pulmonary ossification. J Thorac
Imaging. 19(2):98-102, 2004

I IMAGE GALLERY

(Left) Axial NECT lung window from the same patient as previous page shows a pattern of idiopathic pulmonary fibrosis, but with scattered
calcific densities typical for opo. (Center) Axial NECT in 50ft tissue windows confirms scattered calcific densities typical for OPO within the
distribution of the peripheral honeycombing. (Right) Axial HRCT from this 43 year old woman with IN shows severe fibrosis with multiple
associated calcified nodules, the faller representing extensive OPO.
 LYMPHANGITIC CARCINOMATOSIS

2
52

Axial graphic shows typical features of Iymphangitic Cross pathology shows Iymphangitic tumor. Subpleural
tumor. Irregular septal thickening farrow). Distribution and interlobular septa (arrows) irregularly thickened
also markedly asymmetric, right greater than left. and beaded. Bronchovascular bundles are also
thickened (curved arrow).

ITERMINOlOGY Radiographic Findings

Radiography
Abbreviations and Synonyms
o Chest radiograph may be normal (30-50%)
Lymphangitic tumor o Interstitium
Definitions Reticulonodular opacities, coarse bronchovascular
Permeation of lymphatics by neoplastic cells markings, septal lines, subpleural edema at fissures
Tumor emboli or direct spread to lungs from hilar Kerley B lines common
nodes or bronchogenic carcinoma May resemble interstitial edema but chronic, will
Seen with carcinoma of the lung, breast, pancreas, not respond to diuretics
stomach, colon, prostate and other tumors (typically o Distribution
adenocarcinomas) Confined to 1 lung or lobe in 30%, more common
in the right lung
Unilateral disease: Most commonly due to lung
I IMAGING FINDINGS cancer
Bilateral symmetric disease commonly due to
General Features extra thoracic primary tumor
Best diagnostic clue: Nodular or beaded septal o Associated findings
thickening which may spare whole lobes or lung Hilar and mediastinal lymphadenopathy may be
Location: Usually diffuse but confined to 1 lung or present (30%)
lobe in 30% Pleural effusion common (50%)
Size: Interstitial thickening up to 10 mm CT Findings
Morphology: Irregular or beaded interlobular or
HRCT
bronchovascular thickening
o Secondary pulmonary lobule

DDx: Lymphangitic Carcinomatosis

Idiopathic Pulmonary Fibrosis Scleroderma

 LYMPHANGITIC CARCINOMATOSIS

Key Facts
Terminology Idiopathic Pulmonary Fibrosis
Permeation of lymphatics by neoplastic cells Scleroderma
Lymphoma
Imaging Findings Drug Reaction
Best diagnostic clue: Nodular or beaded septal
thickening which may spare whole lobes or lung Pathology
Frequent form of tumor spread found in 33% to 50%
2
Location: Usually diffuse but confined to 1 lung or
lobe in 30% of those with solid tumors at autopsies 53
Chest radiograph may be normal (30-50%) Common tumors: Breast, stomach, pancreas, prostate,
Hilar and mediastinal lymphadenopathy may be lung
present (30%) Hematogenous metastases: Tumor emboli to small
Pleural effusion common (50%) pulmonary artery branches with subsequent spread
Frequency of involvement: Axial (75%) > axial + along lymphatics
peripheral (20%) > peripheral (5%) Some tumors such as lymphoma spread from hilar
nodes retrograde into pulmonary lymphatics
Top Differential Diagnoses
Pulmonary Edema

Peripheral or axial distribution within lobule Cardiomegaly common

Frequency of involvement: Axial (75%) > axial + Rapidly resolves with treatment
peripheral (20%) > peripheral (5%)
Nodular or beaded thickening of interlobular Idiopathic Pulmonary Fibrosis
septa and or bronchovascular bundles Linear interstitial thickening, not nodular
Septa may be smooth (due to edema rather than Subpleural bilateral basilar distribution
tumor) Slow progression
Small, centrilobular nodules, thickened No adenopathy or pleural effusions
centrilobular bronchovascular bundles Honeycombing and architectural distortion more
Smooth or nodular thickening of interlobar common
fissures Scleroderma
o Lung architecture preserved
Dilated esophagus
o Patchy ground-glass and airspace opacities,
Linear interstitial thickening, not nodular
nonspecific
Subpleural bilateral basilar distribution
Airways may be narrowed from lymphatic Honeycombing and architectural distortion more
permeation leading to atelectasis or obstructive
common
pneumonitis
o Distribution Lymphoma
Basilar predominance Nodules usually larger (> 1 em)
Commonly asymmetric, may spare lobes or lungs Adenopathy common
(50%) Usually secondary or recurrent disease in patient with
o Associated findings known lymphoma
Pleural effusion
Hilar/mediastinallymphadenopathy Drug Reaction
Primary tumor may be obvious in case of lung Drug history, especially chemotherapy drugs
cancer Linear interstitial thickening, not nodular
Other metastatic sites: Bone, liver Honeycombing and architectural distortion more
common
Imaging Recommendations
Best imaging tool Sarcoidosis
o HRCT best imaging to suggest diagnosis, more Nodules but reticular opacities usually not beaded
sensitive and accurate than chest radiographs Typically more common in upper lung zones
Confident diagnosis in 50% of those with No pleural effusions
lymphangitic tumor
Asbestosis
Pleural plaques (80%)
I DIFFERENTIAL DIAGNOSIS Linear interstitial thickening, not nodular
Honeycombing and architectural distortion more
Pulmonary Edema common
Septal thickening smooth not irregular or beaded No pleural effusions or adenopathy
Bilateral pleural effusions also common Hypersensitivity Pneumonitis
Ground-glass opacities in gravity dependent
Antigen exposure
distribution due to edema
 LYMPHANGITIC CARCINOMATOSIS
Diffuse ground-glass opacities or micronodular pattern o If no known malignancy - sputum cytology,
Air-trapping common transbronchial biopsy, fine needle aspiration biopsy
Linear interstitial thickening when present, not or open lung biopsy for diagnosis
nodular
No adenopathy or pleural effusions
Demographics
Age: Incidence increases with age, reflects the age at
Unilateral Lung Disease (Mnemonic PEARL) which tumors develop in the population
2

Pneumonia
Edema
Natural History & Prognosis
Aspiration Poor, 15% survive 6 months
54
Radiation Treatment
Lymphangitic tumor
Aimed at underlying malignancy
o With successful chemotherapy, lymphangitic tumor
may regress
I PATHOLOGY Hospice care
General Features
General path comments
o Frequent form of tumor spread found in 33% to I DIAGNOSTIC CHECKLIST
50% of those with solid tumors at autopsies Image Interpretation Pearls
o Permeation of lymphatics by neoplastic cells
HRCT: Beaded septal thickening in patient with
o Common tumors: Breast, stomach, pancreas,
known malignancy
prostate, lung
Typically adenocarcinomas
Etiology
o Hematogenous metastases: Tumor emboli to small
I SELECTED REFERENCES
pulmonary artery branches with subsequent spread 1. Castaner E et al: Diseases affecting the peribronchovascular
along lymphatics interstitium: CT findings and pathologic correlation. Curr
o Some tumors such as lymphoma spread from hilar Probl Diagn Radiol. 34(2):63-75, 2005
2. Honda 0 et al: Comparison of high resolution CT findings
nodes retrograde into pulmonary lymphatics of sarcoidosis, lymphoma, and Iymphangitic carcinoma: is
o Lung cancer can spread to adjacent lung along there any difference of involved interstitium? ] Comput
lymphatics AssistTomogr. 23(3):374-9, 1999
3. Wu]W et al: Lymphangitic carcinomatosis from prostate
Gross Pathologic & Surgical Features carcinoma.] Comput AssistTomogr. 23(5):761-3, 1999
Interstitial thickening of interlobular septa due to 4. Hirakata Ket al: CT of pulmonary metastases with
tumor cells, desmoplastic response, and dilated pathological correlation. 5emin Ultrasound CT MR.
lymphatics 16(5):379-94,1995
Hilar and mediastinal lymph nodes mayor may not be 5. 1kezoe] et al: Pulmonary Iymphangitic carcinomatosis:
involved chronicity of radiographic findings in long-term survivors.
A]RAm] Roentgenol. 165(1):49-52, 1995
Microscopic Features 6. ]ohkoh T et al: CT findings in 1ymphangitic carcinomatosis
Nests of tumor cells within lymphatics, may be of the lung: correlation with histologic findings and
pulmonary function tests. A]RAm] Roentgenol.
associated with fibrosis
158(6):1217-22, 1992
o Tumor emboli in small adjacent arterioles also 7. Ren H et al: Computed tomography of inflation-fixed
common lungs: The beaded septum sign of pulmonary metastases.]
o Occluded lymphatics may also be edematous or Comput AssistTomogr. 13:411-6, 1989
fibrotic 8. Munk PLet al: Pulmonary Iymphangitic carcinomatosis:
CT and pathologic findings. Radiology. 166(3):705-9, 1988
Staging, Grading or Classification Criteria 9. 5tein MG et al: Pulmonary Iymphangitic spread of
For any malignancy, Iymphangitic tumor places carcinoma: appearance on CT scans. Radiology.
patient in stage IV or end-stage unresectable disease 162(2):371-5, 1987
10. Dennstedt FEet al: Pulmonary Iymphangitic
carcinomatosis from occult stomach carcinoma in young
adults: an unusual cause of dyspnea. Chest. 84(6):787-8,
I CLINICAL ISSUES 1983
11. 50stman HD et al: Perfusion scan In pulmonary
Presentation vascular/lymphangitic carcinomatosis: the segmental
Most common signs/symptoms contour pattern. A]RAm] Roentgenol. 137(5):1072-4, 1981
o Dyspnea, cough, insidious and progressive 12. Trapnell DH: Radiological appearance of lymphangitis
o Usually not first manifestation of underlying tumor, carcinomatosa of the lung. Thorax. 19: 251-60, 1964
usually seen in patients with known malignancy
o Sometimes present with asthma
Other signs/symptoms
o Progressive dyspnea in young adults often from
occult gastric carcinoma
 LYMPHANGITIC CARCINOMATOSIS

I IMAGE GALLERY
Typical
(Left) Axial HRCr shows
marked thickening of the
bronchovascular bundles 2
(arrows) and irregular
thickening of interlobular
septa, some of which are 55
beaded (curved arrow) from
Iymphangitic carcinomatosis.
(Right) Axial CECr in same
patient shows small pleural
effusion (arrow) and right
hilar adenopathy and
bronchial wall thickening of
the right upper lobe
bronchus (curved arrow).

Typical
(Left) Frontal radiograph
shows diffuse reticular
thickening right lung.
Differential includes
pneumonia, edema,
aspiration, radiation therapy,
and Iymphangitic tumor.
(RighI) Axial H RCr shows
irregular and beaded (arrow)
thickening of the interlobular
septa from Iymphangitic
tumor. Bronchovascular
bundles are also thickened
(open arrow).

Typical
(Left) Axial HRCr shows
interlobular septal thickening
and multiple centrilobular
micronodu/es (arrows).
Fissure is beaded (open
arrows). Lymphangitic
carcinomatosis. (Right) Axial
H ReT shows interlobular
and intralobular interstitial
thickening (arrows).
Thickening is relatively
uniform in both lower lobes.
Right middle lobe is
relatively spared (open
arrows).
 lYMPHOCYTIC INTERSTITIAL PNEUMONIA

2
56

Frontal radiograph shows nonspecific mild diffuse Axial HRCT shows multiple thin-walled cysls in Sjogren
inlerstiliallhickening in patient with Sjbgren syndrome syndrome (arrows). CYSls vary in size wilh no lobar
secondary 10lymphocytic interstitial pneumonitis. predilection. Lymphocytic interslilial pneumonilis.

ITERMINOLOGY I IMAGING FINDINGS

Abbreviations and Synonyms General Features
Lymphocytic interstitial pneumonia (LIP), Best diagnostic clue: Thin-walled cysts and
pseudolymphoma, diffuse hyperplasia of bronchial centrilobular nodules
bronchus-associated lymphoid tissue (BALT), Location: Basilar interstitial thickening in adult with
mucosa-associated lymphoid tissue lymphoma (MALT) Sjogren syndrome
Morphology: BALT lymphomas have identical
Definitions radiographic characteristics to non-neoplastic
Diffuse infiltration of alveolar septa by lymphocytic lymphoid lesions
infiltrate
Part of the spectrum of lymphoid disorders Radiographic Findings
o Follicular bronchiolitis to low grade lymphoma Radiography
Diffuse disease commonly referred to as LIP o Diffuse
Focal disease commonly referred to as Diffuse interstitial thickening, predominately
pseudolymphoma basilar
Non-neoplastic lymphoproliferation must be Multiple pulmonary nodular opacities often with
differentiated from lymphoma by immunologic stains air-bronchograms (more common in AIDS)
o Monoclonal cell lines in lymphoma, polyclonal in o Focal
non-neoplastic lymphoproliferative disorders Focal central air-space mass(s), segmental or lobar
in size simulating pneumonia
Over time gradually grow towards the periphery of
the lung
May also arise in lung periphery

DDx: Thin-Walled Cysts

Lymphangiomyomatosis Laryngotracheal Papillomatosis Langerhans Granulomatosis

 lYMPHOCYTIC INTERSTITIAL PNEUMONIA

Key Facts
Terminology Castleman Disease
Diffuse disease commonly referred to as LIP Lymphomatoid Granulomatosis
Focal disease commonly referred to as Hypersensitivity Pneumonitis
pseudolymphoma Pathology
Imaging Findings Pseudo lymphoma and LIP identical histologically
Small lymphocytes and plasma cells
2
Best diagnostic clue: Thin-walled cysts and
centrilobular nodules When centered on small airways: Follicular 57
Location: Basilar interstitial thickening in adult with bronchiolitis
Sjogren syndrome When more florid into alveolar septa: LIP
Morphology: BALT lymphomas have identical Clinical Issues
radiographic characteristics to non-neoplastic Dysproteinemia
lymphoid lesions Gender: Adults: Women primarily
Top Differential Diagnoses May evolve into B-celilymphoma, especially in
Nonspecific Interstitial Pneumonia (NSIP) Sjogren (5%)
Angioimmunoblastic Lymphadenopathy

Unilateral or bilateral No lobar predilection

o Pleural effusions rare Cavitation rare
o Associated findings: Anterior mediastinal mass o Evolution
Thymomas All findings may resolve with therapy except for
Predisposing condition for cysts
hypogammaglobulinemia or myasthenia gravis Centrilobular nodules or consolidation may
either of which may lead to LIP evolve into cysts
Air-space consolidation may evolve into
CT Findings honeycombing
CECT
o Lymph nodes may be enlarged Imaging Recommendations
More common in acquired immunodeficiency Best imaging tool: CT or HRCT to better characterize
syndrome (AIDS) lung and mediastinal pathology
Not demonstrated at chest radiography
o Pleural effusions rare
HRCT I DIFFERENTIAL DIAGNOSIS
o Diffuse (LIP)
Nonspecific ground-glass opacities or Nonspecific Interstitial Pneumonia (NSIP)
consolidation (100%) Cellular or fibrotic, temporally homogeneous at
2-4 mm centrilobular and subpleural nodules in histology
lymphatic distribution (100%) Idiopathic or seen with collagen vascular diseases
Thin-walled cysts the most distinctive Ground-glass opacities in bronchovascular distribution
characteristic finding (80%)
Angioimmunoblastic Lymphadenopathy
Thin-walled cysts range from 1 to 30 mm in
diameter (average 5 mm) Lymphoproliferative disorder associated with
dysproteinemia and immunodeficiency
Thin-walled cysts involve less than 10% of total
lung Generalized lymphadenopathy and
hepatosplenomegaly
Thin-walled cysts may be isolated finding
Thickening of small bronchovascular bundles Skin rash
(tree-in-bud pattern) Pleural effusion
Lung may be normal or have focal mass-like areas of
Septal thickening (80%)
consolidation
Combination of ground-glass opacities,
centrilobular nodules and thin-walled cysts Castleman Disease
common Benign Iymphoproliferative hyperplasia of lymph
Distribution: Bilateral (90%), diffuse (60%), nodes
peripheral distribution (10%) Hilar or mediastinal adenopathy
Rarely fibrosis and honeycombing o Hyaline vascular form: Nodes have intense
o Focal (pseudolymphoma) contrast-enhancement
Air-space mass, consolidation with Lungs less likely to De abnormal (if abnormal may be
air-bronchograms due to co-existing LIP)
Nodules: Peribronchial in distribution
Nodules: 2 to 30 mm in size (average 10 mm) Lymphomatoid Granulomatosis
Bronchiectasis (20%) Multiple pulmonary nodules (may be cavitary)
 lYMPHOCYTIC INTERSTITIAL PNEUMONIA
Skin rash 25% of adults with Sjogren syndrome have LIP
Central nervous system (CNS) disease
Microscopic Features
Hypersensitivity Pneumonitis Pseudolymphoma and LIP identical histologically
Appropriate antigen exposure Small lymphocytes and plasma cells
Diffuse ground-glass opacities and centrilobular o When centered on small airways: Follicular
nodules bronchiolitis
2 Lobular air-trapping
Cysts and adenopathy much less common
o When more florid into alveolar septa: LIP
Lymphocytic population both Band T-cells
58 (polyclonal)
Thin-Walled Cysts Noncaseating granulomas if present usually
Laryngotracheal papillomatosis inconspicuous
Pneumatoceles
o Trauma Staging, Grading or Classification Criteria
o Pneumocystis jiroveci pneumonia or Staphylococcus AIDS
pneumonia o LIP and HIV positive under 13 years of age
o Hydrocarbon ingestion
Langerhans granulomatosis
Lymphangiomatosis I CLINICAL ISSUES
Centrilobular emphysema
Metastases Presentation
Birt-Hogg-Dube syndrome: Multiple renal Most common signs/symptoms
oncocytomas/cancer, skin lesions o Nonspecific cough, dyspnea
o Generalized lymphadenopathy or
hepatosplenomegaly
I PATHOLOGY o Other symptoms related to any predisposing
diseases
General Features o Sjogren syndrome: Sicca complex (dry eyes and dry
General path comments mouth), parotid gland enlargement
o BALTa subset of MALT Other signs/symptoms
o BALTextends from nodal clusters in airway o Dysproteinemia
bifurcations to lymphocyte clusters at proximity of Hypergammaglobulinemia (90%), hypo- (10%)
lymphatics in terminal bronchiole o Rheumatoid factor usually positive in Sjogren
BALTextensive, positioned to handle large o Pulmonary function tests
number of inhaled or circulating antigens Decreased lung volumes and diffusion capacity
Polyclonal proliferation consistent with benign o Diagnosis usually requires surgical biopsy and not
disease, monoclonal proliferation of lymphocytes transbronchial biopsies
consistent with lymphoma; clonal groups
determined by special stains Demographics
BALToma (lymphoma) low grade B-cell primary Age: Adults: 40-70 years of age, average 55
pulmonary lymphoma Gender: Adults: Women primarily
Etiology Natural History & Prognosis
o Chronic antigenic stimulus elicits
Variable, often depends on underlying condition
lymphoproliferative response, may be
May evolve into B-ceillymphoma, especially in
Idiopathic Sjogren (5%)
Autoimmune: Sjogren syndrome, rheumatoid
Lymphomas have good response to treatment
arthritis, other collagen vascular diseases,
myasthenia gravis, primary biliary cirrhosis, Treatment
Hashimoto thyroiditis Non-AIDS: Steroids
Viral infection: HIV and Epstein-Barr virus AIDS: Retroviral drug therapies
(especially in children), human T-cell leukemia BALTlymphoma
virus (HTLV) type 1 o Surgical resection localized disease
Immunodeficiency: Common variable o Radiotherapy and chemotherapy for locally
immunodeficiency, bone marrow transplants and advanced disease
graft-vs-host disease
Drugs: Dilantin (phenytoin)
Multicentric Castleman disease I SELECTED REFERENCES
o Sjogren syndrome
1. Nicholson AG: Lymphocytic interstitial pneumonia and
Proliferation of non-neoplastic I-cells other lymphoproliferative disorders in the lung. Semin
Helper T-cells chronically stimulate B-cells Respir Crit Care Med. 22(4):409-22, 200\
Eventually, malignant B-cell clone develops 2. Johkoh T et al: Lymphocytic interstitial pneumonia:
Epidemiology follow-up CT findings in 14 patients. J Thorac Imaging.
o Most cases in HIV positive children 15(3):162-7,2000
o Sjogren syndrome
 lYMPHOCYTIC INTERSTITIAL PNEUMONIA

I IMAGE GALLERY

(Left) Frontal NECT shows

focal areas of consolidation
nearly lobar in size in both 2
midlungs, right more severe
than the le(t (open arrows).
Patient was asymptomatic. 59
(Right) Frontal radiograph
four years later shows
progressive consolidation of
the area in the left midlung
(open arrows). Patient
remained asymptomatic.
Pseudolymphoma.

Typical
(Left) Axial GCT shows
focal nodular opacities with
surrounding ground-glass
opacities that either have
air-bronchograms or early
cavitation (arrows). Sjogren
syndrome and LIP (Right)
Axial H RCT shows
nonspecific thickening of
bronchovascular bundles
(open arrow), parenchymal
bands (arrows), subpleural
nodules (curved arrows) in
LIP

(Left) Axial NECT shows

reticular interstitial
thickening (open arrows)
and ground-glass opacities
(arrow) in the upper lobes.
Sixty seven year old women
with common variable
immunodeficiency and
thrombocytopenia purpura.
(Right) Axial NECT shows
nodules (arrows) in addition
to the reticular interstitial
thickening. Subcarinal nodes
are also enlarged (open
arrow). LIP at autopsy.
 LYMPHANGIOMYOMATOSIS

2
60

Axial graphic shows typical appearance of LAM: Axial HRCT shows near complete replacement of the
Thin-walled cysts of slightly heterogeneous sizes that lung with variable sized cysts.
lead to diffuse destruction of lung parenchyma.

Morphology: Cysts, pleural air collections, chylous

ITERMINOLOGY effusion
Abbreviations and Synonyms Radiographic Findings
Lymphangioleiomyomatosis (LAM)
Lung
Definitions o Often normal in early stages of disease
Characterized by non-neoplastic hamartomatous o Paradoxical normal or enlarged lung volumes
proliferation of atypical muscle cells that leads to o Reticular interstitial thickening
progressive cystic destruction of lung parenchyma o Course honeycombing
Pleura
o Small to moderate sized pleural effusions (chylous)
I IMAGING FINDINGS o Spontaneous pneumothorax (40%)

General Features CT Findings

Best diagnostic clue Lung
o Reticular thickening on chest radiograph represents
o Radiograph: Paradoxical coarse interstitial
superimposed cysts
thickening in hyperinflated lungs in young women
o Thin-walled cysts uniform in size, increase in size
o HRCT: Large thin-walled cysts diffusely distributed
and number as disease progresses
in the lungs that will eventually replace entire lung
o Cyst show tendency to conflate
parenchyma
o Cysts will eventually replace entire lung
Location: Diffuse in entire lung, no predominance for
o Diffuse distribution, no predilection for any region
anyone area
of the lung
Size: Lesions range from several mm to 8 or more cm
o Scattered ground-glass opacities (may represent
in diameter
hemorrhage)

DDx: Lymphangiomyomatosis

Apica/ Bullae Pan/obu/ar Emphysema Histiocytosis X

 LYMPHANGIOMYOMATOSIS

Key Facts
Terminology Sjogren Syndrome (Lymphocytic Interstitial
Pneumonia)
Characterized by non-neoplastic hamartomatous
proliferation of atypical muscle cells that leads to Neurofibromatosis
progressive cystic destruction of lung parenchyma Laryngotracheal Papillomatosis

Imaging Findings Pathology

Hamartomatous proliferation of smooth muscle
2
Radiograph: Paradoxical coarse interstitial thickening
in hyperinflated lungs in young women around lymphatics and blood vessels 61
HRCT: Large thin-walled cysts diffusely distributed in Etiology: Predilection for premenopausal women
the lungs that will eventually replace entire lung suggests estrogen plays a role in pathogenesis
parenchyma Clinical Issues
HRCT, more sensitive than chest radiograph Five year survival: 50%
Top Differential Diagnoses Diagnostic Checklist
Panlobular Emphysema HRCT findings in appropriate clinical context are
Langerhans Cell Histiocytosis pathognomonic

o Intervening lung appears normal

Neurofibromatosis
Pleura
o Pleural or pericardial effusion (chylous, 60%) Cystic lesions predominately upper lung zones
Other Ground-glass opacities
o Mediastinal adenopathy, borderline enlargement Basilar interstitial lung disease
o Retroperitoneal adenopathy Commonly have cutaneous neurofibromas
o Renal angiomyolipoma (15%) laryngotracheal Papillomatosis
o Pancreatic, adrenal, or uterine angiomyolipoma
Multiple solid or cavitated nodules
(rare)
Thick or thin-walled
Imaging Recommendations Predominantly dorsal distribution
Best imaging tool Tracheal nodules
o HRCT, more sensitive than chest radiograph Idiopathic Pulmonary Fibrosis
o Extent of disease well correlated with expiratory Shows voiume loss from restrictive lung disease
volumes and diffusion capacity
Thickening of interstitial structures with architectural
Protocol advice: Acquire scans in full suspended distortion
inspiration only
Traction bronchiectasis
Cysts from traction bronchiectasis or fibrotic lung
distortion
I DIFFERENTIAL DIAGNOSIS
Chronic Hypersensitivity Pneumonitis
Pan lobular Emphysema Upper lobe predominance
Lower lobe predominance in alpha-I-anti protease Ground-glass opacities and micronodules
deficiency Often have air-trapping at expiratory CT
Lesions have no definable wall Thickening of interstitial structures
No pleural effusion
Ground-glass opacities are uncommon
langerhans Cell Histiocytosis I PATHOLOGY
Occurs in young smokers, male or female General Features
Micronodules are frequent (early disease)
General path comments
Cysts are smaller and of various shapes o Hamartomatous proliferation of smooth muscle
Cysts and nodules preferentially affect upper lung
around lymphatics and blood vessels
zones o Smooth muscle proliferation can also affect airways
No pleural effusion and pleura
Sjogren Syndrome (lymphocytic Interstitial o Muscle proliferation in airway walls may manifest as
bronchial wall thickening
Pneumonia) o Muscle proliferation in venous walls may cause
Older women than LAM pulmonary hypertension and hemoptysis
History of sicca syndrome o Muscle proliferation around lymphatics may cause
1/3 have thin-walled cysts chylous effusions
o Thoracic duct may be markedly enlarged
Genetics
 LYMPHANGIOMYOMATOSIS
o Identical pathologic findings found in 1-2% of Progesterone and oophorectomy
patients with tuberous sclerosis (women only) o Variable, rather modest success
o Nonfamilial (tuberous sclerosis, however, autosomal Oophorectomy
dominant) o Modest success
Etiology: Predilection for premenopausal women Discourage air travel and diving
suggests estrogen plays a role in pathogenesis o Decreases risk of pneumothorax
Epidemiology: Women of child-bearing age Pleurodesis for effusions or pneumothorax
2 Associated abnormalities o May worsen pulmonary function
o Mediastinal adenopathy Lung transplantation
62 o Renal angiomyolipoma (15%) o Disease may recur in transplanted lung
o Uncommon: Angiomyolipomas in pancreas,
adrenals, and uterus
o Extrapulmonary manifestations are incidentally I DIAGNOSTIC CHECKLIST
found on autopsy in up to 55% of patients
Consider
Gross Pathologic & Surgical Features LAM a rare disorder with uncharacteristic clinical
Cysts uniformly distributed throughout the lung presentation that occurs exclusively in women during
Chylous pleural effusion childbearing age
Enlarged thoracic duct HRCT findings in appropriate clinical context are
Lymph nodes with hamartomatous smooth muscle pathognomonic
proliferation
Image Interpretation Pearls
Microscopic Features Chest radiograph may appear normal in early stages of
Normal tissue disorganized, no specific microscopic disease
features HRCT should be performed early because of higher
sensitivity for detection of disease
Often complicated by pneumothorax that may be first
IClINICAllSSUES manifestation
Diffuse cysts, combined with chylous pleural effusions
Presentation are most common findings
Most common signs/symptoms Cyst may eventually replace entire lung parenchyma
o Clinical and functional symptoms may precede the
definite diagnosis by up to 10 years
o Symptoms may mimic asthma, pulmonary fibrosis, I SELECTED REFERENCES
sarcoidosis, tuberculosis, or pulmonary
hemosiderosis 1. Glassberg MK: Lymphangioleiomyomatosis. Clin Chest
Med. 25(3):573-82, vii, 2004
o Dyspnea 2. Pitts 5 et al: Benign metastasizing leiomyoma and
o Pneumothorax, may occur bilaterally Iymphangioleiomyomatosis: sex-specific diseases? Clin
o Hemoptysis (30%) Chest Med. 25(2):343-60, 2004
o Chyloptysis 3. Hancock E et al: Lymphangioieiomyomatosis: a review of
o Chylous pleural and pericardial effusions the literature. Respir Med. 96(1):1-6, 2002
o Pulmonary function tests 4. Pallisa E et al: Lymphangioleiomyomatosis: pulmonary and
Obstructive indices with hyperinflation abdominal findings with pathologic correlation.
Radiographies. 22 Spec No:S185-98, 2002
Mixed obstructive and restrictive patterns
5. KellyJ et al: LymphangioJeiomyomatosis. Am J Med Sci.
Decreased diffusion capacity 321(1):17-25,2001
Other signs/symptoms: Pneumomediastinum 6. Izumi T: Pulmonary Iymphangiomyomatosis--past, present,
and future. Intern Med. 39(9):683-4, 2000
Demographics 7. Matsui Ket al: Extrapulmonary
Age lymphangioleiomyomatosis (LAM):clinicopathologic
o Childbearing age features in 22 cases. Hum Pathol. 31(10):1242-8, 2000
o May become symptomatic during pregnancy 8. Johnson 5: Rare diseases. 1. Lymphangioleiomyomatosis:
o May exacerbate during pregnancy clinical features management and basic mechanisms.
l

o Postmenopausal occurrence related to estrogen Thorax. 54(3):254-64, 1999

substitution therapy
Gender: Women
Natural History & Prognosis
Five year survival: 50%
o Death occurs due to respiratory
occasionally renal failure
Treatment
Pregnancy counseling
o Pregnancy may exacerbate disease
9. Oh YMet al: Pulmonary Iymphangioleiomyomatosis in
Korea. Thorax. 54(7):618-21,1999
10. Sullivan EJ:Lymphangioleiomyomatosis: A review. Chest.
114:1689-703, 1998
11. Kalassian KGet al: Lymphangioleiomyomatosis: new
insights. AmJ Respir Crit Care Med. 155(4):1183-6, ]997
failure or 12. Bonetti F et al: Lymphangioleiomyomatosis and tuberous
sclerosis: where is the border? Eur RespirJ. 9(3):399-40],
1996
13. Muller NL et al: Pulmonary Iymphangiomyomatosis:
Correlation with radiographic and functional findings.
Radiology ]75:335-9, 1990
 LYMPHANGIOMYOMATOSIS

I IMAGE GALLERY

(Leh) Axial HRCT shows

extensive parenchymal
lesions and a subpleural air
collection (arrows). (Right) 2
Axial HRCT shows large
subpleurallesiol1s and an air 63
collection along the
interlobar fissure (arrows).

Typical
(Left) Axial HRCT shows
early LAM wilh subtle
thin-walled cysts (arrows).
The patient presented with
mild dyspnea. (Right) Axial
H RCT in the same patient.
She became aculely
symptomatic because of an
extensive pneumothorax
(arrows) after an air plane
travel.

Other
(Left) Axial H RCT shows
neurofibromatosis type 7.
Cystic lesions (arrows) are
similar to those in LAM but
tend to have a more
heterogeneous appearance.
(Right) Axial H RCT shows
neurofibromatosis type ,.
Extensive ground-glass
opacities prevail over cystic
lesions. Note cutaneous
fibromas (arrows).
 DIFFUSE PULMONARY LYMPHANGIOMATOSIS

2
64

Axial graphic shows diffuse interlobular septal Axial HRCT shows marked thickening of
thickening (curved arrow) and patchy ground-glass bronchovascular bundles (arrows) and septal thickening
opaciUes (arrows) from diffuse pulmonary (curved arrow) from diffuse pulmonary
Iymphangiomatasis. Iymphangiomatasis.

o Smooth uniform thickening of interlobular septa

ITERMINOlOGY and fissures
Abbreviations and Synonyms o Marked smooth thickening of bronchovascular
Lymphangiectasis bundles
o Patchy ground-glass opacities
Definitions o Basilar predominance
Congenital lymphatic disorder characterized by Findings confined to the thorax
proliferation and dilatation of lymphatic channels Diffuse effacement of mediastinal fat
o Mayor may not have slightly enlarged mediastinal
lymph nodes (50%)
I IMAGING FINDINGS Variable sized chylous effusions
o Occasional pleural calcifications
General Features
Best diagnostic clue: Smooth diffuse interlobular septal
thickening with diffuse effacement of mediastinal fat I DIFFERENTIALDIAGNOSIS
Location: Findings confined to the thorax
Lymphangiomyomatosis
Radiographic Findings Young women
Radiography Cysts throughout the lung, chylous pleural or
o Diffuse interstitial thickening 100% pericardial effusions
o Pleural or pericardial effusion SO% No septal thickening
o Mediastinal adenopathy, mild
Lymphangitic Carcinomatosis
CT Findings History of malignancy usually adenocarcinoma
HRCT May have pleural effusions or adenopathy

Lymphangitic Carcinomatosis
 DIFFUSE PULMONARY LYMPHANGIOMATOSIS
Key Facts
Terminology Mediastinal adenopathy, mild
Congenital lymphatic disorder characterized by Top Differential Diagnoses
proliferation and dilatation of lymphatic channels Lymphangiomyomatosis
Imaging Findings Lymphangitic Carcinomatosis
Best diagnostic clue: Smooth diffuse interlobular

Venous Hypertension
Erdheim-Chester Disease
2
septal thickening with diffuse effacement of
mediastinal fat Clinical Issues 65
Diffuse interstitial thickening 100% Medium chain triglyceride high-protein diet
Pleural or pericardial effusion 50%

Septal thickening irregular or beaded non-uniform

throughout lung
ICLINICAl ISSUES
Venous Hypertension Presentation
Most common signs/symptoms: Asthma, wheezing,
Etiologies: Cardiac or mitral valve disease, occlusion
major pulmonary veins, pulmonary veno-occlusive dyspnea
Other signs/symptoms: Pulmonary function:
disease
Restrictive, obstructive, or mixed
Septal thickening smooth and uniform
Ground-glass opacities from pulmonary edema and Demographics
pleural effusions common Age: Pediatric to young adult
Erdheim-Chester Disease Gender: M = F
Perirenal infiltration or aortic soft tissue encasement Natural History & Prognosis
Symmetric sclerotic bone lesions Variable course, more aggressive in the young
Treatment
!PATHOLOGY Medium chain triglyceride high-protein diet
Pleural effusions drained and sclerosed
General Features Radiation therapy successful in case reports
General path comments
o Lymphatic channels grossly dilated
o Chylous pleural or pericardia I effusions I SElECTED REFERENCES
o Lymphangiectasis: Dilatation of pre-existing
1. EI Hajj Let al: Diagnostic value of bronchoscopy, CT and
lymphatic spaces, not increase in size and number transbronchial biopsies in diffuse pulmonary
o Hemorrhage common lymphangiomatosis: case report and review of the
Etiology: Considered congenital but unknown literature. Clin Radiol. 60(8):921-5, 2005
Epidemiology: 4% of chronic interstitial disease in 2. Yekeler E et al: Diffuse pulmonary Iymphangiomatosis:
children imaging findings. Diagn lnterv Radiol. 11(1):31-4, 2005
3. Swensen 5J et al: Diffuse pulmonary Iymphangiomatosis:
Gross Pathologic & Surgical Features CT findings. J Com put Assist Tomogr. 19(3):348-52, 1995
Proliferation in size and number of lymphatic 4. Tazelaar HD et al: Diffuse pulmonary lymphangiomatosis.
channels Hum Pathol. 24(12): 1313-22, 1993

I IMAGE GALLERY

(LeFt) Frontal radiograph shows moderate sized bilateral pleural effusions (black arrow) along with mild widening of the mediastinum (white
arrows) in patient with diffuse pulmonary lymphangiomalOsis. Small calcification (curved arrow). (Center) Axial NECT shows diffuse infiltration
of mediastinal fat (arrows) and mildly enlarged mediastinal lymph node (curved arrow) in diffuse pulmonary lymphangiomalOsis. (Right) Axial
HRCT shows marked thickening of the bronchovascular bundles (arrows). The bronchial lumen is extremely narrowed. Smooth septal thickening
(curved arrows) Diffuse pulmonary Iymphangiomatosis
 Congenital
Cystic Fibrosis, Pulmonary 1-3-2
Tracheobronchomegaly 1-3-6
lmmotile Cilia Syndrome 1-3-10
Bronchial Atresia 1-3-14
Anomalous Bronchi 1-3-18
Alpha-l Antiprotease Deficiency 1-3-22
Para tracheal Air Cyst 1-3-26

Infectious
Rhinoscleroma 1-3-28
Chronic Bronchitis 1-3-30
Bronchiectasis 1-3-34
Mycobacterial Avium Complex 1-3-38
Laryngeal Papillomatosis 1-3-42

Inflammatory - Degenerative
Allergic Bronchopulmonary Aspergillosis 1-3-46
Tracheobronchomalacia 1-3-50
Relapsing Polychondritis 1-3-54
Middle Lobe Syndrome 1-3-58
Saber-Sheath Trachea 1-3-60
Bronchiolitis Obliterans 1-3-64
Asthma 1-3-68
Panlobular Emphysema 1-3-72

Toxic - Metabolic
Langerhans Cell Granulomatosis, Pulmonary 1-3-76
Respiratory Bronchiolitis 1-3-80
Centrilobular Emphysema 1-3-84
Amyloidosis, Pulmonary 1-3-88

Neoplastic
Tracheopathia Osteochondroplastica 1-3-92
Carcinoid, Pulmonary 1-3-94
Kaposi Sarcoma, Pulmonary 1-3-98
 CYSTIC FIBROSIS, PULMONARY

3
2

Graphic shows bronchial abnormalities in cystic fibrosis. Frontal radiograph shows typical features of cystic
Abnormal thick secretions result in bronchiectasis fibrosis with right upper lobe collapse (arrows) and
(arrow), mucus plugging (open arrow) and scattered bronchiectasis bilaterally (open arrows) with
parenchymal destrucUon of the lung (curved arrow). upper lobe predominance.

ITERMINOlOGY o Not essential for diagnosis, primary role in

longitudinal assessment
Abbreviations and Synonyms Usefulness during an exacerbation debatable
Cystic fibrosis (CF), mucoviscidosis (exclude lobar atelectasis or pneumothorax)
o Hyperinflation earliest finding, may be reversible
Definitions early and then permanent (100%)
Disorder due to autosomal recessive gene regulating o Airways
chloride transport which produces thick viscous Bronchial wall thickening and mucoid impaction
secretions affecting multiple organs, primarily the Bronchiectasis: Cylindrical most common, evolves
lung and pancreas into varicose and then saccular forms
Accounts for up to 25% of adult cases of Bronchiectasis usually more severe in the upper
bronchiectasis lobes, especially the right
Lobar atelectasis, especially right upper lobe
10% develop allergic bronchopulmonary
IIMAGING FINDINGS aspergillosis (ABPA)
o Lung
General Features Recurrent pneumonias (often difficult to detect)
Best diagnostic clue: Diffuse bronchiectasis primarily Lung abscess (air-fluid levels usually found in
in upper lobes with mucous plugging bronchiectatic airways)
Location: Upper lobe predominance, often worse in Subpleural blebs can lead to spontaneous
the right upper lobe pneumothorax
o Pleura
Radiographic Findings
Pleural effusions uncommon
Radiography
Spontaneous pneumothorax

DDx: Bronchiectasis

CilialDysmotility Radiation Fibrosis

 CYSTIC FIBROSIS, PULMONARY
Key Facts
Terminology 10% develop allergic bronchopulmonary aspergillosis
Disorder due to autosomal recessive gene regulating (ABPA)
chloride transport which produces thick viscous Pleural effusions uncommon
secretions affecting multiple organs, primarily the Acute increase in heart size from cor pulmonale
lung and pancreas ominous sign

Imaging Findings Top Differential Diagnoses

Not essential for diagnosis, primary role in Allergic Bronchopulmonary Aspergillosis (ABPA)
longitudinal assessment Cilial Dysmotility (Kartagener)
Usefulness during an exacerbation debatable (exclude Post-Infectious Bronchiectasis
lobar atelectasis or pneumothorax) Endobronchial Obstruction
Radiation Fibrosis
Hyperinflation earliest finding, may be reversible
early and then permanent (100%) Tuberculosis 3
Bronchiectasis usually more severe in the upper lobes, Clinical Issues 3
especially the right Diagnosis: Sweat chloride test
Lobar atelectasis, especially right upper lobe

o Hilum o Secretions within peripheral small centrilobular

Adenopathy (from chronic bronchopulmonary bronchioles can give V- or V-shaped opacities -
inflammation) lItree-in-budli
Enlarged central pulmonary arteries from Bhalla scoring system for CT
pulmonary artery hypertension o 25 - point demerit system, similar to Brasfield
o Cardiac scoring system
Acute increase in heart size from cor pulmonale o Presence, extent, and severity of bronchiectasis,
ominous sign peribronchial thickening, mucous plugging,
o Other atelectasis or consolidation, em physema
Osteopenia o Whether this new system will have practical use in
Kyphosis (10%) the assessment of patients and therapeutic
Brasfield scoring system interventions is unclear
o Used to generate age-based severity (ABS) curves to o Total score = 25 - demerit score
assess change over time
o System assesses 5 elements
Angiographic Findings
Bronchial arteriography and embolotherapy for
Air trapping
Linear markings hemoptysis
Nodular cystic lesions Imaging Recommendations
General severity, and Best imaging tool
Large lesion (e.g., pneumonia, segmental or lobar o Chest radiography sufficient for monitoring disease
atelectasis) progression
o First 4 elements are scored 0-4 o HRCT: Best imaging modality for detection of
o 0 for absent bronchiectasis
o 4 used if finding is severe
For large lesion, score of 5 given for multiple
atelectasis I DIFFERENTIAL DIAGNOSIS
For general severity, score of 5 given for
complications (cardiac enlargement, Allergic Bronchopulmonary Aspergillosis
pneumothorax)
(ABPA)
o Total score = 25 - demerit points (3 is most severe)
Normal chest radiographic receives a score of 25 Central bronchiectasis, usually upper lobe
predominant
CT Findings History of asthma, often eosinophilia
Advanced cases may be difficult to distinguish from
Cilial Dysmotility (Kartagener)
fibrosis with honeycombing
CT more sensitive and specific in identifying bronchial No upper lobe predominance
and pulmonary abnormalities Dextrocardia or situs inversus
o CT however not very specific in distinguishing Sinusitis
among different causes of adult bronchiectasis Post-Infectious Bronchiectasis
In setting of a normal radiograph, CT may show Usually unilateral, lobar or sublobar
o Mosaic perfusion secondary to small-airway Often lower lobe
abnormalities
o Air trapping (best demonstrated on expiratory scans)
 CYSTIC FIBROSIS, PULMONARY
Endobronchial Obstruction IClINICAllSSUES
Generally localized, lobar or sublobar, may have
associated volume loss or hyperinflation Presentation
Etiology: Foreign body, carcinoid tumor, bronchial Most common signs/symptoms
atresia o Onset in childhood
Meconium ileus at birth: 15%, failure to thrive,
Radiation Fibrosis recurrent respiratory infections
Cicatricial scarring and bronchiectasis conforming to o With mild disease, may be asymptomatic
radiation port o Wheezing, dyspnea on exertion, recurrent
History of intrathoracic malignancy pneumonias, atypical asthma, pneumothorax
o Digital clubbing nearly universal in symptomatic
Tuberculosis patients in childhood
Reactivation can produce upper lobe volume loss, o Diagnosis: Sweat chloride test
3 bronchiectasis
Often associated with calcifications in lung
o Symptoms parallel development of chronic airways
disease
granulomas and hilar mediastinal lymph nodes Other signs/symptoms
4
o Shwachman-Kulczyski score
Clinical score: Physical exam, nutrition, activity
I PATHOLOGY and chest radiograph
o Hemoptysis, sometimes massive
General Features
General path comments Demographics
o Lungs normal at birth Age
o Airways colonized with Pseudomonas aeruginosa o Most diagnosed by age 3
(mucoid type), atypical mycobacteria, candida and o Occasional mild cases not diagnosed until
aspergillus species adulthood
Genetics: Autosomal recessive: Gene defect that Gender: Male patients less affected than females
regulates chloride transport across cell membrane Ethnicity: More common in Caucasians, rare in
Etiology African-Americans or Asians
o Pathologic changes acquired from abnormal
chloride transport Natural History & Prognosis
Abnormal chloride transport produces thick, More patients surviving into 40's, 50's and older
viscous mucus Death due to cor pulmonale or hemoptysis
Mucus not expectorated, becomes secondarily
Treatment
infected
Repeated infections eventually destroy airways Pancreatic enzymes
o Increased respiratory excursions in lower lobes aids Respiratory therapy: Postural drainage,
removal of secretions, thus upper lobe airways bronchodilators, prophylactic antibiotics, aerosolized
predominately affected rh DNase
Epidemiology: 3,200 cases each year in US, 30,000 Gene therapy promising
cases total in US Segmental lung resection with video-assisted
thoracoscopy (VATS)
Associated abnormalities
o Pancreatic insufficiency Lung transplant
Fatty replacement on CT; may spare pancreatic o Bilateral: Prevents reinfection of transplanted lung
head from native lung
May have macrocysts in pancreas
o Pansinusitis
Almost all will have underdeveloped and opacified I DIAGNOSTIC CHECKLIST
paranasal sinuses on imaging Consider
o Biliary cirrhosis
CF in any adult with unexplained bronchiectasis
o Bone demineralization
Compression fractures are common
Rib fractures
o Infertility
I SELECTED REFERENCES
1. Berrocal T et aI: Pancreatic cystosis in children and young
Microscopic Features adults with cystic fibrosis: sonographic, CT, and MRI
No specific features, chronic inflammation both to findings. AJR.184:1305-9,2005
airway wall and lung 2. Brody A5et al: High-resolution computed tomography in
young patients with cystic fibrosis: distribution of
abnormalities and correlation with pulmonary function
tests. J Pediatr. 145(1):32-8,2004
3. de Jong PAet al: Progressive damage on high resolution
computed tomography despite stable lung function in
cystic fibrosis. Eur RespirJ. 23(1):93-7, 2004
 CYSTIC FIBROSIS, PULMONARY

IIMAGE GALLERY
Typical
(Left) Axial NECT shows
severe cystic and cylindrical
bronchiectasis bilaterally in
the upper lobes with an air
fluid level (arrow) suggesting
acute infection. Spontaneous
pneumomediastinum (open
arrows). (Right) Frontal
radiograph shows severe
upper IDbe vDlume 1055with
upward retractiDn Df hila 3
(arrows) and large cystic
spaces; as well as an
implanted reservoir catheter. 5
NDte the radiDgraphic
opacities are more marked in
the right upper IDbe.

(Left) Frontal radiDgraph

shDws upper lobe
bronchiectasis and mucus
plugging (arrows) frDm cystic
fibrosis. RadiDgraphic
abnormalities are more
severe in the right upper IDbe
as compared tD the left
upper IDbe. (Right) Axial
NEeT shows extensive
bronchiectasis in the right
middle and lower lobe alDng
with typical tree-in-bud
(arrows) findings Df mucous
plugging in smaller airways.

(Left) Axial NECT shDws

complete fatty infiltration of
the pancreas (arrows), with
only minima/linear opacities
remaining. (Right) Axial
NEeT shows extensive
bilateral brDnchiectasis at the
lung apex (white arrows) as
well as a subpleural bleb
(black arrDw), which can
lead to spontaneous
pneumothorax.
 TRACHEOBRONCHOMEGALY

3
6

Frontal radiograph shows dilated trachea (open arrows) Lateral radiograph shows dilated trachea with
and main bronchi (arrows). Scarring left mid-lung, the corrugated walls (open arrows). Diagnosis:
result of remote infection. Mounier-Kuhn syndrome.

I TERMINOlOGY a Men: Left and right mainstem bronchi> 18 or 21

mm
Abbreviations and Synonyms a Women: Right and left mainstem bronchi> 19.8 or
Mounier-Kuhn syndrome 17.4 mm
Morphology: Corrugated trachea, best seen on lateral
Definitions radiograph
Rare disorder characterized by dilation of the trachea
and central bronchi that impairs the ability to clear Radiographic Findings
mucus from the lungs Airways dilated on inspiration, collapse on expiration
a Cine fluoroscopy shows airway collapse with
expiration or cough
I IMAGING FINDINGS Lateral view, trachea is more conspicuous than frontal
Marked dilatation of trachea and central bronchi
General Features a Normal for males: Coronal 13-25; sagittal 13-27 mm
Best diagnostic clue a Normal for females: Coronal 10-21; sagittal 10-23
a Tracheal diameter> 27 mm in men and> 23 mm in mm
women a Main bronchi, normal (right-left, in mm): Men 21,
a Recurrent pulmonary infections; bronchiectasis 18.4; women 19.8, 17.4
Location: Trachea and central bronchi Corrugated effect due to redundant mucosa prolapsing
Size through tracheal rings
a Men: Tracheal sagittal and coronal diameters> 25 Tracheobronchial diverticula
and 27 mm Central bronchiectasis, first to fourth order bronchi
a Women: Tracheal sagittal and coronal diameters> Peripheral bronchi have normal caliber
21 and 23 mm Hyperinflation and emphysema

DDx: Tracheobronchomegaly

Tracheocele Pulmonary Fibrosis

 TRACHEOBRONCHOMEGALY

Key Facts
Terminology Cutis laxa (generalized elastolysis)
Rare disorder characterized by dilation of the trachea Immune deficiency states and recurrent childhood
and central bronchi that impairs the ability to clear infections
mucus from the lungs Ataxia telangiectasia

Imaging Findings Pathology

Tracheal diameter> 27 mm in men and> 23 mm in Mounier-Kuhn: Idiopathic
women Clinical Issues
Recurrent pulmonary infections; bronchiectasis Recurrent infections may lead to bronchiectasis and
Morphology: Corrugated trachea, best seen on lateral pulmonary fibrosis
radiograph
Obstructive airway disease from collapse of trachea
Airways dilated on inspiration, collapse on expiration
Hyperinflation and emphysema
and major bronchi (tracheomalacia)
Usually diagnosed, age 30-50 years
3
Thinning of the tracheal wall Gender: Male to female ratio, 19:1 7
Top Differential Diagnoses
Ehlers-Danlos syndrome

Secondary pulmonary fibrosis from recurrent Immune deficiency states and recurrent childhood
infections, less common infections
o Ataxia telangiectasia
CT Findings o Bruton-type agammaglobulinemia
NECT
o Abnormally large tracheal and bronchial diameters Localized Tracheal Widening
on inspiration Tracheocele
o Normal diameter of subglottic trachea o Localized ballooning of membranous portion of
o Tracheobroncheal collapse with expiration cervical or thoracic trachea
o Thinning of the tracheal wall o Right posterior tracheal wall
o Recurrent pneumonias o Varying size with respiration
o Bronchiectasis, pulmonary fibrosis, hyperinflation Post intubation with overinflated cuff
HRCT: More sensitive for bronchiectasis, emphysema,
pulmonary fibrosis Central Bronchomegaly
Allergic bronchopulmonary aspergillosis
Imaging Recommendations o Asthma, central bronchiectasis
Best imaging tool: CT demonstrates the abnormalities Williams-Campbell syndrome
better than radiographs o Cartilage deficiency in 4th to 6th order bronchi;
Protocol advice: Expiratory studies show collapse of larger airways may be involved
airways o Cystic bronchiectasis distal to first generation
Chest radiographs usually sufficient for diagnosis, segmental bronchi
often overlooked o Normal caliber trachea and central bronchi
o Expiration HRCT shows collapse of bronchi with
distal air trapping
I DIFFERENTIAL DIAGNOSIS o Present in infancy with recurrent pneumonias,
bronchiectasis
Tracheobronchomegaly o Prognosis: Prolonged survival or rapid deterioration
Ehlers-Danlos syndrome Cystic fibrosis
o Inherited connective tissue disorder o Bronchiectasis, upper lobe and central
o One subtype has tracheal and bronchial dilation predominance
o Pulmonary artery stenoses, bronchiectasis, o Hereditary, Caucasians, positive sweat test,
thin-walled cavitary lesions, cysts abnormally thick mucus
Cutis laxa (generalized elastolysis)
o Hereditary connective tissue disorder Tracheomalacia
o Autosomal recessive Causes other than Mounier Kuhn include
o Premature aging, loose skin and subcutaneous tissue o Emphysema: No bronchiectasis, saber sheath trachea
o Tracheobronchomegaly, panacinar emphysema, o After prolonged intubation, endotracheal or
bronchiectasis, aortic aneurysms transtracheal
Mechanical ventilation in preterm neonate o Relapsing polychondritis: Autoimmune disease,
Upper lobe or diffuse pulmonary fibrosis cauliflower ears, saddle nose
o Ankylosing spondylitis
o Sarcoidosis
 TRACHEOBRONCHOMEGALY
If require intubation, should be performed with an
I PATHOLOGY uncuffed tube
General Features Demographics
General path comments: Atrophy or absence of elastic
Age
fibers and thinning of smooth muscle layer in trachea o Mounier-Kuhn may be present at birth
and main bronchi o Rarely causes problems < 20 years
Genetics o Usually diagnosed, age 30-50 years
o Mounier-Kuhn: Congenital in some cases Gender: Male to female ratio, 19:1
o Ehlers-Danlos, cutis laxa, or ataxia telangiectasia: Ethnicity: Predisposition in blacks
Congenital
Etiology Natural History & Prognosis
o Mounier-Kuhn: Idiopathic Prognosis: Variable from minimal disease to
o In some cases familial, autosomal recessive respiratory failure and death
3 o Theories
Deficiency of segmental myenteric plexus, genetic
Even with congenital cause, symptoms usually don't
develop until adulthood, some patients remain
8 predisposition asymptomatic
Congenital spasticity of elastic and muscular Depends on the development of obstructive airways
elements of the tracheobronchial walls disease
Epidemiology
o Most cases are sporadic Treatment
o Approximately 1% of bronchograms show this Managing secretions in the lungs (physiotherapy)
abnormality Reducing risk for infection, pneumococcal
o Rare, usually identified in adults, rare in infants or immunization
children Antibiotic treatment during infectious exacerbations
Smoking cessation
Gross Pathologic & Surgical Features Tracheal stenting has been used in advanced cases
Enlarged trachea with thinning of the tracheal wall,
may contain diverticula
Both airway cartilages and membranous portions of I DIAGNOSTIC CHECKLIST
trachea are affected
Consider
Microscopic Features Inspiratory and expiratory fluoroscopy or CT to
No specific features, absence of elastic fibers, thinning evaluate for tracheomalacia
smooth muscle, abnormal cartilage
Airways distal to fourth-order and fifth-order division Image Interpretation Pearls
are normal in diameter Trachea often blind spot
Evaluate tracheal caliber in patients with
bronchiectasis or recurrent pneumonias
ICLINICAL ISSUES
Presentation I SELECTED REFERENCES
Most common signs/symptoms: Symptoms of 1. Lazzarini-de-Oliveira LC et al: A 38 year old man with
recurrent infection and bronchiectasis tracheomegaly, tracheal diverticulosis, and bronchiectasis.
Symptoms may date back to childhood with Chest. 120(3):1018-20,2001
ineffective cough due to widened trachea and 2. Marom EMet al: Diffuse abnormalities of the trachea and
diverticula main bronchi. AJR.176;(3):713-7,2001
Loud, productive cough, hoarseness, dyspnea, 3. Webb EMet al: Using CT to diagnose nonneoplastic
recurrent pneumonias tracheal abnormalities: appearance of the tracheal wall. AJR
Am J Roentgenol. 174(5):1315-21,2000
Occasional hemoptysis Tanoue LTet al: Pulmonary involvement in collagen
4.
Spontaneous pneumothorax vascular disease: a review of the pulmonary manifestations
Clinical exam to determine other causes, i.e., of the Marfan syndrome, ankylosing spondylitis, Sjogren's
Ehlers-Danlos, Marfan, etc. syndrome, and relapsing polychondritis. J Thorac Imaging.
Finger clubbing 7(2):62-77, 1992
May be asymptomatic or have minor symptoms 5. Padley S et al: Tracheobronchomegaly in association with
Recurrent infections may lead to bronchiectasis and ankylosing spondylitis. Clin RadioI43(2):139-41, 1991
pulmonary fibrosis 6. Woodring et al: Acquired tracheomegaly in adults as a
complication of diffuse pulmonary fibrosis. AJR.152:
Obstructive airway disease from collapse of trachea
743-7, 1989
and major bronchi (tracheomalacia)
Bronchoscopy shows easy collapsibility of large
airways and diverticula
Pulmonary function tests: Increased dead space, total
lung capacity, residual volume and airflow obstruction
 TRACHEOBRONCHOMEGALY

I IMAGE GALLERY
Typical
(Left) Axial CECT in same
patient shows dilated trachea
with thinned wall (curved
arrow). Subpleural blebs at
both upper lobes (arrows)
indicate emphysema, due to
obstructive airways disease.
(Right) Axial CECT in same
patient shows dilated
ma;nstem bronchi (curved
arrows), emphysema
(arrows) and left upper lobe
3
scars (open arrow).
Diagnosis: Mounier-Kuhn 9
syndrome.

Typical
(Left) Axial CECT in same
patient shows bronchiectasis
at both upper lobes
(arrows), emphysema (open
arrow) and the left upper
lobe scars (curved arrow).
(Right) Axial CECT in same
patient shows left lower lobe
consolidation indicating
pneumonia (open arrow),
right lower lobe
bronchiectasis (curved
arrow) and lower lobe
emphysema (arrows).

(Left) Frontal radiograph

shows normal tracheal
caliber at the cervical
trachea (arrow). The
intrathoracic trachea and
mainstem bronchi are dilated
(open arrows). Diagnosis:
Maunier Kuhn syndrome
(Right) Frontal radiograph
bronchogram shows a
corrugated dilated trachea
(open arrow) and right
upper lobe bronchiectasis
(arrow). Bronchography has
been replaced by HRCT.
Diagnosis: MounierKuhn.
 IMMOTILE CILIA SYNDROME

3
10

Frontal radiograph shows situs solitus, bibasilar Axial CECT shows right middle lobe cysUc
bronchial wall thickening (arrows) and right lower lobe bronchiectasis (open arrow), left lower lobe cylindrical
bronchiectasis (open arrow). Electron microscopy bronchiectasis (arrow) and centrilobular nodules
showed ciliaryultrastructuredefects. (curved arrow). Primaryciliarydyskinesia syndrome.

Size: Dilated bronchi, variable caliber from mild to

!TERMINOlOGY severe dilation and cystic change
Abbreviations and Synonyms Morphology: Dilated airways, consolidation
Primary ciliary dyskinesia syndrome (PCD), Kartagener Radiographic Findings
syndrome Situs inversus or dextrocardia (50%), paranasal
Definitions sinusitis, bronchiectasis
Primary ciliary dyskinesia syndrome: Situs solitus, Situs solitus, 50%
chronic sinusitis, bronchiectasis Bronchial wall thickening, segmental atelectasis,
segmental bronchiectasis (often lower lobes)
Kartagener syndrome: Subset of PCD
o 50% of cases of PCD Hyperinflation
o Situs inversus or dextrocardia, nasal polyposis with Recurrent pneumonias
chronic sinusitis, bronchiectasis CT Findings
Functional and/or structural abnormalities of cilia and
NECT
spermatozoa o Bronchial wall thickening
o Signet ring sign: Dilation of bronchi, diameter
greater than accompanying pulmonary artery
IIMAGING FINDINGS CT section perpendicular to bronchus and artery
The "ring" is the dilated bronchus and the "pearl"
General Features
is the accompanying pulmonary artery
Best diagnostic clue: Bronchiectasis, dextrocardia and
o Variable severity of bronchiectasis: Cylindrical,
sinusitis
varicose, cystic bronchiectasis
Location: Bronchiectasis, predilection for middle lobe,
o Bronchiolectasis: Tree in bud, V and Y shaped
lower lobes
peripheral centrilobular opacities

DDx: Chronic Sinusitis and Bronchiectasis

Young Syndrome Cystic Fibrosis Immune Deficiency

 IMMOTILE CILIA SYNDROME

Key Facts
Imaging Findings Top Differential Diagnoses
Situs inversus or dextrocardia (50%), paranasal Young Syndrome
sinusitis, bronchiectasis Sinobronchial Allergic Mycosis
Situs solitus, 50% Cystic Fibrosis
Recurrent pneumonias
Variable severity of bronchiectasis: Cylindrical, Pathology
varicose, cystic bronchiectasis Abnormal ciliary function eventually results in stasis
Bronchiolectasis: Tree in bud, V and Y shaped of secretions in airways, recurrent infections and
peripheral centrilobular opacities bronchiectasis
Peribronchial or confluent airspace opacities Cilia with missing dynein arms, central microtubule
representing pneumonia pairs, inner sheath, radial spokes, or nexin links
Areas of decreased attenuation suggest small airways
disease
Clinical Issues
Infertile males due to immotile spermatozoa
3
Diffuse centrilobular small nodules up to 2 mm in
diameter Gender: Male:Female = 1:1 11
Good prognosis, compatible with normal lifespan

o Peribronchial or confluent airspace opacities
representing pneumonia
o Areas of decreased attenuation suggest small airways
disease
Expiration CT should show air trapping in these
regions
o Pattern of mosaic perfusion may represent
obliterative bronchiolitis
HRCT
o Bronchiectasis and bronchiolectasis, right middle
and lower lobe predominance
o Diffuse centrilobular small nodules up to 2 mm in
diameter
Represents membranous bronchiolitis and
peribronchiolitis
Imaging Recommendations
Best imaging tool
o Chest radiography usually sufficient for diagnosis
o HRCT may be useful to determine presence and
extent of bronchiectasis
o More sensitive for recurrent pneumonias involving
mostly lower lobes and right middle lobe
Protocol advice
o HRCT: Thin-section widths 1-2 mm at 10 mm
intervals without IV contrast
o Window width, approx. 1600 HU, level,
approximately-600
History of asthma, central bronchiectasis with mucoid
impactions, aspergillus sinusitis
No ciliary immotility
Cystic Fibrosis
Genetic disorder, upper lobe bronchiectasis, mucoid
impactions, nasal polyps and chronic sinusitis
Positive sweat test, no ciliary immotility
Immune Deficiency Disorders
Bruton disease, common variable immunodeficiency,
selective immunoglobulin deficiency, AIDS, etc.
o Recurrent pneumonias, bronchiectasis, chronic
sinusitis
o History of immune deficiency disorder
o No ciliary immotility
Yellow Nail Syndrome
Recurrent sinopulmonary infections
Bronchiectasis (25%), pleural effusions
Hypogammaglobulinemia
Yellow nails
No ciliary immotility
Diffuse Panbronchiolitis
Common in Asians
Chronic sinusitis, bronchial inflammation, marked
bronchiectasis, chronic respiratory failure
Responds to erythromycin treatment
No ciliary immotility

I DIFFERENTIAL DIAGNOSIS
Young Syndrome
Chronic sinopulmonary infections
Persistent azoospermia, obstruction
with inspissated secretions
Normal spermatogenesis
No ciliary immotility
Sinobronchial Allergic Mycosis
Allergic bronchopulmonary aspergillosis
Allergic aspergillus sinusitis
I PATHOLOGY
General Features
of the epididymis General path comments: Electron microscopy show
ciliary structure abnormalities
Genetics: Autosomal recessive
Etiology
o Compromised mucociliary transport secondary to
dynein protein structural and functional
abnormalities
o Uncoordinated and ineffective motion of cilia
and/or spermatozoa
 IMMOTILE CILIA SYNDROME
Lack of ciliary motion results in dextrocardia (no
in utero cardiac rotation)
a Abnormal ciliary function eventually results in stasis
of secretions in airways, recurrent infections and
bronchiectasis
Epidemiology
a 1 in 20,000 births
a 20% of patients with dextrocardia have Kartagener
syndrome
Associated abnormalities
a Pyloric stenosis, hypospadias, post-cricoid web
(Paterson-Brown-Kelly syndrome)
a Congenital heart disease, comparable to general
population
3 Corrected transposition of the great vessels,
trilocular or bilocular heart
12
Gross Pathologic & Surgical Features
Dextrocardia or situs inversus
Diffuse bronchiectasis
Microscopic Features
Electron microscopy
a Cilia with missing dynein arms, central microtubule
pairs, inner sheath, radial spokes, or nexin links
a Normally, cilia have 2 central microtubules
connected by radial spokes to 9 outer doublet
microtubules
Disordered ciliary beating and disordered ciliary arrays
on epithelial cell surfaces
a Dyskinetic cilia throughout the body, including
nasal and bronchial cilia
Immotile spermatozoa
Defective neutrophil chemotaxis
Bronchiolitis: Infiltration with lymphocytes, plasma
cells and neutrophils
Obliterative bronchiolitis: Plugging of membranous
bronchioles with granulation tissue
ICLINICAL ISSUES
Presentation
Most common signs/symptoms: Recurrent sinus, ear
and pulmonary infections, male infertility
Productive cough, wheezing, coarse crackles,
exertional dyspnea
Chronic rhinitis, sinusitis, nasal polyposis, otitis media
Recurrent bronchitis, bronchiectasis, small airways
disease
a Due to absent or reduced tracheobronchial
mucociliary clearance
Pneumonias, often with Haemophilus influenzae or
Pseudomonas
Corneal abnormalities, poor sense of smell
Infertile males due to immotile spermatozoa
Female infertility, uncommon; due to immotile cilia in
the fallopian tubes
Diagnosis confirmed by biopsy of nasal and bronchial
epithelium
a Demonstration of ultrastructure defects with
electron microscopy
Demographics
Age: Abnormality present at birth
Gender: Male:Female = 1:1
Natural History & Prognosis
Airways normal at birth
May present as respiratory distress in neonates
Onset of symptoms in childhood or adolescence with
bronchiectasis and recurrent pneumonias
Sequelae of recurrent pneumonias
a Pulmonary scarring, honeycombing, cysts,
bronchiectasis, small airways disease
a Pneumothoraces, due to rupture of blebs, cysts and
cavities into the pleural space
a Aspergillomas, saprophytic infection in pre-existing
cavities
a Hemorrhage, the result of bronchiectasis and
mycetomas
Disability due to severity of bronchiectasis
Good prognosis, compatible with normal lifespan
a Better prognosis than cystic fibrosis
Fatalities from progressive respiratory failure,
pulmonary artery hypertension
Treatment
Rigorous lung physiotherapy with postural drainage
Prophylactic and organism specific antibiotics against
common pulmonary pathogens
Advanced disease
a Surgical intervention for bronchiectasis
a Lung transplantation for end-stage lung disease
Genetic counseling
I DIAGNOSTIC CHECKLIST
Consider
Obtaining paranasal sinus radiographs in patients with
bronchiectasis
Image Interpretation Pearls
Half of patients with PCD have situs solitus
I SELECTED REFERENCES
1. Homma S et al: Bronchiolitis in Kartagener's syndrome. Eur
RespirJ. 14(6):1332-9, 1999
2. Tsang KW et al: Clinical profiles of Chinese patients with
diffuse panbronchiolitis. Thorax. 53(4):274-80,1998
3. Coleman LTet al. Bronchiectasis in children. J Thorac
Imaging. 10(4):268-79, 1995
4. Nadel HR et aI: The immotile cilia syndrome: Radiological
manifestations. Radiology. 154:651-5, 1985
5. Handelsman et al: Young's syndrome. Obstructive
azoospermia and chronic sinopulmonary infections N Engl
J Med. 310:3-9,1984
6. Eliasson R et al: The immotile-cilia syndrome. A congenital
ciliary abnormality as an etiologic factor in chronic airway
infections and male sterility. N Engl J Med. 297:1-6, 1977
 IMMOTILE CILIA SYNDROME
I IMAGE GALLERY
Typical
(Left) Frontal radiograph in a
patient with dextrocardia
(arrow) and chronic sinusitis
shows acute right lower lobe
pneumonia (open arrow).
(Right) Axial NEeT in same
patient shows bronchial wall
thickening, bronchiectasis
(curved arrow) and small
nodules (open arrow). Note
the right lower lobe
pneumonia (arrow).
3
Diagnosis: Kartagener
syndrome. 13

Typical
(Left) Frontal radiograph in a
40 year infertile male shows
opacified left frontal sinus
(curved arrow) and mucosal
thickening in both maxillary
sinuses (arrows). He had a
history of wheezing since
childhood and situs inversus
(not shown). (Right)
Bronchogram in left posterior
oblique position in same
patient shows crowded
airways and bronchiectasis
a t the left lower lobe
(arrow). He had cor
pulmonale. Diagnosis:
Kartagener syndrome.

Variant
(Left) Frontal radiograph in a
patient with immotile cilia
shows dextrocardia (arrow)
and clear lungs. The
left-sided stomach (curved
arrow) and right-sided liver
indicates abdominal situs
solitus. (Right) Axial NEeT in
same patient shows thoracic
situs ;nversus and mild
bronchiectasis at the
left-sided middle lobe
(arrow). Diagnosis:
Kartagener syndrome and
heterotaxy syndrome.
 BRONCHIAL ATRESIA

3
14

Frontalradiograph shows large ellipucalsharply defined Axial HRCT in different patient shows well-defined
perihilar mass (arrow) in the right upper lobe. Distal hyperlucent subsegment (curved arrows) and small
lung hyperlucent (curved arrow). Mucoid impaction bronchocele and mucoid impacuon (arrows) in the
(open arrow). Bronchial atresia. rightlower lobe. Bronchialatresia.

Central nodule or mass representing mucoid

ITERMINOlOGY impaction distal to the atretic bronchus
Definitions (bronchocele)
Congenital atresia of segmental bronchus with normal Hyperlucency of affected segment
distal architecture Hypoperfusion of affected segment with paucity
of vessels
a Bronchocele
Sharply defined rounded or tubular branching
IIMAGING FINDINGS opacities adjacent to the hilum (tear drops,
General Features grape-like clusters, gloved finger appearance)
Best diagnostic clue: Round, sharply-defined, perihilar Bronchocele points towards the hilum
mass with distal hyperinflation Blunt horn-like protrusions distal to the mass
Location: Apicoposterior segment left upper lobe (mucoid impaction in bronchiectatic bronchi)
(50%), followed by right upper lobe (20%), lower lobes Occasionally have air-fluid level
(15% each) and rarely right middle lobe 5%) a Hyperlucent lobe
Size: Bronchocele usually greater than 1 cm in Neonates: Lobe or segment distal to atretic
diameter bronchus fluid-filled, gradually replaced by air
Morphology: Atretic bronchus usually segmental but Resorption of fluid shown to occur within 1st
may be lobar or distally in subsegmental airways week of life
Ventilation from collateral air drift via
Radiographic Findings intraalveolar pores of Kohn, bronchoalveolar
Radiography channels of Lambert across incomplete
a Typical triad intrapulmonary fissures
a Boomerang sign

DDx: Congenital Lucent Lung Lesions

Bronchial Atresia
 BRONCHIAL ATRESIA

Key Facts
Terminology Intralobar Pulmonary Sequestration
Congenital atresia of segmental bronchus with Intrapulmonary Bronchogenic Cyst
normal distal architecture Arteriovenous Malformation
Allergic Bronchopulmonary Aspergillosis (ABPA) or
Imaging Findings Cystic Fibrosis
Location: Apicoposterior segment left upper lobe Solitary Pulmonary Nodule (SPN)
(50%), followed by right upper lobe (20%), lower Carcinoid Tumor, Slow Growing Endobronchial
lobes (15% each) and rarely right middle lobe 5%) Tumor
Central nodule or mass representing mucoid
impaction distal to the atretic bronchus
Clinical Issues
(bronchocele) Often asymptomatic, may not come to attention
Hyperlucency of affected segment until adulthood (50%)
Hypoperfusion of affected segment with paucity of Surgical resection for those with complications:
Recurrent pneumonia or encroachment on normal
3
vessels
pulmonary structures 15
Top Differential Diagnoses
Congenital Lobar Emphysema

Parabolic curve: Junction of the hyperinflated

segment with adjacent normal lung
I DIFFERENTIAL DIAGNOSIS
CT Findings Mucoid Impaction with Hyperinflation
Bronchial atresia
CECT
o Triad: Bronchocele, hyperlucent and hypo perfused Intralobar sequestration
segment diagnostic of bronchial atresia Intrapulmonary bronchogenic cyst
o Bronchocele Congenital Lobar Emphysema
No enhancement of the bronchocele No bronchocele
May be of lower attenuation due to mucoid Left upper lobe also most commonly affected
material Hyperinflated lobe causes mass effect with shift of
Continuation of bronchocele with mucoid filled mediastinum away from the affected lobe
bronchi Usually diagnosed in infancy with respiratory distress
o Rare systemic arterial supply (bronchoarterial
malinosculation) Intralobar Pulmonary Sequestration
Malinosculation: Overlap syndromes May have distal hyperinflation
Abnormal systemic arterial supply, usually from aorta
Nuclear Medicine Findings Most common location left lower lobe in the
V/QScan paravertebral angle
o Hypoperfusion of affected segment
o Delayed ventilation of affected segment with Intrapulmonary Bronchogenic Cyst
delayed washout (air trapping) Usually located in the medial one-third of the lung in
the lower lobes
Ultrasonographic Findings May have distal hyperinflation
Can be detected in utero Cyst may be fluid-filled, air-filled or both (air-fluid
Fluid-filled upper lobe level)
o Differential
Cystic adenomatoid malformation Arteriovenous Malformation
Congenital diaphragmatic hernia Abnormal feeding artery and draining vein
Bronchopulmonary foregut malformations Nodule will enhance with contrast administration
Congenital lobar emphysema No bronchial obstruction, no hyperlucency or
hyperinflation
Imaging Recommendations
Best imaging tool: CT procedure of choice to Mucoid Impaction Associated Conditions
characterize bronchocele, airway anatomy, and distal Endobronchial lesion
hyperinflated lung and other associated anomalies o Bronchogenic carcinoma, extrinsic compression,
Protocol advice foreign body, adenoma
o Expiratory CT demonstrates accentuated Inflammatory
hyperinflation of affected segments o Tuberculosis, cystic fibrosis, asthma, ABPA
o Multidetector CT useful in demonstrating the
anatomy of the atretic bronchus Allergic Bronchopulmonary Aspergillosis
(ABPA) or Cystic Fibrosis
Central bronchiectasis
 BRONCHIAL ATRESIA
Bilateral disease usually more severe in upper lung o Often asymptomatic, may not come to attention
zones especially the right upper lobe until adulthood (50%)
May have mucoid impaction o History of recurrent infections (nearly 20%)
Distal lung usually abnormal; small airways disease - o Decreased breath sounds or wheeze over affected
tubular branching opacities, hyperinflation segment
Other signs/symptoms
Solitary Pulmonary Nodule (SPN) o Bronchoscopy usually not helpful in demonstrating
No hyperinflation distal to nodule the blind-ending bronchus
Typically no mucoid impaction unless lesion Useful if endobronchial lesion cannot be excluded
endobronchial or compresses bronchus
Demographics
Carcinoid Tumor, Slow Growing Age: Up through adulthood, average age at diagnosis
Endobronchial Tumor 22
Mass not as large as mucoid impaction Gender: M:F = 2:1
3 Distal lung usually not hyperexpanded but atelectatic
Natural History & Prognosis
16 Excellent
I PATHOLOGY Treatment
General Features None for asymptomatic patients
Surgical resection for those with complications:
General path comments
Recurrent pneumonia or encroachment on normal
o Obliteration short segment proximal lumen of
pulmonary structures
segmental bronchus
o Aeration distal lung through collateral air drift
across incomplete intrapulmonary fissures
o Distal lung: Normal bronchial architecture but
I DIAGNOSTIC CHECKLIST
alveoli hypoplastic Consider
Etiology Slow growing endobronchial tumor
o Two theories of pathogenesis
o Disconnected cells from bronchial bud Image Interpretation Pearls
Thought to occur between 5th and 6th week of Perihilar nodule or tubular opacity
gestation Distal hyperinflation of affected segment
Cluster of developing cells loses communication Decreased vascularity of affected segment
with a bronchial bud but continues to branch
o Vascular injury
Thought to occur between 5th and 15th week of I SELECTED REFERENCES
gestation
1. Agarwal PP et al: An unusual case of systemic arterial
In-utero vascular insult may lead to bronchial supply to the lung with bronchial atresia. AJRAmJ
injury Roentgenol. 185(1):150-3,2005
Epidemiology: 2nd most common congenital 2. Kamata S et al: Case of congenital bronchial atresia
tracheobronchial malformation after pulmonary detected by fetal ultrasound. Pediatr Pulmonol.
sequestration 35(3):227-9,2003
Associated abnormalities: Bronchogenic cyst, 3. Matsushima H et al: Congenital bronchial atresia:
pulmonary sequestration, congenital adenomatoid radiologic findings in nine patients. J Comput Assist
Tomogr. 26(5):860-4, 2002
malformation, congenital lobar emphysema,
4. Zylak CJ et al: Developmental lung anomalies in the adult:
anomalous pulmonary venous return, aplastic or radiologic-pathologic correlation. Radiographies. 22 Spec
hypoplastic lung, pericardial defect No(S25-43, 2002
5. Petrozzi MC et al: Bronchial atresia: clinical observations
Gross Pathologic & Surgical Features and review of the literature. Clin Pulm Med. 8(2):101- 7,
Mucoid-filled lung-forming mass distal to atretic 2001
bronchus 6. Miyahara N et ai: Bronchial atresia with transient
Distal lung hyperinflated but otherwise normal, no spontaneous disappearance of a mucocele. Intern Med.
anthracotic pigmentation 38(12):974-8,1999
7. Ouzidane L et al: Segmental bronchial atresia--a case report
Microscopic Features and a literature review. EurJ Pediatr Surg. 9(1):49-52,1999
No specific features, nonspecific inflammation distal 8. Ward S et al: Congenital bronchial atresia--presentation of
to atresia three cases and a pictorial review. Clin Radial. 54(3):144-8,
1999
9. Kinsella D et al: The radiological imaging of bronchial
atresia. BrJ Radiol. 65(776):681-5,1992
IClINICAllSSUES 10. Kuhn C et al: Coexistence of bronchial atresia and
bronchogenic cyst: diagnostic criteria and embryologic
Presentation considerations. Pediatr Radiol. 22(8):568-70, 1992
Most common signs/symptoms
 BRONCHIAL ATRESIA
I IMAGE GALLERY

(Left) Frontal radiograph

shows sharply defined
perihilar nodule right lung
(arrow). Faint tubular
densities below main nodule
(open arrows). (Right) Axial
CECT shows air-fluid level
(arrow) in dilated bronchus
(curved arrow). Rare atretic
5ubsegmental bronchus in
right-middle lobe. 3
17

(Left) Frontal scanogram

shows sharply defined left
perihilar nodule (arrow).
Nodule is elliptical in shape
with long axis pointing
towards hilum. (Right) Axial
NECT shows tubular mass
(arrow) adjacent to left
hilum. Distal lung is
hyperlucent and
hypoperfused (curved
arrows).

(Left) Axial NECT again

demonstrates the mucoid
impaction with large
branches arising from the
tubular opacity (arrows).
Distal lung hyperlucent.
(Right) Axial NECT shows
air-fluid level in superior
aspect of bronchocele
(arrow). Apical-posterior
segment hyperlucent and
hypoperfused. Bronchial
atresia.
 ANOMALOUS BRONCHI

3
18

True righllracheal bronchus ("Pigrr bronchus). Coronal Corresponding oblique surface shaded display shows
multiplanar reconstruction shows a tracheal bronchus the RUL bronchus displaced on the trachea (arrows).
(arrow) 2 cm proximal to the carina. Rounded Note the spliWng of the 82 and 83 bronchi.
pneumonia is seen in RUL.

o Anomalies arising from sites typically lacking

ITERMINOlOGY branches
Abbreviations and Synonyms Tracheal bronchus
Congenital anomalies of bronchi Bridging bronchus
Tracheal bronchus: "Pig" bronchus Accessory cardiac bronchus
o Anomalies associated with abnormalities of situs
Definitions Bronchial isomerism: Bilateral left-sided or
Different congenital variations in the number, length, right-sided airway anatomy
diameter, and position of the bronchi o Congenital bronchial atresia
o Agenesia-hypoplasia complex
CT Findings
I IMAGING FINDINGS Accessory superior segmental bronchi
General Features o Two closely aligned bronchi both supplying the
Best diagnostic clue: Variations in the number, length, superior segment of the right lower lobe (RLL)
diameter, and position of the bronchi Axillary bronchi
Location: Variable o Supernumerary segmental bronchus supplying the
Size: Variable lateral aspect of the right upper lobe (RUL)
Other general features Tracheal bronchus
o Anomalies arising from normal higher-order o Various types
bronchial divisions Supernumerary (23%): Coexist with a normal
Accessory superior segmental bronchi branching of upper lobe bronchus
Axillary bronchi

DDx: Focal Recurrent Pulmonary Infections

CCAM Type 1
 ANOMALOUS BRONCHI

Key Facts
Terminology Top Differential Diagnoses
Congenital anomalies of bronchi Endobronchial Obstruction
Aspiration
Imaging Findings Intralobar Pulmonary Sequestration
Best diagnostic clue: Variations in the number, Congenital Cystic Adenomatoid Malformation
length, diameter, and position of the bronchi
Accessory superior segmental bronchi Clinical Issues
Axillary bronchi Usually asymptomatic
Tracheal bronchus Tracheal bronchus, accessory cardiac bronchus, and
Bridging bronchus congenital bronchial atresia: Recurrent infections,
Accessory cardiac bronchus atelectasis, or bronchiectasis
Bronchial isomerism Prognosis: Very good
Congenital bronchial atresia Usually no treatment 3
Agenesis-hypoplasia complex
Best imaging tool: MDCT; consider multiplanar Diagnostic Checklist 19
reformations Anomalous bronchial abnormalities should be
suspected in recurrent pneumonia and/or atelectasis

Displaced (77%): In addition to the aberrant

bronchus one branch of the upper lobe bronchus
is lacking
a Most cases located in the right side
a Tracheal origin of the right upper lobe bronchus
("pig" bronchus)
a Arises from right lateral wall of the trachea
a Usually within 2 em of the carina and up to 6 em
from the carina
a Variable length, sometimes reduced to a
blind-ending pouch
a Left tracheal bronchus: Early origin of the
apicoposterior left upper lobe (LUL) bronchus from
the terminal portion of the left main bronchus
Bridging bronchus
a Ectopic bronchus arising from the left mainstem
bronchus
a Crosses through the mediastinum to supply the RLL
Accessory cardiac bronchus
a Distinct airway originating in the medial wall of the
main bronchus or bronchus intermedius
a Cephalic to the origin of the middle lobe bronchus
a Located in the azygo-esophageal recess
a Demarcated from the RLL by an anomalus fissure
a Associated consolidation (pneumonia)
Bronchial isomerism
a Pattern of bronchial branching and pulmonary lobe
formation identical in both lungs
Bilateral left-sided airway anatomy
Bilateral right-sided airway anatomy
a Equal number of bronchi within each lung
Congenital bronchial atresia
a Ovoid or tubular opacity near the hilum
a Segmental hyperlucency and decreased vascularity
a Air-trapping on expiratory CT scans
Agenesis-hypoplasia complex
a Agenesia: Total absence of bronchus and lung
a Aplasia: Total absence of the lung with a
rudimentary main bronchus
a Hypoplasia: Hypoplastic bronchi and an associated
variable amount of lung tissue
Imaging Recommendations
Best imaging tool: MDCT; consider multi planar
reformations
I DIFFERENTIAL DIAGNOSIS
Endobronchial Obstruction
Children: Foreign bodies
Adults: Bronchogenic carcinoma
a 10% of nonresolving pneumonias due to underlying
carcinoma
May have evidence of volume loss in addition to
chronic consolidation
CT useful to exclude airway obstruction
Aspiration
Predisposing conditions: Alcoholism, neuromuscular
disorders, structural abnormalities of esophagus, reflux
disease
Recurrent opacities in dependent locations
May be unilateral
Esophagram useful to determine esophageal motility
and evaluate for reflux
Intralobar Pulmonary Sequestration
Abnormal pulmonary tissue that does not
communicate with the tracheobronchial tree with a
normal bronchial connection
Anomalous systemic vascularization
Recurrent pulmonary infections usually in the LLL
Congenital Cystic Adenomatoid
Malformation
Usually manifest in the neonatal period
Hamartomatous pulmonary lesion
Recurrent episodes of pneumonia
 ANOMALOUS BRONCHI
I PATHOLOGY
General Features
Etiology
o Congenital
o Pathogenesis
Controversial
Various developmental theories: Reduction,
migration, and selection
Tracheal bronchus: Occurs 29-30 days after
starting differentiation of lobar bronchi
Accessory cardiac bronchus: Always a
supernumerary bronchus
3 Congenital bronchial atresia: Focal obliteration of
a segmental bronchus with normal distal
20 structures, usually involves the upper lobes, in
particular the apicoposterior segment of the LUL
Epidemiology
o Prevalence
Proximal or distal segmental or subsegmental
bronchial displacement is seen in 10% of
individuals
Right tracheal bronchus: 0.1-2%
Left tracheal bronchus: 0.3-1%
Accessory cardiac bronchus: 0.09-0.5%
Associated abnormalities
o Congenital diaphragmatic hernias (CDH) sometimes
are associated with airway anomalies such as
congenital stenosis, abnormal branching of the
bronchi, and pulmonary hypoplasia
o Variable bronchial branching associated with
isolated lobar agenesis, aplasia, or hypoplasia
(hypogenetic lung syndrome)
o Isomerism with bilateral left-sided airway anatomy:
Associated with venolobar syndrome, absence of the
inferior vena cava and azygous continuation, and
polysplenia
Absence of inferior vena cava (IVC) visible on
lateral radiograph and azygous continuation on
frontal radiograph
o Isomerism with bilateral right-sided airway
anatomy: Associated with asplenia and severe
congenital heart disease
o Agenesis-hypoplasia complex: May be associated
with partial anomalous venous return (congenital
pulmonary venolobar syndrome) and pectus
excavatum
I CLINICAL ISSUES
Presentation
Most common signs/symptoms
o Usually asymptomatic
o Tracheal bronchus, accessory cardiac bronchus, and
congenital bronchial atresia: Recurrent infections,
atelectasis, or bronchiectasis
Other signs/symptoms: Intubated patients with
tracheal bronchus may have recurrent or chronic
partial atelectasis of the upper lobe
Demographics
Age: Any age
Natural History & Prognosis
Prognosis: Very good
Treatment
Usually no treatment
Treat complications
o Antibiotics for infections
o Surgery in complicated cases
I DIAGNOSTIC CHECKLIST
Consider
Anomalous bronchial abnormalities should be
suspected in recurrent pneumonia and/or atelectasis
Knowledge of bronchial abnormalities is necessary for
fiberoptic bronchoscopy, endobronchial treatment,
transplantation
I SElECTED REFERENCES
1. Naidich DP et al: Imaging of the airways. Functional and
radiologic correlations. Philadelphia: Lippincott Williams &
Wilkins, 2005
2. Berrocal T et al: Congenital anomalies of the
tracheobronchial tree, lung, and mediastinum:
embryology, radiology, and pathology. Radiographies.
24:eI7- 62, 2003
3. Zylak CJ et al: Developmental lung anomalies in the adult:
radiologic-pathologic correlation. Radiographies Spec.
No:S25-43, 2002
4. Ghaye Bet al: Congenital bronchial anomalies revisited.
RadioGraphies. 21:105-119,2001
5. Nose Ket al: Airway Anomalies in Patients With
Congenital Diaphragmatic Hernia. J Pediatr Surg.
35:1562-1565,2000
6. Ghaye B et al: Accessory cardiac bronchus: 3D CT
demonstration in nine cases. Eur Radiol. 9:45-48, 1999
7. WUJW et al: Variant bronchial anatomy: CT appearance
and classification. AJRAm J Roentgenol. 172:741-744, 1999
8. C. Beigelman et al: Congenital anomalies of
tracheobronchial branching patterns: spiral CT aspects in
adults. Eur Radiol. 8:79-85, 1998
9. Keane MP et al: Accessory cardiac bronchus presenting
with hemoptysis. Thorax. 52:490-491, 1997
10. Mata JM et al: The dysmorphic lung: imaging findings. Eur
Radiol. 6: 403-414, 1996
11. Rappaport DC et al:Congenital bronchopulmonary diseases
in adults: CT findings. AJR. 162: 1295-1299, 1994
12. McGuinness G et al: Accessory cardiac bronchus: CT
features and clinical significance. Radiology. 189: 563-566,
1993
13. Keslar P et al: Radiographic manifestations of anomalies of
the lung. Radiol Clin North Am. 29: 255-270,1991
14. Mata JM et al: CT of congenital malformations of the lung.
Radiographies. 10: 651- 74, 1990
15. Shipley RTet al: Computed tomography of the tracheal
bronchus. J Comput AssistTomogr. 9: 53-55, 1985
16. Yamashita H: Roentgenologic anatomy of the lung.
Stuttgart: Thieme Medical Publishers, 1978
17. Gonzalez-Crussi F et al: "Bridging bronchus" a previously
underscribed airway anomaly. Am J Dis Child.
130:1015-1018,1976
18. Boyden EA:The nomenclature of the bronchopulmonary
segments and their blood supply. Dis Chest. 39:1-6,1961
19. Brock RC:The anatomy of the bronchial tree. Oxford
University. Press, London,1946
 ANOMALOUS BRONCHI

I IMAGE GALLERY
Typical
(Left) Accessory cardiac
bronchus with ventilated
lobulus in an asymptomatic
patient. Coronal MOCT
shows the accessory cardiac
bronchus (arrow) arising
from the intermediate
bronchus. (Right)
Corresponding axial CT
shows an accessory cardiac
bronchus (arrow) separated
by a spur from the proximal
3
part of the intermediate
bronchus (curved arrow). 21

(Left) CT scan of the same

patient obtained more
distally shows a small
ventilated cardiac lobulus
separated from the RLL by an
anomalous fissure (arrow).
(Right) Coronal MOCT of a
patient with heterotaxy
(polysplenia) syndrome
shows bilateral hyparterial
bronchial branching pattern.
(arrows).
Azygous-hemiazygous
continuation is also seen
(open arrow).

Typical
(Left) Axial NECT in a patient
with absent IVC shows a
significant dilatation of the
azygous vein (arrow) and
the characteristic bilateral
hyparterial bronchial
branching pattern
(isomerism). (Right)
Bronchial atresia in a patient
with a left pleural effusion.
NECT shows a small ovoid
lesion (mucocele) in the LLL
(open arrow). Note the
hyperlucent lung
surrounding the lesion
(arrows).
 ALPHA-1 ANTIPROTEASE DEFICIENCY

3
22

Coronal CECT in alpha-l-antiuYPsin deficiency shows Axial CECT in alpha-l-antiuypsin deficiency induced
flanened hemi-diaphragms (curved arrows), areas of liver cirrhosis shows a small liver with an noc/ular
panlobular lung desuuction (arrows), and subpleural contour (arrows), combined with perihepatic ascites
bullae (open arrows). (open arrows).

a Basal emphysema
I TERMI NOLOGY a Combination with bullous disease
Abbreviations and Synonyms o Combination with liver cirrhosis
Alpha-l-antitrypsin deficiency Location: Lower lobe predominance

Definitions Radiographic Findings

Inherited deficiency of alpha-l-antitrypsin Indirect signs of emphysema: Hyperinflation
o So far, alpha-I-deficiency the only genetic o Flat diaphragms
abnormality specifically linked to chronic o Widened retrosternal air space
obstructive pulmonary disease o Widened retrocardiac space
a Common Pi ZZ phenotype: 1 in 2000 o Small and narrow heart
Liver disease in infancy o Lung height increased
a Commonly progresses to liver cirrhosis Direct signs: Emphysema
o Primarily involves lower lobes
Panlobular emphysema
a Emphysema develops prematurely, especially in o Arterial deficiency
smokers o Hypoattenuation
a Predominantly involves lower lobes o Bullae
a Commonly associated with bullous disease o "Increased markings"
Phenomenon is not clearly understood
Assumed to be combination of bronchial wall
thickening and "superimposed" emphysema
I IMAGING FINDINGS Secondary manifestations
General Features o Pulmonary arterial hypertension
Best diagnostic clue Enlarged pulmonary arteries

DDx: Lung Destruction

Centri/obu/ar Emphysema Bullae Langerhans Histiocytosis

 ALPHA-1 ANTIPROTEASE DEFICIENCY
Key Facts
Terminology Pathology
Inherited deficiency of alpha-I-antitrypsin Pi ZZ have 15% normal levels, need 35% to protect
Common Pi ZZ phenotype: 1 in 2000 from emphysema
Liver disease in infancy Epidemiology: As common as cystic fibrosis, Pi ZZ 1
Pan lobular emphysema in 2000
Imaging Findings Clinical Issues
Basal emphysema Most suffer from wheezing and exertional dyspnea
Combination with bullous disease Nearly 80% of patients have a positive family history
Combination with liver cirrhosis of lung disease
Nearly 25% of patients have a positive family history
Top Differential Diagnoses of liver disease
Centrilobular Emphysema Smoking an extremely important cofactor for the 3
Bullae development of disease in alpha-I-deficient
Langerhans Cell Histiocytosis individuals 23
Lymphangiomyomatosis Many cases are discovered as a consequence of family
Neurofibromatosis screening of emphysema patients

Peripheral arterial pruning

lymphangiomyomatosis
CT Findings Occurs in women only
HRCT Thin-walled cysts
o Not as easy to detect as centrilobular emphysema Chylous pleural effusion
o Insensitive to mild disease
o Extensive areas of low attenuation
Neurofibromatosis
o Reduction in size of pulmonary vessels Upper lobe bullae
o No normal lung to accentuate contrast differences Basilar interstitiai lung disease
o Abnormal lung "fades away" Cutaneous fibromas
o Bullae are common Neurofibromas, posterior mediastinal mass
o Bronchial wall thickening
o Bronchiectasis
I PATHOLOGY
Imaging Recommendations
Best imaging tool General Features
o HRCT more sensitive than chest radiography General path comments
o HRCT useful to confirm or exclude concurrent o Panlobular emphysema
bronchiectasis and bullous disease a Liver cirrhosis
Protocol advice: Thoracic CT should be complemented Genetics
by medical and imaging evaluation of the liver a Alpha-I-antitrypsin expressed primarily in the liver
and to a lesser degree in neutrophils and monocytes
a Hepatic alpha-I-antitrypsin escapes into general
I DIFFERENTIAL DIAGNOSIS circulation, where it counteracts neutrophil eiastase
a Alpha-I-antitrypsin blocks proteolytic enzymes
Centrilobular Emphysema a Coded by single gene chromosome 14
Confined to secondary pulmonary lobule o Single level determined by single allele derived from
More heterogeneous appearance than panlobuiar both pa ren ts
emphysema a Normal phenotype (Pi MM)
Not associated with widespread lung parenchymal a Pi MZ have 60% normal levels, no propensity for
destruction emphysema
a Pi ZZ have 15% normal levels, need 35% to protect
Bullae from emphysema
Thin-walled hole in the lung a Z variant single lysine for glutamic acid in M protein
Cavity contains no lung parenchyma Etiology
High natural contrast between normal and diseased a Elastase-antielastase hypothesis
lung Natural elastases from neutrophils and
langerhans Cell Histiocytosis macrophages normally neutralized by
antiproteases
Predominantly upper lung zone
Imbalance causes emphysema
Bizarre shaped cysts
Animal model: Instillation of papain ("meat
Cavitated micronoduies
tenderizer") will induce emphysema
 ALPHA-1 ANTI PROTEASE DEFICIENCY
Epidemiology: As common as cystic fibrosis, Pi ZZ 1 in Ethnicity: Caucasians
2000
Natural History & Prognosis
Gross Pathologic & Surgical Features Life expectancy decreased even in nonsmokers
Emphysema predominantly involves the lower lung Many cases are discovered as a consequence of family
zones screening of emphysema patients
Microscopic Features Treatment
Panlobular emphysema Rehabilitation
o Parenchymal destruction uniformly involves entire o Designed to optimize physical and social
secondary pulmonary lobule performance
o No evidence of fibrosis o Consists of exercise training, patient education,
psychosocial intervention, behavioral intervention,
and regular assessment of outcomes
3 ICLINICAL ISSUES Smoking cessation
o Cornerstone in the management of
24
Presentation alpha-I-antitrypsin deficiency treatment
Most common signs/symptoms Antibiotic prophylaxis
o In non-smokers, symptoms or signs rarely develop o Little evidence supports use of antibiotics in stable
before age 55 disease
o Smokers develop dyspnea age 40 o Yearly influenza vaccination recommended
Most suffer from wheezing and exertional dyspnea o Pneumococcal vaccination recommended
Chronic cough less common Oxygen
o Nearly 80% of patients have a positive family o Aimed to prevent cellular hypoxia with its
history of lung disease deleterious physiological consequences
o Nearly 25% of patients have a positive family Augmentation therapy with IV alpha-I-protease
history of liver disease inhibitor in selected patient groups
o Normal levels of alpha-I-antitrypsin are 20 to 48 o Shown to induce protective levels of
~mol/L alpha-I-antitrypsin in deficient individuals
o Alpha-I-antitrypsin levels should be obtained o Drawback: Expensive and inconvenient treatment
routinely for Lung transplantation
Chronic airflow obstruction in non-smokers o Should be actively considered when prognosis for
Chronic bronchitis in non-smokers emphysema worse than survival statistics for surgery
Patients with bronchiectasis and liver cirrhosis Lung volume reduction surgery
without apparent risks o Designed to relieve dyspnea and improve exercise
Premature emphysema function in severely disabled patients
Basal predominant emphysema Liver transplantation
Patients under age 50 with unremitting asthma o Considered in end-stage hepatic cirrhosis
Patients with a family history of alpha Gene therapy
I-antitrypsin deficiency o Still is in experimental status
o Smoking an extremely important cofactor for the
development of disease in alpha-I-deficient
individuals I SELECTED REFERENCES
Only few lifetime nonsmokers with Pi ZZ develop
1. Barnes PJet al: COPD: current therapeutic interventions
emphysema and future approaches. Eur RespirJ. 25(6):1084-106, 2005
Most have no symptoms, normal lung function, 2. Churg A et al: Proteases and emphysema. Curr Opin Pulm
and near-normal life span Med. 11(2):153-9,2005
Other signs/symptoms 3. McMahon MAet al: Alpha-l antitrypsin deficiency and
o Liver disease computed tomography findings. J Comput AssistTomogr.
Homozygous deficiency in infancy 29(4):549-53,2005
Hepatosplenomegaly, may lead to cirrhosis 4. RanesJet al: A review of alpha-l antitrypsin deficiency.
Hepatoma second most common cause of death Semin Respir Crit Care Med. 26(2):154-66, 2005
5. Strange C et al: Results of a Survey of Patients with Aipha-1
o Hepatic alterations in imaging should suggest liver Antitrypsin Deficiency. Respiration. 2005
cirrhosis 6. Perlmutter DH: Alpha-J-antitrypsin deficiency: diagnosis
Uneven nodular liver contour and treatment. Clin Liver Dis. 8(4):839-59, viii-ix, 2004
Enlarged or small liver 7. Tomashefski JFJr et al: The bronchopulmonary pathology
Inhomogeneous structure of hepatic parenchyma of alpha-J antitrypsin (AAT)deficiency: findings of the
Portal hypertension Death Review Committee of the national registry for
Porto-caval collateral pathways (varices) individuals with 5evere Deficiency of Alpha-J Antitrypsin.
Hum Pathol. 35(12):1452-61, 2004
Demographics 8. 5pouge D et al: Panacinar emphysema: CT and pathologic
Age: Typically mid-forties, with a forced expiratory findings. J Com put AssistTomogr 17:710-3, 1993
volume in one second and a pulmonary diffusing
9. Guest Pl et al: High resolution computed tomography
(HRCT)in emphysema associated with alpha-I-antitrypsin
capacity at or below the 50% predicted levels deficiency. Ciin Radioi 45:260-6, 1992
 ALPHA-1 ANTIPROTEASE DEFICIENCY
I IMAGE GALLERY

(Left) Axial CECT in

alpha-I-antitrYPsin
deficiency shows diffuse
bilateral destruction of lung
parenchyma resulting in
diffuse hypoattenuation.
(Right) Axial CECT in
alpha-I-antitrypsin
deficiency shows extensive
bullae (arrows) in the lower
lobes. 3
25

(Left) Axial CECT in

alpha-I-antitrypsin
deficiency shows ill-defined
but focal areas of
parenchymal destruction
resulting in focal
hypoattenuation (open
arrows). (Right) Axial HRCT
in alpha-I-antitrypsin
deficiency shows subtle
areas of hypoattenuation
(open arrows) suggestive of
emphysematous
parenchymal destruction.

Typical
(Left) Axial CECT in
alpha-I-antitrypsin
deficiency induced liver

Iiii'~'
cirrhosis shows signs of
portal hypertension (arrows)
and venous porto-caval
collaterals (open arrows).
(Right) Axial T7 C+ FS MR in
J I alpha-I-antitrypsin

~~ <.' '4 ..
deficiency induced liver
cirrhosis shows regenerate
nodules
liver.
(arrows) in cirrhotic

\"
 PARATRACHEAL AIR CYST

3
26

Graphic shows paratracheal air cyst (arrow) with Axial CECT shows small right paratracheal cyst (arrow)
narrow communication with the trachea. Cysts are most at the level of lhe thoracic inlel. No wall thickening or
common on lhe right at the level of the thoracic inlet fluid. No demonstrable communication.
bul can occur anywhere along lhe trachea. Emphysemalous change al the right apex.

Multiplanar reformations may be useful to

I TERMINOlOGY demonstrate communication
Abbreviations and Synonyms o Enlarge on expiration, shrink on inspiration
Tracheal diverticulum, tracheocele, lymphoepithelial o Solitary cyst typically S-20 mm in size
cyst Rarely multiple
o Usually associated with emphysema
Definitions
Mucosal herniation through the tracheal wall from
Imaging Recommendations
increased intraluminal pressure Best imaging tool: CT, chest radiographs only 15% of
para tracheal cysts visualized
Protocol advice: HRCT to find channel
I IMAGING FINDINGS communicating with the trachea

General Features
Best diagnostic clue: Small rounded air-filled cyst in I DIFFERENTIAL DIAGNOSIS
the right para tracheal region at the thoracic inlet
Location: Right posterolateral tracheal wall in the
Paraseptal Bleb
thoracic inlet (> 95%) Usually several blebs aligned in rows along the pleura
Size: Usually < 2 cm in diameter Zenker Diverticulum
Morphology Usually located more cephalad and often contain fluid
o No calcification or air-fluid level or lung markings
o Wall thickening uncommon (33%) Apical Lung Hernia
o Rarely tracheal communication identified (10%), Contain lung markings and larger
may not be found on bronchoscopy

DDx: Paratracheal Cyst

Subpleural Bleb Zenker ~iverticulum Apical Lung Hernia

 PARATRACHEAL AIR CYST

Key Facts
Terminology Rarely tracheal communication identified (10%), may
Mucosal herniation through the tracheal wall from not be found on bronchoscopy
increased intraluminal pressure Top Differential Diagnoses
Imaging Findings Para septal Bleb
Best diagnostic clue: Small rounded air-filled cyst in Zenker Diverticulum
the right para tracheal region at the thoracic inlet Apical Lung Hernia
Location: Right posterolateral tracheal wall in the Pathology
thoracic inlet (> 95%) Epidemiology: Autopsy prevalence 1%
Size: Usually < 2 cm in diameter

I PATHOLOGY o Other symptoms, chronic cough and dyspnea

usually from obstructive lung disease
3
General Features Other signs/symptoms 27
o May be large enough for endotracheal tube insertion
Etiology
o Congenital o Pulmonary function test: Obstructive pattern
Supernumerary lung buds contain all layers of Demographics
tracheal wall including smooth muscle and Age: Middle age and older
cartilage, often filled with mucus
o Acquired Treatment
Chronic increased intraluminal pressure: None to surgical resection if location and size can be
Coughing, emphysema shown to result in symptoms
Larger and wider mouth than congenital
diverticula, have respiratory epithelium only
Epidemiology: Autopsy prevalence 1% I DIAGNOSTIC CHECKLIST
Gross Pathologic & Surgical Features Consider
Cyst communicates with the trachea, channel Paratracheal cysts serve as marker for underlying
measures 1.5 to 2 mm in length, 1 mm in diameter obstructive pulmonary disease
Location at transition point between the intrathoracic
and extrathoracic trachea Image Interpretation Pearls
Must not be mistaken for pneumomediastinum or
Microscopic Features pneumothorax
Cyst lined with normal ciliated columnar epithelium

I SELECTED REFERENCES
I CLINICAL ISSUES 1. Goo JM et al: Right paratracheal air cysts in the thoracic
Presentation inlet: clinical and radiologic significance. AJR Am J
Roentgenol. 173(1):65-70, 1999
Most common signs/symptoms
o Usually asymptomatic

I IMAGE GALLERY

(Left) Axial CECTshows close approximation of paratracheal cyst to trachea. Apical lung is abnormal with thickened septa and hyperinflated
lobules. (Center) Axial NECT shows right paratracheal diverticulum with narrow communication (arrow) with the trachea at the junction of the
posterior membrane and the cartilaginous wall. (Right) Sagittal oblique HRCT shows multiple small diverticula along the posterolateral aspect of
the trachea (arrows).
 RHINOSCLEROMA

28

Axial CECT shows subglottic mucosal thickening Coronal minimum intensity projection shows the focal
(arrows) and crypt-like space (curved arrow) in subglottic narrowing and the air-filled crypts (curved
rhinoscleroma. arrows) typical of rhinoscleroma.

o Crypt-like spaces nearly diagnostic

ITERMINOlOGY
Imaging Recommendations
Abbreviations and Synonyms
Best imaging tool: CT or MRI to fully evaluate upper
Scleroma, Klebsiella rhinoscleroma tis respiratory tract
Definitions
Chronic granulomatous infection of the upper
respiratory tract due to Klebsiella rhinoscleromatis I DIFFERENTIAL DIAGNOSIS
laryngotracheal Papillomatosis
Discrete laryngeal and tracheal polypoid masses
I IMAGING FINDINGS May spread to lung and form solid and cavitary
General Features nodules
Best diagnostic clue: Irregular subglottic mucosal Relapsing Polychondritis
thickening with crypt-like spaces Cartilage thickening: Ear, nose, tracheal rings
Location: Nasal vault, nasopharynx, subglottic trachea
Wegener Granulomatosis
CT Findings
Smooth subglottic narrowing
Nasal polyps and enlarged turbinates Multiple thick-walled cavitary lung lesions
Paranasal sinuses characteristically spared unless nasal
disease obstructs ostiomeatal units Post-Intubation Stricture
Thickening of soft tissues nasopharynx History of prolonged or traumatic intubation
o Fascial planes preserved Smooth tracheal narrowing
Subglottic tracheal narrowing

DDx: Tracheal Wall Thickening

Papillomatosis Relapsing Polychondritis

 RHINOSCLEROMA

Key Facts
Terminology Paranasal sinuses characteristically spared unless
Chronic granulomatous infection of the upper nasal disease obstructs ostiomeatal units
respiratory tract due to Klebsiella rhinoscleromatis Crypt-like spaces nearly diagnostic

Imaging Findings Top Differential Diagnoses

Best diagnostic clue: Irregular subglottic mucosal Laryngotracheal Papillomatosis
thickening with crypt-like spaces Relapsing Polychondritis
Location: Nasal vault, nasopharynx, subglottic Wegener Granulomatosis
trachea Post-Intubation Stricture
Nasal polyps and enlarged turbinates

I PATHOLOGY I CLINICAL ISSUES 3

General Features Presentation 29
Etiology Most common signs/symptoms
a Direct inhalation of contaminated droplets a Nasal obstruction
a Cellular immunity impaired with impaired T-cell a Stridor
function and decreased macrophage activation a Epistaxis and rhinorrhea
Epidemiology: Endemic in Central America, Africa, Other signs/symptoms: Cultures positive in only 50%
and India
Demographics
Gross Pathologic & Surgical Features Age: 10-30 years of age
Nasal cavity 95% Gender: Females more common
Nasopharynx 50%
Larynx and trachea 15-40%
Natural History & Prognosis
Chronic debilitating progressive disease
Microscopic Features May relapse with discontinuation of therapy
Mikulicz cells: Large vacuolated macrophage
containing the bacilli Treatment
Long-term antibiotic therapy for months or years
Staging, Grading or Classification Criteria Surgery may be required for obstructive lesions
Stage 1: Catarrh
a Nonspecific rhinitis for weeks or months
Stage 2: Granulomatous I SELECTED REFERENCES
a Nasal polyps and nodular thickening of affected I. Iyengar P et al: Rhinoscleroma of the larynx.
epithelium Histopathology. 47(2):224-5, 2005
Stage 3: Sclerosis 2. Prince jS et al: Nonneoplastic lesions of the
a Fibrosis and scarring of affected structures tracheobronchial wall: radiologic findings with
bronchoscopic correlation. Radiographies. 22 Spec
No(S215-30, 2002

I IMAGE GALLERY

(Leh) Axial CECTshows ci,cumfe,ential mucosal thickening with uypt-like outpouching (arrows). (Center) Axial CECT shows asymmetric
thickening of tissues in the nasopharynx (arrows), a common location for rhinoscleroma. (Right) Axial NECT shows polypoid nodules (arrows) in
the nasal cavity and enlarged turbinate (curved arrow). Adjacent maxillary sinuses are normal, typical of rhinoscleroma.
 CHRONIC BRONCHITIS

3
30

Coronal graphic shows generalized thickening of Frontal radiograph magnification view from a patient
lfachea & cenlfal bronchi. Bronchial walls are coated with chronic bronchitis shows generalized thickening of
with a thick layer of mucus. Inset depicts a thickened central interstitium. Arrows ouUine one thickened
bronchus in cross-section. bronchus (lfamline).

Size: Significantly increased percentage of bronchial

ITERMINOlOGY wall area & bronchial thickness-to-diameter ratio
Abbreviations and Synonyms Morphology: Thickening of tracheobronchial walls
Chronic bronchitis (CB) Radiographic Findings
Chronic obstructive pulmonary disease (COPD) Radiography
Forced expiratory volume in 1 second (FEV!) o CB not a radiographic diagnosis
Forced vital capacity (FVC) o Many, if not most, patients with isolated chronic
Definitions bronchitis are radiographically normal
CB defined clinically, not anatomically o Most important role of radiography is detecting
o Productive cough with sputum production for ~ 3 other conditions in differential diagnosis
months for ~ 2 consecutive years without other o Radiographic features cited for CB are nonspecific
underlying cause o Tramlines or tram tracks
Longitudinally oriented bronchi with thickened
walls
o Thickened ring shadows
I IMAGING FINDINGS Thickened bronchi seen on end
General Features Note: Bronchi are normally seen side-by-side with
Best diagnostic clue: Retained mucus & thickening of pulmonary arteries
trachea & large bronchi in a smoker o Increased interstitial lung markings
Location Increase in linear opacities in central lungs
o Trachea & large bronchi Pathological basis unclear
o Enlarged central pulmonary arteries with rapid Thickening of central interstitium plays a part
peripheral tapering a Hyperinflation

DDx: Bronchial Thickening

Bronchopneumonia Aspergillosis Tracheopathia Osteochondroplastica

 CHRONIC BRONCHITIS

Key Facts
Imaging Findings Pathology
Best diagnostic clue: Retained mucus & thickening of Most important cause: Cigarette smoking
trachea & large bronchi in a smoker 4% of adults over 18 in United States have diagnosis
Many, if not most, patients with isolated chronic of chronic bronchitis
bronchitis are radiographically normal Associated abnormalities: Chronic bronchitis occurs
Tramlines or tram tracks infrequently without associated emphysema
Thickened ring shadows
Increased interstitial lung markings
Clinical Issues
Tortuous pulmonary arteries with blurred margins Most common signs/symptoms: Cough & sputum
Cor pulmonale production
Smoking cessation: Single most important treatment
Top Differential Diagnoses 0, therapy: Backbone of clinical management
Emphysema
3
Diagnostic Checklist
Asthma
Bronchiectasis Clinical diagnostic criteria must be fulfilled, so 31

Acute Bronchitis radiographic findings are only supportive

Pneumonia

o Tortuous pulmonary arteries with blurred margins
o Cor pulmonale
Enlarged right ventricle, dilated central
pulmonary arteries & peripheral arterial pruning
o Saber-sheath trachea strongly associated with CB
Coronal diameter of intrathoracic trachea
measures less than 60% of sagittal diameter
Chronic cough deforms tracheal cartilage
CT Findings
HRCT
o Many patients with CB have no associated CT
abnormality
Bronchial wall thickening
Bronchial lumen mayor may not be narrowed
Mucus in tracheobronchial tree
Enlargement of central pulmonary arteries from
cor pulmonale
o Orlandi, et ai, found differences in patients with
chronic obstructive lung disease (COP D) who have
CB & those who do not
Bronchi in patients with CB had significantly
higher thickness-to-diameter ratio & percentage
wall area
Multivariate analysis showed correlation between
bronchial measurements & indices of bronchial
obstruction
Imaging Recommendations
Best imaging tool: High resolution CT
Protocol advice
o Conventional CT protocols usually suffice
o Since patients are often dyspneic, technologists
should prepare patients to avoid respiratory motion
I DIFFERENTIAL DIAGNOSIS
Emphysema
Emphysema, particularly centrilobular emphysema,
commonly coexists with CB
Characterized by destruction of airspaces, decreases
FEV,
Can be reliably diagnosed on high resolution CT
Asthma
Can coexist with CB (asthmatic bronchitis)
Can have bronchial wall thickening & hyperinflation
Reverses with bronchodilator treatment
Bronchiectasis
Common complication of CB
Tramlines & increased interstitial markings common
Bronchiectasis can be diagnosed on high resolution CT
Acute Bronchitis
Acute bronchitis often superimposed on CB
Acute bronchitis has a sudden onset & usually lasts a
few days or weeks
Acute bronchitis often follows a viral upper respiratory
infection
CB usually has an indolent onset & lasts ~ 3 months
for ~ 2 consecutive years
Pneumonia
Frequent complication of CB
Bronchial thickening from CB sometimes difficult to
differentiate from bronchopneumonia
Pneumonia usually has associated airspace disease
with segmental or lobar distribution
I PATHOLOGY
General Features
Genetics: Linkage to chromosomes 22 & 12p
Etiology
o Most important cause: Cigarette smoking
o Also pipe, cigar & passive smoking
o Air pollution: Especially particulates, sulfur dioxide
& nitrogen oxides
o Infection
Epidemiology
o 4% of adults over 18 in United States have diagnosis
of chronic bronchitis
o Compares with 1.5% of adults over 18 in US with
emphysema
 CHRONIC BRONCHITIS
o Both conditions often undiagnosed
o Prevalence tracks cigarette use
Associated abnormalities: Chronic bronchitis occurs
infrequently without associated emphysema
Gross Pathologic & Surgical Features
Inflamed, erythematous bronchial mucosa with
increased mucus on bronchial surfaces
Microscopic Features
Mucous gland hypertrophy & hyperplasia
o Reid index: Ratio of thickness of bronchial mucous
gland layer to bronchial wall
o Normal Reid index < 0.4
3 o Reid index increased in CB
Goblet cell metaplasia
32 Mucous.plugs in small airways
Bronchial inflammation & fibrosis
Staging, Grading or Classification Criteria
COPD severity is graded by Global Initiative for
Chronic Obstructive Lung Disease (GOLD) criteria
COPD: FEV,/FVC ratio < 70%
Stages
o Mild: Postbronchodilator FEV, '" 80% predicted
o Moderate: Postbronchodilator 30% predicted :s
FEV" < 80% predicted
o Severe: Postbronchodilator FEV, < 30% predicted or
respiratory or right heart failure
GOLD criteria are functional, not symptomatic
Chronic bronchitis is an important component of
COPD, but not part of diagnostic criteria
ICLINICAL ISSUES
Presentation
Most common signs/symptoms: Cough & sputum
production
Other signs/symptoms: Dyspnea, hemoptysis, digital
clubbing & rhonchi
Clinical Profile
o "Blue Bloater": Classical clinical presentation of CB
Stocky build, cyanosis & peripheral edema from
right heart failure
Only a few patients with COPD fit classical profile
of "blue bloater"
Most have a combination of emphysema ("pink
puffer") & CB
For many patients with CO PO, CB is an important
part of their symptom complex
Pulmonary function tests
o FEV,: Standard measure of airway obstruction
o Reid index does not correlate well with FEV,
o FEV, ., FVC ., FEV,/FVC ratio. in CB
o Total lung capacity normal, reSidual volume t in CB
Arterial blood gas measurements
o Increased C02, decreased 02
Demographics
Age: Prevalence in US, 18-44 years (2.9%), 45-64 years
(4.9%),65-74 years (6.3%), 75 years & over (5.4%)
Gender: Prevalence in US, 2.7% of adult males, 5.3%
of adult females
Ethnicity: Prevalence in US, Caucasian (4.1%),
African-American (3.8%)
Natural History & Prognosis
Risk of mortality from CO PO correlates well with
FEV" but not with mucus production
Treatment
Smoking cessation: Single most important treatment
Avoidance of environmental dust exposure
02 therapy: Backbone of clinical management
Bronchodilators, especially for patient with
superimposed asthma or acute bronchitis
Steroids, particularly inhaled corticosteroids
Antibiotic therapy for bacterial bronchitis or
pneumonia
Pulmonary rehabilitation
Immunizations against influenza & pneumococcus
I DIAGNOSTIC CHECKLIST
Consider
Clinical diagnostic criteria must be fulfilled, so
radiographic findings are only supportive
Image Interpretation Pearls
CB patients are usually smokers, so beware of subtle
lung carcinoma!
I SELECTED REFERENCES
1. Lethbridge-Cejku M et al: Summary health statistics for
U.S. adults: National Health Interview Survey, 2003. Vital
Health Stat. 10(225):1-161,2005
2. Orlandi I et al: Chronic obstructive pulmonary disease:
thin-section CT measurement of airway wall thickness and
lung attenuation. Radiology. 234(2):604-10, 2005
3. Molfino NA: Genetics of COPD. Chest. 125(5):1929-40,
2004
4. Pauwels RA et al: Global strategy for the diagnosis,
management, and prevention of chronic obstructive
pulmonary disease. NHLBI/WHO Global Initiative for
Chronic Obstructive Lung Disease (GOLD) Workshop
summary. Am J Respir Crit Care Med. 163(5):1256-76,2001
5. Takasugi JE et al: Radiology of chronic obstructive
pulmonary disease. Radiol Clin North Am. 36(1):29-55,
199B
6. Jamal K et al: Chronic bronchitis. Correlation of
morphologic findings to sputum production and flow
rates. Am Rev Respir Dis. 129(5):719-22, 1984
7. Peto R et al: The relevance in adults of air-flow obstruction,
but not of mucus hypersecretion, to mortality from
chronic lung disease. Results from 20 years of prospective
observation. Am Rev Respir Dis. 128(3):491-500, 1983
8. Greene R: "Saber-sheathll trachea: relation to chronic
obstructive pulmonary disease. AJR Am J Roentgenol.
130(3):441-5, 1978
9. Milne EN et al: The roentgenologic diagnosis of early
chronic obstructive pulmonary disease. J Can Assoc Radiol.
20(1):3-15, 1969
10. American Thoracic Society. Definitions and classification
of chronic bronchitis, asthma and pulmonary emphysema.
Am Rev Respir Dis. 85:762-768, 1962
 CHRONIC BRONCHITIS

I IMAGE GALLERY

(Left) Axial CECT with

mediastinal window settings
shows marked central
bronchial thickening in a
patient with chronic
bronchitis (open arrows).
(Right) Axial CECT in same
patient with lung window
settings again shows
thickening of central bronchi
(open arrows). Bronchial
wall thickening also extends
3
to segmental bronchi
(arrows). 33

(Left) Axial CECT of patient

with chronic bronchitis
shows thickening of right
middle lobe bronchus (open
arrow) & marked luminal
narrowing of multiple lower
lobe segmental bronchi
(arrows). (Right) Lateral
radiograph magnification
view of same patient shows
thickening of middle lobe
bronchus (open arrows),
lower lobe segmental
bronchi (arrows) & bronchus
intermedius (curved arrow).

Typical
(Left) Lateral radiograph
magnification view shows
ring shadows (arrows) in a
patient with chronic
bronchitis. Tramlines (open
arrows) are visible in a lower
lobe segmental bronchus.
(Right) Coronal CECT shows
mild bronchial wall
thickening (open arrows). A
large globule of retained
mucus (arrows) is visible in
right main bronchus.
 BRONCHIECTASIS

3
34

Axial graphic shows cystic bronchial dilatation in right Frontal radiograph in CYSbC fibrosis shows bilateral
upper lobe (arrow). Bronchial wall is thickened by bronchiectasis, worst in right upper lobe, where
fibrosis(open arrows). A focus of organizingpneumonia atelectasis is also visible. Cor pulmonale & adenopathy
(curved arrow) is seen. cause hi/ar enlargement.

ITERMINOLOGY Upper lobe predominance, often unilateral

o Atypical mycobacterial disease
Abbreviations and Synonyms Right middle lobe & lingular predominance
Allergic bronchopulmonary aspergillosis (ABPA) o Viral infection
Cystic fibrosis (CF) Lower lobe predominance
Forced expiratory volume in 1 see (FEV1) Size: Ranges from mild, cylindrical dilatation to severe,
saccular dilatation
Definitions Morphology
Irreversible dilatation of a bronchus or bronchi, often o Dilated bronchi with thickened walls
with thickening of bronchial wall o Bronchi can be filled with gas, fluid or mucus
Radiographic Findings
I IMAGING FINDINGS Radiographic findings can be subtle or nonspecific
Primary radiographic finding - bronchial dilatation
General Features Bronchi
Best diagnostic clue: Thickened, cystic bronchi o Bronchial wall thickening
containing fluid levels o Tramlines or tram tracks
Location Longitudinally oriented bronchi with thickened
o Cystic fibrosis walls
Upper lobe predominance, central & peripheral o Ring shadows
o Allergic bronchopulmonary aspergillosis Thickened bronchi seen on end
Upper lobe predominance, often bilateral, Note: Bronchi are normally seen side-by-side with
asymmetric, central predominance pulmonary arteries
o Tuberculosis o Signet ring sign

DDx: Cystic Lung Disease

Lymphangio/eiomyomatosis Langerhans Cell Histiocytosis Bullae

 BRONCHIECTASIS

Key Facts
Imaging Findings Prevalence lower with antibiotics & immunization
Best diagnostic clue: Thickened, cystic bronchi Bronchial wall dilatation, thickening & chronic
inflammation with granulation tissue & fibrosis
containing fluid levels
Bronchial wall weakness, recurrent infections,
Tramlines or tram tracks
parenchymal volume loss & distortion
Ring shadows
Bronchial artery hypertrophy
Signet ring sign
Top Differential Diagnoses Clinical Issues
Most common signs/symptoms: Cough, sputum
Pneumonia
production & hemoptysis
Chronic Bronchitis
Other signs/symptoms: Digital clubbing, dyspnea,
Cystic Lung Disease
crackles & wheezing

Bronchial Atresia
Atelectasis Diagnostic Checklist
3
Asthma In young patient with diffuse bronchiectasis, confirm 35
Pathology cystic fibrosis by identifying pancreatic atrophy
Defect of mucous clearance

Dilated bronchus abutting an adjacent pulmonary
artery
Normal bronchus usually" size of adjacent
pulmonary artery
o "V" or "Y"opacities, band shadows or
"finger-in-glove" sign
Fluid or mucous-filled bronchi may branch &
point to hilum
Lung
o Volume loss
Subsegmental to lobar
Scarring or endobronchial obstruction
o Compensatory hyperinflation of uninvolved lung
o Cysts
Can contain fluid level
o Blebs
Predispose to pneumothorax
CT Findings
HRCT
o Dilated bronchi with bronchoarterial ratio> 1, no
tapering
Cylindrical bronchiectasis: Uniform diameter
Varicose bronchiectasis: "String of pearls",
alternating dilatation & narrowing
Saccular or cystic bronchiectasis: "Cluster of
grapes", marked dilatation, rounded
o Internal bronchial diameter> adjacent pulmonary
artery
o Signet ring sign
o Bronchial wall thickening
o "V" or "Y" opacities or ltfinger-in-glove" sign
Mucus or secretions in bronchioles or bronchi
o Bronchi seen" 1 cm of costal or paravertebral
pleura
o Bronchi touching mediastinal pleura
Endobronchial secretions do not correlate with
measures of airway obstruction in bronchiectasis
o Bronchial wall thickness & decreased attenuation on
expiratory CT scans do correlate with obstruction
o Correlations suggest that obliterative bronchiolitis is
cause for airway obstruction in bronchiectasis
HRCT of idiopathic bronchiectasis shows interlobular
septal thickening in 60%
o Could be related to impaired lymphatic drainage
Traction bronchiectasis, distortion & honeycombing
occur in pulmonary fibrosis
Imaging Recommendations
Best imaging tool: HRCT for diagnosis &
characterization of severity & extent
Protocol advice: Conventional protocols are usually
adequate for diagnosis
I DIFFERENTIAL DIAGNOSIS
Pneumonia
Reversible bronchiectasis sometimes associated with
pneumonia
Re-image three months after resolution of pneumonia
to confirm that bronchi have returned to normal
Chronic Bronchitis
Can be precursor to bronchiectasis
Can coexist with bronchiectasis
Cough & sputum production are features of both
HRCT can confirm bronchiectasis, if present
Cystic Lung Disease
Langerhans cell histiocytosis
o Irregular cysts of Langerhans cell histiocytosis can
simulate bronchiectasis
Lymphangioleiomyomatosis
o Uniform distribution of cysts in young women
Bullae
o Hyperinflated lung seen in paraseptal emphysema
Laryngotracheal papillomatosis
o Airway nodules; solid & cystic lung nodules
Bronchial Atresia
Dilated, mucous-filled bronchus distal to atretic
segment
Associated with marked hyperlucency &
hypo perfusion of involved segment
 BRONCHIECTASIS
Other signs/symptoms: Digital clubbing, dyspnea,
Atelectasis crackles & wheezing
Central bronchial obstruction can cause atelectasis & Mild bronchiectasis can be asymptomatic
bronchiectasis ! FEV,
Mucus plugs from bronchiectasis can cause atelectasis ! FEV,/forced vital capacity ratio
Asthma Demographics
Bronchiectasis & asthma can coexist, e.g., ABPA
Age
Tramlines, mucous plugs & hyperinflation in both o Prevalence (exclusive of CF) increases with age
Ranges from 4.2 per 100,000 for age 18-34 years to
271.8 per 100,000 for;;,; 75 years
I PATHOLOGY Gender
o Bronchiectasis often more severe in women
General Features
o Prevalence among women higher at all ages
3 General path comments
o Defect of mucous clearance
o Atypical tuberculosis more common in elderly
women
36 o Traction bronchiectasis occurs in special case of
Ethnicity: CF predominantly affects Caucasians
pulmonary fibrosis
Genetics: Wide variety of genetic etiologies Natural History & Prognosis
Etiology Depends on severity & underlying cause
o Cystic fibrosis
o Primary ciliary dyskinesia Treatment
o Mounier-Kuhn syndrome Smoking cessation
o Williams-Campbell syndrome Appropriate vaccinations
o Deficiencies of cellular or humoral immunity Postural drainage
o Allergic bronchopulmonary aspergillosis Antibiotic treatment for superimposed infection
o Post-infection Bronchodilators
Typical & atypical tuberculosis, fungal, bacterial & Bronchial artery embolization to control severe
viral hemoptysis
o Chronic aspiration Surgery for localized disease that is unresponsive to
o Toxic inhalation medical therapy
o Obstruction Lung transplant for selected cases
Tumor, foreign body or lymph node enlargement
o Pulmonary fibrosis
o Rheumatoid arthritis I DIAGNOSTIC CHECKLIST
o Yellow nail syndrome
Epidemiology Image Interpretation Pearls
o - 30,000 patients with CF in US In young patient with diffuse bronchiectasis, confirm
o > 110,000 patients (exclusive of CF) receiving cystic fibrosis by identifying pancreatic atrophy
treatment for bronchiectasis in US
o Prevalence lower with antibiotics & immunization
Associated abnormalities I SELECTED REFERENCES
o Pneumonia, pneumothorax & empyema 1. Sibtain NA et al: Interlobular septal thickening in
o Cor pulmonale, hypertrophic osteoarthropathy Idiopathic bronchiectasis: a thin-section CT study of 94
o Brain abscess & amyloidosis: Rare patients. Radiology. 237(3):1091-6, 2005
2. Weycker D et al: Prevalence and economic burden of
Gross Pathologic & Surgical Features bronchiectasis. Clin Pulm Med. 12(4):205-209,2005
Bronchial wall dilatation, thickening & chronic 3. Morrissey BM et al: Bronchiectasis: sex and gender
inflammation with granulation tissue & fibrosis considerations. Clin Chest Med. 25(2):361-72, 2004
4. Rados C: Orphan products: hope for people with rare
Bronchial wall weakness, recurrent infections,
diseases. FDA Consumer Magazine. 37(6), 2003
parenchymal volume loss & distortion 5. Roberts HR et al: Airflow obstruction in bronchiectasis:
Bronchial artery hypertrophy correlation between computed tomography features and
Lymph node enlargement pulmonary function tests. Thorax. 55(3):198-204, 2000
6. Cartier Y et al: Bronchiectasis: accuracy of high-resolution
Microscopic Features CT in the differentiation of specific diseases. A)R Am)
Edema, inflammation, ulceration, organizing Roentgenol. 173(1):47-52, 1999
pneumonia & fibrosis 7. Austin)H et al: Glossary of terms for CT of the lungs:
recommendations of the Nomenclature Committee of the
Fleischner Society. Radiology. 200(2):327-31, 1996
I CLINICAL ISSUES 8. Woodring )H: Improved plain film criteria for the diagnosis
of bronchiectasis.) Ky Med Assoc. 92(1):8-13,1994
Presentation 9. Naidich DP et al: Computed tomography of bronchiectasis.
) Com put Assist Tomogr. 6(3):437-44,1982
Most common signs/symptoms: Cough, sputum
production & hemoptysis
 BRONCHIECTASIS
I IMAGE GALLERY
Typical
(Left) Axial CECT in same
patient with cystic fibrosis
shows cystic bronchiectasis
with bronchial wall
thickening (open arrow).
Fluid level is visible in a
dilated bronchus (arrow).
(Right) Coronal CECT in
same patient demonstrates a
large bronchial collateral
artery (open arrows) arising
from aorta (arrow).
3
Increased lymphoid tissue is
present in both hila (curved 37
arrows).

Typical
(Left) Axial CECT shows
cylindrical bronchiectasis in
right middle & lower lobes
(arrows). Varicose
bronchiectasis is visible in
right lower lobe (open
arrow). (Right) Coronal
CECT magnification view
shows multiple signet ring
signs, dilated bronchi in
cross-section (arrows).
Compare to normal
bronchus (open arrow) &
paired pulmonary artery
(curved arrow).

Typical
(Left) Frontal radiograph
magnification view shows
band shadows in right lower
lobe, representing dilated
bronchi filled with
inspissated mucus (arrows).
Dilated bronchi can be
followed to hilum. (Right)
Axial CECT in a different
patient shows a branching,
cystic bronchus filled with
inspissated mucus (arrows),
a "finger-in-glove" sign.
 MYCOBACTERIAL AVIUM COMPLEX

3
38

Frontal radiograph in a male smoker wi!h a history of Axial CECT in same patient shows !he cavitary lesion
emphysema shows right upper lobe nodules fcurved (arrow) has a variable !hick wall and irregular shape.
arrows) and a cavitary mass (open arrow). MAC pneumonia.

Location: Right middle lobe and lingular involvement

I TERMINOlOGY
Radiographic Findings
Abbreviations and Synonyms
Radiography
Mycobacterium avium intracellulare complex (MAC), o Consolidative/fibrocavitary disease
non tuberculous mycobacteria (NTM) Linear, nodular or mass-like opacities
Definitions Apical posterior segments upper lobes; superior
NTM pulmonary infection, most commonly caused by segments lower lobes
MAC Unilateral or bilateral
o Other NTM: M xenopi, M fortuitum, and M Thin or thick-walled cavities, usually < 3 cm
chelonae diameter
Five types of disease Adjacent pleural thickening
o Consolidative/fibrocavitary disease, classic form Scarring with volume loss and distortion
o Airways disease o Airways disease
o Solitary or multiple pulmonary nodules Tubular branching lucencies with bronchial wall
o Pneumonia in immunosuppressed host thickening
o Hypersensitivity pneumonia Scattered ill-defined reticulonodular opacities,
nodules
Primarily in right middle lobe and lingula
I IMAGING FINDINGS Hyperinflation
o Nodule(s)
General Features Single or multiple, when multiple may be
Best diagnostic clue: Slowly progressive bronchiectasis, clustered
bronchiolectasis, nodules o Pneumonia in immunosuppressed host, AIDS

DDx: Consolidative/Fibrocavitary Disease

Mycobacterium Tuberculosis Cryptococcus Lung Cancer

 MYCOBACTERIAL AVIUM COMPLEX
Key Facts
Terminology Top Differential Diagnoses
Consolidative/fibrocavitary disease, classic form Post-Primary Tuberculosis
Airways disease Other Infections
Solitary or multiple pulmonary nodules Bronchogenic Carcinoma
Pneumonia in immunosuppressed host
Hypersensitivity pneumonia Pathology
Hot tub lung: Hypersensitivity reaction to MAC
Imaging Findings Peribronchial and peribronchiolar granulomas
Airspace opacities, masses, nodules
Thin walled cavities; thick walled cavities, less Clinical Issues
common Lady Windermere syndrome
Bronchiectasis, distributed mainly in the right middle Almost exclusively in elderly white women
lobe and lingula No underlying lung disease other than nodular 3
Tree-in-bud pattern: Branching centrilobular nodules bronchiectatic disease
Nodules < 10 mm; 10-30 mm Multiple anti mycobacterial drugs for 12-36 months 39
With AIDS, hilar-mediastinallymphadenopathy, Curative in up to 80%
pleural effusions

Normal radiograph with positive sputum cultures, a With AIDS, hilar-mediastinallymphadenopathy,

common pleural effusions
Small scattered alveolar and nodular opacities, a Lymphadenopathy with low density necrotic centers
miliary nodules, mass-like lesions Hypersensitivity pneumonia
Cavitation in non-AIDS immunosuppressed a Diffuse centrilobular micronodules
Hilar/mediastinallymphadenopathy and a Ground-glass opacities
effusions, common and may be isolated
o Hypersensitivity pneumonia Imaging Recommendations
Diffuse interstitial or nodular opacities Best imaging tool
Normal radiograph (22%) a CT, superior to show cavities, nodules, bronchiolitis
and bronchiectasis, lymphadenopathy
CT Findings CT for airways disease with MAC: Sensitivity 80%,
Fibrocavitary disease specificity of 87%, accuracy of 86%
o Airspace opacities, masses, nodules Protocol advice
a Upper lobe predominance a Inspiration and expiration thin-section CT for
a Thin walled cavities; thick walled cavities, less airways disease
common For mosaic attenuation and or air-trapping
a Feeding bronchus
a Bronchogenic spread with 5-15 mm peripheral
centrilobular nodules I DIFFERENTIAL DIAGNOSIS
a Bronchial wall thickening
a Apical pleural thickening Post-Primary Tuberculosis
a Lymphadenopathy, miliary disease, effusion, Identical radiologic appearance
uncommon Unlike NTM, human to human transmission
Airways disease Distinguished by microbiologic features
a Bronchiectasis, distributed mainly in the right
Other Infections
middle lobe and lingula
a Bilateral multifocal bronchiolitis Cryptococcus, sporotrichosis, nocardiosis, abscess
Tree-in-bud pattern: Branching centrilobular Similar radiologic appearance
nodules Clinical presentation may suggest pathogenic etiology
a Well-defined small peribronchial nodules a Renal transplant patient: Cryptococcus, nocardia
o Mosaic pattern of perfusion a Rose gardener: Sporotrichosis
a Scarring, volume loss, distortion a Aspiration: Aerobic and anaerobic abscess
Nodule(s) Bronchogenic Carcinoma
a Nodules < 10 mm; 10-30 mm Similar radiologic appearance
a Miliary, small or medium size Biopsy for diagnosis
Pneumonia in immunosuppressed, AIDS
a Normal or subtle pulmonary findings such as a few Progressive Massive Fibrosis
scattered centrilobular nodules History of coal or silica dust exposure
a Airspace opacification, mass-like opacities, nodules, Background of small sometimes calcified nodules
miliary disease Lymphadenopathy with eggshell calcifications
a Cavitation in non AIDS patients Masses are pancake shaped; may cavitate
Often bilateral symmetrical
 MYCOBACTERIAL AVIUM COMPLEX
Right middle lobe and lingular bronchiectasis
I PATHOLOGY Almost exclusively in elderly white women
General Features Immunocompetent individuals
Chronic pulmonary MAC infection
Etiology
o Water is likely source of human infection Nonsmokers
No underlying lung disease other than nodular
Infection by inhalation, ingestion, or direct
bronchiectatic disease
inoculation
o Hot tub lung: Hypersensitivity reaction to MAC Immunosuppressed, AIDS
o Infection with CD4+ lymphocyte count of < 50 cells
Epidemiology
o Ubiquitous throughout environment per ~L
o Disseminated disease, uncommon 2%
o In water, soil, milk, fish, birds, and animals
o Common infection in southeast U.S. Hypersensitivity lung disease
o Human to human transmission, rare o Hot tub use, aerosolized water in showers

3 Associated abnormalities
o Lung disease: Emphysema, chronic bronchitis,
Demographics
Age: Many infected patients are> 50 years old
40 bronchiectasis, cystic fibrosis Gender
o Cardiac disease: Mitral valve prolapse o Classic infection: Primarily males
o Skeletal anomalies: Pectus excavatum, mild scoliosis, o Lady Windermere syndrome: Females
straight back Ethnicity: More common in whites, excluding patients
o Immunosuppressed patients with AIDS
AIDS, rheumatoid arthritis, diabetes mellitus,
alcoholism Natural History & Prognosis
Lung cancer, non pulmonary malignancies Slowly progressive radiographic abnormalities
o From localized disease to involve other lobes and
Gross Pathologic & Surgical Features contralateral lung
MAC o Progressive fibrosis with volume loss and traction
o Nonphotochromogens colonies, type III, beige or bronchiectasis
white Mycetomas may form in residual cavities
o Does not change color on exposure to light Bronchopleural fistulas
o Low grade pathogen, requires 2-4 weeks to grow in Death from respiratory failure, uncommon
culture AIDS, with antiretroviral treatment, 50% alive 5 years
Consolidation, cavities, bronchiectasis, later
bronchostenosis, bronchopleural fistula, fibrosis,
distortion Treatment
Diagnosis by isolation from transbronchial or open
Microscopic Features lung biopsy specimens
Peribronchial and peri bronchiolar granulomas Isolation from sputum or bronchoalveolar lavage fluid
Bronchiolectasis, centrilobular bronchiolar can represent airway colonization and not infection
granulomas or necrotic debris AIDS patients: Positive sputum or bronchoalveolar
lavage fluid cultures are diagnostic of infection
Multiple antimycobacterial drugs for 12-36 months
I CLINICAL ISSUES o Curative in up to 80%
Presentation Surgery for localized disease, followed by
anti mycobacterial drugs
Most common signs/symptoms
Hypersensitivity pneumonia: Cessation of hot tub use,
o Chronic, minimally productive cough, sputum
steroids, anti mycobacterial drugs
production
o Malaise, fever, weight loss, hemoptysis
o AIDS: Fever, sweats, weight loss, fatigue, diarrhea,
I SElECTED REFERENCES
shortness of breath
o Purified protein derivative (PPD) skin test may be 1. Hanak V et al: Hot tub lung: Presenting features and
positive clinical course of 21 patients. Respir Med. 26, 2005
2. Jeong VJet al: Nontuberculous mycobacterial pulmonary
Other signs/symptoms: In immunocompetent and
infection in immunocompetent patients: comparison of
immunosuppressed thin-section CT and histopathologic findings. Radiology.
Fibrocavitary disease 231(3):880-6, 2004
o Most common type 3. Wittram C et al: Mycobacterium avium complex lung
o Elderly white men disease in immunocompetent patients: radiography-CT
o Underlying lung disease, emphysema, pulmonary correlation. BrJ Radiol. 75(892):340-4, 2002
fibrosis 4. Erasmus JJ et al: Pulmonary non tuberculous mycobacterial
Airways disease infection: radiologic manifestations. Radiographies.
19(6):1487-505,1999
o Second most common type
o Infection with and without pre-existing lung disease
o Lady Windermere syndrome
 MYCOBACTERIAL AVIUM COMPLEX

I IMAGE GALLERY

Typical
(Left) Axial NEeT in an
elderly female shows
bronchiolectasis in the right
middle lobe and lingula
(arrows). Right middle lobe
peribronchiolar opacity.
Airway disease with MAC.
(Right) Axial H RCT in a
middle aged woman with
Lady Windermere disease
shows bronchiectasis and
fibrosis in the right middle 3
lobe and lingula (curved
arrows). Note right middle 41
lobe nodule (open arrow).

Typical
(Left) Frontal radiograph
shows large right upper lobe
mass-like opacity (arrow).
(Right) Axial NEeT in same
patient shows an irregular
mass with indistinct margins
(arrow). Consolidative MAC
pneumonia.

(Left) Frontal radiograph

shows diffuse opacification,
volume loss and cavities
(arrows) in the left lung due
to fibrocavitary MAC
pneumonia. (Right) Axial
CECT in a patient with prior
lung cancer and left upper
lobectomy shows varying
sized nodules (arrows) in the
left lower lobe. Nodules due
to MAC infection.
 lARYNGEAL PAPillOMATOSIS

3
42

Axial graphic shows typical features of laryngotracheal Axial NECT sequence shows multiple cauliflower
papillomatosis. Airway nodules (arrow), peribronchial shaped nonocc/usive polyps in the main trachea.
nodules and cysts. The larger the nodule the more likely Tracheal wall is slightly thickened (arrow). I.J.mgwas
it is to be cystic. normal.

Morphology: Smaller nodules solid, more likely to

ITERMINOlOGY cavitate when larger
Abbreviations and Synonyms Radiographic Findings
Juvenile-onset recurrent respiratory papillomatosis, Radiography
adult-onset recurrent respiratory papillomatosis, o Multiple solid or cavitated nodules
human papilloma virus, invasive papillomatosis As nodules enlarge more likely to cavitate
Definitions Thick or thin wall, typically 2-3 mm in thickness
Laryngeal nodules due to human papilloma virus, Cavitation also seen in squamous cell carcinoma
and lung abscess, common complications of
usually self-limited infection
papillomatosis
o < 1% seed the lung, known as invasive
Slow growth (measured in decades)
papillomatosis
o At risk to develop squamous cell carcinoma (2%) Nodules with rapid growth suspicious for
squamous cell carcinoma
Air-fluid level infrequent; when present suggests
superinfection
I IMAGING FINDINGS Do not spontaneously regress
General Features o Tracheal wall thickening or nodularity
Best diagnostic clue: Multiple solid and cystic nodules Tracheal pathology often missed on chest
Location radiographs
o Perihilar and central location in coronal plane o Atelectasis curiously rare even though papillomas
o Dorsal distribution in axial plane (gravity seeding) grow into the airway lumen
Size: Typically 1-3 cm in diameter o Bronchiectasis secondary complication due to
repeated bronchial obstruction and infection

DDx: laryngeal Papillomatosis

Lymphangiomyomatosis Metastases
 lARYNGEAL PAPillOMATOSIS
I IMAGE GALLERY
Typical
(Left) Frontal radiograph
shows multiple small central
cysts (arrows).
Tracheostomy. Focal mass
right lower lobe. 16 year old
with long history of laryngeal
papillomas. (Right) Axial
NECT shows multiple less
than 1 em solid and
thin-walled cystic nodules.
Nodular carina from tracheal
papillomas (arrow). Larger 3
nodules are cystic, smaller
nodules are solid. 45

Typical
(Left) Axial NECT shows the
right lower lobe mass is a
focal area of consolidation
(arrow). Squamous
papillomas may extend
through the lung in a lipidic
growth pal/ern. Infection
must be excluded. Any
change would suggest
squamous cell carcinoma.
(Right) Axial H RCT shows
several extremely thin-walled
cysts and single nodule in
left lower lobe (arrow). One
nodule communicates with
airway (open arrow).

Typical
(Left) Axial NECT shows tiny
tracheal papilloma (arrow).
Papillomas may be extremely
small or even nonexistent.
(Right) Axial HRCT shows
mixed cystic and solid
nodules. Trachea is lobulated
due to circumferential
papillomas (arrow). Cluster
of ground-glass centriacinar
nodules in dorsal aspect of
right lung (curved arrows).
 AllERGIC BRONCHOPULMONARY ASPERGillOSIS

3
46

Frontal radiograph shows diffuse bronchial dilatation Frontal radiograph shows focal bronchial dilatation in
with a "glove-like" or "Y-shaped" configuraUon typical the RUL (arrows) from mucoid impaction in this patient
for allergic bronchopulmonary aspergillosis. (Courtesy j. with allergic bronchopulmonary aspergillosis. ABPA
Speckman, MO). often involves the upper lobes.

ITERMINOlOGY Radiographic Findings

Radiography
Abbreviations and Synonyms o Mucoid impaction
Allergic bronchopulmonary Aspergillosis (ABPA) Tubular, finger-in-glove increased opacity in
Definitions bronchial distribution
Hypersensitivity reaction to aspergillus fumigatus "Y"-shaped density
o Central bronchiectasis; predominantly cystic
o Colonization of tracheobronchial tree
o Fleeting areas of consolidation
Occurs in conjunction with asthma and cystic fibrosis
Allergic fungal sinusitis may occur alone or with ABPA CT Findings
o Symptoms including chronic sinusitis, purulent HRCT
sinus drainage o Bronchiectasis
May be associated with chronic eosinophilic Often affects multiple lobes
pneumonia or cryptogenic organizing pneumonia Central cystic and/or varicoid variety
(COP) Cystic or saccular bronchiectasis shows ballooned
bronchi that may have air-fluid levels
Varicose bronchiectasis has bulbous appearance
IIMAGING FINDINGS with dilated bronchi and interspersed sites of
constriction
General Features
o Mucus-filled dilated bronchi
Best diagnostic clue
May have air-fluid levels
o Mucoid impaction
o Lobulated masses
o Bronchiectasis
o Centrilobular nodules
Location: Predominantly upper lobes

DDx: Bronchial Obstruction/Dilatation

Bronchial Atresia Cystic Fibrosis

 AllERGIC BRONCHOPULMONARY ASPERGillOSIS
Key Facts
Terminology
Hypersensitivity reaction to aspergillus fumigatus
Occurs in conjunction with asthma and cystic fibrosis
Allergic fungal sinusitis may occur alone or with
ABPA
May be associated with chronic eosinophilic
pneumonia or cryptogenic organizing pneumonia
(COP)
Imaging Findings
Mucoid impaction
Tubular, finger-in-glove increased opacity in
bronchial distribution
Central bronchiectasis; predominantly cystic
Fleeting areas of consolidation
o Combination of above findings highly suggestive of
ABPA
o Atelectasis related to bronchial obstruction
o Areas of consolidation, ground-glass opacity (GGO)
I DIFFERENTIAL DIAGNOSIS
Bronchogenic Carcinoma with Obstruction
Mucoid impaction may have mass-like appearance and
sometimes resected as undiagnosed lung mass
Carcinoid make present as central endobronchial
lesion with mucoid impaction
Differentiating features; look for
o Endobronchial lesion
o Associated adenopathy
o No history of allergies or cystic fibrosis
Cystic Fibrosis and Other Forms of
Bronchiectasis
Cystic fibrosis can have identical radiographic pattern
and be associated with ABPA
History pertinent; positive sweat test
Eosinophilia, cutaneous reactivity to Aspergillus;
increased IgE and other tests as below indicate
superimposed ABPA
Bronchial Atresia
Likely sequela of vascular insult to lung during early
fetal development
Branching, tubular mass, representing mucoid
impaction
Segmental bronchus does not communicate with
central airway
Apical posterior segment of left upper lobe most
common
Difficult to distinguish from ABPA; look for
o Hyperinflation of lung with decreased vascular
markings
o No history of allergies or cystic fibrosis
Top Differential Diagnoses
Bronchogenic Carcinoma with Obstruction
Bronchial Atresia
Airway Obstruction from Foreign Body
Bronchocentric Granulomatosis
Pathology
Type I hypersensitivity reaction with IgE and IgG
release
Septate hyphae branching at 45 degree angle
May be progressive
Clinical Issues 3
Cough, wheezing, low grade fever, malaise
Oral corticosteroids treatment of choice 47
Primary Ciliary Dyskinesia (Immobile Cilia
Syndrome)
Manifested by immotile or dyskinetic cilia; leads to
poor mucociliary clearing and development of
bronchiectasis
Other manifestations include hearing loss and male
infertility
Bronchiectasis in the setting of immotile cilia
Dextrocardia in patients with Kartagener syndrome
Airway Obstruction from Foreign Body
Look for radiopaque foreign body
Air-trapping on expiratory film
History of aspiration
Bronchocentric Granulomatosis
Rare hypersensitivity lung disease may be caused by
Aspergillus species
Can be seen with ABPA or separate from it as response
to infection with mycobacteria, other fungi, or
Echinococcus
Distal airway lumen replacement by necrotizing
granulomas
Radiographically same as ABPA
Can have focal mass or lobar consolidation with
atelectasis
Tuberculosis
Look for associated cavitary changes; lobar
consolidation; more predominant centrilobular
nodularity/septal thickening; adenopathy
Cutaneous reactivity with Tuberculin skin test
Other Parenchymal Masses;Granuloma,
Metastasis, Arteriovenous Malformation
CECT may aid in diagnosis
History and bronchoscopy pertinent for focal masses
Arterial venous malformation shows tubular
serpiginous areas contiguous with vessels; may show
vascular enhancement with CECT
 AllERGIC BRONCHOPULMONARY ASPERGillOSIS
Occurs in 1-2% patients with asthma
Radiographically and Pathologically Distinct Associated with peripheral blood eosinophilia;
from Other Pulmonary Aspergillosis cutaneous reactivity to Aspergillus; increased total
Syndromes Including serum IgE concentration; serum precipitating
antibodies to Aspergillus fumigatus; and increased
Chronic necrotizing (semi-invasive) Aspergillus
serum antibodies of IgE, IgG, or both to Aspergillus
pneumonia
o Subacute process in patients with some degree of fumigatus
immunosuppression (CO PO, alcoholism, diabetes Diagnosis usually determined based upon
mellitus, steroids) . combination of clinical, laboratory, and radiographic
criteria, occasionally will require pathologic diagnosis
o Multiple nodular areas of opacity, consolidation
with or without cavitation or pleural thickening Natural History & Prognosis
Aspergilloma (fungus ball; mycetoma) Recurrent ABPA may result in widespread
o Develops in preexisting lung cavity bronchiectasis and fibrosis
3 o Does not invade the cavity wall
o May cause hemoptysis
35% of exacerbations are asymptomatic but may result
in lung damage
48 Angioinvasive Aspergillosis
o Occurs in severely immunosuppressed patients Treatment
o Halo sign: Nodules surrounded by halo of GGO Oral corticosteroids treatment of choice
o Pleural-based, wedge-shaped consolidation, may o Inhaled steroids not effective
cavitate Addition of oral itraconazole (Sporanox) may allow
o Air crescents similar to mycetomas o Resolution of consolidation and symptoms
o Steroid tapering, lowers maintenance dose
Patients with associated allergic fungal sinusitis benefit
I PATHOLOGY from surgical resection of obstructing nasal polyps and
inspissated mucus
General Features Allergic fungal sinusitis may require endoscopic sinus
Genetics: Higher frequencies of specific HLA-DR2 and surgery to improve drainage
HLA-DR5 genotypes found in association with ABPA Serial measurement of serum IgE useful to monitor
Etiology response to therapy
o Aspergillus fumigatus
Ubiquitous soil fungi
o Type I hypersensitivity reaction with IgE and IgG I SELECTED REFERENCES
release
1. Greene R:The radiological spectrum of pulmonary
Microscopic Features aspergillosis. Med Mycol. 43 Suppll:SI47-54, 2005
2. Moss RB:Pathophysiology and immunology of allergic
Septate hyphae branching at 45 degree angle bronchopuimonary aspergillosis. Med Mycol. 43 Suppi
Plugs of inspissated mucus containing Aspergillus and I:S203-6, 2005
eosinophils 3. Franquet T et al: Aspergillus infection of the airways:
computed tomography and pathologic findings. J Comput
Staging, Grading or Classification Criteria AssistTomogr. 28(1):10-6, 2004
May be progressive 4. Buckingham SJet al: Aspergillus in the lung: diverse and
Five stages coincident forms. Eur Radiol. 13(8):1786-800,2003
o Acute disease 5. Khan AN et al: Bronchopulmonary aspergillosis: a review.
o Remission Curr Probi Diagn Radiol. 32(4):156-68, 2003
o Exacerbation or recurrence 6. Kumar R: Mild, moderate, and severe forms of allergic
o Corticosteroid-dependent asthma bronchopulmonary aspergillosis: a clinical and serologic
evaluation. Chest. 124(3):890-2, 2003
o Endstage fibrosis 7. Gotway MB et al: The radiologic spectrum of pulmonary
Aspergillus infections. J Comput AssistTomogr.
26(2):159-73,2002
ICLINICAL ISSUES 8. McGuinness G et al: CT of airways disease and
bronchiectasis. Radiol Clin North Am. 40(1):1-19, 2002
Presentation 9. Moss RB:Allergic bronchopulmonary aspergillosis. Clin
Most common signs/symptoms RevAllergy Immunol. 23(1):87-104, 2002
o Cough, wheezing, low grade fever, malaise 10. Franquet T et al: Spectrum of pulmonary aspergillosis:
Sputum with thick mucous plugs (contain histologic, clinical, and radiologic findings. Radiographics.
21(4):825-37,2001
hyphae) 11. Vlahakis NE et al: Diagnosis and treatment of allergic
Other signs/symptoms bronchopulmonary aspergillosis. Mayo Clin Proc.
o Prick or intradermal skin testing with Aspergillus 76(9):930-8,2001
antigen positive reaction manifested by wheal and 12. Johkoh T et al: Eosinophilic lung diseases: diagnostic
flare accuracy of thin-section CT in 111 patients. Radiology.
Negative skin test excludes diagnosis of ABPA 216(3):773-80,2000
Occurs in approximately 10% patients with cystic 13. Ward S et al: Accuracy of CT in the diagnosis of allergic
fibrosis bronchopulmonary aspergillosis in asthmatic patients. AJR
Am J Roentgenol. 173(4): 937-42,1999
 AllERGIC BRONCHOPULMONARY ASPERGillOSIS
I IMAGE GAllERY

(Left) Frontal radiograph

shows focal RUL bronchial
dilatation in this asthmatic
patient with ABPA. In an
asthmatic patient, with
elevated IgE and skin
reactivity the diagnosis of
ABPA is likely. (Right) Axial
CECT shows the mucoid
impaction in the RUL
bronchus in this same patient
with ABPA. Evaluation at
3
multiple levels shows the
tubular nature of this 49
mucus-filled structure
(bronchus).

(Left) Axial CECT in the same

patient with ABPA shows
multifocaf bronchiectasis
(arrows) with a more
saccular area of dilatation
containing an air-fluid level
(open arrow). (Right)
Coronal scout topogram
shows a tubular opacity in
the right lower lobe (arrows)
corresponding to an area of
focal involvement with
ABPA. (Courtesy j.
Speckman, MO).

(Left) Axial CECT shows

extensive RLL mucoid
impaction in this same
patient with ABPA. The
location here in the lower
lobe is not as commonly
seen, as the upper lobes are
most commonly affected.
(Right) Axial CECT in the
same patient with ABPA
shows central bronchiectasis
with bronchial wall
thickening. Histologic
evaluation typically reveals
mucous with branching
hyphae and eosinophils.
 TRACHEOBRONCHOMALACIA

3
50

Graphic shows circumferential tracheal stenosis at the Axial NEeT at dynamic expiration shows frown-like
thoracic inlet following prolonged intubation. Stenosis configuration of tracheal lumen consistent with
may be isolated or may be accompanied by tracheomalacia.
lIacheomafacia.

o At end-inspiration, tracheal lumen may be normal

ITERMINOLOGY in size, widened in coronal (lunate trachea) or
Abbreviations and Synonyms sagittal (saber sheath trachea) diameter, or focally
Tracheomalacia, bronchomalacia narrowed (malacia may accompany focal stenosis)
o > 50% reduction in airway lumen at expiration
Definitions diagnostic
Increased compliance and excessive collapsibility of Morphology
trachea or bronchi o Intrathoracic trachea: Collapse with expiration due
to positive extratracheal pressures
o Extrathoracic trachea: Collapse with inspiration due
I IMAGING FINDINGS to negative intratracheal pressures

General Features Radiographic Findings

Best diagnostic clue: "Frown sign" (crescentic Radiography: Tracheobronchomalacia usually escapes
narrowing of tracheal lumen that resembles a frown) detection on routine, end-inspiratory CXR and CT
during expiration scans
Location CT Findings
o Diffuse
May involve entire trachea and/or bronchi NECT
o Paired inspiratory-dynamic expiratory CT
o Focal
Inspiratory CT provides comprehensive
May be seen following intubation, in conjunction
assessment of airway anatomy
with focal stenosis, or at site of long-standing
Dynamic expiratory CT provides assessment of
compression collapse of central airways during one helical
Size acquisition

DDx: Extrinsic Compression Trachea

Bronchogenic Cyst Aortic Arch Anomaly

 TRACHEOBRONCHOMALACIA
Key Facts
Terminology
Increased compliance and excessive collapsibility of
trachea or bronchi
Imaging Findings
Best diagnostic clue: "Frown sign" (crescentic
narrowing of tracheal lumen that resembles a frown)
during expiration
> 50% reduction in airway lumen at expiration
diagnostic
Radiography: Tracheobronchomalacia usually escapes
detection on routine, end-inspiratory CXR and CT
scans
Best imaging tool: Paired inspiratory-dynamic
expiratory helical CT imaging
Malacia defined as > 50% decrease in
cross-sectional area with expiration
Most common finding during dynamic
expiration: Tracheal collapse with crescentic
bowing of the posterior membranous trachea
("frown sign")
Multiplanar and 3D reconstructions not required
for diagnosis but help display extent of disease
o Cine CT during coughing
Coughing most sensitive method for eliciting
tracheal collapse
Can be performed with electron beam CT or
multidetector row CT
Requires multiple acquisitions to cover entire
central airways
MR Findings
50- to 100-msec imaging time allows cine evaluation
of tracheal collapse during coughing
Only limited data in the literature using this technique
Fluoroscopic Findings
Chest Fluoroscopy
o Cine fluoroscopy historically used to evaluate
tracheal wall mobility between inspiration and
forced expiration, or during coughing
o Diagnosis based upon> 50% decrease in airway
lumen during expiration or coughing
o Limitations: Subjective interpretation;
operator-dependent; inability to simultaneously
evaluate anteroposterior and lateral walls of trachea;
limited visualization of tracheal anatomy and
adjacent mediastinal structures
Imaging Recommendations
Best imaging tool: Paired inspiratory-dynamic
expiratory helical CT imaging
Protocol advice
o Helical CT with 2.5 or 3.0 mm collimation
50% overlapping reconstruction intervals for
multi planar reformations and 3D reconstructions
o Perform at suspended inspiration and during
dynamic, forced exhalation
Pathology
General path comments: Weakening of the cartilage
and/or hypotonia of the posterior membranous
trachea, with degeneration and atrophy of the
longitudinal elastic fibers
Acquired form relatively common in adults,
incidence increases with advancing age
Clinical Issues
Intractable cough, dyspnea, wheezing, recurrent
respiratory infections
Patients often misdiagnosed as having asthma
Acquired form usually progressive over time in the 3
absence of therapy
Surgical repair with tracheoplasty procedure for 51
severely symptomatic patients with diffuse malacia
Forced exhalation elicits greater collapse than
end-expiration
o Dynamic expiratory portion of scan can be
performed with low-dose (40 mAs) technique to
reduce radiation exposure
I DIFFERENTIAL DIAGNOSIS
Imaging Clues for Identifying Specific
Underlying Cause of Tracheomalacia
Chronic obstructive pulmonary disease (CaPO): Look
for emphysema or saber sheath trachea
Prior intubation: Look for focal stenosis
Relapsing polychondritis: Look for wall thickening +/-
calcification that spares posterior membranous wall
Long-standing extrinsic compression: Look for mass
adjacent to trachea (e.g., thyroid, vascular)
Radiation: Look for geographically marginated fibrosis
in paramediastinal region with traction bronchiectasis
Mounier-Kuhn: Look for tracheobronchomegaly
I PATHOLOGY
General Features
General path comments: Weakening of the cartilage
and/or hypotonia of the posterior membranous
trachea, with degeneration and atrophy of the
longitudinal elastic fibers
Etiology
o Primary tracheomalacia: Congenital weakness
Abnormal cartilaginous matrix (chondromalacia,
mucopolysaccharidoses such as Hurler syndrome)
Inadequate maturity of cartilage (e.g., premature
infants)
Congenital tracheoesophageal fistula
Mounier-Kuhn syndrome (congenital
tracheobronchomega Iy)
o Secondary, acquired tracheobronchomalacia
Chronic obstructive pulmonary disease
 TRACHEOBRONCHOMALACIA
Prior intubation (endotracheal tube or
tracheostomy tube)
Prior surgery (e.g., lung resection, lung
transplantation)
Radiation therapy
Long-standing extrinsic compression (e.g., thyroid
mass, vascular ring, aneurysm)
Chronic inflammation (e.g., relapsing
polychondritis)
Tracheoesophageal fistula
Idiopathic
Epidemiology
o Congential form more common in premature
infants
3 o Acquired form relatively common in adults,
incidence increases with advancing age
52 o 5-23% of patients undergoing bronchoscopy for
respiratory symptoms
o 5-10% of patients referred to pulmonologists for
chronic cough
o 10% of patients referred for CTA for suspected
pulmonary embolism
o Found in up to 20% of autopsies
Associated abnormalities: Congenital form often
associated with cardiovascular abnormalities,
bronchopulmonary dysplasia, and gastroesophageal
reflux
IClINICAllSSUES
Presentation
Most common signs/symptoms
o Intractable cough, dyspnea, wheezing, recurrent
respiratory infections
o Congenital form usually presents in the first weeks
to months of life with expiratory stridor, cough, and
difficulty feeding
Underdiagnosed condition
Patients often misdiagnosed as having asthma
o If X-ray requisition states asthma, always look for
tracheal stenosis, tracheal mass, or malacia
Rarely, hypoventilation, hypoxemia, hypercarbia,
pulmonary artery hypertension, cor pulmonale
Inspiratory wheeze if lesion extra thoracic; expiratory
wheeze if lesion intrathoracic
Post intubation: Symptoms may appear several weeks
to years after intubation
Bronchoscopic findings in tracheomalacia
o > 50% narrowing of lumen in AP diameter, (normal
< 40%)
o In children, ratio of the expiratory-inspiratory
cross-sectional area ratio < 0.35, (normal 0.82)
Demographics
Age: Neonates to elderly
Gender: Acquired form has male predominance
Natural History & Prognosis
Acquired form usually progressive over time in the
absence of therapy
Congenital form sometimes self-limited (especially in
premature infants with malacia due to immature
cartilage)
Treatment
Conservative therapy for mildly symptomatic patients
Nasal continuous positive airway pressure can help to
relieve nocturnal symptoms
Silicone stents for severely symptomatic patients who
are poor surgical candidates
Surgical repair with tracheoplasty procedure for
severely symptomatic patients with diffuse malacia
o Goals are to remodel trachea and increase its rigidity
by placing marlex graft along posterior wall
Surgical repair with aortopexy procedure when due to
long-standing extrinsic compression by vascular lesion
o Mechanical fixation of trachea releases compression
and widens anteroposterior dimension of trachea
I DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Recognize characteristics of expiratory CT scan to
ensure that expiratory component of scan is diagnostic
o Increased lung attenuation
o Decreased anteroposterior dimension of thorax
o Posterior wall of trachea should be flat or bowed
forward
Malacia defined on the basis of percentage change in
tracheal lumen between inspiration and expiration
o If either component of the scan is not performed
during the appropriate phase of respiration,
diagnostic errors may occur
o Coaching of the patient with careful breathing
instructions necessary to ensure diagnostic study
I SElECTED REFERENCES
1. Baroni RH et al: Tracheobronchomalacia: comparison
between end-expiratory and dynamic expiratory CT for
evaluation of central airway collapse. Radiology.
235(2):635-41, 2005
2. Carden KA et al: Tracheomalacia and
tracheobronchomalacia in children and adults: an in-depth
review. Chest. 127(3):984-1005, 2005
3. Boiselle PM et al: Tracheobronchomaiacia: evolving role of
dynamic multislice helical CT. Radiol Clin North Am.
41(3):627-36,2003
4. Hasegawa I et al: Tracheomalacia incidentally detected on
CT pulmonary angiography of patients with suspected
pulmonary embolism. AJR Am J Roentgenol.
181(6):1505-9,2003
5. Zhang J et al: 2003 AUR Memorial Award. Dynamic
expiratory volumetric CT imaging of the central airways:
comparison of standard-dose and low-dose techniques.
Acad Radiol. 10(7):719-24,2003
6. Gilkeson RC et al: Tracheobronchomalacia: dynamic
airway evaluation with multi detector CT. AJR Am J
Roentgenol. 176(1):205-10,2001
7. Marom EM et al: Focal abnormalities of the trachea and
main bronchi. AJR. 176: 707-11,2001
8. Suto Y et al: Evaluation of tracheal collapsibility in patients
with tracheomalacia using dynamic MR imaging during
coughing. AJR. 171:393-394, 1998
9. Stern EJ et al: Normal trachea during forced expiration:
dynamic CT measurements. Radiology 187:27-31, 1993
10. Breatnach E et al: Dimensions of the normal human
trachea. AJR. 142:903-6, 1984
 TRACHEOBRONCHOMALACIA

I IMAGE GALLERY
Typical
(Left) Axial NEeT at
end-inspiration shows
normal appearance of
tracheal lumen. (Right) Axial
NECT at dynamic expiration
shows frown-like
configuration of tracheal
lumen consistent with
tracheomalacia.

3
53

(Left) Sagittal NECT at

end-inspiration shows
normal caliber of tracheal
(T) lumen. (Right) Sagittal
NEeT at dynamic expiration
shows excessive narrowing
of intrathoracic trachea
consistent with
tracheomaJacia.

(Left) NECT virtual

bronchoscopic image at
end-inspiration shows
normal appearanceof airway
lumen at level of carina.
(Right) NECT virtual
bronchoscopic image at
dynamic expiration at/eve!
of carina shows excessive
bowing of posterior wall
(arrow) consistent with
malacia.
 RELAPSING POLYCHONDRITIS

3
54

Frontal radiograph shows diffuse tracheal wall Axial NEeT shows uniform thickening of the anterior
thickening (arrows) and narrowing of the tracheal and lateral tracheal walls with thickened calcific
lumen due to relapsing polychondritis. cartilage (arrows). Posterior membrane is spared (open
arrow). Relapsingpolychondritis.

Single, multiple, or diffuse foci

ITERMINOlOGY o Lung
Definitions Atelectasis or pneumonia due to proximal airway
Rare autoimmune episodic disorder that destroys stenosis
cartilage, especially of the ear, nose, and Diffuse alveolar hemorrhage associated with
laryngotracheobronchial tree glomerulonephritis
Bronchiectasis (25%) due either to recurrent
infections or direct destruction of cartilage
I IMAGING FINDINGS Air-trapping and bronchomalacia (50%)
o Aorta
General Features Aneurysmal dilatation especially ascending aorta
Best diagnostic clue: Increased thickness and increased Cardiac enlargement: Aortic or mitral valve
attenuation of tracheal wall regurgitation or from pericarditis
Location: Tracheal or lobar bronchi o Tracheal cartilage
Size: Stenosis usually focal and short segment Calcified with chronic disease
Morphology: Diffuse thickening tracheal wall sparing CT Findings
posterior tracheal membrane NECT
Radiographic Findings o Tracheobronchial tree
Radiography Increased attenuation of airway wall
o Tracheal and main bronchi Progressive calcification of airway wall
Diffuse thickening, sparing posterior tracheal Focal or diffuse involvement
membrane Early: Airway wall thickened
Stenosis: Fixed or variable Late: Stenosis and calcification of airway wall

DDx: Tracheal Wall Thickening

Tracheopathia Osteochondroplastica Rhinoscleroma Wegener Granulomatosis

 RELAPSING POLYCHONDRITIS

Key Facts
Terminology Saber-Sheath Trachea
Rare autoimmune episodic disorder that destroys Sarcoidosis
cartilage, especially of the ear, nose, and Amyloidosis
laryngotracheobronchial tree Laryngotracheal Papillomatosis
Rhinoscleroma
Imaging Findings
Morphology: Diffuse thickening tracheal wall sparing
Pathology
posterior tracheal membrane Epidemiology: 3.5 cases per million population
Bronchiectasis (25%) due either to recurrent Clinical Issues
infections or direct destruction of cartilage Prolonged remitting disease, diagnosis usually
Air-trapping and bronchomalacia (50%) delayed 3 years
Aneurysmal dilatation especially ascending aorta
Top Differential Diagnoses
Swelling and redness of ears (90%)
Nasal chondritis (50%) results in saddle nose
3
Wegener Granulomatosis deformity 55
Post-Intubation Stenosis Respiratory complications account for 30% of deaths
Tracheopathia Osteochondroplastica

Stenosis typically smooth, ranges from 1 cm to Nearly all have obstructive airways disease
entire length of trachea
Sarcoidosis
CECT
o Aorta Focal or diffuse nodularity of airway wall
Aneurysmal dilatation of aorta, especially Diffuse interstitial lung disease and or hilar
ascending aorta adenopathy invariably present
Aortic wall thickening Amyloidosis
Expiratory CT Diffuse thickening and nodularity of tracheal wall,
o May show dynamic collapse due to malacia usually circumferential
Nuclear Medicine Findings Nodules often calcify
Ga-67 Scintigraphy: Increased uptake in affected areas laryngotracheal Papillomatosis
Imaging Recommendations Younger patients
Protocol advice: Helical CT with multiplanar Diffuse nodularity of trachea and main bronchi
reconstructions May have multiple nodules or cystic lesions in the
lung
Rhinoscleroma
I DIFFERENTIAL DIAGNOSIS Subglottic circumferential narrowing
Wegener Granulomatosis Retropharyngeal and turbinate involvement
Infection Klebsiella rhinoscleromatis endemic in
Subglottic or diffuse tracheal involvement
Central America and Africa
Nodular or smooth thickening of tracheal wall
Often associated with multiple-thick walled Tracheal Wall Calcification
pulmonary cavities Normal process of aging
Post-Intubation Stenosis Long term warfarin therapy
History of long term intubation Tracheopathia osteochondroplastica
Smooth subglottic narrowing Amyloidosis
Airway wall not calcified
Tracheopathia Osteochondroplastica I PATHOLOGY
Multiple small calcified nodules that arise from
cartilaginous wall General Features
Also spares posterior membrane General path comments
Airway narrowing uncommon o Cartilage: Ribs, tracheobronchial tree, ear lobes,
Older age group nose, peripheral joints involved
o Pathologic findings nonspecific
Saber-Sheath Trachea Genetics: HLA-DR4
Intrathoracic deformity associated with chronic Etiology: Autoimmune disorder: Anticartilage
obstructive lung disease antibodies
o Coronal dimension less than 2/3rds of the sagittal Epidemiology: 3.5 cases per million population
dimension Associated abnormalities
Extrathoracic trachea normal o Other autoimmune disorders
 RELAPSING POLYCHONDRITIS
Systemic vasculitis (5%)
Autoimmune thyroiditis (10%)
Rheumatoid arthritis (5%)
Systemic lupus erythematosus (5%)
Sjogren syndrome (3%)
Inflammatory bowel disease (2%)
Gross Pathologic & Surgical Features
Chondrolysis, chondritis and perichondritis of affected
cartilage
Cartilage replaced by fibrous tissue with loss of volume
Microscopic Features
Cartilage shows nonspecific inflammatory infiltrate
3 acutely, replaced by fibrous tissue late
Staging, Grading or Classification Criteria
56
Diagnosis: Recurrent inflammation of 2 or more
cartilaginous sites
McAdam criteria (3 of 6 clinical features present)
o Bilateral auricular chondritis
o Nonerosive seronegative inflammatory polyarthritis
o Nasal chondritis
o Ocular inflammation
o Respiratory tract chondritis
o Audiovestibular damage
ICLlNICAllSSUES
Presentation
Most common signs/symptoms
o Prolonged remitting disease, diagnosis usually
delayed 3 years
Swelling and redness of ears (90%)
Arthralgias and arthritis (80%) especially
costochondral joints, usually spares forefeet
Nasal chondritis (50%) results in saddle nose
deformity
Skin: Redness and swelling over affected cartilage
Hearing loss (50%), often sudden
Cardiovascular disease (25%): Aortic or mitral
valvular regurgitation, aortic aneurysm,
pericarditis
o Respiratory tract (50%)
Initial presentation in 20%
Dyspnea, cough hoarseness, stridor, wheezing
Respiratory tract may be involved without other
sites
o Cardiac (25%)
Nonspecific shortness of breath, dyspnea on
exertion
o Glomerulonephritis (20%) may be due to circulating
immune complexes
Necrotizing glomerulonephritis or renal vasculitis
o Mouth and genital ulcers with inflammed cartilage
(MAGIC syndrome): Overlap of relapsing
polychondritis with Behcet disease
Other signs/symptoms
o Pulmonary function tests
Variable or fixed flow-volume loop
Reduction FEV1
Demographics
Age: Any age, average age at diagnosis: 50 years
Gender
o Equal
Airway involvement, however, more common in
women (3:1)
Natural History & Prognosis
Respiratory complications account for 30% of deaths
o Other causes of death: Infections secondary to
corticosteroid treatment, systemic vasculitis, renal
failure
75% 5 year survival
Treatment
Corticosteroids bolus with tapered long term
maintenance
Methotrexate in selected cases
Tracheostomy and airway stents for stenosed airways
Aortic aneurysm repair, cardiac valve replacement
I SELECTED REFERENCES
1. Faix LE et al: Uncommon CT findings in relapsing
polychondritis. AJNR Am J Neuroradiol. 26(8):2134-6, 2005
2. Gergely Pet al: Relapsing polychondritis. Best Pract Res
Clin Rheumatol. 18(5):723-38,2004
3. Kent PD et al: Relapsing polychondritis. Curr Opin
Rheumatol. 16(1):56-61, 2004
4. 5egel MJ et al: Relapsing polychondritis: reversible airway
obstruction is not always asthma. Mayo Clin Proc.
79(3):407-9, 2004
5. Braman 55: Diffuse tracheal narrowing with recurrent
bronchopulmonary infections. Relapsing polychondritis.
Chest. 123(1):289,90,2003
6. Behar JV et al: Relapsing polychondritis affecting the lower
respiratory tract. AJR Am J Roentgenol. 178(1):173-7,2002
7. Prince J5 et al: Nonneoplastic lesions of the
tracheobronchial wall: radiologic findings with
bronchoscopic correlation. Radiographics. 225pec
No(52 I5-30, 2002
8. 5taats BA et al: Relapsing polychondritis. 5emin Respir Crit
Care Med. 23(2):145-54, 2002
9. Yamazaki K et al: Large vessel arteritis in relapsing
polychondritis. J Laryngol Otol. 115(10):836-8,2001
10. Heman-Ackah YD et al: A new role for magnetic resonance
imaging in the diagnosis of laryngeal relapsing
polychondritis. Head Neck. 21(5):484-9,1999
11. 5arodia BD et al: Management of airway manifestations of
relapsing polychondritis: case reports and review of
literature. Chest. 116(6):1669-75, 1999
12. Lee-Chiong TL et al: Pulmonary manifestations of
ankylosing spondylitis and relapsing polychondritis. Clin
Chest Med. 19(4):747-57, ix, 1998
13. Tillie-Leblond I et al: Respiratory involvement in relapsing
polychondritis. Clinical, functional, endoscopic, and
radiographic evaluations. Medicine (Baltimore).
77(3):168-76,1998
14. Trentham DE et al: Relapsing polychondritis. Ann Intern
Med. 129(2):114-22, 1998
15. Adliff M et al: Treatment of diffuse tracheomalacia
secondary to relapsing polychondritis with continuous
positive airway pressure. Chest. 112(6):1701-4, 1997
16. Zeuner M et al: Relapsing polychondritis: clinical and
immunogenetic analysis of 62 patients. J Rheumatol.
24(1):96-101,1997
 RELAPSING POLYCHONDRITIS

IIMAGE GAllERY
Typical
(Left) Coronal reconstruction
shows diffuse thickening of
the tracheal wall and slight
narrowing of the distal lumen
(arrows) due to relapsing
polychondritis. (Right) Axial
NECT shows diffuse uniform
thickening of the anterior
and lateral tracheal wall
(arrows) with sparing of the
posterior tracheal
membrane. Putty-like
3
dystrophic calcification
(curved arrows). 57

(Left) Axial NECT shows

diffuse uniform thickening of
the anterior and lateral
tracheal walls with sparing of
the posterior tracheal
membrane (arrows) due to
relapsing polychondritis.
(Right) Axial NECT at a
narrow window better
demonstrates the increased
attenuation of the tracheal
wall (arrows). Wall is
thickened anteriorly and
laterally. Posterior tracheal
membrane spared. Relapsing
polychondritis.

Typical
(Left) Axial CECT at full
inspiration shows uniform
thickening of the tracheal
wall anteriorly and laterally
with sparing of the posterior
tracheal membrane (arrows).
Relapsing polychondritis.
(Right) Axial CECT in same
patient at full expiration
shows diffuse tracheal
malacia (arrows). Expiratory
CT useful to demonstrate
malacia and air trapping.
 MIDDLE LOBE SYNDROME

3
58

Frontal radiograph shows focal opacification of the right lAteral radiograph shows an anterior wedge-shaped
middle lobe and silhouetting of the superior right heart opacity from the collapsed right middle lobe (arrows).
border (arrow). Patient initially refused treatment.

Wedge-shaped opacity on lateral radiography

ITERMINOlOGY sharply marginated by major and minor fissures
Abbreviations and Synonyms CT Findings
Peripheral middle lobe syndrome, right middle lobe
CECT
syndrome o Bronchus
Definitions Extrinsic compression from lymph nodes or
Recurrent or fixed atelectasis or consolidation of the broncholiths
right middle lobe or lingula Endobronchial obstruction from tumor, foreign
body
o Lung
IIMAGING FINDINGS Varying degrees of atelectasis and consolidation
Bronchiectasis
General Features
Imaging Recommendations
Best diagnostic clue: Chronic right middle lobe
Best imaging tool: CT to exclude central obstructing
collapse or opacification
lesion and to evaluate for bronchiectasis
Location: Right middle lobe most common followed
by lingula
Radiographic Findings I DIFFERENTIALDIAGNOSIS
Radiography
Pneumonia
o Signs of chronic right middle lobe collapse
Silhouette right heart border on frontal film Acute illness, antibiotics should resolve in 4-6 wks
May be initial event in evolution of middle lobe
syndrome

DDx: Right Middle Lobe Syndrome

Mycobacterium Avium Complex Pneumonia Pectus Excavatum

 MIDDLE LOBE SYNDROME
Key Facts
Terminology Top Differential Diagnoses
Recurrent or fixed atelectasis or consolidation of the Pneumonia
right middle lobe or lingula Mycobacteria Avium Complex
Allergic Bronchopulmonary Aspergillosis (ABPA) or
Imaging Findings Cystic Fibrosis I
Location: Right middle lobe most common followed
by lingula Pathology
Best imaging tool: CT to exclude central obstructing Central obstruction (30%)
lesion and to evaluate for bronchiectasis Peripheral obstruction (70%)

Mycobacteria Avium Complex

Complete fissures: Right major fissure (200A,),
minor fissure (22%), left major fissure (27%)
3
Typically elderly females 59
Ventral bronchiectasis of middle lobe, lingula, or both Gross Pathologic & Surgical Features
Scattered nodules in affected and nonaffected lobes Combinations of bronchiectasis, bronchitis,
May be a cause of right middle lobe syndrome bronchiolitis, and organizing pneumonia
Allergic Bronchopulmonary Aspergillosis
(ABPA) or Cystic Fibrosis I CLINICAL ISSUES
Chronic mucus plugging may produce chronic middle
lobe or lingular atelectasis Presentation
Bronchiectasis usually involves other lobes Most common signs/symptoms: Chronic cough and
nonspecific chest pain
Pectus Excavatum Other signs/symptoms: Hemoptysis with central
Vague opacity in region of right middle lobe on obstructing lesions (rare with peripheral atelectasis)
frontal radiograph
Sternum depressed on lateral radiograph Demographics
Age: Any age, more common middle-aged
Gender: Women more common
I PATHOLOGY Treatment
General Features Bronchoscopy useful for diagnosis and treatment of
endobronchial disease
Etiology
o Central obstruction (30'1',,) Long term antibiotics with macrolides successful in
Extrinsic lymph nodes or bronchostenosis case reports
Endobronchial tumors (bronchogenic carcinoma, Surgical lobectomy for recurrent pneumonia
foreign bodies, carcinoid, papillomas)
o Peripheral obstruction (70%)
Lack of collateral ventilation due to complete I SELECTED REFERENCES
fissures rendering cough ineffective in clearing 1. Kwon KYet al: Middle lobe syndrome: a
secretions or inflammatory material clinicopathological study of 21 patients. Hum Pathol.
26(3):302-7, 1995

I IMAGE GALLERY

(Left) Frontal radiograph 14 months later again shows focal opacification of the right middle lobe. Patient now complained of hemoptysis.
(Center) Lateralradiograph shows wedge-shaped collapse of the right middle lobe. Diagnosis of right middle lobe syndrome. (Right) Axial CECT
shows a mass (arrows) obstructing the right middle lobe. Right middle lobe bronchus (curved arrow). Diagnosis: Carcinoid tumor causing
chronic right middle lobe collapse.
 SABER-SHEATH TRACHEA

3
60

Axial graphic just below the level of the thoracic inlet Axial CECT shows narrowing o( the trachea (curved
shows side-to-side narrowing of the intrathoracic arrow) in a patient with fibrosis (open arrow).
trachea (arrow). Saber-sheath trachea was diagnosed.

o Normal trachea
ITERMINOlOGY Sagittal diameter of 13-27 mm in men and 10-23
Abbreviations and Synonyms mm in women
Tracheal narrowing, tracheomalacia Coronal diameter of 13-25 mm in men and 10-21
mm in women
Definitions o Saber sheath tracheal measurements
A trachea in which the coronal dimension is less than Coronal and sagittal diameters < 13 mm in men
or equal to two thirds of the sagittal dimension and 10 mm in women
o Extrathoracic trachea normal Frontal tracheal diameter (FTD) / lateral tracheal
diameter (LTD) < 2/3
Morphology: Saber-sheath deformity: Narrowed
I IMAGING FINDINGS trachea on frontal view, widened on lateral view

General Features Radiographic Findings

Best diagnostic clue Posteroanterior chest radiograph shows diffuse
o "Blind spot" narrowing of coronal diameter of intrathoracic trachea
Tracheal pathology often overlooked Extrathoracic trachea is normal in diameter
Location FTD / LTD < 2/3
o Proximal thoracic airway o Specificity for chronic obstructive pulmonary
May involve entire intrathoracic trachea disease (COPD) 95%
Main bronchi are normal o Sensitivity for COPD < 10%
o Early: Thoracic inlet CT Findings
o Late: Intrathoracic trachea
Side to side narrowing of the trachea at and below the
Size
thoracic inlet

DDx: Tracheal Anomalies

Tracheomalacia Tracheomegaly
 SABER-SHEATH TRACHEA
Key Facts
Terminology Tracheopathia osteochondroplastica
A trachea in which the coronal dimension is less than Wegener granulomatosis
or equal to two thirds of the sagittal dimension Amyloidosis
Extrathoracic trachea normal Relapsing polychondritis
Sarcoidosis
Imaging Findings
Pathology
Posteroanterior chest radiograph shows diffuse
narrowing of coronal diameter of intrathoracic Etiologic and physiologic mechanisms responsible for
trachea the saber-sheath shape are uncertain
Extrathoracic trachea is normal in diameter Narrowing of trachea caused by deformity of tracheal
Best imaging tool: CT cartilage
Mucosa and submucosa are normal
Top Differential Diagnoses
Clinical Issues
3
Tracheal Stenosis
Tracheobronchomalacia Gender: Usually males 61
Tracheobronchomegaly
Infectious diseases

No tracheal wall thickening a Recurrent iower respiratory tract infections

Severe tracheal narrowing in its sagittal dimension a Bronchiectasis
Extrathoracic trachea remains normal in configuration Can be seen in association with other congenital or
connective tissue disorders
Imaging Recommendations a Cystic fibrosis
Best imaging tool: CT a Ankylosing spondylitis
Protocol advice: CT during forced expiration or a Marfan syndrome
Vaisalva maneuver shows inward bowing of tracheal
walls Diffuse Tracheal Narrowing
Infectious diseases
a Bacterial
I DIFFERENTIAL DIAGNOSIS Klebsiella rhinoscleromatis: Rhinoscleroma
a Fungal
Tracheal Stenosis a Viral
Short or long segment involvement Tracheopathia osteochondroplastica
a Narrowing often concentric a Rare
Side-to-side narrowing of the intrathoracic trachea on a Associated with aging
CT performed during or after forced expiration a Characterized by multiple submucosal cartilaginous
Stenosis typically occurs in the subglottic region at the or osseous nodules that project into tracheal lumen
cuff site of an endotracheal tube a Spares the posterior tracheal wall
a In patients who have undergone tracheostomy, Wegener granulomatosis
postextubation stenosis typically occurs at the stoma a Necrotizing granulomatous vasculitis
site a Characteristically involves upper and lower
respiratory tract
Trach eob ronchom alacia
a Primarily tracheal involvement subglottic
Causes tracheai narrowing with negative intratracheal
Amyloidosis
narrowing a Characterized by deposition of autologous fibrillar
Tracheal wall softening protein
a Due to an abnormality of the cartilaginous ring and a Tracheobronchial amyloid most common form of
hypotonia of the myoelastic elements respiratory system involvement
Thought to be caused by congenital immaturity of the a Multifocal or diffuse submucosal plaques or masses
tracheal cartilage in airway
Involves most of the trachea and other major airways a Any portion of tracheal wall including posterior wall
Can be associated with pulmonary sling Relapsing polychondritis
Traditionally viewed as a disease of infants and a Rare multisystem disorder
neonates a Results in inflammation and destruction of
Tracheobronchomegaly cartilaginous tissue
Marked dilatation of the trachea and mainstem Sarcoidosis
bronchi a Results from presence of perilymphatic granulomas
Also referred to as Mounier-Kuhn syndrome a Focal nodular thickening of segmental and
Frequently associated with subsegmental bronchi
a Tracheal diverticulosis a Tracheal involvement rare
 SABER-SHEATH TRACHEA
Stent fracture
I PATHOLOGY Stent migration
General Features Surgical options
General path comments o Tracheopexy
o Normal trachea has a coronal diameter of 13-25 mm o Tracheal resection
in men and 10-21 mm in women o Tracheal reconstruction
o Probably an acquired deformity of the trachea Treatment of underlying CO PO
May be related to abnormal pattern and
magnitude of intrathoracic pressure changes in
chronic obstructive pulmonary disease (COPD) I SELECTED REFERENCES
o Saber-sheath shape has been associated with 1. Carden KA et al: Tracheomalacia and
pulmonary dysfunction, specifically obstructive tracheobronchomalacia in children and adults: an in-depth
airways disease review. Chest. 127(3):984-1005,2005

3 Etiology
o Etiologic and physiologic mechanisms responsible
2. Epstein 5K: Late complications of tracheostomy. Respir
Care. 50(4):542-9, 2005
3. Berrocal T et al: Congenital anomalies of the
62 for the saber-sheath shape are uncertain tracheobronchial tree, lung, and mediastinum:
Trapped gas volume of upper lobe obstructive embryology, radiology, and pathology. Radiographies.
lung disease greatly restricts the potential 24(l):eI7,2oo4
side-to-side dimensions of the para tracheal 4. McNamara VM et al: Tracheomalacia. Paediatr Respir Rev.
mediastinum 5(2):147-54,2004
Forcing the trachea to remodel itself into a 5. Barnes NA et al: Bronchopulmonary foregut
saber-sheath configuration in some patients with malformations: embryology, radiology and quandary. Eur
Radiol. 13(12):2659-73,2003
COPD
6. Fukai I et al: Saber-sheath malacic trachea remodeled and
o Deformity of tracheal cartilage or chronic injury fixed into a normal shape by long-term placement and
Associated abnormalities: Emphysema then removal of gianturco wire stent. Ann Thorac 5urg.
76(2):597-8,2003
Gross Pathologic & Surgical Features 7. Wright CD: Tracheomalacia. Chest Surg Clin N Am.
Narrowing of trachea caused by deformity of tracheal 13(2):349-57, viii, 2003
cartilage 8. Franquet T et al: The retrotracheal space: normal anatomic
Mucosa and submucosa are normal and pathologic appearances. Radiographies. 22 Spec
Cartilage rings are usually calcified or ossified both No:S231-46, 2002
clinically and radiologically 9. Marom EM et al: Focal abnormalities of the trachea and
main bronchi. A)R Am) Roentgenol. 176(3):707-11, 2001
Inner wall of trachea usually smooth
10. Webb EM et al: Using CT to diagnose nonneoplastic
Microscopic Features tracheal abnormalities: appearance of the tracheal wall. A)R
Am) Roentgenol. 174(5):1315-21,2000
Selective destruction of cartilate that is infiltrated by
11. Stark Pet al: Imaging of the trachea and upper airways in
lymphocytes and macrophages patients with chronic obstructive airway disease. Radiol
Clin North Am. 36(1):91-105,1998
12. Imaizumi H et al: Reversible acquired
I CLINICAL ISSUES tracheobronchomalacia of a combined crescent type and
saber-sheath type.) Emerg Med. 13(1):43-9, 1995
Presentation 13. Trigaux J Pet al: CT of saber-sheath trachea. Correlation
Most common signs/symptoms: Those related to with clinical, chest radiographic and functional findings.
COPD and emphysema (up to 95%): Dyspnea, Acta Radiol. 35(3):247-50, 1994
14. Kwong)S et al: Diagnosis of diseases of the trachea and
shortness or breath
main bronchi: chest radiography vs CT. AJR Am)
Other signs/symptoms: Chronic cough Roentgenol. 161(3):519-22, 1993
Demographics 15. Kwong)S et al: Diseases of the trachea and main-stem
bronchi: correlation of CT with pathologic findings.
Age Radiographies. 12(4):645-57, 1992
o Nonspecific 16. Callan E et al: &quot;Saber-sheath&quot; trachea. Ann
Most emphysema patients tend to be older Otol Rhinol Laryngol. 97(5 Pt 1):512-5, 1988
(greater than 50 years) 17. Gamsu G et al: Computed tomography of the trachea:
o Not seen in children normal and abnormal. A)R Am J Roentgenol. 139(2):321-6,
Gender: Usually males 1982
18. Greene R: &quot;Saber-sheath&quot; trachea: relation to
Natural History & Prognosis chronic obstructive pulmonary disease. A)R Am)
Prognosis varied Roentgenol. 130(3):441-5, 1978
19. Greene R et ai: &quot;Saber-Sheath&quot; Trachea: A
o Dependent on severity of narrowing and degree of
Clinical and Functionai Study of Marked Coronal
COPD Narrowing of the Intrathoracic Trachea. Radiology.
Treatment 115(2):265-8, 1975

Tracheal stenting
o Complications and limitations include
Granulation tissue formation
 SABER-SHEATH TRACHEA
IIMAGE GALLERY

(Left) Axial CECT shows

normal caliber trachea at the
level of the thoracic inlet
(arrow). (Right) Frontal
radiograph shows long
segment narrowing of the
intrathoradc airway (arrow).

3
63

(Left) Magnification view of

previous image shows long
segment narrowing of the
intrathoracic trachea. There
is no significant airway
deviation or discernible
mass. (Right) Axial CECT
shows marked (greater than
SO%) narrowing of the
trachea (arrow) in a patient
with COPD.

(Left) Coned down axial CT

image at the thoracic inlet
shows elongated narrowing
of the airway in a patient
with saber-sheath trachea.
(Right) Axial CECT shows
marked narrowing of the
trachea (arrow) in this
patient with emphysema.
Saber-sheath trachea was
diagnosed.
 BRONCHIOLITIS OBLITERANS

3
64

Axial NECT shows subtle bronchial wall thickening Axial NECT in the same patient shows that after
(arrows)and areas of hypoattenuatlon (open arrows) in expiration the areas of hypoallenuatlon (open arrows)
a patient with 80 after bone marrow transplantation. becomes more conspicuous.

Diminished pulmonary vasculature

ITERMINOlOGY
Abbreviations and Synonyms
Bronchiolitis obliterans (BO)
Obliterative bronchiolitis (OB)
Constrictive bronchiolitis (CB)
Definitions
Concentric luminal narrowing of the membranous
and respiratory bronchioles secondary to submucosal
and peri bronchiolar inflammation and fibrosis
without intraluminal granulation tissue and polyps
Swyer-James syndrome: Postinfectious obliterative
bronchiolitis primarily involving one lung
IIMAGING FINDINGS
Radiographic Findings
Radiography
o Radiographic features of BO are nonspecific,
absent in all but the most severe cases
Overinflation of the lungs
Bronchial wall thickening ("tram lines")
o Radiographic findings of hyperinflation are prone to
substantial observer variation
o Unilateral hyperlucent lung
Swyer-James syndrome
CT Findings
HRCT
o Abnormalities of the macroscopic airways
Some degree of bronchial wall thickening and
dilatation the rule in patients with BO
Severity of bronchial abnormalities variable
In immunologically mediated BO (post
transplantation) marked dilatation of bronchi
frequent
Bronchiectasis common with post-infectious BO
o Air trapping at expiratory HRCT
Regional inhomogeneities of lung density are
accentuated on sections obtained at end
expiration
and Inspiratory scans may be completely normal
Involved lung variable in size: Individual lobules
to entire lobes

DDx: Bronchiolitis Obliterans

Pan/obu/ar Emphysema Hypersensitivity Pneumonitis Langerhans Granu/omatosis

 BRONCHIOLITIS OBLITERANS

Key Facts
Terminology Langerhans Cell Granulomatosis
Concentric luminal narrowing of the membranous Hypersensitivity Pneumonitis
and respiratory bronchioles secondary to submucosal Pathology
and peribronchiolar inflammation and fibrosis Idiopathic
without intraluminal granulation tissue and polyps Postinfectious
Imaging Findings Inhalational injury
Bronchiectasis common with post-infectious BO Connective tissue disorders
Air trapping at expiratory HRCT Drugs
Transplant recipients (occurs in 50% of long term
Inspiratory scans may be completely normal
lung transplant survivors)
Caveat: In patients with widespread disease,
end-expiratory CT sections may appear virtually
identical to inspiratory CT sections because air
Clinical Issues
Clinical course may resemble chronic obstructive
3
trapping extensive pulmonary disease, with the notable exception that 65
Top Differential Diagnoses the course of BO substantially more rapid
Asthma

Cross-sectional areas of affected lungs do not o CT window settings have a marked effect on
decrease in size on expiratory CT apparent thickness of bronchial wall and on lung
Density of affected lung areas does not increase on attenuation
expiratory CT o For imaging BO, CT window settings with centers
Expiratory CT helpful in differentiating between around -500 HU and widths around 1500 HU
the three main causes of mosaic pattern recommended
(infiltrative lung disease, occlusive pulmonary o HRCT protocol
vasculature disease, small airways disease) Full inspiration: 1 mm thin-sections at 10 mm
Caveat: In patients with widespread disease, intervals from lung apices to costophrenic angles
end-expiratory CT sections may appear virtually Full expiration: 1 mm thin-sections at 10 mm
identical to inspiratory CT sections because air from aortic arch and right hemidiaphragm
trapping extensive o Respiratory coaching by an experienced technologist
Compare the size of the hemithorax between the can substantially improve image quality
inspiratory and expiratory scan; lack of change
either due to patient cooperation or severe air
trapping I DIFFERENTIAL DIAGNOSIS
o Atelectasis
Common in post-infectious BO Asthma
From subsegmental or segmental in size No micronodules
Mosaic perfusion Mosaic perfusion may be identical
o Areas of decreased density of the lung parenchyma Panlobular Emphysema
Can have poorly defined margins or a sharp
Destruction of lung parenchyma
geographical outline
Lower lobe predominance
Relatively higher attenuation regions represent
No mosaic attenuation
relatively increased perfusion of normally
ventilated lung Langerhans Cell Granulomatosis
When severe, lung may be of homogeneously Micronodules more profuse
decreased attenuation Micronodules may cavitate
o Reduction in caliber of the macroscopic pulmonary Upper lobe predominance
vessels
In areas of decreased attenuation, perfusion Desquamative Interstitial Pneumonia
reduced Diffuse ground-glass opacities
In acute bronchiolar obstruction, this represents Patchy and subpleural
physiological reflex hypoxic vasoconstriction Not bronchocentric
In chronic state, vascular remodeling occurs and
reduced perfusion becomes irreversible
Hypersensitivity Pneumonitis
Similar radiographic findings
Imaging Recommendations Chronic disease will have more fibrosis
Best imaging tool: HRCT much more sensitive to
detect and characterize small airways disease
Protocol advice
o Expiratory sections are mandatory to visualize
potential air trapping
 BRONCHIOLITIS OBLITERANS
I PATHOLOGY ICLINICALISSUES
General Features Presentation
General path comments Most common signs/symptoms
o Nonspecific reaction from insults to the small o Clinical onset of BO related to the development of
airways (respiratory bronchioles) bronchitic symptoms associated with cough and
o Reactive submucosal and peri bronchiolar mucopurulent sputum
inflammation o Clinical course may resemble chronic obstructive
o May affect airways visible on HRCT and airways pulmonary disease, with the notable exception that
below the resolution of HRCT the course of BO substantially more rapid
o Subsequent concentric scarring with obliteration of o Upper and lower respiratory tract infections
the membranous and respiratory bronchioles frequently complicate BO
o Air flows rapidly down conducting airways (trachea o Infections may result in permanent colonization of
3 to terminal bronchioles) and then velocity decreases
rapidly to allow gas exchange
the airways with bacteria and fungi
o Early BO associated with decreases in the
66 o Small particles 5 microns) escape impacting into effort-independent flow-rates and a concavity of the
larger airways and are deposited on the expiratory flow pattern on the flow volume loop
nonconducting airways (respiratory bronchioles) o At later stages, effort dependent flow-rates decrease
Etiology o Larger airway malfunction may develop with further
o Idiopathic progression of disease
Truly idiopathic BO exceedingly rare o Tissue confirmation of BO by trans bronchial biopsy
o Postinfectious problematic because disease heterogeneously
Adenovirus distributed and biopsy may harvest false-negative
Respiratory syncytial virus tissue
Influenza
Mycoplasma pneumoniae Demographics
Mycobacterial Age: Depends on etiology
o Inhalational injury Gender: Idiopathic more common in women
Nitrogen dioxide ("Silo-filler's disease") Natural History & Prognosis
Sulfur dioxide
Pulmonary function may not improve and slowly
Ammonia decline with time
Phosgene
Hot gases Treatment
o Connective tissue disorders Steroids often tried with little benefit
Rheumatoid arthritis: May be related to drug Pulmonary rehabilitation program
treatment with gold and penicillamine Immunosuppressive therapy for transplant recipients
Sjogren syndrome
Systemic lupus erythematosus
o Drugs I SELECTED REFERENCES
Penicillamine
1. Bankier AAet al: Air trapping in heart-lung transplant
Lomustine recipients: variability of anatomic distribution and extent
o Transplant recipients (occurs in 50% of long term at sequential expiratory thin-section CT. Radiology.
lung transplant survivors) 229(3):737-42,2003
Lung or heart-lung transplant recipients 2. Boehler A et al: Post-transplant bronchiolitis obliterans. Eur
Bone marrow transplant recipients RespirJ. 22(6):1007-18, 2003
o Other conditions 3. Carris PA: Lung transplantation. Bronchiolitis obliterans
Inflammatory bowel diseases syndrome. Chest Surg Clin N Am. 13(3):543-57,2003
Bronchiectasis 4. RyuJH et al: Bronchiolar disorders. Am J Respir Crit Care
Med. 168(11):1277-92, 2003
Cystic fibrosis 5. Erasmus JJ et al: Drug-induced lung injury. Semin
Asthma Roentgenol. 37(1):72-81, 2002
Hypersensitivity pneumonitis 6. Estenne M et al: Bronchiolitis obliterans after human lung
Microcarcinoid tumorlets transplantation. Am J Respir Crit Care Med. 166(4):440-4,
Sauropus androgynus ingestion: Asiatic shrub used 2002
for weight control 7. Kim EAet al: Interstitial lung diseases associated with
Para neoplastic pemphigus collagen vascular diseases: radiologic and histopathologic
findings. Radiographies. 22 Spec No:SI5] -65, 2002
Microscopic Features 8. Schlesinger C et al: Bronchiolitis: update 2001. Curr Opin
Concentric fibrosis narrowing or obliterating Pulm Med. 8(2):112-6, 2002
bronchioles 9. Sharma V et al: The radiological spectrum of small-airway
diseases. Semin Ultrasound CT MR. 23(4):339-51, 2002
o Intraluminal granulation tissue polyps absent 10. Shaw RJet al: The role of small airways in lung disease.
Respir Med. 96(2):67-80, 2002
1]. Waitches GM et al: High-resolution CT of peripheral
airways diseases. Radiol Clin North Am. 40(1):21-9, 2002
 BRONCHIOLITIS OBLITERANS

I IMAGE GALLERY
Typical
(Left) Axial CECT in a lung
transplant recipient with 80
shows bronchial wall
thickening (open arrows)
and subtle peribronchial
opacities (arrow). (Right)
Axial CECT in a patient with
80 after bone marrow
transplantation shows
complicating infection
manifesting as patchy
ill-defined peribronchial 3
opacities (arrows).
67

(Left) Axial NECT at end

expiration in a patient with
80 shows bronchiectasis
(arrow) and diffuse air
trapping (open arrows).
Higher attenuation lung
(curved arrows) is normal.
(Right) Axial CECT in a
patient with postinfectious
80 shows bronchial wall
thickening (arrow) and
intrabronchial mucous plug
(open arrow).

(Left) Axial HRCT in a

patient with post-toxic 80
shows subtle attenuation
inhomogeneities of the lung
parenchyma (arrows).
Inspiratory scans may be
normal or near normal in
80 (Right) Axial HRCT at
end expiration reveals
extensive areas of air
trapping in the right lung
(open arrows). Vesselsin the
hypoattenuated lung are
small compared to those in
the higher attenuating lung.
 ASTHMA

3
68

Axial NECT shows airway with markedly thickened wall Axial NECT shows airway (arrow) with its distal part
and irregular inner lining of the lumen (arrows) in obstructed by a mucous plug (open arrow) in asthmadc
asthmatic padent. patient.

Small segments receive most of the air

I TERMINOlOGY (hyperventilated)
Definitions Summation of hypo and hyperventilated lung
Intermittent reversible obstruction to air flow in the often results in normal chest radiography
lung Bronchospasm leads to
Asthma is defined in physiological terms and requires o Hyperinflation: Flattened diaphragms, deep
physiological tests to establish diagnosis retrostemal space
Status asthmaticus: Medical emergency in which o Bronchial wall thickening: More common in
asthmatic attack refractory to bronchodilator therapy chronic asthma
o Atelectasis: Subsegmental to lobar due to airways
obstruction from mucus plugs
IIMAGING FINDINGS o Pulmonary artery hypertension (due to hypoxic
vasoconstriction of large portions of the pulmonary
Radiographic Findings vascular bed)
Limited role in diagnosis, important for complications Acute complications
of and processes that mimic asthma o Pneumomediastinum (5%)
In general, the more severe the bronchoconstriction, o Pneumothorax (0.3%)
the more likely the chest radiograph abnormal o Pneumonia (up to 2%)
Radiograph usually normal (75%) Chronic complications
o Obstruction to airflow in non-uniformly scattered o Allergic bronchopulmonary aspergillosis (ABPA)
throughout the lungs Up to 10% of steroid-dependent asthmatics
Large segments receive a small fraction of each Central bronchiectasis with sparing of distal
breath (hypoventilated) airways
More marked in the upper lung zones

DDx: Asthma

Cystic Fibrosis Bronchial Bleeding

 ASTHMA
Key Facts
Terminology Chronic upper airway obstruction
Intermittent reversible obstruction to air flow in the Airway foreign bodies
lung Cardiac asthma
Recurrent pulmonary embolus
Status asthmaticus: Medical emergency in which
asthmatic attack refractory to bronchodilator therapy Recurrent aspiration
Eosinophilic pneumonia
Imaging Findings Polyarteritis nodosa
In general, the more severe the bronchoconstriction, Carcinoid syndrome
the more likely the chest radiograph abnormal Vocal cord dysfunction (factitious asthma)
Radiograph usually normal (75%)
Clinical Issues
Status asthmaticus: Paradoxically chest radiograph
Mortality 2 deaths per 100,000 (last 2 decades 100%
often normal
Bronchiectasis suggests development of ABPA and
increase in death rate) 3
will change treatment Diagnostic Checklist 69
Top Differential Diagnoses Requisition for as~hma common, not everything that
wheezes asthma, consider the asthma mimics
Asthma Mimics

Status asthmaticus: Paradoxically chest radiograph

often normal
I DIFFERENTIAL DIAGNOSIS
o Stage 1: Hyperventilate to maintain oxygenation, Asthma Mimics
lung volumes increased Chronic upper airway obstruction
o Stage 2: Hyperventilate but unable to maintain o Subglottic stenosis (Wegener, post-intubation
oxygenation, lung volumes increased stricture)
o Stage 3: Fatigue, unable to hyperventilate, PC02 o Substernal thyroid and extrinsic tracheal narrowing
normalizes, hypoxic, lung volumes decrease to (vascular rings)
normal Airway foreign bodies
o Stage 4: Respiratory failure, PC02 rises, hypoxia, Cardiac asthma
lung volumes normal o Congestive heart failure, edema of airway wall
CT Findings narrows lumen
Recurrent pulmonary embolus
HRCT Recurrent aspiration
o More sensitive than chest radiography
Airtrapping: Mosaic lung attenuation, expiratory Eosinophilic pneumonia
air-trapping (20-50%) Polyarteritis nodosa
Churg-Strauss vasculitis
Bronchi: Bronchial wall thickening, mucoid
impaction, centrilobular nodules and tree-in-bud Carcinoid syndrome
opacities (20%) Sarcoidosis
Bronchocentric granulomatosis
o Bronchial dilatation: Subsegmental bronchi larger
Vocal cord dysfunction (factitious asthma)
than adjacent artery
o Conversion disorder, oxygenation normal, responds
True bronchiectasis mayor may not be related to
to anti-anxiety agents
ABPA
Artifactual due to hypoxic vasoconstriction
Physiologic due to ventilation at large lung
volumes I PATHOLOGY
o Emphysema General Features
Debatable whether secondary to asthma, usually
General path comments
only seen in those who smoke o Pathophysiology: Hyperinflation
Imaging Recommendations Intrinsic: Airway obstruction from pathologic
Best imaging tool airway narrowing
o HRCT useful to evaluate for bronchiectasis Physiologic: Increase in lung volume enlarges
Bronchiectasis suggests development of ABPA and airway caliber and offsets the intrinsic airway
will change treatment obstruction
Protocol advice Genetics
o Choose display window widths >1500 HU to avoid o Genetic variants associated with asthma present on
artificial bronchial wall thickening chromosomes 3, 5, 6, 11, 12, and 20
o Perform expiratory scans to visualize air-trapping o Polymorphisms in interleukin (IL)-13, located on
chromosome 5q, are associated to have a high IgE
and airway hyperresponsiveness
 ASTHMA
o Interaction of polymorphisms in the lL-13 and IL-4R
gene are associated with a fivefold increased risk for
asthma
Epidemiology
o Prevalence of childhood asthma 2-8%
o Prevalence of adult asthma 3%
Associated abnormalities: Rhinosinusitis up to 85%
Microscopic Features
Bronchial wall thickening: Mucus plugs, edema,
infiltration by eosinophils, lymphocytes and plasma
cells; smooth muscle hypertrophy, mucus gland
hypertrophy
3 Gastroesophageal reflux
ICLINICAllSSUES
70
Presentation
Most common signs/symptoms
o Severity of bronchospasm correlates with clinical
features
o Episodic wheezing, chest tightness, breathlessness
Symptoms may worsen at night, awakening the
patient
o History of allergic rhinitis or atopic dermatitis
o Prolonged phase of forced exhalation
o Increased nasal secretion, mucosal swelling,
sinusitis, rhinitis, or nasal polyps
Other signs/symptoms
o Peak expiratory flow rate (PEFR)
Two or three daily measurements in hospital, at
home, or at work
Asthma diagnosed when there is greater than 20%
diurnal variation on 3 or more days in a week for
2 weeks on a peak flow diary
o Spirometry
Airways obstruction determined by drop of forced
expiratory volume in 1 second (FEV,) below 80%
of predicted, or if ratio of FEV, to forced vital
capacity is less than 75%
o Other lung function tests
Flow-volume curves: Can help differentiate
between asthma and chronic obstructive
pulmonary disease (COPD)
Single-breath gas transfer factor: Can be normal in
asthma and reduced in COPD
o Skin prick testing for atopic state
May identify potential trigger
Lifestyle or workplace modifications for known
allergen
o Measurement of airway hyperresponsiveness
FEV, measurements during incremental
administration of histamine or methacholine
Administration stopped once a 20% decrease of
FEV, reached
Result expressed as "provocative dose"
Provocative dose reflects degree of airway
sensitivity that can reflect asthma severity
o Sputum examination
Sputum eosinophil counts may serve as marker for
airway inflammation
Rises in sputum eosinophils may predict
imminent loss of asthma control
o Asthma triad: Nasal polyps, urticaria, asthma
following aspirin ingestion (up to 10% of
asthmatics)
o Trigger factors
Infections: Rhinoviruses, influenza virus,
respiratory syncytial virus
Exercise: Especially in cold weather
Changes in climate: Thunderstorms
Pollution: Ozone and sulfur dioxide
Occupational factors: Dusty, cold, and wet rooms
Drugs: Aspirin, beta-blockers, nonsteroidal
anti-inflammatory agents
Allergens: Pet allergens, house dust, cockroach
allergens, pollens
Smoking
Clinical Profile
o Step 1: Mild and intermittent
;s; 2 days with symptoms per week, ;s; nights with
symptoms per week, '" 80% peak expiratory flow,
< 20% peak expiratory flow variability
o Step 2: Mild and persistent
3-6 days with symptoms per week, 3-4 nights with
symptoms per month, '" 80% peak expiratory
flow, 20-30% peak expiratory flow variability
o Step 3: Moderate and persistent
Daily symptoms, '" 5 nights with symptoms per
month, > 60 to < 80% peak expiratory flow, > 30%
peak expiratory flow variability
o Step 4: Severe and persistent
Continuous symptoms, frequent nights with
symptoms, ;s; 60% peak expiratory flow, > 30%
peak expiratory flow variability
Natural History & Prognosis
Mortality 2 deaths per 100,000 (last 2 decades 100%
increase in death rate)
Treatment
Medical therapy directed towards bronchoconstriction
and inflammation of airway wall
o Bronchodilators: Long-acting B-agonists,
theophyllines, corticosteroids
o Anticholinergic agents: Ipratropium bromide
Preventive therapies
o Inhaled corticosteroids
o Cromoglycate: Mast cell stabilizer
o Leukotriene modifying agents
I DIAGNOSTIC CHECKLIST
Consider
Requisition for asthma common, not everything that
wheezes asthma, consider the asthma mimics
I SElECTED REFERENCES
1. de Jong PAet al: Computed tomographic imaging of the
airways: relationship to structure and function. Eur Respir
J. 26(1):140-52, 2005
2. Mitsunobu F et al: The use of computed tomography to
assess asthma severity. CUff Opin Allergy Clin Immuno!.
5(1):85-90, 2005
 ASTHMA

I IMAGE GALLERY
Typical
(Left) Frontal radiograph
during asthma attack. The
hemidiaphragms are
flattened (arrows) from
hyperinflation. Requisition
for asthma should also
initiate examination for
asthma mimics. (Right)
Frontal radiograph after
administration of
bronchodilators. The
hemidiaphragms have 3
regained their normal shape
(arrows). Return to norma! 71
also seen in status
aslhmaticus as patient tires
and goes into respiratory
failure.

(Left) Frontal radiograph

shows masking of the right
heart border ("silhouette"
sign) caused by middle lobe
pneumonia in a patient with
asthma (arrows). (Right)
Axial CECT shows
pneumomediastinum in an
asthmatic patient after cough
attack. Note air around
esophagus (arrow), aorta
(curved arrow), and
aOrlopulmonary window
(open arrow).

(Left) Axial CECT shows

extensive pneumothorax
after cough attack in a
patient with severe asthma.
Pneumothorax much less
common than
pneumomediastinum.
(Right) Axial CECT shows
eosinophilic pneumonia in a
patient with asthma. Note
peripheral opacities of
ground-glass allenuation
(arrows) in the upper lobes.
 PANLOBULAR EMPHYSEMA

3
72

Axial graphic shows typical appearance of PLf: Axial HRCT shows widespread areas of parenchymal
Unevenly and heterogeneous areas of lung destruction destruction (arrows). Other than in CLf, the borders of
that transgress the structure of the secondary pulmonary the secondary pulmonary lobule are not preserved.
lobule.

I TERMI NOLOGY IIMAGING FINDINGS

Abbreviations and Synonyms General Features
Panacinar emphysema (PLE) Best diagnostic clue: Ill-defined absence of lung
parenchyma, distinction between normal and
Definitions emphysematous lung may be very difficult
Emphysema general: Abnormal permanent Location: Homogeneously distributed in the lung,
enlargement of any or all parts of the acinus, occasionally with lower lobe predominance
accompanied by destruction of alveolar tissue, but Size: Larger than CLE, can range from 1-2 cm to 10-15
without fibrosis cm
PLE: Enlargement and destruction of respiratory Morphology
bronchioles that involves the entire secondary o Destruction of lung parenchyma that uniformly
pulmonary lobule, and that can transgress and destroy affects the secondary pulmonary lobule
this anatomical structure o Ill-defined margins between normal and
Commonly associated with alpha-I-antitrypsin emphysematous lung creates homogeneous
deficiency appearance
Less common than centrilobular emphysema (CLE)
Chest radiograph insensitive for mild disease, which Radiographic Findings
emphasizes the role of CT Radiography
o Mild disease: Very insensitive, radiography may be
completely normal
Problem is recognition of loss of normal lung

DDx: Panlobular Emphysema

PLE (Right) And CLE (Left) Langerhans Granulomatosis Lymphangioleiomyomatosis

 PANLOBULAR EMPHYSEMA
Key Facts
Terminology Ill-defined margins between normal and
PLE: Enlargement and destruction of respiratory emphysematous lung creates homogeneous
bronchioles that involves the entire secondary appearance
pulmonary lobule, and that can transgress and Top Differential Diagnoses
destroy this anatomical structure Technical Considerations
Commonly associated with alpha-I-antitrypsin Athletic Hyperinflation
deficiency Centrilobular Emphysema
Chest radiograph insensitive for mild disease, which
Asthma
emphasizes the role of CT
Pathology
Imaging Findings
Familial alpha-I-antitrypsin deficiency (common
Best diagnostic clue: Ill-defined absence of lung
parenchyma, distinction between normal and
etiology) 3
emphysematous lung may be very difficult Diagnostic Checklist
Location: Homogeneously distributed in the lung, 73
PLE diffusely involves the entire lung parenchyma,
occasionally with lower lobe predominance has a homogeneous appearance, and is difficult to
distinguish from normal lung parenchyma

Normal lung on chest radiograph 90% air, making Wide windows may cause false negatives
detection of slight increases in air nearly
impossible to detect Athletic Hyperinflation
Consequence: Weak correlations between Lung normal, young athlete
functional indices and radiographic findings Centrilobular Emphysema
o Advanced disease: May be visible on radiograph
More common in upper lung zones
Attenuation inhomogeneities
Lesions are more subtle and have distinct borders
Vascular distortion and disruption
Appearance more heterogeneous
Increased branching angle of remaining vessels
Distinction between normal and emphysematous lung
o Hyperinflation
easier
Flat hemidiaphragms
Sagittal diameter of thorax increased Asthma
Widened retrosternal space No parenchymal destruction
Widened retrocardiac space Hyperinflation may be reversible with bronchodilators
Small and narrow heart
o Secondary manifestations Bulla
Pulmonary arterial hypertension: Enlarged central Emphysematous space, commonly subpleural, greater
pulmonary arteries, peripheral arterial pruning than 1 cm in the distended state
May coexist with PLE
CT Findings
HRCT Adenomatoid Malformation
o HRCT more sensitive than chest radiography Typical clinical presentation
o Can detect clinically and functionally "silent" PLE Well-defined margins with completely destroyed lung
o Emphysematous lesions usually have no discernible areas
wall
Langerhans Cell Granulomatosis
o PLE can destroy anatomical structure of the
secondary pulmonary lobule Lesions are smaller, well-defined, and have distinct
o Distinction between normal and emphysematous borders
lung can be very difficult Interstitial markings are pronounced
o Objectively measured by assuming that lung with a Lesions predominate near bronchovascular bundles
threshold HU < -960 is emphysematous lung Lym phangioleiomyomatosis
Imaging Recommendations Lesions are well-defined and have distinct borders
Best imaging tool: HRCT for detection Interstitial markings are pronounced
Protocol advice Ground-glass opacities usually absent in PLE
o Acquire scans in full suspended inspiration only
o Expiratory scans are of little value in PLE
!PATHOLOGY
General Features
I DIFFERENTIAL DIAGNOSIS Genetics: Familial PLE associated with
Technical Considerations alpha-I-antitrypsin deficiency
Low dose techniques may have false negatives Etiology
 PANLOBULAR EMPHYSEMA
a Familial alpha-I-antitrypsin deficiency (common
etiology)
a Familial PLE not associated with alpha-I-antitrypsin
deficiency, genetic correlates unknown
a Incidental PLE: May be found in 5-10% of random
autopsies
a PLE associated with CLE and chronic airflow
obstruction
a Congenital bronchial atresia
a Intravenous drug abusers
Epidemiology: Strongly depends on etiology
Associated abnormalities
a Chronic bronchitis
a Hyperinflation
3 a Recurrent infection
a Secondary pulmonary hypertension
74 Pathologic functional correlation
a Patients may have anatomic emphysema without
alteration of pulmonary function
a Approximately 30% of the normal lung must be
destroyed before pulmonary function deteriorates
a Pulmonary function tests global summation of
airways and lung, but HRCT provides regional
information
Gross Pathologic & Surgical Features
PLE substantially more homogeneous than CLE
Involves entire secondary pulmonary lobule that will
eventually be transgressed and destroyed
Homogeneous distribution with slight lower lobe
predominance
Microscopic Features
Early stages primarily involve alveolar ducts and sacs
Alveoli then become enlarged and flattened
Extensive destruction of alveolar walls the key feature I SELECTED
Staging, Grading or Classification Criteria
Because PLE defined in strictly morphological terms,
correlation with pathology and microscopy should
always be sought
HRCT allows for objective quantification of
emphysema
ICLINICAl ISSUES
Presentation
Most common signs/symptoms
a Mild disease
Often asymptomatic
May be incidental finding on HRCT 6.
a Advanced disease
Dyspnea, shortness of breath 7.
Increased total and residual lung volumes
Residual volume> 120% predicted 8.
Forced expiratory volume in one second < 80%
predicted
Diffusion capacity decreased < 80% predicted 9.
Other signs/symptoms
a Pulmonary hypertension
a Recurrent infection
Demographics
Age: Depends on etiology
Gender: Slight male predominance
Natural History & Prognosis
Progresses rapidly towards end-stage disease if
untreated
Treatment
Exercise
Nutritional and physical therapy support
Bronchodilators
Prevention of infections
Lung volume reduction surgery
a Undergoing randomized trial
a Candidates primarily those with inhomogeneous
emphysema
Bullectomy
a When bullae> 50% of hemithorax
Lung transplantation
I DIAGNOSTIC CHECKLIST
Consider
PLE diffusely involves the entire lung parenchyma, has
a homogeneous appearance, and is difficult to
distinguish from normal lung parenchyma
Image Interpretation Pearls
Ill-defined areas of lung destruction
No clearly visible borders
Best seen on HRCT
REFERENCES
1. Rankier AA et al: CT quantification of pulmonary
emphysema: assessment of lung structure and function.
Crit Rev Com put Tomogr. 43(6):399-417, 2002
2. Copley SJ et al: Thin-section CT in obstructive pulmonary
disease: discriminatory value. Radiology. 223(3):812-9,
2002
3. Sugi K et al: The outcome of volume reduction surgery
according to the underlying type of emphysema. Surg
Today. 31(7):580-5, 2001
4. Rankier AA et al: Pulmonary emphysema: subjective visual
grading versus objective quantification with macroscopic
morphometry and thin-section CT densitometry.
Radiology. 211(3):851-8, 1999
5. Slone RM et al: Preoperative and postoperative imaging in
the surgical management of pulmonary emphysema.
Radiol Clin North Am. 36(1):57-89, 1998
Webb WR: Radiology of obstructive pulmonary disease.
AJR Am J Roentgenol. 169(3):637-47, 1997
Stern EJ et al: CT of the lungs in patients with pulmonary
emphysema. Semin Ultrasound CT MR. 16(5):345-52, 1995
Stern EJ et al: CT of the lung in patients with pulmonary
emphysema: Diagnosis, quantification, and correlation
with pathologic and physiologic findings. AJR 162:791-8,
1994
Stern EJ et al: Pan lobular pulmonary emphysema caused by
Lv. injection of methylphenidate (Ritalin): findings on
chest radiographs and CT scans. AJR AmJ Roentgenol.
162(3):555-60, 1994
 PANLOBULAR EMPHYSEMA

I IMAGE GAllERY

(Left) Axial H RCT shows

panlobular emphysema.
Note that disease is equally
distributed in upper (white
arrows) and lower (black
arrows) lobe. (Right) Axial
CECT shows patient with
extensive PLE and bilateral
pneumonia (arrows) as
manifested by ground-glass
opacities. Due to
emphysema, the distribution 3
of pneumonia is atypical
75

Typical
(Left) Axial CECT shows
patient with extensive PLE.
One of the left apical
emphysematous lesions has
been c%nia/ized with
aspergillus (arrow). (Right)
Axial CECT shows PLE in a
cigarelle smoker !left).
Follow-up 6 months later
(right) shows newly
occurred adenocarcinoma
(open arrow) with
ground-glass halo (arrows).

Typical
(Left) Axial HRCT shows PLE
combined with chronic
bronchitis. Note areas of
lung destruction with thick
walled bronchi (curved
arrows), some filled with
mucus (arrow). (Right)
Coronal NECT shows
extensive PLE.
Emphysematous destruction
is equally present at the apex
(black arrows) and the base
(white arrows) of the lung.
 LANGERHANS CELL GRANULOMATOSIS, PULMONARY

3
76

Graphic shows solid and cavitary nodules, and thin Axial HRCT shows multiple cysts and nodules (arrows).
walled cysts in upper and mid lungs. Cysts may be The cysts have variable wall thickness and shape.
septated or lobulated and typically have visible thin Septated and bizarre-shaped cysts are shown (curved
walls.Sparing o( the costophrenic angles is typical. arrows).

o Cysts with variable wall thickness

ITERMINOLOGY
Radiographic Findings
Abbreviations and Synonyms Radiography
Eosinophilic granuloma or histiocytosis X; Langerhans o Diffuse symmetric nodular and reticulonodular
cell histiocytosis (LCH) is preferred opacities
Definitions o Multiple ill-defined nodules, 1-10 mm in diameter
Diffuse destructive disorder of distal airways from o Cysts, 1-3 cm in diameter
granulomas that contain Langerhans cells Walls of cysts may be imperceptible
o Upper and mid lung, sparing costophrenic angles
o Lung volume preserved or increased
IIMAGING FINDINGS o Pneumothorax, recurrent, unilateral or bilateral
o May see rib involvement: Lytic expansile lesion(s)
General Features with beveled edges
Best diagnostic clue: Reticulonodular opacities in o Uncommon features
upper and middle lung zones in a smoker Lymphadenopathy
Location Airspace opacification
o Upper and mid lung Solitary nodule
o Bilateral symmetric Pleural effusion
o Spares costophrenic angles May have a normal radiograph
Size: Nodules, 1-10 mm in diameter; cysts, 1-3 cm in CT Findings
diameter
NECT: Upper and mid lung predominance, sparing
Morphology costophrenic angles
o HRCT: Irregular stellate nodules
HRCT

DDx: Cysts, Cavities, or Nodules

LT Papillomatosis LAM
 LANGERHANS CELL GRANULOMATOSIS, PULMONARY

Key Facts
Imaging Findings Pneumocystis Jiroveci Pneumonia
Best diagnostic clue: Reticulonodular opacities in Sarcoid
Hypersensitivity Pneumonitis (e.g., Farmer's Lung)
upper and middle lung zones in a smoker
Spares costophrenic angles Pathology
Lung volume preserved or increased LCH probably an allergic reaction to some
Pneumothorax, recurrent, unilateral or bilateral constituent of smoke
May see rib involvement: Lytic expansile lesion(s)
with beveled edges Clinical Issues
HRCT: Irregular, small nodules and bizarre shaped May regress, resolve completely, become stable, or
cysts; surrounded by normal lung progress to advanced cystic disease
Cysts: Spherical, lobulated, septated or partially Burned out disease may resemble panacinar
septated, bilobed, cloverleaf, confluent,
bizarre-shaped
emphysema or honeycombing 3
Diagnostic Checklist
Top Differential Diagnoses 77
Characteristic HRCT appearance may obviate need for
Lymphangioleiomyomatosis (LAM) biopsy
Laryngotracheal Papillomatosis (LT Papillomatosis)

o HRCT findings may be characteristic Cysts usually lower lobes and dorsal aspect of lung
o HRCT: Irregular, small nodules and bizarre shaped
cysts; surrounded by normal lung
Pneumocystis Jiroveci Pneumonia
o Nodules: Centrilobular, peribronchial, Pneumatoceles that resemble cysts
peri bronchiolar Cysts occur in areas of ground-glass opacification,
Indistinct irregular or smooth nodules, 1-10 mm, unlike LCH
maybe> 1 cm Silicosis
Some cavitate with thick walls Often upper lobe, nodules located in lymphatics and
Few or innumerable nodules; solitary nodule, rare will also be seen along pleura
Progression of nodules to cavitary nodules to cysts Egg-shell calcification lymph nodes
o Cysts: More common than nodules Progressive massive fibrosis, peripheral emphysema
Cysts 1-10 mm, may be > 1 cm
No cysts
Cysts with thin or thick walls
Cysts: Spherical, lobulated, septated or partially Sarcoid
septated, bilobed, cloverleaf, confluent, Often upper lobe, nodules located in lymphatics and
bizarre-shaped will also be seen along pleura
Cysts alone or with nodules o Unusual for nodules of Langerhans cell histiocytosis
o Ground-glass opacities, interstitial lines, septal lines, Lymphadenopathy, hilar/mediastinal; nodule
irregular bronchovascular bundles cavitation, very rare
o Fibrosis, honeycombing Endstage disease: Upper lobe fibrosis, cysts,
honeycombing
Imaging Recommendations o Resembles end stage Langerhans cell histiocytosis
Best imaging tool: HRCT: To show characteristic
appearance of nodules and/or cysts Hypersensitivity Pneumonitis (e.g., Farmer's
Protocol advice: HRCT, 1-3 mm thick cuts, supine and Lung)
prone
May be upper lobe, also spares costophrenic angles
Nodules identical to LCH
May have cysts but usually few in number
I DIFFERENTIAL DIAGNOSIS
Tuberculosis
Lymphangioleiomyomatosis (LAM) Sputum smears and cultures to distinguish
Unique to females, unless related to tuberous sclerosis
Spherical cysts, uniformly distributed, involves Bullous Emphysema
costophrenic angles Identical appearance with end stage LCH
Cysts surrounded by normal lung
Nodules uncommon
Chylothorax (pleural effusions rare in LCH) I PATHOLOGY
Pneumothoraces, unilateral, bilateral, recurrent
General Features
Laryngotracheal Papillomatosis (LT General path comments: Diffuse destructive disorder
Papillomatosis) of distal airways caused by granulomas containing
Laryngeal and tracheal nodules Langerhans cells
Nodules may cavitate Etiology
 LANGERHANS CELL GRANULOMATOSIS, PULMONARY
a LCH probably an allergic reaction to some o Hand-Schuller-Christian Disease: Young adults and
constituent of smoke adolescents
a Smoke may stimulate cytokine production causing Involves lung, bone and pituitary - diabetes
activation of Langerhans cells insipidus (adults and adolescents)
a Reported following radiation treatment or o Letterer-Siwe: Infants, multiorgan involvement,
chemotherapy for Hodgkin disease malignant Langerhans cells, poor prognosis
Epidemiology Pulmonary function tests: Reduced carbon monoxide
a Uncommon smoking-related lung disease (95% diffusing capacity, normal total lung capacity
smoke) Diagnosis: HRCT can be characteristic
Only a small percent of smokers develop LCH o Transbronchiallung biopsy
a Bone involvement and diabetes insipidus, < 10% o Bronchioalveolar lavage (BAL) with> 5% COlA
Associated abnormalities: Associated with lymphoma, positive Langerhans cells
leukemia, lung cancer and other solid nonlymphoid a Open lung biopsy, if all else fails
tumors
3 Gross Pathologic & Surgical Features
Demographics
Age: Most common ages 20-40, range 1-69 years old
78 Majority of adult patients with pulmonary Gender: M = F
involvement have disease limited to the lung Ethnicity: Caucasian adults, less likely in
Cellular and fibrotic lesions with variable cyst African-Americans
formation
End stage fibrosis, honeycombing, cysts and Natural History & Prognosis
emphysema Early phase, predominantly nodular pattern; later
phase, predominantly cystic pattern
Microscopic Features May regress, resolve completely, become stable, or
Proliferation of Langerhans cells in the bronchial and progress to advanced cystic disease
bronchiolar epithelium 75% of patients eventually have resolution or stable
a Contain Birbeck granules, seen with electron disease
microscopy May recur up to 7 years after presentation, even with
a Numerous surface dendritic processes smoking cessation
a Intracellular protein S-100 and surface marker COlA May recur in transplanted lung
a Need T-cell lymphocytes to be activated into Burned out disease may resemble panacinar
granulomas emphysema or honeycombing
a Lymphocytes, macrophages and eosinophils mediate Pulmonary artery hypertension, 33%
tissue damage Variable prognosis from complete remission to
Nodules: Bronchiolocentric, stellate-shaped centered respiratory failure
in walls of terminal and respiratory bronchioles o Mortality is < 5%, worse in men, elderly, and in
o Typically < 1 em in diameter, but may be as large as patients with recurrent pneumothoraces
1.5-2 em
o Separated by relatively normal or somewhat Treatment
distorted lung Smoking cessation
o Frequently cavitate; cavity represents enlarged Steroids if disease is progressing
airway lumina Chemotherapy for childhood disease
o Form thick- and thin-walled cysts Lung transplantation for advanced disease
Adjacent lung may show desquamative interstitial
pneumonitis (DIP), bronchiolitis obliterans-organizing
pneumonia (BOOP) and respiratory bronchiolitis I DIAGNOSTIC CHECKLIST
Progression from dense cellular nodules to cavitary
Consider
nodules to increasing degrees of fibrosis that may
extend along alveolar walls LCH in adult smokers with interstitial lung disease
a Fibrotic scars surrounded by enlarged distorted Image Interpretation Pearls
airspaces Characteristic HRCT appearance may obviate need for
biopsy
ICLINICAllSSUES
Presentation I SELECTED REFERENCES
Most common signs/symptoms 1. Abbott GF et al: From the archives of the AFlP:pulmonary
a Non-productive cough, dyspnea, fatigue, chest pain, Langerhans cell histiocytosis. Radiographies. 24(3):821-41,
fever, weight loss, or asymptomatic (25%) 2004
2. Brauner MW et al: Pulmonary Langerhans cell
o Heavy smokers histiocytosis: Evolution of lesions on CT scans. Radiology.
o Pneumothorax in 25% during course of disease, 204:497-502,1997
unilateral, bilateral, and may be recurrent 3. Moore AD et al: Pulmonary histiocytosis X: Comparison of
Variants radiographic and CT findings. Radiology. 172:249-54, 1989
 LANGERHANS CELL GRANULOMATOSIS, PULMONARY

I IMAGE GALLERY

Typical
(Left) Frontal radiograph in a
patient with LCH shows
bony destructive lesion left
fifth rib (arrow). Note the
vague bilateral upper lobe
reticulonodufar opacities.
(Right) Axial HRCT in same
patient shows destruction
and soft tissue replacement
of the posterolateral left fifth
rib. The transition
shows a characteristic
zone
3
beveled edge (arrow).
79

(Left) Axial HRCT in same

patient shows sma/J cavitary
nodules (arrows) and cysts
(curved arrows) that were
imperceptible on the frontal
radiograph. (Right) Coronal
NECT shows bilateral
irregular nodules (arrows)
and cysts (curved arrows).
Note sparing of disease at
the lung bases.

Typical
(Left) Axial NECT shows
bilateral upper lobe stellate
nodules (arrows).
Hyperfucent peripheral lung
(open arrows), i.e., thin
walled cystic change,
resembles bullous
emphysema. End stage LCH.
(Right) Axial HRCT shows
diffuse thin walled cystic
change. Some cysts are> 7
em in diameter are partially
septated and confluent
(curved arrows). Diagnosis:
End stage LCH.
 RESPIRATORY BRONCHIOLITIS

3
80

Axial CECT in patient with RB-ILD shows very subtle Axial HRCT in patient with RB-/LD shows
nodular ground-glass-like opacities (white arrows) that bronchocentric ground-glass opacities (arrows) in both
contrast with attenuation of normal lung areas (black upper lobes. No other abnormalities are seen.
arrows).

Location: Gradient: More predominant in upper lung

I TERMI NOLOGY zone diminishing to the lung bases
Abbreviations and Synonyms Size: Centrilobular nodules 3-5 mm in diameter
Respiratory bronchiolitis (RB) Radiographic Findings
Respiratory bronchiolitis-interstitial lung disease Radiography
(RB-ILD) o Respiratory bronchiolitis: Chest radiograph normal
Definitions o Respiratory bronchiolitis-interstitial lung disease
RB histologic reaction to dusty environments, Chest radiograph normal in up to 50% of patients
especially cigarette smoke Normal lung volumes
RB-lLD and desquamative interstitial pneumonia (DIP) Poorly defined hazy areas of increased density
are regarded as a spectrum of smoking induced Bronchial wall thickening
interstitial lung diseases Fine reticular or reticulonodular pattern (rare)
o DIP more extensive form of pigmented alveolar Small peripheral ring shadows
macrophage accumulation in bronchioles and CT Findings
alveoli
HRCT
o Respiratory bronchiolitis
HRCT often normal (sensitivity 25%)
I IMAGING FINDINGS Micronodular faint centrilobular opacities
General Features Patchy ground-glass opacities (abnormal lobules
adjacent to normal lobules)
Best diagnostic clue: Centrilobular ground-glass
Ground-glass opacities tend to be more
opacities in the upper lobes
widespread
Predominant upper lung zones

DDx: RB-ILD

Desquamative Interstitial Pneumonia Hypersensitivity Pneumonitis Pneumonia

 RESPIRATORY BRONCHIOLITIS
Key Facts
Terminology
Respiratory bronchiolitis-interstitial lung disease
(RB-ILD)
RB histologic reaction to dusty environments,
especially cigarette smoke
RB-ILD and desquamative interstitial pneumonia
(DIP) are regarded as a spectrum of smoking induced
Interstitial lung diseases
Imaging Findings
Best diagnostic clue: Centrilobular ground-glass
opacities in the upper lobes
Location: Gradient: More predominant in upper lung
zone diminishing to the lung bases
Size: Centrilobular nodules 3-5 mm in diameter
Respiratory bronchiolitis: Chest radiograph normal
May have associated centrilobular emphysema if
older
a Respiratory bronchiolitis-interstitial lung disease
Upper lobe centrilobular nodules more
pronounced
Distinct reticular pattern (from mild fibrosis)
primarily in lower lobes
Centrilobular emphysema more common
Mild bronchial wall thickening
a May be combined with other sequelae of smoking
(bronchogenic carcinoma)
Imaging Recommendations
Best imaging tool
a HRCT most sensitive examination
Expiratory HRCT sections can display air trapping
Subtle bronchocentric and nodular ground-glass
opacities can be difficult to detect
I DIFFERENTIAL DIAGNOSIS
Desquamative Interstitial Pneumonia (DIP)
Ground-glass opacities are more diffuse
Opacities usually more subpleural or patchy
Not as bronchocentric as RB-ILD
Intervening alveolar spaces between bronchioles are
diffusely filled with macrophages
Subtle signs of fibrosis seen in advanced cases
Part of the spectrum of smoking related disease
Hypersensitivity Pneumonitis
Similar radiographic and CT findings
More widespread than bronchocentric
Opacities less subtle than in RB-lLD
Chronic hypersensitivity pneumonitis will have more
fibrosis
Contact with inhalational allergen important history
Incidence of hypersensitivity pneumonitis decreased
in smokers
Top Differential Diagnoses
Desquamative Interstitial Pneumonia (DIP)
Hypersensitivity Pneumonitis
Langerhans Cell Granulomatosis
Pathology
Respiratory bronchioles are filled with pigmented
macrophages
Clinical Issues
RB: Universal histologic response in smokers
Virtually all patients with RB-ILD are heavy smokers,
typically unfiltered cigarettes 3
Respiratory bronchiolitis may be precursor to
centrilobular emphysema 81
Smoking cessation
Langerhans Cell Granulomatosis
Probably due to allergy to some constituent of
cigarette smoke
Often "bronchocentric" as RB-lLD
Micronodules more dense and more profuse
Similar predominance upper lung zones
Micronodules may cavitate and show cystic
transformation
May lead to complete lung destruction
Typical Langerhans cell granuloma in biopsy specimen
Acute Pneumonia
Ground-glass opacities are either more diffuse or
localized
Opacities may occur in all parts of the lung
More widespread than bronchocentric
Can be accompanied by pleural effusion
Typical clinical presentation with fever and cough
I PATHOLOGY
General Features
General path comments
a Insult to the respiratory bronchioles ("small
airways") by
Contents of cigarette smoke
Other inhaled fumes
a Reactive accumulation of macrophages within
lumen of respiratory bronchioles (2nd order) and
surrounding alveoli occurs
Etiology
a Airflow dynamics for small particulate material
Air flows rapidly down conducting airways
(trachea to terminal bronchioles)
Then velocity decreases rapidly to allow gas
exchange
Small particles included in smoke 5 microns)
escape impacting into larger airways
Particles are then deposited in the respiratory
bronchioles
Epidemiology
 RESPIRATORY BRONCHIOLITIS
o RB histologic reaction to inhaled dusts, especially
cigarette smoke
o With functional alterations causing disease, then
known as RB-ILD
Gross Pathologic & Surgical Features
RB commonly encountered as incidental finding in
lung specimens removed from cigarette smokers
Often associated with centrilobular emphysema in
smoking
Mild bronchial wall thickening
Microscopic Features
Respiratory bronchioles are filled with pigmented
macrophages
o Macrophages may spill into surrounding alveoli
82 Macrophages typically have a brown cytoplasm with
some black particles
Wall of bronchioles may show mild chronic
inflammation
Advanced cases
o Remodeling of bronchial wall
o Interstitial fibrosis that extends along surrounding
alveolar walls
Epithelial lining of these airspaces range from cuboidal
to bronchiolar-type, pseudostratified and ciliated
respiratory epithelium
Bronchiolar epithelial cells may show goblet cell
metaplasia
Cuboidal cell hyperplasia can be seen along alveolar
ducts and alveoli neighboring bronchioles
If alveolar macrophage absent, alveolar parenchyma
between bronchioles is relatively normal and without
interstitial fibrosis
IClINICAllSSUES
Presentation
Most common signs/symptoms
oRB: Normal
o RB-ILD: Cough, dyspnea
Fine bibasilar end-expiratory crepitations
Other signs/symptoms
o Clubbing (rare)
o Pulmonary function tests may be normal (especially
in respiratory bronchiolitis)
When abnormal: Mixed restrictive-obstructive
pattern
When abnormal: Isolated increase of residual
volume
Slightly reduced diffusing capacity common
o Mild hypoxia may be present at rest or with exercise
Demographics
Age: Mean age at onset: 36 years (range, 22-53 years)
Gender: No gender predilection
Ethnicity: No ethnic predilection
Natural History & Prognosis
RB: Universal histologic response in smokers
Virtually all patients with RB-ILD are heavy smokers,
typically unfiltered cigarettes
Respiratory bronchiolitis may be precursor to
centrilobular emphysema
o Respiratory bronchiolitis occurs early after smoking
o Same location in the 2nd order respiratory
bronchioles
o Evolution of centrilobular nodules (presumed RB) to
centrilobular emphysema has been demonstrated in
longitudinal studies performed at 5-10 year intervals
Treatment
Smoking cessation
o Histologic abnormalities reversible with cessation of
smoking
Removal from dusty environment
Steroids helpful in a small number of patients
I DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
HRCT
o Nodular opacities of ground-glass attenuation
o Bronchocentric distribution
o Upper lobe predominance
o Smoking history mandatory, usually heavy
unfiltered cigarettes for RB-ILD
o Usually no signs of fibrosis
I SElECTED REFERENCES
1. Lynch DA et al: Idiopathic interstitial pneumonias: CT
features. Radiology. 236(1):10-21, 2005
2. Davies G et al: Respiratory bronchiolitis associated with
interstitial lung disease and desquamative interstitial
pneumonia. Clin Chest Med. 25(4):717-26, vi, 2004
3. Wittram C: The idiopathic interstitial pneumonias. Curr
Probl Diagn Radiol. 33(5):189-99, 2004
4. Desai SRet al: Smoking-related interstitial lung diseases:
histopathological and imaging perspectives. Clin Radiol.
58(4):259-68,2003
5. RyuJH et al: Bronchiolar disorders. Am J Respir Crit Care
Med. 168(11):1277-92,2003
6. Wittram C et al: CT-histologic correlation of the AT5/ERS
2002 classification of idiopathic interstitial pneumonias.
Radiographies. 23(5):1057-71, 2003
7. American Thoracic Society; European Respiratory Society:
American Thoracic Society/European Respiratory 50ciety
International Multidisciplinary Consensus Classification of
the Idiopathic Interstitial Pneumonias. This joint
statement of the American Thoracic Society (ATS),and the
European Respiratory Society (ERS)was adopted by the AT5
board of directors, June 2001 and by the ERSExecutive
Committee, June 2001. Am J Respir Crit Care Med.
165(2):277-304,2002
8. Nicholson AG: Classification of idiopathic interstitial
pneumonias: making sense of the alphabet soup.
Histopathology. 41(5):38]-9], 2002
9. SzakacsJG: Pathologist's approach to diffuse lung disease.
Semin Ultrasound CT MR. 23(4):275-87, 2002
]0. Aubry MC et al: The pathology of smoking-related lung
diseases. Clin Chest Med. 2](]):11-35, vii, 2000
] 1. Guckel C et al: Imaging the 'dirty lung'nhas high
resolution computed tomography cleared the smoke? Clin
Radiol. 53(10):7]7-22, ]998
12. Wells AU:Computed tomographic imaging of bronchiolar
disorders. Curr Opin Pulm Med. 4(2):85-92, 1998
 RESPIRATORY BRONCHIOLITIS

I IMAGE GALLERY
Typical
(Left) Axiall-IRCT shows
bronchocentric ground-glass
nodules (arrows) and
bronchial wall thickening
(open arrows) in patient with
R8-ILD. (Right) Axiall-fRCT
shows bronchocentric
ground-glass nodules
(arrows), more widespread
ground-glass opacities
(curved arrows), and 3
bronchial wall thickening
(open arrows) in patient with
R8-ILD. 83

Typical
(Left) Axial CECT shows
patient with R8-ILD and
centrilobular emphysema
(arrows). Centrilobular
lesions are "highlighted" by
attenuation increase caused
by nodular ground-glass
opacities (open arrows).
(Right) Axial HRCT in patient
with R8-ILD shows upper
lobe predominance of
R8-ILD. Upper lobes
(arrows) have increased
attenuation, whereas lower
lobes (open arrows) have
normal allenuation.

Typical
(Left) Axial HRCT shows
patient with R8-ILD.
Peripheral ground-glass
opacities (open arrows) are
combined with centrilobular
emphysema (arrows).
Subpleural regions show very
subtle signs of fibrosis.
(Right) Axial HRCT in the
same patient with R8-ILD
(open arrows) shows
incidentally discovered
spiculated and cavitated
peripheral squamous cell
carcinoma in the right lower
lobe (arrow).
 CENTRILOBULAR EMPHYSEMA

84

Graphic shows bilateral upper lobe bullous Axial HRCT shows subtle CLf lesions (arrows). Lesions
emphysema. Apical blebs may rupture and cause are surrounded by normal lung parenchyma and are
spontaneous pneumothorax. located near the center of the secondary pulmonary
lobule.

Location: Predominantly involves upper lung zones

I TERMI NOlOGY (lung apex, apical segments of lower lobes)
Abbreviations and Synonyms Size
Centrilobular emphysema (CLE) o Mild disease: 1-2 mm centrilobular holes
Centriacinar emphysema (CAE) o Advanced disease: May occupy the entire secondary
pulmonary lobule
Definitions Morphology
Emphysema general: Abnormal permanent o Destruction of lung parenchyma, classically near the
enlargement of any or all parts of the acinus, central arteriole and bronchiole of the secondary
accompanied by destruction of alveolar tissue, but pulmonary lobule
without fibrosis o Well-defined margins between normal and
CLE: Enlargement and destruction of respiratory emphysematous lungs create inhomogeneous
bronchioles, classically located near (but not exactly appearance of CLE
at) the center of the secondary pulmonary lobule
Radiographic Findings
Radiography
I IMAGING FINDINGS o Mild disease: Very insensitive, radiography may be
completely normal
General Features Problem is recognition of loss of normal lung
Best diagnostic clue Normal lung in chest radiographs is 90% air,
o Well-defined holes in the centrilobular portion of making detection of slight increases in air nearly
the secondary pulmonary lobule on HRCT impossible to detect
o Anatomical borders of the secondary pulmonary Consequence: Weak correlations between
lobule are preserved functional indices and radiographic findings

DDx: Centriacinar Emphysema

Langerhans Granulomatosis Tracheobronchial Papillomatosis Metastasis

 CENTRILOBULAR EMPHYSEMA

Key Facts
Terminology Top Differential Diagnoses
CLE: Enlargement and destruction of respiratory Technical Considerations
bronchioles, classically located near (but not exactly Athletic Hyperinflation
at) the center of the secondary pulmonary lobule Panlobular Emphysema
Langerhans Cell Histiocytosis
Imaging Findings
Asthma
Well-defined holes in the centrilobular portion of the
secondary pulmonary lobule on HRCT Pathology
Anatomical borders of the secondary pulmonary CLE strongly associated with cigarette smoking
lobule are preserved Approximately 30% of the normal lung must be
Location: Predominantly involves upper lung zones destroyed before pulmonary function deteriorates
(lung apex, apical segments of lower lobes)
Well-defined margins between normal and Diagnostic Checklist 3
emphysematous lungs create inhomogeneous CLE very common "incidental" finding in cigarette
appearance of CLE smokers 85
Can detect clinically and functionally "silent" CLE

o Advanced disease: May be visible on radiograph

Attenuation inhomogeneities Athletic Hyperinflation
Vascular distortion and disruption Lung normal, young athiete
Increased branching angle of remaining vessels Pan lobular Emphysema
Hyperinflation
Pattern of destruction is more homogeneous than CLE
o Flat hemidiaphragms
Uniform distribution, no upper lobe predominance
o Sagittal diameter of thorax increased
Uniform destruction of the secondary pulmonary
o Widened retrosternal space
lobule
o Widened retrocardiac space
o Small and narrow heart langerhans Cell Histiocytosis
Secondary manifestations Also smoking related
o Pulmonary arteriai hypertension: Enlarged centrai Initially nodules that evolve into cysts
pulmonary arteries, peripheral arterial pruning Similar upper lung zone distribution
CT Findings Long standing disease may be identical to
centrilobuiar emphysema
HRCT
o HRCT more sensitive than chest radiography Asthma
o Can detect clinically and functionally "silent" CLE No parenchymal destruction
o Emphysematous lesions usually have no discernible Hyperinflation may be reversible
wall
o Emphysematous lesions are surrounded by normal Bleb
lung parenchyma Collection of air within the layers of the pleura
o Central artery sometimes remains visible surrounded
by destroyed lung
Cyst
o Borders of secondary pulmonary lobule are Closed cavity lined by bronchiolar epithelium or
preserved fibrous tissue
o Objectively measured by assuming that lung with a Bulla
threshold HU < -950 is emphysema Emphysematous space, commonly subpleural, greater
Imaging Recommendations than 1 cm in the distended state
Best imaging tool: HRCT for detection "Cystic" lesions
Protocol advice Tracheobronchial papillomatosis, metastases
o Acquire scans in full suspended inspiration only Other than CLE, all these lesions have visible borders
o Expiratory scans are of little value in CLE
o Pay particular attention to upper lung zones (lung Constrictive Bronchiolitis Obliterans
apex, apical segments of lower lobes) No parenchymal destruction, mosaic attenuation
pattern

I DIFFERENTIAL DIAGNOSIS
Technical Considerations
I PATHOLOGY
Low dose techniques may have false negatives General Features
Wide windows may cause false negatives Genetics
 CENTRILOBULAR EMPHYSEMA
o Potential genetic predisposition to CLE
Such predisposition could explain varying extent
of CLE in individuals with comparable smoking
habits
Etiology
o CLE strongly associated with cigarette smoking
o Smoking-associated CLE time and dose related
o CLE also occurs after inhalation of industrial dusts
(silica)
Epidemiology
o Very common disease in the industrial world
o Geographic variations according to regional
smoking habits
Associated abnormalities
3 o Respiratory bronchiolitis
o Chronic bronchitis
86 o Hyperinflation
o Secondary pulmonary hypertension
Pathologic functional correlation
o Patients may have anatomic emphysema without
alteration of pulmonary function
o Approximately 30% of the normal lung must be
destroyed before pulmonary function deteriorates
o Pulmonary function usually determined by
structural integrity of lower lung zones
o Pulmonary function tests are global summation of
airways and lung, but HRCT provides regional
information
Gross Pathologic & Surgical Features
Centrilobular location secondary pulmonary lobule
o Dilatation 2nd order respiratory bronchioles in
secondary lobule
o Primarily involves upper lung zones
o Precursor may be respiratory bronchiolitis
Microscopic Features
Enlargement and destruction of alveolar walls
Emphysematous spaces become confluent in series
and in parallel within the acinus
Staging, Grading or Classification Criteria
Because CLE defined in strictly morphological terms,
correlation with pathology and microscopy should
always be sought
HRCT allows for objective quantification of
emphysema
ICLINICAL ISSUES
Presentation
Most common signs/symptoms
o Mild disease
Often asymptomatic
May be incidental finding on HRCT
o Advanced disease
Dyspnea, shortness of breath
Increased total and residual lung volumes
Residual volume> 120% predicted
Forced expiratory volume in one second < 80%
predicted
Diffusion capacity decreased < 80% predicted
CLE most common form of emphysema associated
with symptomatic or fatal chronic airway
obstruction
Other signs/symptoms: Pulmonary hypertension
Demographics
Age: Incidence peak between 4S and 7S years
Gender: Slight male predominance, due to smoking
habits
Natural History & Prognosis
With smoking cessation: Stabilization or slow
progression
Without smoking cessation: Accelerated progression to
clinically symptomatic form that requires treatment
Treatment
Smoking cessation
o Pulmonary function will continue to decline
Bronchodilators
Lung volume reduction surgery
o Undergoing randomized trials
o Candidates primarily those with inhomogeneous
emphysema (usually upper lobe predominant)
Lung transplantation. unfortunately too few organs
for those in need
I DIAGNOSTIC CHECKLIST
Consider
CLE very common "incidental" finding in cigarette
smokers
Image Interpretation Pearls
Well-defined areas of destruction surrounded by
normal lung
Predominates in upper lung zones
Best seen on HRCT
I SELECTED REFERENCES
1. Watz H et al: Micro-CT of the human lung: imaging of
alveoli and virtual endoscopy of an alveolar duct in a
normal lung and in a lung with centrilobular
emphysema--initial observations. Radiology.
236(3):1053-8,2005
2. Bankier AAet al: CT quantification of pulmonary
emphysema: assessment of lung structure and function.
Crit RevComput Tomogr. 43(6):399-417, 2002
3. 5ugi Ket al: The outcome of volume reduction surgery
according to the underlying type of emphysema. 5urg
Today. 31(7):580-5, 2001
4. Bankier AAet al: Pulmonary emphysema: subjective visual
grading versus objective quantification with macroscopic
morphometry and thin-section CT densitometry.
Radiology. 211(3):851-8,1999
5. 510ne RM et al: Preoperative and postoperative imaging In
the surgical management of pulmonary emphysema.
Radiol Clin North Am. 36(1):57-89,1998
6. Webb WR: Radiology of obstructive pulmonary disease.
A]RAm] Roentgenol. 169(3):637-47, 1997
7. 5tern E]et al: CT of the lungs in patients with pulmonary
emphysema. Semin Ultrasound CT MR. 16(5):345-52, 1995
 CENTRILOBULAR EMPHYSEMA
I IMAGE GALLERY

(Left) Axial HRCT shows

slightly bigger CLE lesions
(arrows). Lesions
nevertheless remain confined
to the center of the
secondary pulmonary lobule.
(Right) Axial HRCT shows
CLE lesions of larger extent
(open arrows). In some
lesions, CLE occupies the
entire secondary pulmonary 3
lobule (arrows).

87

(Left) Axial HRCT shows

patient with CLE and
parenchymal bleeding.
Bleeding manifests as diffuse
ground-glass opacities and
consolidations (arrows) that
delimit diffuse CLE lesions.
(Right) Coronal CECT shows
apical CLE (white arrows)
and basal fibrosis (black
arrows). Macroscopical
coexistence of these entities
does not conflict with
histological definition of
emphysema.

Typical
(Left) Axial HRCT shows
widespread asymptomatic
emphysema (arrows) in a
patient with breast cancer
(breast implant! and pleural
metastases (open arrows).
Because smoking is
common, incidental
emphysema is common.
(Right) Axial HRCT shows
subpleural blebs anteriorfy
(arrows) and centriacinar
emphysema (open arrows)
some of which has become
confluent (curved arrow).
 AMYLOIDOSIS, PULMONARY

3
88

Axial graphic shows one of the varied manifestations of Axial NECT shows significant circumlerentialthickening
amyloidosis, multiple small pulmonary nodules which 01 the tracheal wall with irregular inner margins.
may calcify. Peripheral ground-glass opacities are Amyloidosis
nonspecific.

o Pleural
I TERMI NOlOGY 10% of patients with multiple myeloma develop
Definitions amyloidosis
Generic term for a heterogeneous group of disorders Bleeding common due to amyloid deposition in
characterized by abnormal extracellular accumulation vessels
of insoluble fibrillar proteins Tracheobronchial> pulmonary nodular> adenopathy
Types: Primary AL type (amyloid light chain) and > diffuse septal
secondary AA type (amyloid A, ()(globulin) Calcification more common in localized deposits
o Primary amyloidosis (AL type): Excessive deposition
of protein secreted by B lymphocytes or plasma cells
(amyloidosis with monoclonal gammopathy or I IMAGING FINDINGS
multiple myeloma)
General Features
o Secondary or reactive systemic amyloidosis (AA
type): Extracellular protein deposition caused by Best diagnostic clue: Multiple calcified tracheal or
pulmonary nodules
underlying chronic inflammatory diseases
(infections, bronchiectasis, rheumatic diseases, Radiographic Findings
neoplasms, age related (senile, SA type), familial (AF Tracheobronchial form
type) also with Mediterranean fever o Focal or diffuse tracheal thickening
Major clinical forms: Systemic and localized o Nodular deposits more common than diffuse
Thoracic amyloidosis thickening
o Tracheobronchial o Multiple concentric or eccentric strictures
o Nodular o Subglottic location most common
o Diffuse o Foci of calcification (30% of cases)
o Lymph nodes Pulmonary nodular form

DDx: Diffuse Bilateral Nodular Pattern

Sarcoidosis
 AMYLOIDOSIS, PULMONARY

Key Facts
Terminology Diffuse micronodular, reticulonodular, or linear
pattern
Generic term for a heterogeneous group of disorders
characterized by abnormal extracellular accumulation Thickening of the bronchovascular bundles
of insoluble fibrillar proteins Top Differential Diagnoses
Imaging Findings Primary benign and malignant tumors
Tracheopathia osteochondroplastica
Focal or diffuse tracheal thickening
Relapsing polychondritis
Single or multiple nodules
Alveolar microlithiasis
Miliary nodules
Metastatic calcification
Honeycombing
Nodal calcification: Stippled, diffuse, or eggshell Silicosis
Cardiac enlargement due to amyloid deposition Sarcoidosis
Irregular pleural thickening with associated Mesothelioma
calcification Clinical Issues 89
Thickening of the airway wall Prognosis poor for diffuse disease (survival < 2 years)
Submucosal foci of calcification

o Single or multiple nodules Diffuse pulmonary disease usually associated with

o Size: 0.5 to 5 em in diameter adenopathy
o 20% calcify, growth very slow
o Sharply-marginated, peripheral, round or lobulated
Imaging Recommendations
o Cavitation extremely rare Best imaging tool
o Chest radiograph usually sufficient to document
Diffuse (septal) form
o Miliary nodules extent of thoracic involvement
o Bilateral basal involvement o HRCT: More sensitive in detecting tracheobronchial
o Diffuse fine linear or reticulonodular interstitial involvement, lymphadenopathy, presence of
pattern calcification, and subtle parenchymal abnormalities
o Honeycombing
Adenopathy
o Isolated finding or associated with interstitial I DIFFERENTIAL DIAGNOSIS
involvement Tracheobronchial Amyloid
o Usually multiple lymph node groups involved
Primary benign and malignant tumors
o May be massive
o Usually focal, not diffuse
o Nodal calcification: Stippled, diffuse, or eggshell
Tracheopathia osteochondroplastica
Other o Nodules located only along anterior and lateral walls
o Cardiac enlargement due to amyloid deposition
o Amyloid circumferential
o Pleural effusions rare, usually associated with cardiac
Relapsing polychondritis
disease
o No nodules
o Irregular pleural thickening with associated
o Clinical findings in sclerae or ears
calcification
Rhinoscleroma
CT Findings o Paranasal sinus disease
NECT o Culture for Klebsiella
o Tracheobronchial abnormalities Nodular
Thickening of the airway wall
Differential for solitary pulmonary nodule (SPN) and
Intraluminal nodules multiple pulmonary nodules including primary
Submucosal foci of calcification
carcinoma, metastases, granulomatous disease, benign
o Parenchymal abnormalities
metastasizing leiomyomas, rheumatoid nodules
Diffuse micronodular, reticulonodular, or linear
pattern Diffuse Septal
Calcification seen in 20-50% of nodules Differential for interstitial lung disease including
Nodules may cavitate (rare) idiopathic pulmonary fibrosis (IPF), scleroderma,
Ground-glass opacities and honeycombing rheumatoid arthritis, bronchiolitis obliterans
Thickening of the bronchovascular bundles organizing pneumonia, drug toxicity
o Adenopathy
Stippled, diffuse, or eggshell calcifications Diffuse or Multifocal Lung Calcification
o Other Granulomatous infections (tuberculosis,
Soft tissue deposition histoplasmosis, healed varicella)
More sensitive than chest radiograph, more sensitive Alveolar microlithiasis
for calcification Metastatic calcification
 AMYLOIDOSIS, PULMONARY
Silicosis a Tracheal compression and superior vena caval
Sarcoidosis obstruction (rare)
Dendritic calcification in lung fibrosis Primary or myeloma associated (AL type protein)
a Most patients with amyloid have monoclonal spike
Adenopathy o Conversely < 25% with monoclonal gammopathy
Lymphoma: Does not calcify prior to treatment develop amyloidosis
Sarcoid o 10% of patients with multiple myeloma develop
a Symmetrical enlargement amyloidosis
a Often associated peribronchial interstitial lung o Other organs involved: Heart, kidney, tongue, GI
disease tract, skin, muscle
Tuberculosis: Nodes often have rim enhancement Secondary (AA type)
Metastases: Not likely to calcify (unless from bone or a Inflammation: RA, bronchiectasis, CF, osteomyelitis,
chondroid tumor) Crohn
Pleural Thickening a Malignancies: Renal cell, medullary thyroid
Mesothelioma carcinoma, Hodgkin
o Familial (AF type) also with Mediterranean fever
90 Metastases
o Senile (AS type)
Generally asymptomatic, common (90% over 90
I PATHOLOGY years of age)
Associated with cardiac deposition
General Features
Natural History & Prognosis
General path comments
Prognosis poor for diffuse disease (survival < 2 years)
a Extracellular protein deposition
a Vascular deposition leads to fragility and bleeding Treatment
Microscopic Features Resection to alleviate symptoms in tracheobronchial
obstruction
Large sheets of protein deposition o Often recurs
Amyloid material surrounding airways
No known treatment for diffuse forms, supportive
Amyloid deposits also in the media of small blood therapy only
vessels and interstitium
a Deposits: Uniform and linear or as multiple small
nodules
Calcification and foreign body giant cell reaction may
I SElECTED REFERENCES
be present 1. Chung, MJ et al: Metabolic lung disease: imaging and
Apple-green birefringence under polarized light after histopathologic findings. Eur J Radiol. 54:233-245, 2005
2. Jeong, YJ et al: Amyloidosis and Iymphoproliferative
Congo red staining
disease in Sjogren syndrome: thin-section computed
tomography findings and histopathologic comparisons. J
Comput Assist Tomogr. 28:776-81, 2004
IClINICAllSSUES 3. Prince, JS et al: Nonneoplastic lesions of the
tracheobronchial wall: radiologic findings with
Presentation bronchoscopic correlation. Radiographies. 22:S215-230,
Most common signs/symptoms: Different clinical 2002
presentations and nonspecific symptoms 4. Gillmore JD et al: Amyloidosis and the respiratory tract.
Tracheobronchial form Thorax 54:444-451,1999
5. Lee, KSet ai: Diffuse micronodular lung disease: HRCT and
a Male 2:1, average age 50
pathologic findings. J Com put Assist Tomogr. 23: 99-106,
a Depends on airway involvement: Trachea and/or 1999
proximal bronchi chronic wheezing, dyspnea, 6. Curtin, JJ et al: Thin-section spiral volumetric CT for the
cough, hemoptysis, and recurrent pneumonia assessment of lobar and segmentai bronchiai stenoses. Clin
a Usually asymptomatic Radiol. 53:110-115, 1998
a Chronic wheezing, dyspnea, cough, hemoptysis, and 7. Kirchner, J et al: CT findings in extensive tracheobronchiai
recurrent pneumonia amyloidosis." Eur Radiol. 8(3):352-354,1998
a Simulates asthma 8. Faik RH et ai: The systemic amyloidoses. N Engl J Med.
25:898-909,1997
Nodular form
9. Pickford HA et al: Thoracic cross-sectional imaging of
a No gender prevalence, average age 65 amyloidosis. AJR. 168:351-5, 1997
a Usually asymptomatic 10. Urban BA et al: CT evaluation of amyloidosis: spectrum of
a Occasionally, cough and hemoptysis disease. Radiographies. 13:1295-1308, 1993
a Need to be distinguished from neoplasia 11. Ayuso MC et al. CT appearance of localized pulmonary
Diffuse septal form amyloidosis. J Com put Assist Tomogr. 11:197 -199, 1987
a No gender prevalence, average age 55 12. Hui AN et al: Amyloidosis presenting in the lower
a Usually symptomatic: Dyspnea and respiratory respiratory tract Clinicopathologic, radiologic,
insufficiency immunohistochemical, and histochemicai studies on 48
cases. Arch Pathol Lab Med. 110:212-218, 1986
Adenopathy 13. Gross BH. Radiographic manifestations of lymph node
a Rarely associated with localized form involvement in amyloidosis. Radiology. 138:11-14, 198]
 AMYLOIDOSIS, PULMONARY

I IMAGE GALLERY
Typical
(Left) Axial H RCT shows
extensive bilateral small
nodules in the subpleural
regions and interlobar
fissures (black arrows).
Nodular thickening of the
interlobular septa is visible
(white arrow). (Right) Axial
HRCT shows numerous
subpleural nodules and
septal thickening (arrows).
Irregular thickening of major
3
interlobar fissures is a/so
clearly demonstrated 91
(curved arrow).

(Left) Axial HRCT shows

marked circumferential
thickening of the trachea
(arrow) associated with
increased attenuation due to
submucosal deposition of
calcium. (Right) Axial CECT
shows irregular narrowing
and thickening of main
bronchi. (Courtesy K. Lee,
MO).

Typical
(Left) Axial NECT shows
bilateral pleural thickening
and a huge well-marginated
pleural-based mass in the
upper right hemithorax.
Calcifications are seen in
both, left thickened pleura
and mass. (Right) Axial
NECT shows marked right
pleural thickening Note the
presence of a calcified lymph
node in the 5ubcarinal region
(arrow). (Courtesy /.
Caceres, MO).
 TRACHEOPATHIA OSTEOCHONDROPLASTICA

92

Coronal NECT shows diffuse calcified nodularity of the Axial NECT shows diffuse calcified nodularity of the
lower trachea and main bronchi from tracheopathia main bronchi. Left upper lobe bronchus is narrowed by
osteochondroplastica. Bronchus intermedius stented larger calcified nodules (arrow). (Courtesy C. Fuhrman,
(curved arrow). (Courtesy C. Fuhrman, MO). MO).

o Nodularity or undulating thickening of trachea and

I TERMI NOlOGY bronchi
Abbreviations and Synonyms o Large nodules may cause recurrent pneumonias or
Tracheobronchopathia osteochondroplastica, atelectasis
tracheo-osteoma, tracheitis chronica ossificans CT Findings
Definitions NECT
Rare benign proliferation of bone and cartilage in the o Calcified nodular thickening of anterior and lateral
tracheal wall walls of trachea
Spares posterior wall which does not have
cartilage
I IMAGING FINDINGS o Involves lower two-thirds of trachea and may extend
into main segmental and lobar bronchi
General Features
Imaging Recommendations
Best diagnostic clue: Small nodules arising from
Best imaging tool: CT examination of choice to
anterolateral tracheal cartilaginous rings
evaluate tracheal abnormalities
Size: 2-3 mm in diameter
Radiographic Findings
Radiography I DIFFERENTIAL DIAGNOSIS
o Normal chest radiograph (tracheal abnormalities
often overlooked)
Amyloid
o Calcification of nodules usually not evident on Calcified nodules also involve posterior tracheal
radiograph membrane

DDx: Tracheal Wall Thickening

..-.....
.~
.-

Laryngotracheal Papillomatosis Relapsing Polychondritis

.,,~--
.~

.. -.-
 TRACHEOPATHIA OSTEOCHONDROPLASTICA

Key Facts
Terminology Top Differential Diagnoses
Rare benign proliferation of bone and cartilage in the Amyloid
tracheal wall Laryngeal Papillomatosis
Wegener Granulomatosis
Imaging Findings Endobronchial Sarcoid
Best diagnostic clue: Small nodules arising from Relapsing Polychondritis
anterolateral tracheal cartilaginous rings
Spares posterior wall which does not have cartilage Pathology
Epidemiology: 0.5% prevalence at autopsy

laryngeal Papillomatosis Microscopic Features

3
Nodules don't calcify Nodules or spicules of cartilage and bone in the 93
May have multiple cystic lesions in lung submucosa of the trachea and bronchi
Wegener Granulomatosis
Noncalcified, diffuse nodular or smooth thickening of IClINICALISSUES
tracheal wall
Often associated with multiple thick-walled Presentation
pulmonary cavities Most common signs/symptoms
a Most asymptomatic
Endobronchial Sarcoid a Occasionally dyspnea, hoarseness, cough,
Nodules rarely calcify expectoration, wheezing, hemoptysis, recurrent
Interstitial lung disease and hilar adenopathy common pneumonias
Relapsing Polychondritis a Sometimes discovered at time of intubation
Noncalcified diffuse narrowing of the trachea and Demographics
main bronchi Age: Usually> 50 years of age
Same distribution along the anterior and lateral wall of Gender: M > F
the trachea
Natural History & Prognosis
Progresses very slowly, usually incidental discovery at
I PATHOLOGY autopsy
General Features Treatment
Epidemiology: 0.5% prevalence at autopsy Endoscopic therapy or surgery for obstructing lesions
Gross Pathologic & Surgical Features
Beaded polypoid appearance of trachea and bronchi I SElECTED REFERENCES
with intact mucosa
1. Restrepo S et al: Tracheobronchopathia
osteochondroplastica: helical CT findings in 4 cases. J
Thorac Imaging. 19(2):112-6,2004

IIMAGE GALLERY
 CARCINOID, PULMONARY

3
94

Frontal radiograph demonstrates a 5 cm well-defined Lateral radiograph confirms the perihilar location. No
perihilar mass in this 51 year old man with a chronic calcification, enlarged lymph nodes or cavitation. A
cough. No post obstructive lung changes are present. typical carcinoid neoplasm with negative lymph nodes
was found at surgery.

o Typical carcinoid: 85% develop in main, lobar or

I TERMI NOlOGY
segmental bronchi
Abbreviations and Synonyms 15% are peripheral in location (subsegmental and
"Bronchial adenoma" has been used in the past beyond)
o All carcinoid tumors are malignant and therefore, Tracheal location is very rare
the term "adenoma" should be avoided o Atypical carcinoid: Most develop in lung periphery
Enlarged hilar lymph nodes more commonly seen
Definitions Size: Usually between 1-5 em; atypical carcinoids tend
Uncommon pulmonary neuroendocrine neoplasm to be larger
Low grade malignancy with metastatic potential
o Arise from neuroendocrine cells normally scattered
Radiographic Findings
throughout tracheobronchial epithelium Well-defined nodule/mass with secondary changes of
o Lung is second most common location with the post obstructive atelectasis or pneumonia
gastrointestinal (GI) tract accounting for about 90% o Hyperinflation of the lung/lobe is occasionally
of all carcinoids present with bronchial obstruction (ball-valve
mechanism)
o 4% have ossification seen on the radiograph
I IMAGING FINDINGS Lymph node enlargement not usually seen

General Features CT Findings

Best diagnostic clue: Well-defined hilar/perihilar mass NECT
o 30% of central carcinoids have a variable amount of
+/- associated post obstructive atelectasis, pneumonia
or mucus plugging calcification
This is less common with peripheral tumors
Location
CECT

DDx: Carcinoid Differential

Adenoid Cystic Hamartoma

 CARCINOID,
Terminology
Uncommon pulmonary neuroendocrine neoplasm
Low grade malignancy with metastatic potential
Imaging Findings
Typical carcinoid: 85% develop in main, lobar or
segmental bronchi
Atypical carcinoid: Most develop in lung periphery
Typical carcinoid: Smooth borders without necrosis
or cavitation
Avid contrast enhancement a common feature
Pathology
Two distinct pathologic types
Low-grade "typical" carcinoid (80-90%)
Intermediate-grade "atypical" carcinoid (10-20%)
o Typical carcinoid: Smooth borders without necrosis
or cavitation
Larger size, evidence of necrosis and hilar lymph
node enlargement favors atypical carcinoid
Not all lymph node enlargement represents
malignancy since reactive nodes occur as a
response to pneumonias
Avid contrast enhancement a common feature
o Atypical carcinoid
Enhancement more variable
MR Findings
T2WI
o High signal intensity is common
o MRI does not offer much diagnostic advantages
CT
Nuclear Medicine Findings
Octreotide scan (somatostatin analogue) has been used
successfully to diagnosis and locate carcinoid tumors
o Utilizes tumor's somatostatin-binding sites
Imaging Recommendations
Best imaging tool: Contrast-enhanced CT scan with
thin collimation (5 mm or less)
Protocol advice: IV contrast important since carcinoid
tumors are quite vascular and typically, but not
universally, demonstrate avid enhancement
I DIFFERENTIAL DIAGNOSIS
Adenoid Cystic Carcinoma
Salivary gland malignancy, which often arises in
trachea or main bronchi
o Only 10% develop in lung periphery
A more locally aggressive tumor, careful evaluation
extra luminal or mediastinal extension required
Pulmonary Hamartoma
May have fat detectable on CT exams, characteristic of
tumor
Calcification common and enhancement is less than
carcinoid tumors
PULMONARY
Key Facts
Carcinoid tumors likely have a histologic and clinical
spectrum with the more aggressive large cell
neuroendocrine and small cell carcinomas
Clinical Issues
Cough the most common symptom with hemoptysis
present at some point in 50%
Recurrent pneumonia, post obstructive
atelectasis/pneumonitis also very common, especially
with central tumors (30% presentation)
Typical carcinoid: 5% have lymph node metastasis at
presentation
Atypical carcinoid: 50-60% have lymph node 3
metastasis at presentation
Complete surgical excision remains most effective 95
therapy
Intrabronchiallocation seen in 4%
Mucoepidermoid Carcinoma
A rare salivary gland tumor, which arises in lobar or
segmental bronchi
o Tracheal location very uncommon
Commonly presents as a solitary central mass with
post obstructive atelectasis or pneumonitis
Oval, polypoid or lobulated with well-defined margins
o Calcifications present in 50%
Lung Carcinoma
Adenocarcinomas are often peripheral and may have
subtle "bubble-like" air bronchograms
over Squamous cell carcinomas tend to cavitate
Small cell carcinomas usually have extensive lymph
node enlargement
Margins usually ill-defined, lobulated, or spiculated
Patients tend to be older
History of smoking (smoking dose and time
dependent)
Foreign Body Granuloma or Broncholith
Small intrabronchial nodule with dense calcification
I PATHOLOGY
General Features
Etiology
o Not well understood
Genetic mutations and asbestosis exposure were
theories, but little supporting clinical evidence
Atypical carcinoid has some association with
smoking
Typical carcinoid not associated with smoking
for Epidemiology: Represents 2% of all lung tumors
Associated abnormalities
o Metastatic disease usually spreads to liver, bone,
brain and adrenals
Osseous metastasis often sclerotic rather than lytic
o Small pulmonary tumorlets are occasionally
associated with carcinoid tumors
 CARCINOID, PULMONARY
Tumorlets represent benign neuroendocrine
hyperplastic growth, which often develop in areas
of bronchiectasis or prior surgery
Gross Pathologic & Surgical Features
Well-defined spherical tumor with margins composed
of a thin layer of compressed connective tissue
Most arise within the central bronchi
o Carcinoid can demonstrate predominately
extraluminal growth into the surrounding lung with
only a small portion attached to the bronchus
("iceberg" growth)
Microscopic Features
3 Two distinct pathologic types
o Low-grade "typical" carcinoid (80-90%)
96 o Intermediate-grade "atypical" carcinoid (10-20%)
Carcinoid tumors likely have a histologic and clinical
spectrum with the more aggressive large cell
neuroendocrine and small cell carcinomas
Typical carcinoid: Uniform sheets of variable cells
separated by a thin fibrovascular stroma
o Cytoplasm is moderate and has numerous
neurosecretory granules
o Mitotic figures are rare and necrosis is absent
Atypical carcinoid: Typical features with at least one of
the following
o Presence of necrosis
o Loss of typical architecture with increased cellularity
o Mitotic figures> 5-10 per 10 high-power fields
o Increased nuclear/cytoplasmic ratio or nuclear
pleomorphism
Histology of both demonstrates significant variability
of cells between different tumors and within the same
tumor
Ossification/calcification a common feature
These neuroendocrine cells are referred as Kulchitsky
cells
o Grade 1 for typical carcinoid tumors
o Grade 2 for atypical carcinoid tumors
o Grade 3 for small cell carcinoma; felt to be part of a
histological spectrum
o Large cell neuroendocrine malignancy has recently
replaced large cell carcinoma nomenclature
It is an intermediate-high grade malignancy
between atypical carcinoid and small cell
carcinoma
ICLINICAL ISSUES
Presentation
Most common signs/symptoms
o Cough the most common symptom with
hemoptysis present at some point in 50%
Central carcinoid tumors present clinically earlier
than peripheral tumors
o Recurrent pneumonia, post obstructive
atelectasis/pneumonitis also very common,
especially with central tumors (30% presentation)
o Cushing syndrome [hypercortisolism from ectopic
corticotropin (ACTH) secretion] rare, but
well-described paraneoplastic presentation
Bronchial carcinoids represent most common
source of ectopic ACTH
Can occur with small tumors and without
metastasis
o Acromegaly may occur by tumor-related growth
factor release, which acts on the pituitary gland
o Carcinoid syndrome very uncommon with
pulmonary carcinoid
2-5% of patients and almost all have hepatic
metastasis
Other signs/symptoms: Non-responsive "asthma" or
wheezing isolated to one lung are well-described
presentations (ball-valve mechanism)
Demographics
Age
o Typical carcinoid usually presents between 30-60
years of age
Atypical carcinoid generally presents a decade
later
Gender: Females slightly more than males
Natural History & Prognosis
Typical carcinoid: 5% have lymph node metastasis at
presentation
o 5 year survival at 90-95% without lymph node
involvement and 76-88% with positive nodes
Atypical carcinoid: 50-60% have lymph node
metastasis at presentation
o 5 year survival between 40-70%, depending on stage
at presentation
Tumor size, histological subtype and lymph node
spread are the best predictors of recurrence/survival
Imaging follow-up after excision should be prolonged
since carcinoids tend to be slow growing
Treatment
Complete surgical excision remains most effective
therapy
Radiation therapy for local control
Chemotherapy has had variable results
I SELECTED REFERENCES
1. Divisi D et al: Carcinoid tumors of the lung and
multimodal therapy. Thorac Cardiovasc Surg. 53(3):168-72,
2005
2. Renshaw AAet al: Distinguishing carcinoid tumor from
small cell carcinoma of the lung: correlating cytologic
features and performance in the College of American
Pathologists Non-Gynecoiogic Cytology Program. Arch
Pathol Lab Med. 129(5):614-8,2005
3. Mi-YoungJeung et a!. Bronchial Carcinoid Tumors of the
Thorax: Spectrum of Radiologic Findings. Radiographies.
22: 351-365, 2002
4. Faser and Pare Diagnosis of Diseases of the Chest. 4th ed.
Volume 1I,pages 1229-1243, 1999
5. Rosado de Christenson et a!. Thoracic Carcinoids:
Radiologic-Pathologic Correlation. Radiographies.
19:707-736,1999
 CARCINOID, PULMONARY

I IMAGE GAllERY
Typical
(Left) Axial CECT shows
complete middle lobe
collapse. An enhancing 7.5
em nodule ;5 present within
the proximal bronchus
(arrow). Patient presented
with recurrent pneumonias
and mild hemoptysis. (Right!
Axial CECT demonstrates the
convex smooth margin of the
endobronchial enhancing
nodule (arrow). At surgery,
3
this was a typical carcinoid
tumor. Lymph nodes were 97
negative.

Variant
(Left) Frontal radiograph in a
39 year old women with a 4
cm left lower lobe
well-marginated mass (black
arrow) and mildly enlarged
left hilum (white arrow).
(Right) Lateral radiograph
shows the left hilar lymph
node enlargement (arrow).
The left lower lobe mass
(open arrow) was an
atypical carcinoid with
positive lymph nodes at
surgery.

(Left) Axial NEeT in a 30

year old male with chronic
respiratory symptoms
demonstrates lower lobe
collapse from a centrally
calcified endobronchial
nodule (arrow). Lobectomy
revealed a typical carcinoid.
(Right! Axial NECT
demonstrates the left distal
main bronchial nodule,
which caused air-trapping on
the expiration images. A
typical carcinoid tumor was
found at surgery.
 KAPOSI SARCOMA, PULMONARY

98

Axial graphic shows typical features of Kaposi sarcoma. Coronal CECT shows extensive thickening along the
Tumor infiltrates along bronchovascular bundles, bronchovascular bundles, consistent with KS.
extending from the hilum to the lung periphery. Tumor
clusters may be noncontiguous.

Lymphadenopathy (50%); marked enhancement

ITERMINOlOGY following intravenous contrast
Abbreviations and Synonyms Imaging Recommendations
Kaposi sarcoma (KS)
Best imaging tool
Definitions o CT findings usually highly suggestive
AIDS-related multicentric neoplasm with propensity to o Gallium-thallium imaging complementary tool for
involve skin, lymph nodes, GI tract, and lungs indeterminate cases
KS gallium-negative but thallium-positive

IIMAGING FINDINGS
I DIFFERENTIAL DIAGNOSIS
General Features
Best diagnostic clue: Thickening of bronchovascular
Sarcoidosis
bundles Bronchovascular bundle thickening, lung nodules, and
Location: Perihilar septal thickening (often nodular) may mimic KS
Morphology Lymphadenopathy more symmetrical than KS and
o Thoracic manifestations does not typically enhance
Bronchovascular bundle thickening progressing to Lymphoma
coalescent, flame-shaped perihilar consolidation
Bronchovascular bundle thickening and lung nodules
Poorly defined nodules may mimic KS
Reticular and nodular opacities with basilar Lung nodules vary in size but are often larger than
predominance those associated with KS; air bronchograms more
common in lymphoma nodules than in KS nodules

DDx: Bronchovascular BU!ldle Thickening

Lymphangitic Carcinomatosis
 KAPOSI SARCOMA, PULMONARY

Key Facts
Terminology Pathology
Kaposi sarcoma (KS) Most common AIDS related neoplasm, but decreased
AIDS-related multicentric neoplasm with propensity prevalence in current era of highly active
to involve skin, lymph nodes, GI tract, and lungs antiretroviral therapy
CD4 count usually < 100
Imaging Findings Associated abnormalities: Skin lesions present in 8S%
Best diagnostic clue: Thickening of bronchovascular of patients with pulmonary involvement
bundles
CT findings usually highly suggestive

Lymphangitic Carcinomatosis Microscopic Features

3
Bronchovascular bundle thickening and septal Spindle-shaped stromal cells, abnormal endothelial 99
thickening (often nodular) may mimic KS lining of vascular channels, and slit-like spaces of
Unilateral distribution favors lymphangitic extravasated red cells
carcinomatosis from primary lung cancer over KS
Bacillary Angiomatosis ICLINICALISSUES
Rare infection due to Bartonella henselae
Skin lesions, enhancing lymph nodes, and lung Presentation
nodules mimic KS, but bronchovascular bundle Most common signs/symptoms: Dyspnea, cough
thickening uncommon Other signs/symptoms: Hemoptysis
Demographics
I PATHOLOGY Gender: Homosexual or bisexual male AIDS patients

General Features Natural History & Prognosis

Etiology: Human herpes virus 8 (KS-associated herpes Poor prognosis
virus) Treatment
Epidemiology Highly active antiretroviral therapy with or without
o > 90-95% cases occur in male AIDS patients with risk
chemotherapy
factor of homosexual contact
o Postulated to be sexually transmitted
o Most common AIDS related neoplasm, but decreased I SElECTED REFERENCES
prevalence in current era of highly active
antiretroviral therapy 1. Cheung MC et al: AIDS-Related Malignancies: Emerging
Challenges in the Era of Highly Active Antiretroviral
o CD4 count usually < 100
Therapy. Oncologist. 10(6):412-26,2005
Associated abnormalities: Skin lesions present in 85% Boiselle PM, et al: Update on Lung Disease in AIDS.
2.
of patients with pulmonary involvement Seminars in Roentgenology. 37(1); 54-71, 2002
3. Moore EH et al: Bacillary angiomatosis in patients with
AIDS: multiorgan imaging findings. Radiology.
197(1):67-72,1995

I IMAGE GALLERY

(Left) Axial NEeT shows bronchovascular bundle thickening and poorly defined lung nodules due to KS. (Center) Axial HRCT shows
bronchovascufar bundle thickening, poorly defined lung nodules, and septal thickening due to KS. (Right) Axial CECTshows bronchovascular
bundle thickening, poorly defined lung nodules, and septal thickening due to KS.
 Mediastinum

Introduction and Overview

Mediastinum rn 4
Aorta and Great Vessels rn
Heart and Pericardium []]
Pulmonary Vasculature [I]
 MEDIASTINAL COMPARTMENTS

Differential Diagnosis o
Anterior Mediastinal Mass (1's) long lesions Traversing the Entire length of 3
Thyroid the Mediastinum
Thymoma Aorta (dissecting aneurysm)
Teratoma Esophageal dilatation (achalasia)
Terrible lymphoma Fat (mediastinal lipomatosis)
Middle Mediastinal Mass Lymph nodes (lymphoma)
Lymphadenopathy Extrathoracic Tumors that Tend to
Congenital cysts
Pathology of visceral organs: Airways, esophagus, Metastasize to Mediastinum
aorta Genitourinary tumors: Renal cell carcinoma,
transitional cell carcinoma, testicular tumors,
Posterior Mediastinal Mass prostatic carcinoma, uterine and ovarian tumors
Neurogenic origin (90%) Head & neck tumors
Breast cancer
Melanoma

o Tboradc duct
o Sympathetic chain &: intercostal nerves I PATHOLOGY-BASED IMAGING
o Fat &: lymph nodes ISSUES
KeyConcepts or Questions
IANATOMY-BASED IMAGING ISSUES I How do the surgeons divide the mediastinum?
o 4 mediastinal compartments
KeyConcepts or Questions Superior: Above the aortic arch
Is the mass in the lung or mediastinum? Anterior - middie - posterior: Below the arch
o Lung lesions usually have edges which are similar to radiographic description
indistinct, irregular, or spiculated, mediastinal Exception: Middie mediastinum contains a
lestons have smooth edges portion of radiographic posterior mediastinum:
o Lung lesiOnScontacting the mediastinal pleura Descending aorta, esophagus, azygos and
usually have acute angles whereas mediastinal hemiazygos veins, and thoradc duct
lesions have obtuse angles How do surgeons approach mediastinal lesions?
How many lymph nodes are there in the o Mediastinoscopy: Scope descends down the right
mediasttnum? paratracheal space to the level of carina (known as
o In cadavers, the normal mediastinum contains Barety space)
apptQXtmately 50 lymph nodes. the majority of No access to anterior mediastinum, left-sided
wblchare n~evident on cr mediastinal lesions, posterior mediastinum,
lesions posterior to the carina or inferior to the
Norma1 ~~nts heart
WMtts tbe~ lymph node size? o Chamberlain: Anterior parasternal mediastinotomy
o A~ _*es depending on the region and are Used primarily to access left-sided mediastinal
mNSllnrd using the short-axis diameter (maximum lesions in the aortopulmonary window
shOrt-am.dWbeter) o Thoracoscopy: Can access nearly any mediastinal
Low patatrlicbeal and subcarinallymph nodes: 11 lesion
nun
Superior med1astinallymph nodes: 7 mm
Right hllar and paraesophageallymph nodes: 10 I CLINICAL IMPLICATIONS
mm
Left hllar an~ ~ageallymph nodes: 7 mm Clinical Importance
Perldiaphragmatic: S nun Top S primary mediastinal tumors by frequency:
o in practice, lymph nodes are considered enlarged If Lymphoma, thymic tumors, neurogenic tumors, germ
they exceed 10 mm ceil tumors, aneurysm
How accurate is cr for staging mediastinal lymph
nodes? Function-~nction
o Poor: SeDJftlvity60%, speQ8dty 80% with positive SO%medlastinallesions are asymptomatic discovered
JI{l!4lctlve~ue of SO%and ne&ati~ pnl!Ilctive Inddentallyon chest radiographs
value of 1lO9fI Symptoms related to compression and direct invasion
of surrounding structures or paraneoplastic syndromes
o In general asymptomatic lesions tend to be benign,
symptomatic lesions maUgnant
 .l~ M_E_D_I_A_ST_I_N_A_L_C_O_M_P_l\-R-T-M-E-N-T-S-----
o
4

Lateral radicJ&raph shcoNs sharply marginated mass

(arrCNV)located (NfN the aonIc arch and trachea. This
mass could be dassifIed as superior mediastinum or
middle mediastinum.
 MEDIASTINAL COMPARTMENTS
I IMAGE GALLERY o
5
(Left) Coronal aCT shows
diffuse mediastinal widening
from lipomatosis (arrows).
Mediastinal lesions traversing
the entire length of the
mediastinum include fat,
aortic dissection, esophageal
dilatation, or
lymphadenopathy. (Right!
Axial CECT shows
pneumomediastinum with air
(arrows) extending
throughout the mediastinal
compartments. Mediastinum
contains no fascial planes
that restrict the spread of
disease.

,
(Left) Axial CECT shows low

1.,. ,
attenuation (fat) mass
(arrow) in the posterior

~'.
mediastinum due to a

lipoma. Differential includes
diaphragmatic hernia.

f';~'..... (Right) Axial CECT shows

large cystic mass (arrows) in
the middle mediastinum
~ ./ ,.' .. '
displacing the superior vena
cava (open arrow) due to
mediastinal abscess.
Differential includes
bronchogenic cyst,
~ .. - ~ ~ lymphangioma, and lymph
~- ,. . node metastases.

(Left) Axial CECT shows

small enhancing anterior
mediastinal mass (arrows)
from a parathyroid adenoma.
Differential includes thyroid,
carcinoid tumor,
paraganglioma, and
adenopathy (Castleman).
(Right! Axial NECT shows
multiple mediastinal lymph
nodes with egg-shell
calcification (arrows) from
silicosis. Differential includes
sarcoidosis or treated
Hodgkin disease .
 Congenital
Thoracic Cysts 11-1-2

Inflammatory - Degenerative
Mediastinal Fibrosis 11-1-6
Achalasia 11-1-10
Esophageal Diverticuli 11-1-12
Hiatal and Paraesophageal Hernias 11-1-14

Neoplastic
Goiter, Mediastinum 11-1-18
Hodgkin Lymphoma, Mediastinum 11-1-22
Non-Hodgkin Lymphoma, Mediastinum 11-1-26
Thymoma 11-1-30
Thymic Tumors Other Than Thymoma 11-1-34
Thymic Rebound 11-1-38
Germ Cell Tumors, Mediastinum 11-1-40
Lipomatosis, Mediastinum 11-1-44
Castleman Disease, Mediastinum 11-1-48
Angioimmunoblastic Lymphadenopathy 11-1-52
Hemangioma - Lymphangioma, Mediastinum 11-1-54
Nerve Sheath Tumors, Mediastinum 11-1-58
Sympathetic Ganglion Tumors, Mediastinum 11-1-62
Extramedullary Hematopoiesis, Mediastinum 11-1-66

Vascular
Varices, Mediastinum 11-1-70
 THORACIC CYSTS
1
2

Anteroposterior radiograph shows bUNerfly vertebra Coronal oblique T2WI MR in same patient shows a
(arrow). (Courtesy B. Karmazyn, MO). vertically oriented tubular-shaped fluid-filled structure
that represents a neurenteric cyst (arrow). (Courtesy B.
Karmazyn, MO).

Anterior mediastinal: Thymic cyst, mature cystic

I TERMI NOLOGY teratoma (dermoid), pericardial, lymphangioma
Abbreviations and Synonyms Middle mediastinal: Bronchogenic, esophageal
duplication, neurenteric, pericardial,
Cystic adenomatoid malformation (CAM)
lymphangioma
Definitions Posterior mediastinal: Meningocele, neurenteric,
Foregut malformations: Lung "bud" anomalies bronchogenic, lymphangioma
o Bronchogenic, esophageal duplication, neurenteric, Size: Variable, 2-10 cm in size
CAM and others Morphology: Round, elliptical or tubular
Lymphatic origin: Lymphangiomas; mesothelial Radiographic Findings
origin: Pericardial cysts
Thymopharyngeal duct: Congenital thymic cysts Radiography
o Sharply marginated homogeneous mass-like opacity
Leptomeningeal origin: Meningocele; ~ two germ
o May contain air, or air and fluid
layers: Mature cystic teratoma
Mediastinal bronchogenic cyst: Often subcarinal,
protruding toward right hilum
Pulmonary bronchogenic cyst: In lower lobe, usually
I IMAGING FINDINGS medial 1/3
General Features CAM: Usually unilateral, lower lobes; single or
Best diagnostic clue: Most cysts near tracheal carina multiple
are bronchogenic o Expansion of the involved hemithorax
Location o Compensatory shift of the mediastinum
o Mediastinal cysts Esophageal and neurenteric cysts

DDx: Thoracic Cystic Lesions

Duplication Cyst Dermoid Cyst Lymphangioma

 THORACIC CYSTS

Key Facts
1
Terminology Pericardial cyst: Usually anterior cardiophrenic angle, 3
Foregut malformations: Lung "bud" anomalies on right side
Bronchogenic, esophageal duplication, neurenteric, High signal intensity T2, parallel to cerebrospinal
CAM and others fluid

Imaging Findings Top Differential Diagnoses

Best diagnostic clue: Most cysts near tracheal carina Mediastinal Cystic Tumors
are bronchogenic Pancreatic Pseudocyst
Size: Variable, 2-10 em in size Pathology
Morphology: Round, elliptical or tubular Bronchogenic cysts: 1/5 of mediastinal masses, up to
Sharply marginated homogeneous mass-like opacity 90% are mediastinal
Vertebral anomaly with neurenteric cysts CAM: 1/4 of all congenital lung abnormalities
Homogeneous attenuation; usually in the range of
water, serous fluid, 0-20 HU Clinical Issues
Increased attenuation, viscous, mucoid, blood, Most common signs/symptoms: Usually
calcium oxalate contents asymptomatic
Observation, for most cases

o Tubular, oriented vertically along esophagus and Unilocular, multilocular; thin septations; may also
spine involve neck/chest wall
o Vertebral anomaly with neurenteric cysts Complications: Airway compression, infection,
chylothorax, chylopericardium
CT Findings o Meningocele: Leptomeninges herniates through
NECT intervertebral foramen or vertebral body defect
o Sharply defined, thin-wall, round, elliptical or CT myelography shows cyst filling with contrast
tubular cyst CECT: Enhancement of the cyst wall; no enhancement
o Attenuation of cyst contents of cyst contents
Homogeneous attenuation; usually in the range of
water, serous fluid, 0-20 HU MR Findings
Increased attenuation, viscous, mucoid, blood, TlWI
calcium oxalate contents o Usually, low signal intensity, parallel to
o Infection of cyst: Shaggy wall that enhances cerebrospinal fluid
Communication with airway, air-filled or air-fluid o Variable T1 signal intensity, due to protein, blood or
level mucous contents
o Bronchogenic cyst: Wall may contain calcium T2WI
Moldable, rarely cause obstruction; rarely, in o High signal intensity T2, parallel to cerebrospinal
pleura or diaphragm fluid
o CAM: Complex conglomeration of multiple cysts in Regardless of the nature of cyst contents
a lower lobe
o Pericardial cyst: Usually anterior cardiophrenic Fluoroscopic Findings
angle, on right side Esophagram: Duplication cyst: Extrinsic or intramural
Adjacent to pericardium; may be pedunculated compression
o Thymic cyst: Unilocular or multilocular cysts; wall Nuclear Medicine Findings
may contain calcium
Tc-99m sodium pertechnetate: Uptake in gastric
o Mature cystic teratoma (dermoid)
mucosa in duplication cysts
May contain soft tissue, fluid, fat, calcium, bone,
teeth; fat-fluid level Ultrasonographic Findings
15%: Contents only fluid Grayscale Ultrasound: Pleural or cardiodiaphragmatic
o Esophageal duplication cyst: Adjacent to esophagus location: Anechoic thin-walled cyst with increased
Tubular vertical shape, often right sided; cyst wall through transmission
may be thick
May ulcerate into esophagus or airway (air-fluid Imaging Recommendations
level) Best imaging tool: CT suffices in most cases
o Neurenteric cyst Protocol advice: Standard CT, without and with
Posterior mediastinum, right-sided; long vertical contrast
tubular lesion
Vertebral anomalies, superior to cyst;
hemivertebra, sagittal clefts
Most commonly, upper thoracic spine
o Lymphangioma (cystic hygroma)
 THORACIC CYSTS
1 I DIFFERENTIAL DIAGNOSIS
4 Mediastinal Cystic Tumors
Thymoma, Hodgkin disease, germ cell tumor,
metastases, schwannomas, neurofibromas
History of prior tumor, radiation, chemotherapy
May have a solid or nodular component
Cystic schwannoma: MR may show intraspinal
extension
Pancreatic Pseudocyst
History of pancreatitis; lower posterior mediastinum;
abdominal pseudocyst not always present
Mediastinal Abscess
Patient usually septic
Pneumatocele
Eventually disappears spontaneously
Sequestration
Aberrant systemic arterial supply
Bronchogenic Carcinoma
Smooth thin walled cavitary lesions, uncommon
Fine needle aspiration biopsy for diagnosis
I PATHOLOGY
General Features
General path comments: Cysts contain clear, serous
fluid or thick mucoid material
Etiology
o Foregut malformation, congenital abnormal
budding from ventral foregut
Precursor of trachea and major bronchi
Early duplication anomaly, mediastinal; late
duplication anomaly, pulmonary
Notochord adjacent to foregut and may give rise
to neurenteric cysts
o CAM
Adenomatoid proliferation of bronchioles that
form cysts instead of normal alveoli
o Acquired thymic cysts
After radiation for Hodgkin, in association with
thymic tumors, after thoracotomy
Multilocular cysts following thymic inflammation;
children with HIV
Epidemiology
o Bronchogenic cysts: 1/5 of mediastinal masses, up to
90% are mediastinal
o CAM: 1/4 of all congenital lung abnormalities
o Esophageal duplication cysts, rare
o Thymic cysts, uncommon
Associated abnormalities
o Bronchogenic cysts, associated with sequestration,
congenital lobar emphysema
o CAM associated with sequestration
o Meningocele, associated with neurofibromatosis
Gross Pathologic & Surgical Features
Unilocular cyst containing mucus, watery fluid, or
purulent material
Microscopic Features
Bronchogenic cyst: Lined with pseudostratified
columnar respiratory epithelium
o Walls contain cartilage, smooth muscle, mucous
glandular tissue
Esophageal duplication cyst: Gastric or pancreatic
tissue may cause hemorrhage, ulceration, perforation
Neurenteric cyst: Admixture of gastric and neural
tissue elements
Cystic adenomatoid malformation
o Type I: Cysts measuring 2-10 em, most common
variety
o Type II: Numerous smaller, more uniform cysts
measuring 0.5-2 em in diameter
o Type Ill: Solid-appearing lesions, microscopically
demonstrate tiny cysts
Pericardial cysts: Lined by connective tissue and a
single layer of mesothelial cells
Mature cystic teratoma: Lined by tall mucus secreting
epithelial cells
o Filled with sebaceous material, hair, skin, glandular,
muscle tissue
ICLINICAL ISSUES
Presentation
Most common signs/symptoms: Usually asymptomatic
Other signs/symptoms: Chest pain, cough, dyspnea,
wheezing, fever, purulent sputum, dysphagia
May be life-threatening if there is
o Compression of airway, infection, hemorrhage,
rupture, pneumothorax
Demographics
Age
o Discovered at any age, usually < 35 years
o Lymphangiomas, CAM, usually less than 2 years old
Gender: M:F = 1:1
Natural History & Prognosis
Increasing size of cyst, consider hemorrhage, infection;
rule out neoplasm
Treatment
Observation, for most cases
Needle aspiration for atypical cysts
o Increasing size or malignancy suspected
o Cyst usually recurs after aspiration
Surgical resection for symptomatic lesions
I SELECTED REFERENCES
1. Jeung MY et al: Imaging of cystic masses of the
mediastinum. Radiographies. 22 Spec No:S79-93, 2002
2. Zylak CJ et al: Developmental lung anomalies in the adult:
radiologic-pathologic correlation. Radiographies. 22 Spec
No:S2S-43, 2002
 THORACIC CYSTS

I IMAGE GALLERY 1
5
Typical
(Left) Frontal radiograph
shows cystic changes at the
right lung (arrows). Vague
opacity (curved arrow) may
represent adjacent
compressed lung. Marked
shilt 01 heart and
mediastinum (open arrow).
(Right) Axial NECT in same
patient shows complex cystic
structure (arrow) in the right
lower lobe. Diagnosis: Cystic
adenomatoid malformation.

Typical
(Left) Axial CECT shows cyst
with imperceptible wall
(arrow) that is either
mediastinal or
intra-pulmonary in origin.
(Right) Coronal T2WI FS MR
in same patient shows high
signal contents (arrow). The
cyst is probably
intrapulmonary. Diagnosis:
Bronchogenic cyst.

(Left) Frontal radiograph

shows an air-filled cyst in the
right lower lung with a short
air-I'uid level (arrow). (Right)
Axial CECT in same patient
shows the right middle lobe
cyst (arrow) has a shaggy
wall and an air-Iluid level
(curved arrow). Cyst was
resected and proved /0 be
an infected bronchogenic
cyst.
 MEDIASTINAL FIBROSIS
1
6

Frontal radiograph shows enlarged right hilum (arrows) Axial CECT shows soft tissue mass in the subcarinal
and vague peripheral area of consolidation in the right region engulfing the right pulmonary artery extending
upper lobe (curved arrow) in 42 year old man with along the superior vena cava. Right upper lobe opacity
chest pain and shortness of breath. was a pulmonary infarct. Diagnosis mediastinal fibrosis.

ITERMINOLOGY I IMAGING FINDINGS

Abbreviations and Synonyms General Features
Fibrosing mediastinitis, idiopathic mediastinal fibrosis, Best diagnostic clue: Calcified mediastinal mass with
mediastinal granuloma SVC obstruction
Location: Most common in right para tracheal or
Definitions subcarinallocation
Focal (80%) or diffuse (20%) mediastinal mass either Morphology: Calcification usually central in mass
idiopathic or due to granulomatous infection
Focal form Radiographic Findings
o Calcification common in focal disease Radiography
o Focal disease usually secondary to immune reaction o Focal hilar or mediastinal mass or widening
from histoplasmosis Right para tracheal region most common
o Focal disease obstructs superior vena cava (SVC), Focal mass usually calcified (60-90%)
airways, pulmonary veins in that order Calcified peripheral lung granulomas up to 3 cm
Diffuse form in diameter common
o Diffuse disease usually idiopathic, associated with o Diffuse mediastinal widening
other autoimmune disease, or from drug reaction Calcification less common
Treatment directed toward stenting airways and Smooth or lobulated mediastinal contours
vessels o Secondary findings
Collaterals from SVC obstruction, aortic nipple
(dilated left superior intercostal vein) common
Lobar collapse or pneumonitis with airway
obstruction

DDx: Fibrosing Mediastinitis

Hodgkin SVC Syndrome Aberrant Artery Aneurysm

 MEDIASTINAL FIBROSIS
Key Facts
1
Terminology Goiter 7
Focal (80%) or diffuse (20%) mediastinal mass either Achalasia
idiopathic or due to granulomatous infection Erdheim-Chester Disease
Calcification common in focal disease Pathology
Imaging Findings Large biopsy specimen required to exclude
Focal hilar or mediastinal mass or widening lymphoma
Collaterals from SVC obstruction, aortic nipple Focal disease thought to arise from abnormal
(dilated left superior intercostal vein) common immune response to histoplasmin antigen in
susceptible individuals
Focal mass
Often associated with other immune phenomenon:
Top Differential Diagnoses Retroperitoneal fibrosis, orbital pseudotumor,
Lymphoma systemic lupus erythematosus, rheumatoid arthritis,
Bronchogenic Carcinoma Riedel thyroiditis
Cystic Foregut Malformations < 10% of all mediastinal masses
Mediastinal Germ Cell Tumors Most common non-malignant cause of superior vena
Aortic Aneurysms cava syndrome

Pulmonary venous hypertension and interstitial

edema from pulmonary vein obstruction
I DIFFERENTIAL DIAGNOSIS
o Bronchial narrowing Lymphoma
Main bronchi most commonly followed by upper Noduiar sclerosing Hodgkin may be difficult to
lobe bronchi and bronchus intermedius differentiate pathologically
Often multiple airways affected o Hodgkin usually anterior mediastinum
CT Findings o Fibrosing mediastinitis usually hilar, subcarinal and
paratracheal
NECT
o Focal mass o Hodgkin noncalcified prior to treatment
2-5 cm in diameter o Nodal calcification post treatment either mulberry
type or egg-shell
80% calcified
o Calcification o Usually does not affect surrounding mediastinal
Large central component structures, even when bulky
Amorphus or "putty-like" Bronchogenic Carcinoma
o Diffuse form usually not calcified Generally older adult with smoking history
o Other signs of granulomatous infection Carcinoma does not calcify
Calcified granulomas in lung, often large with Primary lung lesion common
central nidus of calcification
CECT: No contrast-enhancement of mass in either Cystic Foregut Malformations
diffuse or focal form Cystic mass in subcarinal region, mediastinal fibrosis
not cystic
MR Findings
Calcification only in wall
Limited due to inability to detect calcification Generally does not affect mediastinal structures
Similar to CT in ability to evaluate vascular
involvement Mediastinal Germ Cell Tumors
Usually anterior mediastinum
Fluoroscopic Findings Calcification small component of tumor (teratomas
Esophagram only)
o Extrinsic compression from mediastinal fibrosis
Most common in subcarinallocation Aortic Aneurysms
o Fistula to the mediastinal mass possible May compress adjacent mediastinal structures
Calcification in wall or in thrombus
Imaging Recommendations
Enhance with intravenous contrast
Best imaging tool: CECT, focal calcified mediastinal
mass affecting surrounding mediastinal structures Goiter
diagnostic of mediastinal fibrosis Anterior mediastinal mass
Protocol advice: CT procedure of choice to Connected or adjacent to nodular thyroid
demonstrate anatomical relationship to surrounding Often calcified and of high density due to iodine
veins and airways content
Achalasia
Dilated esophagus the cause of mediastinal widening
Air-fluid level upper mediastinum
 MEDIASTINAL
1 Lack of stomach bubble chest radiograph
Erdheim-Chester Disease
8 Presentation
Diffuse encasement aorta and great vessels
Pleural thickening and perirenal soft tissue
encasement
Sclerotic bone lesions
Does not calcify
I PATHOLOGY
General Features
General path comments
a Large biopsy specimen required to exclude
lymphoma
a Location
Right para tracheal lesion affects SVC
Subcarinallesion affects esophagus, pulmonary
veins, bronchi, and pulmonary arteries
Etiology
a Whether focal form evolves into diffuse debatable
a Infection
Focal disease thought to arise from abnormal Treatment
immune response to histoplasmin antigen in
susceptible individuals
Tuberculosis also known etiology but much less
common
Theory of spillage or seepage of ruptured lymph
nodes now considered unlikely
a Idiopathic
Diffuse disease usually of unknown cause I DIAGNOSTIC
Often associated with other immune
phenomenon: Retroperitoneal fibrosis, orbital
pseudotumor, systemic lupus erythematosus,
rheumatoid arthritis, Riedel thyroiditis
a Drugs
Methysergide, used for migraine headaches (rarely
used now)
Epidemiology
a More common in geographic areas endemic for
Histoplasmosis
a < 10% of all mediastinal masses
a Most common non-malignant cause of superior
vena cava syndrome
Gross Pathologic & Surgical Features
Focal disease (known as mediastinal granuloma)
a Calcified matted nodes containing abundant fibrous
tissue
Diffuse disease I SELECTED REFERENCES
a Fibrous tissue replacing mediastinal fat
a Must be differentiated from Hodgkin disease
(nodular sclerosing)
Microscopic Features
Benign inflammatory cells, granulomatous response 3.
Spectrum from necrotizing granulomatous
inflammation to mature fibrous tissue
Lung calcifications larger than 4 mm and nodal 4.
calcifications larger than 1 em are 80% more likely to
come from histoplasmosis than tuberculosis
FIBROSIS
ICLINICAL ISSUES
Most common signs/symptoms
a Asymptomatic unless obstruction of mediastinal
contents
a Hemoptysis, dyspnea, cough most common
a SVC syndrome uncommon, slow progression of
obstruction allows time for collaterals to develop
Other signs/symptoms: Sputum cultures negative
Demographics
Age: Adults of any age, most between 21 and 40
Natural History & Prognosis
Long protracted history, airway compromise,
respiratory failure, or cor pulmonale
Subcarinal involvement most likely to lead to death
because of close proximity to carina, pulmonary veins,
and pulmonary arteries
Nodal or histoplasmoma growth 1-2 mm per year
a To reach 3 em in diameter will take 10-20 years
Antifungals and steroids ineffective for focal form
Steroids may benefit diffuse form
Surgery difficult and little benefit
Palliation
a Intravascular and airway stents
CHECKLIST
Consider
Calcified mediastinal mass
a Fibrosing mediastinitis
a Aortic aneurysm
a Histoplasmosis, tuberculosis, Pneumocystis jiroveci
infection
a Goiter
a Silicosis, sarcoidosis
a Treated lymphoma
a Teratoma
Image Interpretation Pearls
Calcified mediastinal mass with encroachment on
adjacent mediastinal structures hallmark of fibrosing
mediastinitis
1. Ryu DSet al: Fibrosing mediastinitis with peripheral airway
dilatation and central pulmonary artery occlusion. J Thorac
Imaging. 19(3):204-6, 2004
2. RossiSEet al: Fibrosing mediastinitis. Radiographies.
21:737-57,2001
Flieder DBet al: Idiopathic fibroinflammatory
(fibrosing/sclerosing) lesions of the mediastinum: a study
of 30 cases with emphasis on morphologic heterogeneity.
Mod Pathal. 12(3):257-64, 1999
Sherrick AD et al: The radiographic findings of fibrosing
mediastinitis. Chest. 106:484-9, 1994
 MEDIASTINAL FIBROSIS
I IMAGE GALLERY 1
9

(Left) Frontal radiograph

shows enlarged right hilum
in a 30 year old
asymptomatic patient.
(Right) Axial CECT shows
large subcarinal mass
extending into the right
lower lobe. Mass contains
large central clumps of
calcification. Mass has
partially narrowed the
bronchus intermedius
(arrow).

Typical
(Left) Axial NECT shows
subcarinal mass with
amorphous putty-like
calcification narrowing the
carina. Left upper lobe has
collapsed. (Right) Axial
CECT shows calcified
subcarinal mass and soft
tissue rind engulfing the
bronchus intermedius and
encroaching on the right
interlobar pulmonary artery.
Right hemithorax is small.
Airway has been stented.
Note collateral flow from
enlarged intercostal artery
(arrow).

Typical
(Left) Axial CECT shows
subcarinal calcified mass and
50ft tissue mass obstructing
the right pulmonary veins
and surrounding the right
middle lobe bronchus and
bronchus intermedius.
(Right) Axial HRCT shows
marked septal thickening
(arrow) from pulmonary
venous hypertension in same
patient Mosaic attenuation
(curved arrow). Enlarged
central bronchovascu/ar
bundles (open arrow) from
pulmonary arterial
hypertension.
 ACHALASIA
1
10

Graphic shows Achalasia. Esophagus is markedly Frontal radiograph shows bulging of azygoesophageal
dilated with an air-fluid level at the upper esophagus line (white arrows) and dilated esophagus in the upper
and beaking at the esophagogastric junction. Air-fluid mediastinum (black arrow). Note absence of gastric air
level in the stomach typically absent. bubble.

o Absence primary peristalsis, smooth tapering distal

ITERMINOLOGY esophagus ("beak-like" appearance)
Definitions Radiographic Findings
Primary achalasia: Primary motility disorder of the Radiography
esophagus (smooth muscle) o Mediastinal widening (double contour)
Secondary or pseudoachalasia: Involvement of o Marked dilated esophagus
gastroesophageal junction by other abnormalities o Anterior tracheal bowing
(Chagas disease, tumor) o Retro-tracheal air-fluid level
Diffuse mediastinal widening with air-fluid level o "Bird-beak" deformity of distal esophagus
suggests achalasia o Little or absent gastric air bubble
Esophagram key to evaluate motility, reflux, and
aspiration CT Findings
NECT
o Esophageal dilatation with air-fluid level
I IMAGING FINDINGS o Abrupt, smooth narrowing at distal esophagus
o Esophageal squamous cell carcinoma in
General Features long-standing achalasia
Best diagnostic clue Irregular wall thickening
o Smooth esophageal dilatation Mediastinal lymphadenopathy
o "Bird-beak" configuration at esophagogastric o Aspiration pneumonia
junction on esophagram
Esophagram findings Imaging Recommendations
o Procedure of choice to evaluate esophageal motility, Best imaging tool: Barium study
reflux, and aspiration

DDx: Mediastinal Air-Fluid level

Zenker Diverticulum Abscess Hiatal Hernia

 ACHALASIA

Key Facts
1
Terminology Top Differential Diagnoses 11
Primary achalasia: Primary motility disorder of the Scleroderma
esophagus (smooth muscle) Esophageal Carcinoma
Dissecting Aortic Aneurysm
Imaging Findings
Mediastinal widening (double contour) Clinical Issues
Retro-tracheal air-fluid level Dysphagia (90%)
Little or absent gastric air bubble Esophageal carcinoma in 2-7% of cases
Heller myotomy (longitudinal incision of lower
esophageal sphincter)

o Dysphagia (90%)
I DIFFERENTIAL DIAGNOSIS o Recurrent aspiration pneumonias
Scleroderma Demographics
Dysmotility, dilated esophagus
Age
Air-fluid level in stomach o Primary achalasia: Younger patients (30-50 years)
Esophageal Carcinoma o Secondary achalasia: Older patients
Esophagus minimally dilated Gender: M = F
Focal mass at site of tumor Natural History & Prognosis
Dissecting Aortic Aneurysm Esophageal carcinoma in 2-7% of cases
Displaced intimal calcification Treatment
No air-fluid level in esophagus Smooth muscle relaxants
Pneumatic dilatation (risk of perforation)
Heller myotomy (longitudinal incision of lower
I PATHOLOGY esophageal sphincter)
General Features
Etiology I DIAGNOSTIC CHECKLIST
o Unknown
Myenteric plexus neuropathy with incomplete Image Interpretation Pearls
relaxation lower esophageal sphincter Marked dilated esophagus, "bird-beak" deformity of
Microscopic Features distal esophagus
Decreased number of ganglion cells in myenteric
esophageal plexus
I SELECTED REFERENCES
1. Franquet T et a!. The retrotracheal space: normal anatomic
ICLINICAl ISSUES and pathologic appearances. RadioGraphies. 22:S23]-S246,
2002
Presentation 2. Stark Pet al: Manifestations of esophageal disease on plain
chest radiographs. AJR.1SS:729-34, ] 990
Most common signs/symptoms

I IMAGE GAllERY

(Left) Lateral radiograph shows an air-fluid level (arrows) in the retrotrachealspace due to dilated esophagus. (Center) Esophagram shows
dilated, aperistaltic esophagus with beak-like narrowing at gastroesophageal junction (arrow). Note that esophagus is filled with large amounts of
alimentary content. (Right) Axial HRCT shows bulging of azygoesophagealstripe (arrow) due to achalasia. Note multiple centrilobular opacities
("tree-in-bud") in the left upper lobe due to aspirative bronchiolitis (open arrows).
 ESOPHAGEAL DIVERTICULI
1

.,
12

,~~
......... .. , -

'~

.. '

V ..

Graphic shows different types of esophageal diverticu/i:

Zenker, traction and epiphrenic.
i
"
Lateral esophagram shows large Zenker diverticulum
(arrow) displacing and compressing the posterior wall
of the proximal esophagus.

o Air-fluid level in superior mediastinum (Zenker) or

ITERMINOlOGY in midesophagus (traction)
Abbreviations and Synonyms o Barium esophagram
Esophageal saccular protrusion or outpouching True and pulsion diverticulum: Barium-filled sac;
True diverticulum; pulsion diverticulum; traction rounded contour
diverticulum Traction diverticulum: Barium-filled tented or
triangular shaped outpouching
Definitions Location
True diverticulum: Formed by mucosa and submucosa o Pharyngoesophageal junction: Pulsion (Zenker)
without a muscular layer o Midesophagus: Traction
Pulsion diverticulum: Formed by increased o Distal esophagus: Epiphrenic
intraluminal esophageal pressure (e.g., Zenker Size: Variable; Zenker: Range (0.5-8 em)
diverticulum; epiphrenic) Morphology
Traction diverticulum: Due to fibrosis in adjacent o Rounded or saccular: True, pulsion
periesophageal tissues (granulomatous mediastinal o Triangular: Traction
disease)
Imaging Recommendations
Pseudodiverticulum: Small outpouchings of mucosal
glands Best imaging tool: Barium esophagram

IIMAGING FINDINGS I DIFFERENTIALDIAGNOSIS

General Features Pseudodiverticulosis
Best diagnostic clue Tiny outpouchings either diffuse (50%) or segmental
in long rows parallel to long axis of esophagus

DDx: Esophageal Outpouchings

Esophageal Ulcer Esophageal Webs Phrenic Ampulla

 ESOPHAGEAL DIVERTICULI

Key Facts
1
Imaging Findings Esophageal Webs 13
Air-fluid level in superior mediastinum (Zenker) or in Esophageal Ulcer
midesophagus (traction) Phrenic Ampulla
Pharyngoesophageal junction: Pulsion (Zenker) Clinical Issues
Midesophagus: Traction Aspiration pneumonia; bronchitis; bronchiectasis
Distal esophagus: Epiphrenic Risk of perforation after endoscopy or placement of
Best imaging tool: Barium esophagram nasogastric tube
Top Differential Diagnoses Erosion; inflammation; perforation; fistula
Pseudodiverticulosis

o Zenker
Esophageal Webs Upper esophageal dysphagia
1-2 mm wide, shelf-like filling defect along anterior Regurgitation and aspiration of undigested food
wall of cervical esophagus Halitosis; hoarseness; neck mass
Esophageal Ulcer Demographics
Solitary ring-like or stellate shaped large ulcer with Age
halo of edema o Zenker: M > F; 50% of cases seen in 7th-8th decade
o Traction: M = F; usually seen in elderly patients
Phrenic Ampulla
2-4 em long luminal dilation between esophageal "A" Natural History & Prognosis
and rings
!IBI!
Complications
o Zenker
Aspiration pneumonia; bronchitis; bronchiectasis
I PATHOLOGY Risk of perforation after endoscopy or placement
of nasogastric tube
General Features o Traction
Etiology Erosion; inflammation; perforation; fistula
o Pharyngoesophageal junction: Mucosal herniation
through an area of anatomic weakness in the region Treatment
of cricopharyngeal muscle Asymptomatic: No treatment
o Traction diverticulum: Common in areas of endemic Large or symptomatic: Surgical diverticulectomy or
tuberculosis and histoplasmosis endoscopic repair
Gross Pathologic & Surgical Features
Posterior hypopharyngeal saccular outpouching
broad or narrow neck (Zenker)
with I SELECTED REFERENCES
1. Sidow DB et al: Radiographic findings and complications
after surgical or endoscopic repair of Zenker diverticulum
in 16 patients. A]R Am] RoentgenoJ. 177:1067-1071, 2001
ICLINICALISSUES 2. Duda M et al: Etiopathogenesis and classification of
esophageal diverticula. Int Surg. 70:291-295, 1985
Presentation
Most common signs/symptoms

I IMAGE GALLERY

(Left) Esophagram shows multiple outpouchings at the proximal and midesophagus. Hiatal hernia is also visible. Consolidation is evident in the
left lower lobe (post-aspirative!. (Center) Esophagram shows multiple outpouchings within the esophageal wall. This finding is characteristic of
intramural pseudodiverticufosis. (Right) Esophagram shows large epiphrenic diverticulum located near the esophago-gaslric junction (arrows).
 HIATAL AND PARAESOPHAGEAL HERNIAS
1
14

Coronal graphic shows typical findings of a Frontal radiograph shows a large hiatal hernia.
moderate-sized sliding hiatal hernia. Diaphragmatic Retrocardiac air-fluid level (arrows). LArge hernias have
hiatus has enlarged allowing stomach to herniate into mass effect, here displacing the mediastinal structures to
chest. Commonly hernia sac contains air and fluid. the right.

Size: Ranges from barely discernible to entire stomach

ITERMINOlOGY in chest
Abbreviations and Synonyms Radiographic Findings
Hiatus hernia (HH), sliding hiatal hernia Sliding hiatal hernia
Definitions o Smooth hemispherical left-sided retrocardiac mass
Prottusion of a part of the stomach through the o Usually contains air or air-fluid level
esophageal hiatus of the diaphragm Paraesophageal hernia
Sliding (axial) hiatal hernia: Gastroesophageal (GE) o Smooth hemispherical retrocardiac mass
junction and gastric cardia pass through esophageal Protrusion anterior and lateral to esophagus
hiatus of diaphragm into thorax o Usually contains air or air-fluid level
Paraesophageal (rolling) hernia: GE junction below Gastric volvulus
diaphragm, gastric fundus intrathoracic o Organoaxial rotation versus mesenteroaxial volvulus
based on type of rotation
Mesenteroaxial gastric volvulus
IIMAGING FINDINGS o Distended stomach appears spherical on supine
images
General Features o Two air-fluid levels are visible on the upright film: 1
Best diagnostic clue inferiorly in fundus, 1 superiorly in antrum
o Intrathoracic air-containing retrocardiac mass o Upright image may demonstrate a beak at
o Gastric volvulus: Double air-fluid level above and gastroesophageal (GE) junction
below diaphragm o On upper gastrointestinal imaging (UG!), if barium
Location: Retrocardiac, usually left-sided and medial moves past the GE junction, stomach configuration
upside down

DDx: Hiatal Hernia

Epiphrenic Diverticulum Gastric Pull-Up

 HIATAL AND PARAESOPHAGEAL HERNIAS

Key Facts
1
Terminology Top Differential Diagnoses 15
Protrusion of a part of the stomach through the Epiphrenic Diverticulum
esophageal hiatus of the diaphragm Bochdalek Hernia
Sliding (axial) hiatal hernia: Gastroesophageal (GE) Gastric Pull-Up or Colonic Interposition
junction and gastric cardia pass through esophageal Duplication Cyst
hiatus of diaphragm into thorax Bronchogenic Cyst
Paraesophageal (rolling) hernia: GE junction below Intralobar Sequestration
diaphragm, gastric fundus intrathoracic
Pathology
Imaging Findings Common (sliding hiatal hernia 99%) to rare
Intrathoracic air-containing retrocardiac mass (paraesophageal 1%)
Gastric volvulus: Double air-fluid level above and
Clinical Issues
below diaphragm
New unexplained pleural effusion, suspect gastric Mortality rate for acute gastric volvulus is
ulcer in hernia sac approximately 50%

Obstruction usually at the antrum-duodenal as it Presence of B-ring (Schatzki ring) above

courses back into the abdomen hemidiaphragm confirms diagnosis of hiatal
Organoaxial gastric volvulus hernia
o Difficult to diagnose on plain films Mucosal complications, esophagitis, ulcers and
o Stomach lies horizontally and contains a single strictures in up to 5%
air-fluid level on upright views
o No characteristic beak seen
o UGI shows GE junction lower than normal with I DIFFERENTIAL DIAGNOSIS
marked gastric dilatation and slow passage of
contrast past site of twisting Epiphrenic Diverticulum
Complications Occurs in setting of long-standing peptic esophagitis
o Pleural effusion and strictures
New unexplained pleural effusion, suspect gastric Large outpouching with wide neck above GE junction
ulcer in hernia sac seen by UGI
o Pneumoperitoneum UGI may provide clues to underlying motility
Free intraperitoneal air in hernia sac if gastric wall disturbances that may be involved in diverticular
perforated formation
Peritoneum attaches to GE junction Bochdalek Hernia
CT Findings Congenital hernia in the posterolateral diaphragm
NECT 85% left-sided
o Pseudomass: Complex mass with soft tissue, fat, air, Contain fat, rarely bowel or spleen
and oral contrast Gastric Pull-Up or Colonic Interposition
o Widened esophageal hiatus
Air containing stomach or bowel above
Dehiscence of diaphragmatic crura (> 15 mm);
hemidiaphragm
increased distance between crura and esophageal
Surgical history pertinent
wall
o Gastric volvulus and paraesophageal hernias require Duplication Cyst
careful delineation of course of alimentary tract to Usually no air
differentiate from sliding HH Contain air if ulcerate into gastrointestinal (GI) tract
Multiplanar reconstructions may be helpful Paraesophageal in location
Fluoroscopic Findings Bronchogenic Cyst
Esophagram Subcarinallocation
o Lower esophageal mucosal "B" ring observed 2 em or Usually no air
more above diaphragmatic hiatus Uncommonly will contain air if communicates with
o Gastric folds or areae gastricae within herniated GI tract or bronchi
portion of fundus Water density by CT; fluid signal intensity by MRI
o Sensitivity: Single-contrast 100%
Intralobar Sequestration
Imaging Recommendations Left paraspinallocation, systemic arterial supply
Best imaging tool May contain air-fluid level (abscess)
o UGI/esophagram
 HIATAL AND PARAESOPHAGEAL
1 Morgagni Hernia
16 Congenital hernia in the anterior diaphragm
May contain bowel, usually stomach or colon
Right anterior paracardiac mass
Neurogenic Tumors: Schwannoma,
Neurofibroma
Posterior mediastinal location
No air
Enhances by CT, may be heterogeneous
Pericardial Cyst
No air, generally anterior and right-sided
Water density by CT; fluid signal intensity by MR
I PATHOLOGY
General Features
Etiology
a Most are not congenital
Stretching of phrenoesophageal membrane;
progressive wear and tear caused by constant
swallowing; abdominal pressure> thoracic
(exacerbated by pregnancy and obesity)
Atrophy of crura or diaphragmatic muscle from
aging
Epidemiology
a Common (sliding hiatal hernia 99%) to rare
(paraesophageal 1%)
a Sliding: 10% over age 50
Gross Pathologic & Surgical Features
Sliding hiatal hernia
a Atrophy of crura or diaphragmatic muscle
a Esophageal hiatus enlarges from increased
intra-abdominal pressure
a Rotation and mobility of stomach may give rise to
gastric volvulus
Paraesophageal hernia
a Widened hiatus permits the fundus of the stomach
to protrude into the chest
a Protrusion is anterior and lateral to the body of the
esophagus
a GE junction remains below the diaphragm initially,
with large hernias will also be in chest
a As hiatus widens, increased herniation of greater
curvature and sometimes gastro-colic omentum
a Fundus eventually comes to lie above GE junction
a Pylorus is pulled towards diaphragmatic hiatus
Gastric volvulus
a Rotation of all or part of stomach more than 180 0
a Classified on basis of axis of rotation
Organoaxial volvulus (majority): Stomach rotates
on longitudinal axis (line connecting cardia and
pylorus)
Mesenteroaxial volvulus: Stomach rotates about
vertical axis passing through middle of greater and
lesser curvatures, seen in young children and
associated with ligamentous laxity
a Associated with gastric strangulation
HERNIAS
Staging, Grading or Classification Criteria
Surgical classification
a Type 1: GE junction and gastric cardia intrathoracic
(sliding HH)
o Type 2: GE junction normal, gastric fundus
intrathoracic (paraesophageal hernia)
o Type 3: Both GE junction and fundus in chest
(paraesophageal hernia)
o Type 4: GE junction and entire stomach in chest
(paraesophageal hernia)
I CLINICAL ISSUES
Presentation
Most common signs/symptoms
o Sliding HH: Usually asymptomatic, symptoms may
include
Reflux, "heartburn", regurgitation and dysphagia
Occult bleeding
Referred chest pain may mimic cardiac disease
Large hernias may cause respiratory compromise
o Paraesophageal: Asymptomatic to life-threatening
emergency
o Triad of Borchardt (gastric volvulus)
Severe epigastric pain
Retching, unsuccessful vomiting
Inability to pass nasogastric tube
Demographics
Age: Prevalence increases with age
Gender: F > M
Natural History & Prognosis
Mortality rate for acute gastric volvulus is
approximately 50%
Treatment
Medical treatment same as gastrointestinal reflux
disease (GERD)
o Primary treatment is anti reflux therapy and protein
pump inhibitors (PPls)
Surgery for symptomatic disease
o Needed in minority of patients despite aggressive
treatment for GERD
o Other indications: Recurrent aspiration pneumonia,
chronic cough, or hoarseness from GERD
a Nissen fundoplication performed laparoscopically
360 fundic wrap around the gastroesophageal
0
junction, diaphragmatic hiatus also repaired
Paraesophageal hernia may strangulate and frequently
is operated on prophylactically to prevent
strangulation
Gastric volvulus
o Anterior gastropexy or gastrostomy to fix stomach
in anatomically correct position
o Partial or total gastrectomy in the setting of necrosis
I SElECTED REFERENCES
1. Eren S et al: Diaphragmatic hernia: diagnostic approaches
with review of the literature. Eur J Radial. 54(3):448-59,
2005
 HIATAL AND PARAESOPHAGEAL HERNIAS
I IMAGE GAllERY 1
17
Variant
(Left) Frontal radiograph
shows a large ruptured hiatal
hernia. Intrathoracic
pneumoperitoneum
(arrows). The wall of the
herniated portion of the
stomach is also seen (curved
arrow), the double-wall sign
of pneumoperitoneum.
(Right) Lateral radiograph
again shows the large
ruptured hiatal hernia.
Intrathoracic
pneumoperitoneum
(arrows). Peritoneum
attaches to the esophagus
and is pulled into the chest
with the hernia.

Variant
(Left) Frontal radiograph
shows a farge hiatal hernia
with an air-fluid level
(arrows). Hernias may be
either sliding (common) or
paraesophageal (rare).
(Right) Esophagram shows
paraesophageal type gastric
volvulus. Body and antrum
of the stomach are upside
down (arrows). Obstruction,
when it occurs, is usually at
the efferent duodenum
(open arrow).

Other
(Left) Frontal radiograph
shows a large gastric
volvulus. Much of the
stomach is displaced into the
mediastinum with a large
air-fluid level (arrows).
Smaller air-fluid level also
present inferiorly (open
arrow). (Right) Coronal
CECT shows a large
contrast-filled gastric
volvulus. Fundus (arrows)
below the hemidiaphragm
and antrum (open arrows) in
thorax. Surgical repair is
paramount.
 GOITER, MEDIASTINUM
1
18

Axial graphic shows heterogeneous cystic thyroid lesion Coronal oblique CfCT shows heterogeneous mass with
in anterior mediastinum insinuating itself between punctate calcification (arrow) descending from the left
trachea and left common carotid artery with deviation neck between the trachea and aortic arch with
of trachea to right. deviation of the trachea to the right.

a Superior mediastinum with continuation into the

ITERMINOLOGY neck
Abbreviations and Synonyms a 75% descend anteriorly; arise from isthmus or lower
Substernal goiter gland
Lie anterior to recurrent laryngeal nerve and
Definitions anterolateral to trachea
Primary: Due to migrational anomaly, separate from a Posterior goiters arise from posterolateral aspect of
thyroid gland gland
a Derives blood supply from intrathoracic vessels Descend posterior and to right of great vessels
Secondary: Diffuse or asymmetric enlargement of Size: Variable; may be quite extensive at time of
thyroid gland with greater than 50% of mass diagnosis
descending into thorax Morphology
a Primary blood supply from neck a Smooth or lobular mass
a Commonly calcify (coarse, punctate, or rings)

I IMAGING FINDINGS Radiographic Findings

Radiography
General Features a Tracheal deviation at level of thoracic inlet
Best diagnostic clue Variable tracheal compression
a Tracheal deviation at level of thoracic inlet a Anterior-superior mediastinal mass or
Most common cause of tracheal deviation posterior-superior mediastinal mass; calcification in
Other superior mediastinal masses less likely to 25%
deviate trachea a Lateral radiograph
Location

DDx: Mass at Thoracic Inlet

~:iF~~
,
~ '~-i
Thyroid Carcinoma Lymphoma Metastasis
 GOITER, MEDIASTINUM
Key Facts
1
Terminology Enhances strongly with IV contrast 19
1123 and 1131 diagnostic but often unnecessary
Primary: Due to migrational anomaly, separate from
thyroid gland Top Differential Diagnoses
Secondary: Diffuse or asymmetric enlargement of Thymoma
thyroid gland with greater than 50% of mass Teratoma
descending into thorax Lymphoma
Imaging Findings Pathology
Tracheal deviation at level of thoracic inlet Develop in 5% of people worldwide
Superior mediastinum with continuation into the Up to 20% descend into mediastinum
neck
75% descend anteriorly; arise from isthmus or lower Clinical Issues
gland Usually asymptomatic and mildly hypothyroid or
High attenuation due to natural iodine; 70-120 HU euthyroid
Regions of low attenuation due to colloid cysts Surgery treatment of choice for large goiters,
Course calcifications, calcification in cyst walls in up symptomatic disease or suspected malignancy
to 75%

Anterior goiters fill in retrosternal clear space; Absence of uptake does not necessarily exclude
deviate trachea posterior goiter
Posterior goiters obscure Raider triangle; deviate o Technetium pertechnetate not used due to high
trachea anteriorly blood pool activity in mediastinum

CT Findings Ultrasonographic Findings

NECT Often useful for documenting presence of large
o Sharply demarcated heterogeneous mass cervical goiter
o High attenuation due to natural iodine; 70-120 HU o Substernal component often difficult to visualize
Regions of low attenuation due to colloid cysts Heterogeneous echotexture often with multiple
Course calcifications, calcification in cyst walls in nodules of varying size
up to 75% o Uniformly hypoechoic cysts
o May have large pericardial effusion if hypothyroid o Echogenic foci with posterior shadowing due to
CECT calcification
o Enhances strongly with IV contrast
Imaging Recommendations
o Anterior to trachea (75%)
Left side predominant Best imaging tool
o Posterior to trachea (25%) o Chest radiograph with confirmation by CT as
Right side predominate; esophageal displacement necessary
CT useful for surgical planning
MR Findings o Radionuclide iodine study for symptomatic
TlWI hyperthyroid
o Intermediate signal, slightly higher than muscle Detect autonomous functioning "hot" nodules
o Cyst may have high signal due to hemorrhage or Protocol advice: Extend CT into neck to evaluate
proteinaceous material connection to gland
T2Wl
o Slightly higher signal than surrounding structures
o Variable signal in cysts depending on contents and I DIFFERENTIAL DIAGNOSIS
age of hemorrhage
Overall, goiters have heterogeneous signal with Tl and Anterior
T2 imaging Thymoma
o Less calcification, more caudad in mediastinum, not
Fluoroscopic Findings high attenuation
Esophagram Teratoma
o Extrinsic compression of upper esophagus due to o May have coarse calcification, not of high
posterior goiter attenuation, more caudad in mediastinum, often
o Synchronous movement of mass seen with contains fat
swallowing Lymphoma
o Not calcified prior to treatment, usually multiple
Other Modality Findings nodes
Nuclear medicine findings Castleman disease
o 1123 and 1131 diagnostic but often unnecessary o May enhance intensely, no connection to thyroid
Hot nodule in hyperthyroid patients gland, may be multi focal
 GOITER, MEDIASTINUM
Posterior
20 Bronchogenic cyst
o May be of high attenuation due to milk of calcium,
nonenhancing
Gastrointestinal stromal tumor (GIST) esophagus
o Not of high attenuation, no calcification
I PATHOLOGY
General Features
General path comments
o Early: Diffuse enlargement with cellular hyperplasia
o Late: Follicles form; mixed thyroid enlargement and
regional atrophy and fibrosis
Etiology
o Primary: Remnant of tissue related to abnormal
embryologic migration
o Secondary: Related to hypersecretion of thyroid
stimulating hormone (TSH)
Sporadic goiter: Low T4 production results in
increased TSH leading to glandular enlargement
Ingestion of substance that block formation of
thyroid hormone: Turnip, cabbage, kale
Endemic goiter: Deficiency in iodine intake leads
to low serum T4
Epidemiology
o Develop in 5% of people worldwide
Up to 20% descend into mediastinum
2-21% of patients undergoing thyroidectomy have
substernal component
o Represent up to 7% of mediastinal tumors
o Frequency of large goiters declining
Associated abnormalities
o Pericardial effusion due to severe hypothyroidism
and myxedema
o Tracheomalacia due to compressive effects on
trachea
Gross Pathologic & Surgical Features
Enlarged thyroid, heterogeneous, cystic degeneration,
hemorrhage, calcification
Microscopic Features
Irregularly enlarged follicles with flattened epithelium
and abundant colloid
Microscopic nests of cancer may be present in 5-15%
o Psammomatous calcification: Punctate calcifications
5-100 microns, associated with malignancy
I CLINICAL ISSUES
Presentation
Most common signs/symptoms
o Usually asymptomatic and mildly hypothyroid or
euthyroid
o Incidental discovery on routine chest radiography
Cough and choking sensation
Signs due to tracheal compression
o Dyspnea, wheezing, stridor
o May develop precipitously requiring emergency
airway management
Dysphagia due to esophageal compression
Dysphonia due to compression of recurrent laryngeal
nerve
Pemberton sign: Neck vein distention with upper
extremity elevation due to venous obstruction
Plummer disease: Development of autonomous
functioning nodule leading to hyperthyroidism
Myxedema due to severe hypothyroidism
Thyrotoxicity due to autonomous functioning nodule,
iodide ingestion or radiographic contrast material
Demographics
Age: Increases in frequency with advancing age
Gender: M:F = 1:3
Natural History & Prognosis
Patients with goiters should have thyroid function
tests
Slow growth unless underlying cause is corrected;
usually remain asymptomatic
Treatment
Observation, particularly small lesions and elderly
patients
Surgery treatment of choice for large goiters,
symptomatic disease or suspected malignancy
Thyroid hormone replacement for hypothyroidism;
can be used in euthyroid to suppress TSH with
regression of some goiters
I DIAGNOSTIC CHECKLIST
Consider
Goiter as cause of asymptomatic tracheal deviation
Image Interpretation Pearls
When mediastinal goiter is suspected connection to
cervical thyroid should be confirmed
Radionuclide iodine imaging if planned should
precede CECT
o Iodine load from intravenous contrast may cause
falsely low radioactive iodine uptake
I SELECTED REFERENCES
1. Chin SC et al: Spread of goiters outside the thyroid bed: a
review of 190 cases and an analysis of the incidence of the
various extensions. Arch Otolaryngol Head Neck Surg.
129(11):1198-202,2003
2. Hedayati N et al: The clinical presentation and operative
management of nodular and diffuse substernal thyroid
disease. Am Surg. 68(3):24S-S1; discussion 2S1-2, 2002
3. Jennings A: Evaluation of substernal goiters using
computed tomography and MR imaging. Endocrinol Metab
Clin North Am. 30(2):401-14, ix, 2001
4. BuckleyJA et al: Intrathoracic mediastinal thyroid goiter:
imaging manifestations. AJRAm J Roentgenol.
173(2):471-5, 1999
 GOITER, MEDIASTINUM
IIMAGE GALLERY
1
21

(Left) Frontal radiograph

shows large mass in superior
mediastinum (arrows)
displacing trachea to right
continuing into the neck.
Borders of mass are not
visible above clavicles
confirming anterior location.
(Right) Lateral radiograph
shows large mass descending
in anterior superior
mediastinum (arrows)
displacing trachea posteriorly
and obscuring visualization
of the anterior transverse
aortic arch.

(Left) Axial CECT shows

uniform thyroid enlargement
(arrows) with (ocal
hypodensities characteristic
or early multinodular goiter.
Note also endotracheal tube
(open arrow) for airway
protection. (Right) Axial
CECT shows relatively
homogeneous high
attenuation mass due to
iodine content of thyroid
(arrow) which compresses
and deviates the trachea to
the right at thoracic inlet
(open arrow).

Variant
(Left) Lateral radiograph
shows mass descending
posterior to trachea (arrows)
and displacing trachea
anteriorly. Mass rills in
anterior aspect of Raider
triangle. (Right) Axial CECT
shows heterogeneous mass
descending between trachea
and left common carotid and
subclavian arteries (arrows)
extending posterior to the
trachea with resulting airway
compression.
 HODGKIN LYMPHOMA, MEDIASTINUM
1
22

Graphic shows anterior mediastinal mass with Axial CECT shows rounded, somewhat heterogeneous
displacement of surrounding mediastinal structures. anterior mediastinal soft tissue mass in a patient with
Unusual for lymphoma to obstruct adjacent veins or known HD (arrow). Note the lack of vascularinvasion.
airways.

Predilection for the anterior mediastinum,

ITERMINOLOGY especially the thymus
Abbreviations and Synonyms Size; Variable: Depending on chronicity at time of
Hodgkin disease (HD) presentation
Lymphoma Radiographic Findings
Definitions Radiography
Cancer of the lymphatic system: Systemic disease o Utility to detect initial disease and evaluate
Presence of Reed-Sternberg cells in a background of complications related to treatment with
lymphocytes, macrophages, fibroblasts and chemotherapy and radiation therapy
granulocytes Allow evaluation of complications related to
chemotherapy and radiation therapy
o Most commonly involves anterior, superior
I IMAGING FINDINGS mediastinal nodes
Prevascular and para tracheal lymph nodes are
General Features most commonly affected
Best diagnostic clue: Intrathoracic disease: Mediastinal o Calcifications
adenopathy Nodes rarely calcify prior to treatment
Location Following radiation therapy 20% calcify
o In the thorax, mediastinal involvement is most Two types of calcification: Rim or multiple
common discrete deposits (mulberry)
Nodular sclerosing histologic subtype of HD is the o 65-75% have abnormal chest radiographic findings
most common at presentation

DDx: Anterior Mediastinal Masses

Seminoma Thymic Cyst Thymoma

 HODGKIN LYMPHOMA, MEDIASTINUM
Key Facts
Imaging Findings
Predilection for the anterior mediastinum, especially
the thymus
Nodes rarely calcify prior to treatment
Multiple, rounded soft tissue masses or bulky soft
tissue masses
Best imaging tool: CT the modality of choice for
initial staging and follow-up monitoring of HD
Top Differential Diagnoses
Germ Cell Tumor
Thymoma
Metastases
Thyroid Goiter
Of patients with abnormal radiographic findings,
90% have bilateral asymmetric nodal disease
o Pleural effusions (15%)
o Skeletal lesions
Anterior scalloping of vertebrae can be detected
on lateral radiograph
Sclerotic lesions from osteoblastic metastases
CT Findings
CECT
o Imaging should be performed in all patients
Can depict additional areas of lymphadenopathy
that are not obvious on radiograph
Help in formulating treatment plans and radiation
therapy
o Lymphadenopathy: Due to nodal aggregation
Nodes minimally enhance following contrast
administration
Multiple, rounded soft tissue masses or bulky soft
tissue masses
Necrosis, hemorrhage or cyst formation are rare
o Discrete or infiltrating thymic mass
o Additional findings: Displacement, compression,
and invasion of mediastinal or chest wall structures
o Ill-defined or well-defined nodules: Unilateral or
bilateral
Nodules rarely cavitate (10-20%)
MR Findings
TlWI
o Tumor involvement: Homogeneous masses with low
signal intensity
Signal intensity similar to that of muscle
T2Wl
o High signal intensity equal to or slightly greater
than fat can result from tumoral edema,
inflammation, immature fibrosis or granulomatous
tissue
o Low signal intensity on post-therapeutic T2
weighted images rules out relapse in most patients
o Dense fibrosis may demonstrate low signal intensity
on T2 weighted images
Not the primary modality for use in evaluating HD
1
Pathology 23
Minimal mass effect (non-obstructive) compared to
carcinoma
No definite genetic link has been established
Hodgkin: Reed-Sternberg cell
Clinical Issues
Asymptomatic cervical or supraclavicular
lymphadenopathy
Painless adenopathy - nontender
Bimodal distribution
Peak incidence: 3rd to 8th decade of life
90% cure
Diagnostic Checklist
Intrathoracic HD usually associated with disease
elsewhere
Multiplanar capability useful in assessing chest wall
invasion, pleural, pericardial and brachial plexus
involvement
MRI is more sensitive in detecting bone marrow
involvement associated with lymphoma
Nuclear Medicine Findings
PET
o Uptake of FOG increased in malignant cells
Increased metabolic activity
FOG scintigraphy may be as good as CT for
staging lymphoma
o Used in the detection of relapse
May cause upstaging of disease because of bone
marrow involvement
Ga-67 Scintigraphy
o Helpful in distinguishing residual disease from
post-treatment fibrosis in bulky mediastinal HD
o Post-treatment Ga-67 uptake a poor prognostic
factor
o Sensitivity and specificity of gallium scanning in HD
ranges from 85-97% and from 90-100%, respectively
Imaging Recommendations
Best imaging tool: CT the modality of choice for initial
staging and follow-up monitoring of HD
Protocol advice: CECT when possible
I DIFFERENTIAL DIAGNOSIS
Germ Cell Tumor
Teratoma, seminoma, nonseminoma (embryonal cell,
endoderm ai, choriocarcinoma, mixed germ cell)
Younger patients, tumor usually inhomogeneous
10% of anterior mediastinal tumors
Derived from all 3 cell lines or embryologic cell layers
May contain calcification, fat, fluid and soft tissue
Thymoma
Anterior mediastinal mass
o Slow growing
1/3 calcification, rim or punctate
 HODGKIN LYMPHOMA, MEDIASTINUM
1 1/3 cystic, generally larger tumors
Drop metastasis to pleura
o Asymptomatic cervical or supraclavicular
Iym ph adenopathy
24 Painless adenopathy - non tender
Metastases Other signs/symptoms
History of GU or head and neck tumors o Cough or chest pain may be present as a result of
Usually no calcification mediastinal involvement
Thyroid Goiter o Splenomegaly
o B symptoms (characterized by presence of one of the
Soft tissue higher attenuation due to iodine content
following)
Commonly calcify (coarse, punctate, or rings)
Most common cause of tracheal deviation Unexplained weight loss> 10% baseline during 6
months prior to staging
Recurrent unexplained fever> 38 degrees C
I PATHOLOGY Recurrent night sweats

General Features Demographics

Age
General path comments o Bimodal distribution
o Minimal mass effect (non-obstructive) compared to
Peak incidence: 3rd to 8th decade of life
carcinoma
In industrialized nations, first peak: People aged
Invasion of other organ systems can occur
approximately 20 years; second peak: Patients
Genetics aged 55 years or older
o Clustering of cases within families or racial groups
In developing countries, first peak: Shifted into
has supported the idea of a genetic predisposition or
childhood, usually before adolescence
common environmental link
Gender: M> F = 1.4:1
No definite genetic link has been established
Ethnicity: No ethnic predisposition
Epidemiology
o Several epidemiologic studies have suggested links Natural History & Prognosis
between HD and certain viral illnesses Good
Epstein-Barr virus (EBV) 090% cure
Human herpes virus 6 (HHV6) also under o 2nd tumors: AML, non-Hodgkin
investigation Low grade tumors may evolve to higher grade
o Increased frequency of HD in patients with
congenital or acquired immunodeficiency Treatment
syndromes Optimal treatment depends on histologic subtype and
stage of disease, and age of patient
Gross Pathologic & Surgical Features o Standard treatment of early stage Hodgkin
Nodes usually "soft", tend not to obstruct or displace lymphoma: Brief chemotherapy followed by
mediastinal structures unless large irradiation
Calcification rare prior to treatment Mantle radiation therapy
Microscopic Features Chemotherapy
o Standard chemotherapy of advanced Hodgkin
Hodgkin: Reed-Sternberg cell
lymphoma: ABVD (Adriamycin, bleomycin,
Staging, Grading or Classification Criteria vinblastine, dacarbazine) regimen
Hodgkin: Nodular sclerosis (70%); mixed cellularity Bone marrow transplant (BMT)
(20%); lymphocytic predominant (5%); lymphocytic o Reserved for patients receiving aggressive
depleted (5%) chemotherapy and XRT
Staging
o Stage I: Involvement of a single lymph node region
(I) or localized involvement of a single I DIAGNOSTIC CHECKLIST
extralymphatic organ
o Stage II: Involvement of 2 or more lymph node
Image Interpretation Pearls
regions on the same side of the diaphragm Intrathoracic HD usually associated with disease
elsewhere
o Stage 1Il: Involvement of lymph node regions on
both sides of the diaphragm
o Stage IV: Diffuse/disseminated involvement of 1 or
more extra lymphatic organs with or without I SELECTED REFERENCES
associated lymph node involvement 1. Jeung MYet al: Imaging of cystic masses of the
mediastinum. Radiographies. 22 Spec No:S79-93, 2002
2. Strollo DC et al: Primary mediastinal tumors: Part II.
Tumors of the middle and posterior mediastinum. Chest.
I CLINICAL ISSUES 112:1344-57, 1997
Presentation
Most common signs/symptoms
 HODGKIN LYMPHOMA, MEDIASTINUM

I IMAGE GAllERY 1
25

(Left) Frontal radiograph

shows an anterior
mediastinal mass obscuring
the right heart border
(arrow), HD was
subsequently diagnosed.
(Right) Lateral radiograph
from the same patient shows
lobulated anterior
mediastinal opacities in a
patient with HD (arrows).

(Left) Axial CECT shows soft

tissue anterior mediastinal
mass which surrounds SVC
and vascular structures
without obstruction, lypical
of Hodgkin lymphoma
(arrows). (Right) Frontal
radiograph after multiple
rounds of intensive
chemotherapy shows
resolution of anterior
mediastinal mass which
previously obscured the right
heart border.

(Left) Frontal radiograph

shows rounded anterior
mediastinal mass
representing HD (inferior
margin delineated by arrow).
Note the splaying of the
carina (open arrow). (Right)
Frontal radiograph shows
mulberry calcifications in a
patient with treated HD
(arrows).
 NON-HODGKIN LYMPHOMA, MEDIASTINUM
1
26

Coronal graphic shows normal mediastinum rich in Frontal radiograph shows bulky mediastinal and
lymphoid tissue throughout all compartments. Up to 50 bilateral hilar adenopathy (open arrows) in a patient
normal sized lymph nodes present in the mecNastinum. with NHL. Note the bulky subcarinal/paraesophageal
LAD (curved arrow).

o Single or multiple nodules, airspace opacities,

I TERMI NOLOGY
masses or pleural effusion also seen
Abbreviations and Synonyms Non-Hodgkin lymphoma
Non-Hodgkin lymphoma (NHL) o Intrathoracic involvement in 50% of newly
International prognostic index (IPI) diagnosed cases (vs. 85% in HD)
o 20% present with mediastinal adenopathy
Definitions o Lung (perihilar/juxtamediastinal in location
Heterogeneous group of lymphoproliferative although peripheral and subpleural locations also
malignancies common)
Single or multiple discrete pulmonary nodules less
well-defined and less dense than carcinoma, and
I IMAGING FINDINGS may cavitate (10-20%)
Consolidation with air bronchograms (solitary or
General Features multiple, includes pseudolymphoma)
Best diagnostic clue: Bulky mediastinal bilateral hilar Diffuse reticulonodular opacities (lymphocytic
lymphadenopathy (LAD): Can be asymmetric Interstitial pneumonia)
Location: Superior mediastinal nodes (pre-vascular and Atelectasis due to nodal compression
para-tracheal) are predominantly involved in o Pleura
approximately 75% of patients Pleural effusions seen in 10% of patients at
presentation, due to lymphatic or venous
Radiographic Findings
obstruction
Radiography Most patients have mediastinal adenopathy
o Commonly presents with bulky, bilaterally Effusion, may resolve with irradiation of
asymmetrical, mediastinal-hilar adenopathy mediastinal lymph nodes

DDx: Mediastinal Adenopathy

Metastasis Sarcoidosis Tuberculosis

 NON-HODGKIN LYMPHOMA, MEDIASTINUM
Imaging Findings
Commonly presents with bulky, bilaterally
asymmetrical, mediastinal-hilar adenopathy
Single or multiple nodules, airspace opacities, masses
or pleural effusion also seen
Intrathoracic involvement in 50% of newly
diagnosed cases (vs. 85% in HD)
Calcification rarely seen prior to treatment
Masses from lymphoma more likely to encase and
displace the mediastinal structures rather than
constrict or invade them
Usually mild enhancement post Gd-DTPA
CECT of the chest: Imaging modality of choice in
patients with NHL
Top Differential Diagnoses
Hodgkin Disease (AD)
Pleural masses rare
o Pericardial
Pericardial effusion mostly coexistent with
adenopathy adjacent to pericardial margins
Associated with high grade peripheral T
lymphoma, large B cell lymphoma and
post-transplantation Iymphoproliferative
(PTLD)
o Chest wall
Invasion with rib destruction uncommon
PTLD
o Nodules: Peripheral and basilar, no
air-bronchograms, rarely cavitate
o Focal consolidation or diffuse reticulonodular
opacities
o Hilar and mediastinal adenopathy
CT Findings
NECT
o Enlarged discreet homogeneous soft tissue
attenuation lymph nodes to large conglomerate
masses with lobulated margins
o Calcification rarely seen prior to treatment
CECT
o Slight to moderate uniform enhancement following
IV contrast, marked enhancement unusual (low
attenuation in 20% of cases)
o Masses from lymphoma more likely to encase and
displace the mediastinal structures rather than
constrict or invade them
MR Findings
T1WI: Lymph nodes exhibit homogeneous low signal
intensity (51) - similar to muscle
T2WI: Homogeneous high 51 or areas of low 51
(fibrotic tissue) and high 51 (cystic degeneration or
necrosis)
Usually mild enhancement post Gd-DTPA
Other Modality Findings
Gallium-67 scintigraphy
o Useful in differentiating residual mediastinal
from post treatment fibrosis
Key Facts
1
Metastases 27
Sarcoidosis
Tuberculosis
Pathology
AIDS related lymphoma (ARL): Most cases are
aggressive NHLs of B-cell origin
Absence of Reed-Sternberg cell with clonal
proliferation either of T and B-cell origin
Ann Arbor staging system
Clinical Issues
Gender: More common in males (M:F = 1.4:1)
Ethnicity: Higher frequency in Caucasians
Chemotherapy (most cases), combination of
radiation and chemotherapy (few patients) and bone
marrow transplantation
o Post treatment uptake correlates with viable tumor
(uptake unlikely with necrotic or fibrotic tissue)
PET
o Similar or greater accuracy as CT for initial staging
o Sensitive in detecting NHL at extranodal sites and
post treatment residual tumor
disorders
Imaging Recommendations
Best imaging tool
o CECT of the chest: Imaging modality of choice in
patients with NHL
CT more sensitive for the detection of disease than
conventional radiographs, revealing additional
sites of involvement
I DIFFERENTIAL DIAGNOSIS
Hodgkin Disease (HD)
NHL more advanced disease at presentation
Predilection for the anterior mediastinum, especially
the thymus
Multiple, rounded soft tissue masses or bulky soft
tissue masses
Multiple nodal group involvement more common
than NHL
Metastases
Exthoracic neoplasms: Genitourinary tumors, head
and neck tumors, breast, melanoma
Best diagnostic clue: Variable sized sharply defined
multiple pulmonary nodules
Routes for spread: Hematogenous, lymphangitic,
bronchogenic
Sarcoidosis
Symmetric hilar and mediastinal lymphadenopathy;
without or with pulmonary opacities
May show eggshell calcification
Predilection for posterior (sub)segment upper lobes
and su perior segments lower lobes
disease Progressive massive fibrosis, architectural distortion,
honeycombing, cysts, bullae
 NON-HODGKIN LYMPHOMA, MEDIASTINUM
Tuberculosis
28 Primary TB: LAD asymmetric and ipsilateral with the
consolidation, often have pleural effusion
Rim-enhancing necrotic nodes
I PATHOLOGY
General Features
General path comments: Minimal mass effect (not
obstructive) compared to carcinoma
Etiology
o Unknown
o Association between Epstein Barr virus and African
Burkitt lymphoma
o Association of human lymphotropic virus type-1
(HTLV-1) with peripheral T-cell lymphoma
Epidemiology
o NHLs account for approximately 3% of all newly
diagnosed cancers in US
o 4 times more common than HD
o Ranked third in frequency in childhood cancers
(behind leukemia and CNS lymphoma)
Associated abnormalities
o AIDS related lymphoma (ARL): Most cases are
aggressive NHLs of B-cell origin
Frequently involve extranodal sites
Microscopic Features
Absence of Reed-Sternberg cell with clonal
proliferation either of T and B-cell origin
Staging, Grading or Classification Criteria
Non-Hodgkin (low - intermediate - high grade)
o Small (lymphocytic or non-cleaved cell),
immunoblastic
o Follicular (small cleaved, mixed, or large cell)
o Diffuse (small cleaved, mixed, large cell),
lymphoblastic
Ann Arbor staging system
o Stage I: Involvement of a single lymph node region
(I) or a single extralymphatic organ or site (stage I E)
o Stage II: Involvement of two or more lymph node
regions on the same side of diaphragm (II) or
localized involvement of an extra lymphatic organ or
site (stage II E)
o Stage III: Lymph node involvement on both sides of
diaphragm (Ill) or with localized involvement of an
extralymphatic organ or site (stage III E) or spleen
(stage III S), or both (stage III SE)
o Stage IV: Presence of diffuse or disseminated
involvement of one or more extra lymphatic organs
(e.g, liver, bone marrow, lung), with or without
associated lymph node involvement
o Presence or absence of systemic symptoms noted
with each stage designation (A = asymptomatic, B =
B symptoms)
I CLINICAL ISSUES
Presentation
Most common signs/symptoms: LAD may be
asymptomatic or cause pressure symptoms of cough,
chest pain, dyspnea, dysphagia, hemoptysis or
superior vena cava syndrome (3-8%)
Other signs/symptoms
o B symptoms (40%): Fever, weight loss and night
sweats, more common in aggressive lymphomas
(47%) versus indolent types (25%)
o Fatigue, malaise, pruritus 10%)
o Para neoplastic signs: Pruritus, erythema nodosum,
autoimmune phenomenon, coagulopathy,
hypercalcemia
PTLD
o Incidence 5% solid organ transplants, children more
susceptible
o Greatest incidence among heart (2-3%), lung (12%)
and heart-lung (5-9%) transplant recipients:
Majority of the cases are NHL of B-cell origin
o Peak incidence is 3-4 months after transplant, may
develop as early as 6 days post transplant
Risk Factor
o 40-100 times greater risk of intermediate and high
grade NHL in patients with impaired immune
system
Demographics
Gender: More common in males (M:F = 1.4:1)
Ethnicity: Higher frequency in Caucasians
Natural History & Prognosis
Prognosis depends upon histopathology, age, presence
of extranodal disease and stage at diagnosis
International prognostic index for NHL: Following 5
factors correlate significantly with shorter survival
o Age> 60
o Serum lactate dehydrogenase (LDH) concentration
greater than normal
o ECOG performance status more than or equal to 2
o Ann Arbor clinical stage III or IV
o Number of involved extranodal disease sites> 1
o Low risk - [PI score of zero or one; low intermediate
risk - score of two; high intermediate risk - score of
three; high risk - score of four or five
Low grade tumors may evolve to higher grade
Treatment
Chemotherapy (most cases), combination of radiation
and chemotherapy (few patients) and bone marrow
transplantation
I SElECTED REFERENCES
1. Ansell SM et al: Non-Hodgkin lymphoma: diagnosis and
treatment. Mayo Clin Proc. 80(8):1087-97, 200S
2. Lu P: Staging and classification of lymphoma. Semin Nucl
Med. 35(3): 160-4, 2005
3. Strollo DC et al: Primary mediastinal tumors: Part II.
Tumors of the middle and posterior mediastinum. Chest.
112:1344-57,1997
 NON-HODGKIN LYMPHOMA, MEDIASTINUM

I IMAGE GALLERY 1
29

(Leh) Axial CECT shows
superior mediastinal

,.
adenopathy (open arrow).
Note the mass effect on the
SVC (curved arrow). (Right)

~,.
~ Transverse CECT shows
I" bulky mediastinal

~
adenopathy in the right
paratracheal.
aortopulmonary and
prevascular regions (arrows).
This patient was diagnosed
withNHL.

"-k"'"'- ~
/
.-'""""'1

Typical
(Left) Transverse CECT
shows retrocrural and celiac
axis adenopathy (arrows) in
a patient with NHL. Note the
mass effect exerted on the
descending aorta. (Right)
Axial CECT shows multiple
bilateral if/-defined nodules
and masses (arrows) in a
patient with parenchymal
NHL.

Typical
(Left) Coronal CECT shows
bulky bilateral hilar and
mediastinal adenopathy
- ~ (arrows) in an NHL patient.
(Right) Transverse NECT
shows calcified bilateral hilar
~t .~ .. ', '

". '-"\ ..
lymph nodes (arrows) in a

'<~-
" -- , patient with treated
lymphoma .

,\.,
I ~ . '9 \:0' ~ ,
 THYMOMA
1
30

Thymomas are epithelial neoplasms of the anterior Frontal radiograph shows an eccentric anterior
mediasUnum, which often project to one side. They are mediastinal mass projecting to the left side in this 40
the most common anterior mediasUnal mass, especially year old female. A large encapsulated thymoma was
in patients over 40 years old. found at surgery:

Displaces mediastinal structures posteriorly when

I TERMINOlOGY
Definitions
Thymic epithelial neoplasms with a variable amount
of lymphocytes
Most common primary anterior mediastinal mass,
especially in patients> 40
o Thymomas represent about 20% of all mediastinal
tumors
Thymic carcinoma has significant cellular atypia on
histology and is considered a separate neoplasm
I IMAGING FINDINGS
General Features
Best diagnostic clue: Round or oval anterior
mediastinal mass, which is usually smooth or
lobulated
Location
o Occurs anywhere in anterior mediastinum
Usually projects to one side, but occasionally
alter the mediastinal contour bilaterally
large
Size: Usually between 4-10 em in diameter
Morphology: Most are encapsulated, although 1/3
demonstrate some degree of capsular invasion
Radiographic Findings
Radiography
o Lopsided, oval mass within anterior mediastinum,
which is usually centered over heart
Often best seen on lateral view
o Calcification is usually thin, linear and peripheral
corresponding to calcium deposition in the capsule
and is seen on radiograph in 10% of patients
o Invasive thymoma can present with predominately
unilateral pleural nodules or masses, mimicking
pleural metastatic adenocarcinoma or mesothelioma
Other than pleural involvement, determining an
invasive thymoma on radiograph is not reliable
CT Findings
CECT
may o Oval or lobulated mass within anterior mediastinum

DDx: Thymoma

Hodgkin Lymphoma Germ Cell Tumor

 THYMOMA
Key Facts
Terminology
Thymic epithelial neoplasms with a variable amount
of lymphocytes
Most common primary anterior mediastinal mass,
especially in patients> 40
Imaging Findings
Oval or lobulated mass within anterior mediastinum
Cystic regions and necrosis are common (1/3),
especially with larger tumors and may be a dominate
feature
Always evaluate for indistinctness or obliteration of
surrounding fat planes, mediastinal structures and
chest wall as signs of possible invasion
Homogeneous enhancement is common with
small tumors, heterogeneous enhancement for
larger thymomas
o 1/3 have calcification present on CT, which is often
thin and linear within capsule
Scattered punctate calcification is less commonly
seen
o Cystic regions and necrosis are common (1/3),
especially with larger tumors and may be a
dominate feature
o Always evaluate for indistinctness or obliteration of
surrounding fat planes, mediastinal structures and
chest wall as signs of possible invasion
Pericardial thickening or encasement of vessels are
consistent with an Invasive thymoma
Spread to the pleural (unilateral> > bilateral) and
transdiaphragmatic extension are diagnostic
o Differentiation of thymic tumors by CT is not
reliable
Lobulated borders, indistinct mediastinal fat and
pericardial thickening favor a high risk thymoma
and a concurrent higher rate of recurrence
Smooth borders and an oval/spherical shape
correlates with a low risk thymoma
MR Findings
TIWI: Isointense relative to muscle and low signal in
cystic regions
T2WI: Hyperintense, approaching that of fat along
with higher signal for cystic components
Thymoma
o Invasive tumors may have a multinodular
appearance and often demonstrate more
heterogeneous enhancement
o MRI has no definite advantage over CT, especially
since multiplanar imaging is now commonly
performed
Imaging Recommendations
Best imaging tool
o Thoracic CT scan detects and characterizes anterior
mediastinal masses
1
Pathology 31
Classification is based on predominant cell type:
Epithelial or lymphocytic
Encapsulated and Invasive thymomas are
histologically identical
Diagnosis of Invasive thymoma relies on visualizing
gross or microscopic extension beyond its capsule
Entire capsule needs evaluation and therefore most
thymomas require surgical excision
Since this is not a histological malignancy, the term
malignant thymoma is not considered appropriate
Clinical Issues
Most common thymic abnormality seen with
myasthenia gravis is follicular thymic hyperplasia
(65%), not thymoma
Important to search for any evidence of invasion
through capsule or involvement of nearby
structures
Protocol advice: If a mass is known by radiograph,
unenhanced images through lesion followed by a
routine contrast CT scan may be helpful to evaluate
for cysts, calcifications and measure enhancement
I DIFFERENTIAL DIAGNOSIS
Germ Cell Tumor
Younger patients 40) more common
o Seminoma's tend to be well-defined and
homogeneous
o Non-seminoma tumors often heterogeneous with fat
commonly seen in teratomas
Thyroid Goiter
Often located in superior mediastinum in patients>
40 yo
o Higher attenuation (due to iodine) and often
demonstrates a connection to thyroid gland
Lymphoma
Anterior and middle mediastinal nodal masses, often
quite large
o Involvement of other lymph node groups is
common and enhancement is usually
heterogeneous
Thymic Carcinoma
Histologically; a malignant neoplasm with nuclear
atypia, necrosis and extensive mitotic activity
Similar age of presentation although uncommon to
demonstrate any paraneoplastic syndrome
Lobulated, poorly defined borders with heterogeneous
enhancement and necrosis
o Calcification in 10-40%
Enlarged mediastinal nodes (40%), invasion of great
vessels (40%), and metastasis to lung or liver (30%)
Prognosis of thymic carcinoma is quite poor
 THYMOMA
1 Metastases
Large heterogeneous mass (often> 10 cm), usually an
32
adenocarcinoma
o GU, breast or neck malignancy should be initially
considered, but unknown primary is well-described
Thymic Carcinoid/Neuroendocrine
Neoplasms
Uncommon neoplasms often manifesting as large
masses with lymph node and local mediastinal spread
Paraneoplastic syndromes seen in about 1/3 of
patients with thymic carcinoid, usually Cushing
syndrome
o Some associated with MEN syndromes
Thymolipoma
Rare, large predominantly fat-containing neoplasm
o Soft and mobile, often changes shape or position on
different radiographs
I PATHOLOGY
General Features
General path comments
o Normal gland is not lobulated with a maximal
thickness of < 1.3 cm for patients> 20 yo
Normal fatty involution with age, thus making
thymomas more difficult to image and diagnosis
in younger patients
Convex borders favor a thymoma over persistent
thymic tissue
o Embryology-anatomy
Origin: 3rd & 4th brachial cleft (absence:
DiGeorge syndrome)
Microscopic Features
Classification is based on predominant cell type:
Epithelial or lymphocytic . metastases - 50% 5 yr survival
o An equal amount (biphasic) is most common With
predominant epithelial cells the least
Encapsulated and Invasive thymomas are
histologically identical
o Diagnosis of Invasive thymoma relies on visualizing
gross or microscopic extension beyond its capsule
Entire capsule needs evaluation and therefore
most thymomas require surgical excision
o Since this is not a histological malignancy, the term
malignant thymoma is not considered appropriate
Staging, Grading or Classification Criteria
WHO Staging/grading criteria: 1999
o Low risk thymoma: Types A, AB and B1 - similar
cells of epithelial and/or lymphocytes
o High risk thymoma: Types Bl, B2 and B3 -
polygonal cells, some mild atypia . prognosis? AJRAm J Roentgenol. 183(2):283-9,2004
o Thymic carcinoma: Type C - severe nuclear atypIa
and mitotic figures
I CLINICAL ISSUES
Presentation
Most common signs/symptoms
o 3 common clinical presentations
Asymptomatic, discovered incidentally
25-30% have symptoms related to local
compression or invasion
40% have some para neoplastic syndrome
Other signs/symptoms
o Para neoplastic syndromes
Myasthenia gravis (35%); thymoma in myasthenia
gravis (15%)
Pure red cell aplasia (5%); thymoma in red cell
aplasia (50%)
Hypogammaglobulinemia (10%); thymoma in
hypogammaglobulinemia (5%)
o Most common thymic abnormality seen with
myasthenia gravis is follicular thymic hyperplasia
(65%), not thymoma
Demographics
Age: 70% present in the 5th and 6th decades
Gender: M = F
Natural History & Prognosis
Surgical excision is usually required for all thymomas
except in cases of widespread invasion
o Some myasthenia gravis patients have an
improvement in their symptoms following excision
o Patient's with myasthenia gravis tend to have a
better prognosis, potentially from earlier diagnosis
Thymoma staging criteria
o Stage I: Intact capsule - 93% 5 yr survival
o Stage II: Microscopic capsular invasion - 86% 5 yr
survival
o Stage III: Invades local structures - 70% 5 yr survival
o Stage IV: (a) pleural metastases; (b) distant
Treatment
Surgery is indicated to ensure it is encapsulated and
reduce its recurrence rate
Radiation therapy is somewhat controversial, but is
often recommended for local control of disease
Chemotherapy results are variable for invasive
thymoma
I SElECTED REFERENCES
1. Kim DJet al: Prognostic and clinical relevance of the
World Health Organization schema for the classification of
thymic epithelial tumors: a clinicopathologic study of 108
patients and literature review. Chest. 127(3):755-61,2005
2. Jeong VJet al: Does CT of thymic epithelial tumors enable
us to differentiate histologic subtypes and predict
3. Morgenthaler TI et al: Thymoma. Mayo Clin Proc.
68:1110-23,1993
4. Rosado-de-Christenson ML et al: Thymoma:
Radiologic-pathologic correlation. Radiographies.
12:151-68,1992
 THYMOMA

I IMAGE GALLERY 1
33

(Left) Axial NECT shows a

1.2 em nodule in the region
of the thymic gland (arrow)
in this 60 year old male with
a diagnosis of myasthenia
gravis. At surgery, an
encapsulated thymoma was
removed. (Right) Frontal
radiograph demonstrates a
subtle increased convexity
along the right mediastinum,
near the level of the hilum
(arrow).

(Left) Lateralradiograph
better demonstrates an
anterior mediastinal mass
located in the
mid-retrosternal region
(arrow). Thymoma's may be
best seen on the lateral
projection. (Right) Axial
CECTshowsth~same
patient's smooth oval and
homogeneously enhancing
mass. Surgery found an
encapsulated thymoma.

Variant
(Left) Frontal radiograph
demonstrates multiple
pleural masses surrounding
the left lung. There is
involvement of the
mediastinal pleura,
characteristic for a
malignancy. (Right) Axial
NECT demonstrates an
anterior thymoma with
calcification. The pleural
metastasis are an
uncommon, but
well-described manifestation
of invasive thymoma. This
was biopsy confirmed.
 THYMIC TUMORS OTHER THAN THYMOMA

34

Axial CECT in this 39 yo male with Cushing syndrome Axial CECT 1 year later demonstrates fat plane
and enhancing 50ft tissue mass within the thymus gland. obliteration of the great vessels, direct chest wall
Fine needle biopsy was non-diagnostic. extension and a sternal osteoblastic metastasis (arrow).
Biopsy reveled thymic carcinoid.

I TERMINOlOGY I IMAGING FINDINGS

Definitions General Features
Thymolipoma Best diagnostic clue
o Uncommon benign anterior mediastinal mass, o Thymolipoma: Large, pliable, sharply marginated
which consists of normal thymic tissue and fat fatty mass that changes shape or position with
Thymic cysts decubitus views
o Unilocular or multilocular o Thymic cysts: Single dominant or multiple
Likely originated from remnants of fetal thin-walled cysts within thymic soft tissue
thymopharyngeal duct o Thymic carcinoma: Large irregular mass with
Thymic carcinoma aggressive local spread and invasion into
o Aggressive epithelial carcinoma with typical mediastinal vessels
malignant histological features of cellular atypia, o Thymic neuroendocrine neoplasm: Large anterior
necrosis and numerous mitotic figures mediastinal mass that resembles thymomas on
Well-differentiated subtype falls between the imaging studies
spectrum of thymic carcinoma and thymoma Location: All occur in anterior mediastinum
Thymic neuroendocrine neoplasm
o Uncommon spectrum of thymic tumors with Radiographic Findings
histological and clinical features of neuroendocrine Thymolipoma: Large anterior mediastinal mass that
tumors may mimic an elevated diaphragm or cardiomegaly
Carcinoid is most common subtype o Low density of mass can be seen in about 50%
o Characteristically changes shape or position on
follow-up radiographs or decubitus views

DDx: Thymic Tumors other than Thymoma

Liposarcoma Lymphoma Germ Cell Tumor

 THYMIC TUMORS OTHER THAN THYMOMA
Key Facts
Imaging Findings
Thymolipoma: Large, pliable, sharply marginated
fatty mass that changes shape or position with
decubitus views
Thymic cysts: Single dominant or multiple
thin-walled cysts within thymic soft tissue
Thymic carcinoma: Large irregular mass with
aggressive local spread and invasion into mediastinal
vessels
Thymic neuroendocrine neoplasm: Large anterior
mediastinal mass that resembles thymomas on
imaging studies
Pathology
Thymic carcinoma: Features are typical of epithelial
malignancy with abundant nuclear atypia, necrosis
and mitosis
Thymic cysts: Well-marginated anterior mediastinal
mass, well-marginated which can extend into the neck
o When large, it may simulate cardiomegaly or an
elevated diaphragm
Thymic carcinoma: Lobulated, irregular marginated
large anterior mediastinal mass
Thymic neuroendocrine neoplasm: Large size is
common, averaging around 10-12 cm
CT Findings
Thymolipoma: Fat constitutes 50-85% of the mass
o Nonenhancing soft tissue usually appears as linear
strands interwoven within fat
o Connection to thymus often seen
Thymic cysts: Anterior mediastinal mass with a single
dominant or multiple thin-walled cysts
o Cysts do not enhance and often have water density
measurements
o Occasionally have soft tissue septations, hemorrhage
and/or calcification
o Cysts range in size from microscopic to > 15 cm
Thymic carcinoma: Usually heterogeneous
enhancement with areas of necrosis
o Calcification in 10-40%
o Mediastinal fat plane obscuration, lymph node
enlargement, extension to pericardium and pleura
and extra-thymic metastatic disease often present
o Invasion into great vessels and mediastinal
structures seen in about 40%
Thymic neuroendocrine neoplasm: About 20-30%
have evidence of local mediastinal spread or metastasis
at presentation
o Lobulated or smooth soft tissue mass centered in the
region of the thymus, which may mimic a thymoma
o Metastatic disease to lung, brain, lymph nodes and
pleural most common
o Osseous metastasis often osteoblastic
o Calcification has been reported
MR Findings
Thymolipoma: T1 weighted images demonstrate
characteristic high fat signal
1
Well-differentiated subtype of thymic carcinoma has 35
some cortical differentiation and may represent a less
aggressive variant of thymic neoplasms
This subtype has been referred to as an "atypical
thymoma", denoting its less aggressive clinical course,
yet it has distinctive histological manifestations of a
low grade malignancy
Thymic neuroendocrine neoplasm: Gross and
histological features are much like bronchial
carcinoids, especially atypical form
Clinical Issues
1/3 of thymic carcinoids have a paraneoplastic
syndrome, usually Cushing syndrome
20% are associated with type 1 MEN syndrome
(Wermer syndrome)
Thymic cysts: T1 weighted images demonstrate low
signal and T2 has high signal intensity
Imaging Recommendations
Best imaging tool: CT best imaging for mediastinal
masses
Protocol advice
o If a mediastinal mass known, unenhanced images
through mass followed by a contrast-enhanced scan
through thorax
Help identify calcification, cysts and quantify
enhancement
I DIFFERENTIAL DIAGNOSIS
Thymoma
Most common thymic tumor
Often projects to one side of mediastinum and may
have calcifications and cystic regions when large
Smaller thymomas may mimic neuroendocrine
neoplasms of thymus
lymphoma
Lymphoma of thymus is essentially part of a
generalized disease
o Often involves other intrathoracic nodal regions,
especially middle mediastinum, internal mammary
and pericardiophrenic nodes
o Can be large and heterogeneous with cystic areas
o Hodgkin lymphoma is most common to involve
thymus (40-55%)
Germ Cell Tumor
Seminoma usually homogeneous and often not
contiguous with thymus
Teratomas tend to be heterogeneous with areas of fat,
soft tissue and calcification
Choriocarcinoma, embryonal cell and endodermal
sinus tumors often have areas of necrosis and
hemorrhage
Serum B-HCG or alpha-fetoprotein levels may be
elevated
 THYMIC TUMORS OTHER THAN THYMOMA
1 Thymic Hyperplasia
Increase in thymus size, but maintains a normal
36
architecture and histology
o Follicular lymphoid hyperplasia subtype commonly
associated with myasthenia gravis
liposarcoma
Usually large mediastinal masses with abundant
and enhancing soft tissue
o Does not change shape or position, unlike
thymolipoma
Metastatic Adenocarcinoma
Large bulky nodal mass, which heterogeneously
enhances and has areas of necrosis
o Can mimic thymic carcinoma or lymphoma
Cystic Hygroma/Lymphangioma
Often very young patients &: extension from neck into
mediastinum is common
o An uncommon adult form occurs in lower anterior
mediastinum as a well-marginated cystic mass
Multilocular cysts represent dilated lymphatic spaces
lined by endothelial cells
I PATHOLOGY
General Features
Etiology
o Thymic cysts: Most are congential, likely arising
from thymopharyngeal tissue
Some associated with HIV disease, chemotherapy,
surgery or radiation treatment
Gross Pathologic & Surgical Features
Thymic cysts: Cysts are unilocular or multilocular
soft tissue septations
Microscopic Features
Thymic carcinoma: Features are typical of epithelial
malignancy with abundant nuclear atypia, necrosis
and mitosis
o Various histological patterns, but squamous cell
carcinoma is most common
o Well-differentiated subtype of thymic carcinoma has
some cortical differentiation and may represent a
less aggressive variant of thymic neoplasms
This subtype has been referred to as an "atypical
thymoma", denoting its less aggressive clinical
course, yet it has distinctive histological
manifestations of a low grade malignancy
Thymic neuroendocrine neoplasm: Gross and
histological features are much like bronchial
carcinoids, especially atypical form
o Local invasion and areas of necrosis are common
ICLINICALISSUES
Presentation
Most common signs/symptoms
o Thymolipoma: Usually asymptomatic and benign
o Thymic cysts: Usually asymptomatic, but symptoms
may occur from local mass effect
o Thymic carcinoma: Almost all are symptomatic at
presentation with chest pain, weight loss, fatigue
and night sweats
Paraneoplastic syndromes rare
o Thymic neuroendocrine neoplasm: Most present
with local symptoms from mass effect or
fat
mediastinal/chest wall invasion
Chest pain, SVC syndrome and non-productive
cough
1/3 of thymic carcinoids have a para neoplastic
syndrome, usually Cushing syndrome
20% are associated with type 1 MEN syndrome
(Wermer syndrome)
Inappropriate secretion of ACTH and polymyositis
also seen, but no case of carcinoid syndrome has
been described
Demographics
Age
o Thymic carcinoma: Usually in 5th or 6th decade
o Neuroendocrine neoplasm: Average age 40 years old,
although wide variability
Gender: M:F = 3:1 for neuroendocrine neoplasms
Natural History & Prognosis
Thymic carcinoma: Very poor prognosis with
progressive local growth and distant metastatic disease
common
Neuroendocrine neoplasms: Local invasion, recurrence
and metastatic disease common with 5 year survival
around 30%
o Thymic carcinoid associated with type 1 MEN has a
worse prognosis and is a significant cause of
mortality for this group of patients
with
Treatment
Thymic carcinoma: Prognosis is poor, approximately
30% 5 year survival
o Well-differentiated subtype of thymic carcinoma
significantly better
Neuroendocrine neoplasms: Aggressive surgical
resection of primary, metastatic lesions and recurrent
disease
o Radiation therapy has been helpful for local
recurrence, but chemotherapy role not well known
I SELECTED REFERENCES
1. Kim OJ et al: Prognostic and clinical relevance of the
World Health Organization schema for the classification of
thymic epithelial tumors: a clinicopathologic study of 108
patients and literature review. Chest. 127(3):755-61,2005
2. Kim JH et al: Cystic tumors in the anterior mediastinum.
Radiologic-pathological correlation. J Com put Assist
Tomogr. 27(5):714-23, 2003
3. Jung, K et al: Malignant thymic epithelial tumors:
CT-pathologic correlation. AJR. 176:433-439,2001
4. Mueller et al: Radiologic diagnosis of diseases of the
chesUst ed. Philadelphia, Saunders. 694-702, 2001
 THYMIC TUMORS OTHER THAN THYMOMA

I IMAGE GALLERY 1
37

(Left) Axial CECT in lhis 58

year old male wilh chesl
pain, swelling of his upper
extremities and face. A mass
is present in his anterior and
superior mediastinum with
numerous venous collaterals.
(Right) Axial CECT shows
invasion and occlusion of the
cava (arrow) and portion of
the azygos vein. 8iopsyof
mediastinal portion
demonstrated a
poorly-differentiated thymic
carcinoma.

Typical
(Leh) Axial CECT in this 9
year old boy wilh painless
left neck swelling
demonstrates a
predominantly cystic mass
with some soft tissue
involving the anterior and
superior mediastinum.
(Right) Axial CECT shows lhe
extension into the right
laleral neck from lhe
mediastinum. Presurgical
diagnosis of a lymphangioma
was made, bul palhology
demonstrated multilocular
thymic cysts.

(Left) Frontal radiograph

shows a large mediastinal
mass displacing the hearl
and barium filled esophagus.
A lhymolipoma was found.
(Right) Axial CECT in a 4 yo
with a surgically confirmed
thymolipoma. There is
extensive thymic 50ft tissue
with abundant areas of fat
lhroughout lhe mass.
(Courtesy Kalharine
Hopkins, MD).
 THYMIC REBOUND
1
38

Axial CECT in this 6 year old with leukemia after Axial CECT 3 months after chemotherapy demonstrates
completion of chemotherapy. The thymic gland has thymic rebound hyperplasia. The gland is smooth and
atrophied during treatment. without nodularity. (Courtesy Marilyn Siegel, MO).

o Age> 5 years old

I TERMI NOLOGY Thymus gradually becomes more triangular
Abbreviations and Synonyms Thymic margins slowly straighten
o Age> 15 years old
Thymic hyperplasia
Thymic margins should be straight or concave
Definitions
Radiographic Findings
Thymic enlargement following atrophy from severe
Uncommon to see rebound thymic hyperplasia on the
illness, corticosteroid treatment or chemotherapy
radiograph, except in very young patients
CT Findings
IIMAGING FINDINGS CECT
o CT scan helpful to assess the shape and to apply age
General Features
appropriate measurements ..
Location: Anterior mediastinum/thymic gland
Measure in transverse (short aXIs) or antenor (long
Size axis), although short axis most often used
o Age < 20 yo should not exceed 1.8 cm in maximum
o 90% demonstrate thymic atrophy following
transverse (short axis) thickness
chemotherapy
Age> 20 yo should not exceed 1.3 cm in
Gland grows back over ensuing 2-8 months in
maximum transverse thickness
children and about 9 months in adults
o Exceeding these measurements = thymic hyperplasia
Rebound thymic growth occurs more quickly
Morphology following corticosteroid therapy
o Age < 5 years old . o Thymus grows back> baseline volume
Normal thymus quadrilateral shaped with convex
12-25% exceed baseline thymus by 50%
margins

DDx: Thymic Rebound Hyperplasia

Lymphoma Teratoma Normal Thymus 3 yo

 THYMIC REBOUND
Key Facts
1
Terminology 12-25% exceed baseline thymus by 50% 39
Thymic enlargement following atrophy from severe Chemical-shift imaging may be helpful to distinguish
illness, corticosteroid treatment or chemotherapy hyperplastic thymus (fat tissue) from lymphoma
PET scan occasionally helpful to differentiate thymic
Imaging Findings rebound versus recurrent tumor
Age < 20 yo should not exceed 1.8 cm in maximum Standardized uptake value (SUY) > 4 suggests
transverse (short axis) thickness malignancy
Age> 20 yo should not exceed 1.3 cm in maximum Thymic contour and asymmetry more helpful
transverse thickness
Thymus grows back> baseline volume

MR Findings Gross Pathologic & Surgical Features

Chemical-shift imaging may be helpful to distinguish Generalized thymic gland enlargement
hyperplastic thymus (fat tissue) from lymphoma o Weight is > expected for patients age or > 100 grams
Nuclear Medicine Findings Microscopic Features
PET scan occasionally helpful to differentiate thymic Histologically, thymic gland has large nuclear centers
rebound versus recurrent tumor in lymph follicles, but is otherwise normal
o Benign thymic uptake demonstrates significant
overlap with malignancy
Standardized uptake value (SUV) > 4 suggests ICLINICALISSUES
malignancy
PET scan more helpful in young adults (reduced Demographics
thymic activity) Age: Children or young adults, usually < 25 yo
Thymic contour and asymmetry more helpful Treatment
Asymptomatic (almost universal): No treatment
IDIFFERENTIAL DIAGNOSIS
Recurrent Malignancy I SELECTED REFERENCES
Hematological, germ cell or testicular most common 1. Ferdinand B: Spectrum of thymic uptake at F-FDG PET.
Although suggestive, CT, MRI and nuclear medicine Radiographies. 24:1611-1616, 2004
are not consistently diagnostic 2. Takahashi, K et al: Characterization of the normal thymus
and hyperplastic thymus on chemical-shift MR imaging.
AJR. 180: 1265-1269,2003
3. Fraser R et al: Fraser and Pare's Diagnosis of diseases of the
I PATHOLOGY chest. 4th ed. Philadelphia, WB. Saunders. 2877-2879,
1999
General Features
Etiology: Proposed immunologic rebound
phenomenon involving lymph follicles and plasma
cells accounts for the hyperplasia, following
chemotherapy induced thymic atrophy

I IMAGE GALLERY

(Left) Axial CECT in a 6 year old boy with Burkiulymphoma demonstrates the thymus gland at presentation. (Center) Axial CECT shows an
expected interval decrease in the size and volume of the thymus after 3 cycles of chemotherapy. (Right) Axial NECT 3 months later demonstrates
rebound thymic hyperplasia. It is greater in size than the baseline scan. The gland is smooth, symmetrical and without nodularity.
 GERM CELL TUMORS, MEDIASTINUM
1
40

Graphic demonstrates the typical anterior mediastinal Axial CECT in a 25 year old female with lymphoma
location of U,ese uncommon tumors, which often grow shows a well encapsulated mass with enhancing 50ft
to be quite large. They usually arise near the thymus tissue, (at and calcification. The mass (arrows) was
gland. found to be a mature teratoma at surgery.

Seminoma (10-20%): Most common malignant form

I TERMINOLOGY NSGCT (10-20%): Embryonal cell, endodermal sinus
Abbreviations and Synonyms (yolk sac), choriocarcinoma (trophoblastic) and mixed
Germ cell tumor (GCT) germ cell tumors
o Nonseminomatous germ cell tumor (NSGCT)
Seminomas also known as germinoma
I IMAGING FINDINGS
Definitions
Heterogeneous group of tumors with common General Features
histological features related to the 3 primitive germ Best diagnostic clue: Large mediastinal mass
cell layers from testis or embryonic cell lines Location
o 10% of primary mediastinal tumors o Anterior mediastinum most common, usually near
Classification: Teratoma, seminoma & NSGCT thymus
Teratomas: Most common mediastinal GCT (70%); 3 Mediastinum: Most common extragonadal GCT
manifestations site
o Mature teratoma are well-differentiated benign Size: Variable, but often quite large
tumors representing the vast majority Morphology: Spectrum: Solid, cystic, or necrotic
o Immature teratoma consists of> 10% immature Radiographic Findings
neuroectodermal & mesenchymal tissue
Low potential for malignancy Teratoma
o Lopsided, lobulated, sharply-marginated mass in
o Teratoma with additional malignant components,
anterior mediastinum (85%), middle mediastinum
(TAMC) represent aggressive malignancies
(5%) or multiple compartments (10%)
Malignant teratoma or teratocarcinoma no longer
May be quite large
used
o 25% have calcification

DDx: Germ Cell Tumors

Lymphoma Thymoma Thyroid Goiter

 GERM CELL TUMORS, MEDIASTINUM

Key Facts
1
Terminology Nonseminomatous GCT 41
Mature teratoma are well-differentiated.benign Large, irregular mass with ill-defined margins
tumors representing the vast majority Regions of low attenuation from necrosis and
Seminoma (10-20%): Most common malignant form hemorrhage
NSGCT (10-20%): Embryonal cell, endodermal sinus Pathology
(yolk sac), choriocarcinoma (trophoblastic) and NSGCT often contain other elements, including
mixed germ cell tumors seminomatous tissue
Imaging Findings Clinical Issues
Best diagnostic clue: Large mediastinal mass Elevated serum tumor markers: B-human chorionic
Anterior mediastinum most common, usually near gonadotropin (B-HCG) or alpha fetoprotein (AFP)
thymus 5year survival near 100% for pure seminoma
Teratoma: Mature & immature NSGCT: Poor prognosis, especially with metastasis
Multiloculated cystic mass with variable thickness Elevated serum tumor markers associated with
walls (89%) reduced survival
Seminoma
Lobulated homogeneous mass

Bone or teeth are rare o Pulmonary metastasis, pleural & pericardial

Seminoma effusions common
o Bulky, lobulated, anterior mediastinal mass straddles
midline
MR Findings
May have enlarged hilar and mediastinal lymph T1 WI: Teratoma has hyperintense areas from fat
nodes content
o Calcification rare Imaging Recommendations
Nonseminomatous GCT Best imaging tool: Contrast-enhanced CT
o Large, irregular-shaped, anterior mediastinal mass
Protocol advice
o Pleural effusions & pulmonary metastasis common o Initial images through mass without contrast
CT Findings followed by routine contrast enhanced exam
Teratoma: Mature & immature May help to identify calcification, cysts and
o Multiloculated cystic mass with variable thickness degree of enhancement
walls (89%)
Septa may enhance
o Internal foci of fat (75%) I DIFFERENTIAL DIAGNOSIS
Fat-fluid level characteristic
Thymoma
o Calcification (50%)
o Soft tissue elements common, but amount variable Lopsided, smoothy marginated anterior mediastinal
mass, often in patients> 40 yo
Solid teratoma uncommon, although more often
malignant Associated with paraneoplastic syndromes, especially
o TAMC often large heterogeneous masses with myasthenia gravis
necrosis and hemorrhage Lymphoma
Indistinct margins & mediastinal invasion may be Involvement of other nodal stations common
present o Systemic clinical symptoms usually present
Seminoma Lymphoma rarely calcifies before treatment
o Lobulated homogeneous mass
Midline location, although could project to one Thymic Carcinoid
side Often has clinical & histological features of an atypical
o Mild homogeneous enhancement carcinoid
o Calcification, necrosis and cysts rare May present with para neoplastic syndrome (usually
o Dominant multilocular cysts uncommon, but Cushing) & osteoblastic bone metastases
well-described
May mimic multilocular thymic cysts Thyroid Goiter/Malignancy
o May metastasize to regional lymph nodes or bone Usually connected with thyroid gland
Invasion of adjacent structures uncommon o Often dense from iodine accumulation
Nonseminomatous GCT
o Large, irregular mass with ill-defined margins
o Regions of low attenuation from necrosis and I PATHOLOGY
hemorrhage
General Features
Heterogeneous enhancement
o Obliterates adjacent fat planes Genetics: Familial NSGCT reported
 GERM CELL TUMORS, MEDIASTINUM
1 Etiology: Possible abnormal
during embryogenesis
migration of germ cells o Associated with hematologic malignancies not
related to chemotherapy
42 o 20% Klinefelter syndrome (gynecomastia, testicular
Gross Pathologic & Surgical Features atrophy, increased FSH)
Teratoma: Large encapsulated tumors
o Cysts and solid components common Demographics
Seminoma: Large unencapsulated tumors Age
NSGCT: Large unencapsulated tumors with necrosis o Teratoma: 20-30 year old
o Seminoma and NSGCT: 30-40 year old
Microscopic Features Gender
Mature teratoma o Benign lesions> in females
o One or more germ cell layers with some primitive o Malignant tumors> in males
organ formation Teratoma: M = F
Skin, hair, cartilage, pancreatic tissue and rarely Seminoma and NSGCT: Almost exclusively males
teeth or bone
Immature teratoma Natural History & Prognosis
o Immature elements: Neuroectodermal> Mature teratoma: Excellent prognosis
mesenchymal tissue Immature teratoma: Excellent prognosis, especially in
Teratoma with additional malignant components children
o Contain foci of carcinoma, sarcoma or malignant o Slightly more aggressive in young adults
GCT Teratoma with additional malignant components
o Adenocarcinoma, angiosarcoma or o Aggressive tumors that are poorly responsive to
rhabdomyosarcoma most common chemotherapy
Seminoma Seminoma
o Uniform sheets of round cells admixed with o Syear survival near 100% for pure seminoma
lymphocytes Nonseminomatous elements significantly reduces
Nonseminomatous GCT survival
o Embryonal: Large malignant cells arranged in sheets NSGCT: Poor prognosis, especially with metastasis
o Endodermal: Glandular cords of neoplastic cells o Elevated serum tumor markers associated with
o Choriocarcinoma: Large, round, multinucleated cells reduced survival
(syncytiotrophoblastic); hemorrhage o Primary mediastinal NSGCT have worse prognosis
NSGCT often contain other elements, including than gonadal counterpart
seminomatous tissue Clinical and biological behavior more aggressive,
despite similar histology
Staging, Grading or Classification Criteria
Stage I: Well-circumscribed tumor without invasion of Treatment
local structures Mature and immature teratoma: Surgery
Stage II: Gross or microscopic invasion of local Seminoma: Radiation and/or chemotherapy
structures NSGCT: Chemotherapy and surgery
Stage Ill: Metastatic disease o Persistently elevated tumor serum markers post
o A: Metastasis confined to intrathoracic organs chemotherapy predicts high likelihood of surgical
o B: Extrathoracic metastasis failure

IClINICAL ISSUES I SELECTED REFERENCES

1. Sakurai H et al: Management of primary malignant germ
Presentation cell tumor of the mediastinum. Jpn J Clin Oneal.
Most common signs/symptoms 34(7):386-92, 2004
o Teratoma: Asymptomatic or symptoms from mass 2. Takeda S et a1:Primary germ cell tumors in the
effect mediastinum: a SO-yearexperience at a single Japanese
o Seminoma: Chest pain/pressure and dyspnea institution. Cancer. 97(2):367-76, 2003
o NSGCT: Chest pain/pressure, dyspnea or cough 3. Strollo DC et al: Primary mediastinal malignant germ cell
Elevated serum tumor markers: B-human chorionic neoplasms: imaging features. Chest Surg Clin N Am.
12(4):645-58,2002
gonadotropin (B-HCG) or alpha fetoprotein (AFP) Moran CA: Germ cell tumors of the mediastinum. rathol
4.
Teratoma Res Pract. 195(8):583-7, 1999
o Erosion into bronchus with trichoptysis (hair
expectoration) reported
Seminoma
o 20% are asymptomatic at initial diagnosis
o Occasional elevated B-HCG
Elevated AFP indicates presence of concurrent
NSGCT components
Nonseminomatous GCT
o Elevated AFP often present
 GERM CELL TUMORS, MEDIASTINUM
I IMAGE GALLERY 1
43

(Left) Frontal radiograph in a

27 year old male with chest
pressure and dyspnea. A
large anterior mediastinal
mass, projecting
predominantly towards the
right side is present (Right)
Axial CECT demonstrates a
homogeneous mass
compressing the mediastinal
structures posteriorly. No
necrosis, cysts or
calcification. A mediastinal
seminoma was found on
biopsy.

Variant
(Left) Axial CECT in a 37
year old male with dyspnea.
Large cystic mass with
enhancing septa and
pulmonary metastasis. This is
an uncommon presentation
of mediastinal seminoma
(surgically confirmed).
(Right) Axial CECT in a 20
year old male with an
incidental mediastinal mass
on radiograph. It has both
50ft tissue and definite fat
attenuation (arrow). Mature
teratoma was surgically
confirmed.

Typical
(Left) Frontal radiograph in a
35 year old male with chest
pain and dyspnea. A large
irregular anterior mediastinal
mass is projecting to the right
with concurrent pulmonary
nodules (arrows). (Right)
Axial CECT demonstrates a
large heterogeneous,
necrotic mass. B-HCC was
1,250 (normal < 5). Biopsy
demonstrated a
choriocarcinoma. It did not
respond to chemotherapy.
 LIPOMATOSIS, MEDIASTINUM
1
44

Coronal graphic shows exuberant lipomatous tissue Frontal radiograph shows wide mediastinum with lateral
displacing mediastinal pleura laterally with extension displacement of AP reffection (arrows) and convex low
lateral to transverse aortic arch (arrow). Note lack of attenuation to the right of the trachea (open arrow) due
mass effect on trachea. to excessive mediastinal fat.

o Lipomatous hypertrophy of interatrial septum

I TERMINOlOGY (LHIS)
Definitions o Esophageal lipomatosis: Rare, associated with
Exuberant deposition of unencapsulated adipose tissue exogenous steroid use
in mediastinum Size: Variable, often diffuse throughout mediastinum
a Encapsulated adipose tissue termed lipoma Morphology
Anterior pleural reflection (AP reflection): Superior o Insinuates around normal structures without
mediastinal interface with anterior mediastinal invasion or compromise
structures on PA radiograph o Lipoma: Well-circumscribed, may cause compression
o On left should be medial to transverse aortic arch of adjacent structures
Radiographic Findings
Radiography
IIMAGING FINDINGS o Smooth widening of superior mediastinum with
General Features lateral displacement of AP reflection
o Low attenuation lesion right cardiophrenic angle or
Best diagnostic clue
along left heart border
o Superior: Smooth displacement of AP reflection
o Should be able to visualize normal pulmonary
o Inferior: Low attenuation opacity filling
vessels through opacity
cardiophrenic sulcus
If normal vasculature cannot be seen consider
Location pulmonary origin
o Superior mediastinum most frequent location for
lipomatosis CT Findings
May be associated with extensive extra pleural fat NECT
o Enlarged epicardial and/or pericardial fat pads o Homogeneous fat attenuation -70 to -130 HU

DDx: lipomatous Mass

Liposarcoma Hiatal Hernia with Mesentery Extramedullary Hematopoiesis

 LIPOMATOSIS, MEDIASTINUM

Key Facts
1
Terminology Top Differential Diagnoses 45
Exuberant deposition of unencapsulated adipose Anterior Mediastinal Mass
tissue in mediastinum Liposarcoma
Mediastinal Adenopathy
Imaging Findings
Insinuates around normal structures without Pathology
invasion or compromise Mediastinal lipomatosis associated with development
Smooth widening of superior mediastinum with of hypertension and insulin resistance
lateral displacement of AP reflection
Low attenuation lesion right cardiophrenic angle or
Clinical Issues
along left heart border Usually asymptomatic; detected as incidental finding
Homogeneous fat attenuation -70 to -130 HU on imaging
Presence of contrast-enhancement or enhancing Diagnostic Checklist
septa suggests neoplasm On chest radiograph, lipomatosis should have
Signal dropout with fat suppression on both T1 and straight or slightly concave border, AP reflection
T2 sequences displaced laterally

o Does not compress or invade adjacent structures

o Absence of soft tissue component
Hibernoma
o Focal collection of fat in mediastinum, pericardium Benign tumor of fetal brown fat; rarely intrathoracic
or interatrial septum Heterogeneous with fibrous and lipomatous
LHIS: Dumbbell shape, spares fossa ova lis, does components; may enhance with contrast
not invade adjacent structures Lipoblastoma
CECT Age less than 3, intratumoral soft tissue stranding
o As for NECT; does not enhance with contrast
Presence of contrast-enhancement or enhancing Liposarcoma
septa suggests neoplasm Rare; contains variable amount of soft tissue, when
May see small arteries or veins traversing fat large has mass effect on adjacent structures
MR Findings Mediastinal Adenopathy
T1 WI: Uniform high signal similar to subcutaneous fat Usually produces lobular or convex contours
T2WJ: Uniform high signal similar to subcutaneous fat
T1 C+: May be difficult to distinguish gadolinium Mediastinitis
enhancement from lipomatous tissue without fat Hazy infiltration of fat, discrete fluid collections with
suppression or without air bubbles
T1 C+ FS Most often related to post-operative chest, particularly
o No enhancement should be evident following median sternotomy
gadolinium administration May be associated with sternal osteomyelitis
Gadolinium-enhancement or enhancing septa
Morgagni Hernia
suggests neoplasm
Connection to mesentery, may contain bowel
Signal dropout with fat suppression on both T1 and T2
sequences Pericardial Cyst
Imaging Recommendations Cardiophrenic mass, contains simple or proteinaceous
fluid
Best imaging tool: CT for confirmation of
homogeneous fat attenuation
I PATHOLOGY
I DIFFERENTIAL DIAGNOSIS General Features
Aberrant Vasculature General path comments
o With small specimen can be difficult for pathologist
Persistent left su perior vena cava may deviate AP
to definitively diagnose as benign adipose tissue
reflection laterally
Imaging features need to be correlated with final
Anterior Mediastinal Mass pathologic diagnosis
Teratoma: Contains variable amount of fat, soft tissue Genetics
and calcification o Genetic predisposition to weight gain and obesity
Thymolipoma: Contains fat and linear whorls of soft Environmental factors may be as important as
tissue; usually drapes around heart genetics
o Prader-Willi and Angelman syndrome
 LIPOMATOSIS, MEDIASTINUM
1 Related to deletion, maternal disomy or
imprinting defect on chromosome 15
46 Abnormal chromosome may come from father
(Prader-Willi) or mother (Angelman)
o Familial multiple lipomatosis
Autosomal dominant disorder of adipose
regulation
Etiology
o Obesity
o Exogenous steroids: Prescribed for
immunosuppression, asthma, chronic obstructive
pulmonary disease (COPD)
o Cushing syndrome
Endogenous prod uction of corticosteroids by
adrenal or pituitary functioning tumor
o Ectopic production of adrenocorticotrophic
hormone (ACT H) as paraneoplastic syndrome: Small
cell carcinoma 50% of cases
Other neoplasms: Thymoma, thymic carcinoid,
medullary thyroid carcinoma, islet cell tumor of
pancreas
o Multiple symmetric lipomatosis (Launois-Bensaude
disease, Madelung disease)
Rare; associated with high ethanol intake and
autonomic and somatic neuropathy
o Genetic disease: Prader- Willi, Angelman, familial
multiple lipomatosis
Associated abnormalities
o Excess lipomatous tissue in other anatomic regions
Retroperitoneum, mesentery, pelvis, extremities
o Mediastinal lipomatosis associated with
development of hypertension and insulin resistance
Quantitatively related to height of blood pressure
and degree of insulin resistance
o Stigmata of Cushing syndrome
Centripetal obesity, buffalo hump, facial plethora,
striae
Hirsutism in 80% affected females
o Functional adrenal adenoma or pituitary tumor
Gross Pathologic & Surgical Features
Diffuse adipose tissue without surrounding capsule
Lipoma: Encapsulated mature adipose tissue
LHIS: Unencapsulated adipose tissue in interatrial
septum
Microscopic Features
Mature adipocytes and cellular hyperplasia
o May be difficult to differentiate from low grade
liposarcoma at histology
I CLINICAL ISSUES
Presentation
Most common signs/symptoms
o Usually asymptomatic; detected as incidental
finding on imaging
o May be present in newly diagnosed hypertensives
Other signs/symptoms
o Obstructive sleep apnea due to obesity
o Lipoma: Mass effect may lead to dyspnea, dysphagia,
cough, jugular distention
o LHIS associated with atrial arrhythmias
Demographics
Age
o Obesity onset usually prior to age 45
o Cushing syndrome age 20-40
Gender
o No gender predilection for generalized obesity
o M:F = 1:8 for Cushing syndrome
Natural History & Prognosis
Incidentally detected lipomatosis often has a benign
indolent course
Multiple symmetric lipomatosis associated with
substantial morbidity and mortality
Treatment
None necessary; correction of underlying cause may
cause regression
Resection may be appropriate for symptomatic
lipomas
I DIAGNOSTIC CHECKLIST
Consider
Presence of mediastinal mass accounting for contour
abnormality on radiographs
Mediastinal lipomatosis as cause of mediastinal
widening in patients on steroids
Image Interpretation Pearls
Assess for other clues of excess adipose tissue
On chest radiograph, lipomatosis should have straight
or slightly concave border, AP reflection displaced
laterally
Fat should be homogeneous at CT; soft tissue
component should raise possibility of liposarcoma
I SElECTED REFERENCES
1. Sironi AMet al: Visceral fat in hypertension: influence on
insulin resistance and beta-cell function. Hypertension.
44(2):127-33,2004
2. Enzi G et al: Multiple symmetric lipomatosis: clinical
aspects and outcome in a long-term longitudinal study. Int
JObes Relat Metab Disord. 26(2):253-61, 2002
3. Gaerte SC et al: Fat-containing lesions of the chest.
Radiographics. 22 Spec No:S61-78, 2002
4. Boiselle PM et al: Fat attenuation lesions of the
mediastinum. J Comput Assist Tomogr. 25(6):881-9, 2001
5. BogaertJ et al: Esophageal lipomatosis: another
consequence of the use of steroids. Eur Radiol.
10(9):1390-4, 2000
6. Nguyen KQet al: Mediastinal lipomatosis. South Med).
91(12):1169-72,1998
7. Meaney JF et al: CT appearance of lipomatous hypertrophy
of the interatrial septum. AJRAm J Roentgenol.
168(4):1081-4,1997
8. Homer MJ et al: Mediastinal lipomatosis. CT confirmation
of a normal variant. Radiology. 128(3):657-61, 1978
 LIPOMATOSIS, MEDIASTINUM

I IMAGE GALLERY
1
47

(Left) Axial CECT shows

abundant homogeneous
mediastinal fat that displaces
the AP reflection laterally
(arrows) without mass effect
on superior vena cava or
tracheal carina. (Right) Axial
CECT shows homogeneous
mediastinal fat surrounding
the pericardium without
mass effect on pericardium
or cardiac chambers
(arrows). Note also
increa,sed fat in posterior
mediastinum (open arrow).

Variant
(Left) Axial CECT shows
well-defined collection of fat
situated within inter-atrial
septum (black arrow)
between left atrium and
superior vena cava (open
arrow) and right atrial
appendage (white arrow).
(Right) Axial NECT shows
lipomatous hypertrophy
between left and right atria
with sparing and elongation
of the fossa ova/is (open
arrow).

(Left) Coronal T7WI MR

shows extensive fat (arrows)
on both sides of
mediastinum secondary to
ACTH secreting
adrenocortical carcinoma
(open arrow). (Right) Axial
CECT shows extensive
mediastinal fat lateral to
ascending aorta and main
pulmonary artery (curved
arrows) and anterior to
pericardium as a result of
chronic steroid therapy.
 CASTLEMAN DISEASE, MEDIASTINUM

48

Graphic demonstrates a large hilar lymph node with Axial CTA in an asymptomauc 35 year old male with a
increased vasculature, characteristic for localized Castleman (hyaline vascular) lymph node. Note the
hyaline vascular Castleman disease. mu/Uple large peripheral vessels, which avidly enhance
during the arterial phase (arrows).

ITERMINOlOGY I IMAGING FINDINGS

Abbreviations and Synonyms
Giant cell lymph node hyperplasia
Angiofollicular lymph node hyperplasia
Definitions
Unusual benign lymphoproliferative hyperplasia of
lymph nodes
First described in 1956 as localized mediastinal lymph
node hyperplasia by a pathologist, Benjamin
Castleman
Two classification systems of Castleman disease
o Histological: Hyaline vascular (90%), plasma cell
(9%) and mixed forms (rare)
o Distribution: Localized and multicentric
Localized: Hyaline vascular (90%) and
asymptomatic
Multicentric: Plasma cell (80%) and often
symptomatic
General Features
Best diagnostic clue: Solitary or multiple lymph node
enlargement with avid contrast-enhancement
Location
o 70% occurs in the thorax
10-15% in neck and 10-15% in abdomen,
retroperitoneum and pelvis
o Localized: Lymph node involvement often along
tracheobronchial lymph nodes in mediastinum or
hilum
Atypical thoracic involvement occur in almost 1/3
of patients
Involvement of pleura, axillary, intercostal,
pericardium and lung are reported
Atypical locations still related to the presence of
lym ph tissue
o Multicentric: Extensive intrathoracic lymph node
and pulmonary involvement often occurs
Widespread extra thoracic involvement is common
Size: Variable, often between 2-6 em

DDx: Castleman Disease

Pheochromocytoma Schwannoma Kaposi Sarcoma

 CASTLEMAN DISEASE, MEDIASTINUM
Key Facts
Terminology
Unusual benign Iymphoproliferative hyperplasia of
lymph nodes
Two classification systems of Castleman disease
Histological: Hyaline vascular (90%), plasma cell (9%)
and mixed forms (rare)
Distribution: Localized and multicentric
Localized: Hyaline vascular (90%) and asymptomatic
Multicentric: Plasma cell (BO%) and often
symptomatic
Imaging Findings
Localized form
Smooth or lobulated enlarged node
Avid uniform contrast-enhancement is characteristic,
especially hyaline vascular type
Multicentric form
Radiographic Findings
Localized form
o Solitary smooth or lobulated hilar mass most
common
o Mediastinal involvement usually in middle or
posterior compartments
Rarely seen in anterior mediastinum
Multicentric form
o Enlarged lymph nodes involve multiple mediastinal
compartments
o Pulmonary involvement with ground-glass and
septal thickening may be seen
CT Findings
Localized form
o Smooth or lobulated enlarged node
5-10% demonstrate calcifications
o Avid uniform contrast-enhancement is
characteristic, especially hyaline vascular type
Prominent feeding vessels may be seen
Reflects its hypervascular tissue
o Atypical enhancement patterns well-described
Uniform poor enhancement from necrosis,
fibrosis and degeneration
Peripheral rim-enhancement from extensive
central necrosis
Mass with involvement of surrounding structures
or matted lymphadenopathy confined to a single
mediastinal compartment
o Lung involvement rare
Multicentric form
o Numerous lymph node enlargement of mediastinum
and hilum
Involvement of extra thoracic locations very
common, especially abdomen
Sparing of intrathoracic sites may occur
o Plasma cell type demonstrates less intense
enhancement
o Pulmonary involvement with ground-glass, septal
thickening, centrilobular ill-defined nodules,
scattered cysts and bronchovascular wall thickening
1
Numerous lymph node enlargement of mediastinum 49
and hilum
Pulmonary involvement with ground-glass, septal
thickening, centrilobular ill-defined nodules,
scattered cysts and bronchovascular wall thickening
Pathology
Human herpes virus-B DNA often found in lymph
nodes of multicentric Castleman disease
May occur concurrently with Kaposi sarcoma
Clinical Issues
Localized Castleman disease
Management involves complete surgical excision,
which is diagnostic and therapeutic
Multicentric Castleman disease
Surgical biopsy for diagnosis only
MR Findings
Tl WI: Hypointense compared to fat, but hyperintense
with muscle
T2WI: High signal intensity
Tl C+: Enhancement commonly intense
Imaging Recommendations
Best imaging tool: Contrast-enhanced CT
Protocol advice: Initial unenhanced through mass may
be helpful
I DIFFERENTIAL DIAGNOSIS
lymphoma/leukemia
Major differential with multicentric form
Enlarged lymph nodes usually do not demonstrate
intense enhancement
Clinical symptoms and imaging overlap
o Surgical tissue sampling often required to
differentiate these diseases
Fine needle aspiration or needle biopsy may not
yield enough tissue for confident diagnosis
Kaposi Sarcoma
Overlaps with multicentric Castleman disease
o Both associated with herpes virus-B
Kaposi and Castleman may be concurrently present in
HIV patients
Ill-defined nodules radiating in a bronchovascular
distribution into lung
o Bronchovascular structures commonly thickened
Enlarged lymph nodes may also avidly enhance
Cutaneous involvement common with Kaposi
Mediastinal Neurogenic Tumor
Schwannoma or neurofibroma may resemble localized
mediastinal form
o Enhancement less intense with neurogenic tumors
Central enhancement (target sign) described with
some neurofibromas on MRI
o Distribution along known mediastinal nerves
Posterior mediastinum> middle mediastinum
 CASTLEMAN DISEASE, MEDIASTINUM
1 Pheoch romocytoma/Paraganglioma
50 Uncommon tumor of the mediastinum
Most are asymptomatic
Overlap imaging manifestations, especially as a
solitary well-marginated mass
o Enhancement often intense
Rarely seen in hilum
o Most located in posterior mediastinum
Aortic pulmonary recess, subcarinal or pericardiac
region well-described locations
Angioimmunoblastic lymphadenopathy
Uncommon lymphoproliferative disease/lymphoma
o Often multiple enlarged mediastinal and hilar
lymph nodes with variable lung involvement
o Clinical and imaging mimic of multicentric form
Lymph node biopsy required for diagnosis
lymphocytic Interstitial Pneumonitis (LIP)
Pulmonary manifestation of multicentric disease
o Reflects polyclonallymphoproliferation involving
the lungs
Lymph node enlargement is unusual with LIP
!PATHOLOGY
General Features
Etiology
o Proposed etiologies included chronic inflammatory
state, autoimmune reaction, immunodeficiency or
lymphoid hamartomatous hyperplasia
o Human herpes virus-8 DNA often found in lymph
nodes of multicentric Castleman disease
Kaposi sarcoma also strongly associated with
herpes virus-8
Epidemiology
o HIV patients have an increased risk of multicentric
Castleman disease
May occur concurrently with Kaposi sarcoma
Gross Pathologic & Surgical Features
Hyaline vascular nodes are well-circumscribed by a
thick fibrous capsule
Microscopic Features
Hyaline vascular
o Germinal centers within a large number of mature
lymphocytes
Germinal centers have concentric hyaline sclerosis
and an onion-skin layer of lymphocytes
Prominent interfollicular capillary proliferation
o Areas of necrosis may be-present, especially in nodes
>5cm
Plasma cell
o Sheets of mature plasma cells between hyperplastic
germinal centers
o Variable increase in capillaries
Multicentric form with lung involvement
o Parenchymal findings closely reflect lymphocytic
interstitial pneumonitis
ICLINICAL ISSUES
Presentation
Most common signs/symptoms
o Localized form: Usually discovered incidently on
routine imaging
Occasional local symptoms from mass effect
o Multicentric form
Systemic symptoms such as fever, weight loss and
anemia are common
Other signs/symptoms
o Plasma cell form demonstrates splenomegaly,
hepatomegaly and extensive multiple enlarged
lymph nodes
Polyclonal hypergammaglobulinemia also seen
Demographics
Age
o Adults 20-50 year old
Rare in children
Gender: M = F
Treatment
Localized Castleman disease
o Management involves complete surgical excision,
which is diagnostic and therapeutic
Recurrence rare
o Since most are hyaline vascular, requires a cautious
approach given increased vascularity and propensity
to bleed
Radiation and chemotherapy have had limited success
Multicentric Castleman disease
o Surgical biopsy for diagnosis only
o Progression to lymphoma can occur, especially
plasma blastic lymphoma
Herpes virus-8 has been detected in these
plasmablasts
Patients often have a rapid and fatal outcome,
although splenectomy and chemotherapy may
improve survival
o Chemotherapy with highly active anti-retroviral
therapy (HAART), vinblastine and alpha interferon
Since this form is likely a viral driven disease, the
addition of anti-herpes virus therapy is currently
being examined
I SELECTED REFERENCES
1. Cohen A et al: Kaposi's sarcoma-associated herpesvirus:
clinical, diagnostic, and epidemiological aspects. Crit Rev
Clin Lab ScL 42(2):101-53, 2005
2. Hillier )C et al: Imaging features of multicentric
Castleman's disease in HIV infection. Clin Radial.
59(7):596-601, 2004
3. Ko SF et al: Imaging features of atypical thoracic Castleman
disease. Clin Imaging. 28(4):280-5, 2004
4. Ko SF et al: Imaging spectrum of Castleman's disease. A)R
Am) Roentgenol. 182(3):769-75,2004
5. Waterston A et al: Fifty years of multicentric Castleman's
disease. Acta Oneal. 43(8):698-704, 2004
6. Travis W: Non-neoplastic disorders of the lower respiratory
tract. AFIP, Washington, DC. 282-283, 2002
7. )ohkoh T et al: Intrathoracic multicentric Castleman
disease: CT findings in 12 patients. Radiology.
209(2):477-81, 1998
 CASTLEMAN DISEASE, MEDIASTINUM
I IMAGE GALLERY 1
51

(Left) Frontal radiograph in

this 48 year old female
demonstrates an inddental
well-defined, right inferior
hilar mass. The patient had
symptoms of an upper
respiratory infection. (Right)
Lateral radiograph confirms
the right inferior hilar mass
(arrow). There were no
comparison films. A
noncontrast CT scan was
done (contrast allergy) for
further evaluation.

Typical
(Left) Axial NECT in the 48
year old female shows the
smooth non-calcified right
hilar mass (arrow). Surgical
resection demonstrated a
single large node, diagnosed
as Castleman (hyaline
vascular type). (Right) Axial
CECT in this 32 year old
male with a posterior
mediastinal mass seen
incidentally on radiograph.
The 6 em enhancing mass
was resected and Castleman
disease (hyaline vascular)
was found.

Variant
(Left) Axial CECT in this 56
year old male with fever and
fatigue. Multiple enhancing
axillary (white open arrows)
and mediastinal (black open
arrow) lymph nodes are
seen. Bilateral effusions also
present. (Right) Axial CECT
slightly more caudal in this
patient with multicentric
Castleman disease. These
enhancing axillary and
mediastinal lymph nodes
demonstrated the hyaline
vascular form on biopsy.
 ANGIOIMMUNOBLASTIC LYMPHADENOPATHY
1
52

Graphic demonstrates enlarged mediastinal and hilar Frontal radiograph in an asymptomatic 46 year old male
lymph nodes, common with this disease. Consolidative shows a lobulated mass in the right lower lobe (white
and bilateral reticular opacities have also been arrow), a small right effusion (black arrow) and mild
described. mediastinallhilar lymphadenopathy.

ITERMINOlOGY Radiographic Findings

Enlarged lymph nodes in mediastinum and hilum
Abbreviations and Synonyms
o Pleural effusions relatively common
Angioimmunoblastic lymphadenopathy with
Parenchymal involvement variable
dysproteinemia (AILO)
CT Findings
Definitions
Lymph node involvement often extends to axillary,
Systemic Iymphoproliferation associated with cervical and abdominal regions
immunodeficiency
o Nodes may demonstrate avid enhancement
o Clonal T-cell & B-celllymphoma common, termed
Variable pulmonary involvement
angioimmunoblastic lymphoma
o Consolidation, nodule/masses, reticular opacities
May represent a spectrum of the
Pleural effusions present in 40%
lymphoproliferative process
Important to search for opportunistic infections
Some propose lymphoma represents a more
advanced stage of AILO
I DIFFERENTIALDIAGNOSIS
I IMAGING FINDINGS Lymphoma
General Features AILO - lymphoma: Subtype of non-Hodgkin
Lymphoma staging not applicable to AILO
Best diagnostic clue: Enlarged mediastinal & hilar
o AILO - lymphoma> clinical manifestations and poor
lymph nodes +/- pulmonary involvement
prognosis despite low grade histological appearance
Location: Multisystem involvement
Imaging unlikely to distinguish between them
o Lymph node biopsy required

DDx: Angioimmunoblastic Lymphadenopathy

Lymphocytic Leukemia Tuberculosis Castleman Disease

 ANGIOIMMUNOBLASTIC LYMPHADENOPATHY

Key Facts 1
Terminology Pleural effusions present in 40% 53
Systemic lymphoproliferation associated with Pathology
immunodeficiency
Lymph node and/or bone marrow biopsy required
Clonal T-cell & B-celi lymphoma common, termed
angioimmunoblastic lymphoma Clinical Issues
Imaging Findings Most common signs/symptoms: Fever, fatigue, weight
loss (60%), enlarged nodes (90%), anemia
Best diagnostic clue: Enlarged mediastinal & hilar
4 year survival reduced with> clinical symptoms
lymph nodes +/- pulmonary involvement
Death often from opportunistic infection
Nodes may demonstrate avid enhancement

Castleman Disease IClINICAllSSUES

Lymph node hyperplasia, localized or multicentric
Hyaline form (90%) often localized
Presentation
o Intense nodal enhancement characteristic Most common signs/symptoms: Fever, fatigue, weight
Plasma cell form (9%) often multicentric loss (60%), enlarged nodes (90%), anemia
o Clinical and imaging overlap with AlLD Other signs/symptoms
o Hepatosplenomegaly, skin rash, pruritus, ascites
Tuberculosis (TB) o Induces significant T-cell immunosuppression
Lymphadenopathy, fever and night sweats Pneumocystis jiroveci, cytomegalovirus and
o Lymph nodes often necrotic, unusual for AILD bronchopneumonia common
o Lung biopsy only helpful if acid-fast TB recovered
AILD & TB histology overlap Demographics
Age: Middle to older age; mean age is 60
Gender: Slight male predominance
I PATHOLOGY Natural History & Prognosis
General Features 75% deteriorate regardless of intervention
o Median survival: 24 months
General path comments
4 year survival reduced with> clinical symptoms
o Laboratory & imaging findings not diagnostic
o 85% asymptomatic & 15% > 3 symptoms
o Lymph node and/or bone marrow biopsy required
Death often from opportunistic infection
Extranodal biopsy inconsistent results
Etiology: Abnormal immune response to an antigen, Treatment
viral infection or an allergic drug reaction Chemotherapy used for AILD T-cell lymphoma
(doxycycline, azithromycin & salazosulfapyridine)
Microscopic Features
I SElECTED REFERENCES
Follicular architecture obliterated with polymorphic
plasma cells, immunoblasts & small lymphocytes 1. Noah 5: Angioimmunoblastic lymphadenopathy with
o Generalized postcapiIlary venule enlargement dysproteinemia. E-Medicine.com, 2005
2. Siegert, W: AILD T-cell lymphoma: Prognostic impact of
Evaluation for T-cell lymphoma required
clinical observations and laboratory findings at
o B-celllymphoma & leukemias < common presentation. Annals of Oncology. 6: 659-664, 1995

I IMAGE GALLERY
 HEMANGIOMA - LYMPHANGIOMA, MEDIASTINUM
1
54

Axial graphic shows lymphangioma of the upper left Axial CfCr shows a well marginated, irregularly
middle mediasUnum, deviating the trachea and enhancing mass along the left portion of the anterior
brachiocephalic vessels. mediastinum (arrows).

o Lymphangioma: Large "soft" multicystic mass

I TERMINOLOGY extending from the neck into the mediastinum
Abbreviations and Synonyms Location
o Most often anterior and superior mediastinum
Lymphangioma
Hemangioma o Rarer locations possible: Lung, chest wall, pleural,
Hemolymphangioma cardiac
Lymphangiohemangioma Size: Extremely variable in size, from 2-20 em
Cystic hygroma Morphology
o Lobular smooth masses with small or large vascular
Definitions channels
Congenital malformation of lymphatic and or vascular o Well-circumscribed but unencapsulated
channels Lack of capsule allows tumor to infiltrate into
Mediastinal vascular tumors include hemangiomas, surrounding structures
hemangioendotheliomas, and benign and malignant
hemangiopericytomas
Radiographic Findings
o 10-30% of all vascular tumors malignant Hemangiomas
o Anterior-superior mediastinal mass (70%)
Posterior mediastinum less common (20%)
I IMAGING FINDINGS Can occur anywhere in thorax
o Smooth or lobulated with sharp margins
General Features o Phleboliths in 10%
Best diagnostic clue o Occasional adjacent bone hypertrophy (due to
o Hemangioma: Anterior mediastinal mass with increased vascularity)
phleboliths Lymphangiomas

DDx: Cystic Lesions

l\. 'I
. ,,~... /
." 'i..'-._, .. ~.. - ".:'-'T

Bronchogenic Cyst Metastasis (Testicular) Teratoma

 HEMANGIOMA - LYMPHANGIOMA, MEDIASTINUM

Key Facts 1
Terminology Enhancement centrally very characteristic of 55
Congenital malformation of lymphatic and or hemangiomas
vascular channels Tl weighted images may show linear areas of high
signal intensity from stromal fat
Imaging Findings MR may detect cystic nature that is not evident on
Hemangioma: Anterior mediastinal mass with CT
phleboliths Delayed images necessary to find draining vein,
Lymphangioma: Large "soft" multicystic mass important for surgical planning
extending from the neck into the mediastinum
Most often anterior and superior mediastinum
Top Differential Diagnoses
Lack of capsule allows tumor to infiltrate into Bronchogenic Cyst
surrounding structures Teratoma
May be "soft" tumors without displacement of normal Necrotic Mediastinal Metastases
mediastinal structures Clinical Issues
Lymphatic malformation usually cystic with variable
Because lymphangiomas "softer" usually larger at
sized cysts presentation than hemangiomas

a Anterior-superior mediastinal mass usually Delayed imaging useful to demonstrate draining

connecting with major component in the neck veins for surgical planning
(60%) a Lymphangioma
a Smooth or lobulated with sharp margins Enhancement uncommon
Margins may be indistinct when infiltrative
a May be "soft" tumors without displacement of MR Findings
normal mediastinal structures Hemangiomas
a Calcification rare a T1 weighted images may show linear areas of high
a Occasional adjacent bone erosion signal intensity from stromal fat
a May be associated with chylothorax Lymphangioma
a Rarely can present as solitary pulmonary nodule a MR may detect cystic nature that is not evident on
a Rarely can present as pleural mass CT
MR may be better for detection of macroscopic
CT Findings vascular channels within lesions
NECT
a Hemangioma Other Modality Findings
Often heterogeneous lesions with mixed Fine needle aspiration often non-diagnostic
attenuation a Can lead to significant bleeding in hemangiomas
Phleboliths more commonly demonstrated (30%) a Can produce chylous leaks into pleura with
Phleboliths: Small rounded or ring-like lymphangiomas
calcifications, often with central lucency Imaging Recommendations
a Lymphangioma
Best imaging tool
Lymphatic malformation usually cystic with a CECT to characterize mass in relation to
variable sized cysts surrounding structures
Can appear solid because of high density contents a NECT for phleboliths
(protein or hemorrhage)
Protocol advice
MR better confirms the cystic nature a Ideally with and without IV contrast
Spontaneous hemorrhage will acutely increase the Delayed images necessary to find draining vein,
size of the mass important for surgical planning
a If involution occurs, may lead to change in
appearance
Shrinkage in size I DIFFERENTIAL DIAGNOSIS
Development of dystrophic calcifications
Increased fat within lesion Castleman Disease
CECT Mediastinal nodes that may enhance to a marked
a Hemangioma degree
Heterogeneous enhancement, may be delayed May be mistaken for vascular tumors
Enhancement centrally very characteristic of
hemangiomas Bronchogenic Cyst
Peripheral enhancement less common Usually unilocular subcarinal middle mediastinal mass
Enhancement depends of size of venous channels May contain calcification, especially in wall
(capillary channels enhance less) Enhancement unusual unless cyst infected
 HEMANGIOMA - LYMPHANGIOMA, MEDIASTINUM
1 Teratoma
Capillary lesions in either hemangioma
lymphangioma will appear solid
or

56 Anterior mediastinal mass

Often heterogeneous with fat and calcification Microscopic Features
Endothelial lined vascular channels may include areas
Necrotic Mediastinal Metastases of fibrosis or sclerosis
Testicular or squamous cell tumors Vascular channels can be the size of small veins or
May appear cystic capillaries
Can calcify Cavernous form can have smooth muscle proliferation
Epithelioid Hemangioendothelioma Some show papillary histology
Thyroid transcription factor-1 (TTF-1) positive
Slow growing low grade malignancy of vessel walls
May contain calcifications
May enhance brightly
I CLINICAL ISSUES
Angiosarcoma
Highly malignant
Presentation
Often present with diffuse metastases Most common signs/symptoms
o May be asymptomatic
Neuroblastoma Because lymphangiomas "softer" usually larger at
Occur in pediatric age group presentation than hemangiomas
Posterior mediastinal tumor, may be vascular o Generally present with nonspecific symptoms
Chest pain
o May have history of cervical cystic hygroma
I PATHOLOGY (lymphangioma) in childhood
Even after resection, can recur
General Features May require multiple surgeries to remove
Etiology; Often present at birth Other signs/symptoms
Epidemiology o Dyspnea or cough
o Hemangiomas; 0.5% of all mediastinal tumors o Chylothorax, if lesion develops connection to pleura
o Lymphangiomas; 3% of all mediastinal tumors o Chylopericardium, if lesion develops connection to
Lymphangiomas usually extend from neck mass pericardium
(75%) into mediastinum, 15% arise exclusively in
mediastinum Demographics
Associated abnormalities Age; Most common in children and young adults
o Gorham disappearing bone disease Gender
Resorption of bone adjacent to areas of o Hemangiomas M = F
lymphangioma o Lymphangiomas male predilection
o Hemangiomas may be multifocal: Skin, liver, spleen
and kidneys
Natural History & Prognosis
o Klippel-Trenaunay syndrome Benign lesions, generally produce symptoms through
Mediastinal hemangiomas or lymphangioma, compression
nevus, arteriovenous fistulas, deep venous May grow in size over time
malformations and varicosities (at risk for Rarely invasive but unencapsulated and may infiltrate
pulmonary embolism) into mediastinal structures

Gross Pathologic & Surgical Features Treatment

Lobular, well-marginated (but unencapsulated) mass
Hemangiomas and lymphangiomas have infiltrative
tendencies making surgical removal difficult
Hemangiomas
o Classified according to size of vascular space
Capillary
Cavernous
Venous
o 90% mediastinal hemangiomas are capillary or
cavernous
Lymphangiomas
o Classifies according to size of lymphatic channels
Simple (capillary-sized), most common in skin
Cavernous (dilated lymphatic channels), usually
affects organs in the thorax, abdomen and bone
Cystic (or cystic hygroma), few mm to several cm
in size, most common in the neck
Hemangiomas
o May spontaneously involute
o Steroids can sometimes shrink
o May treat with sclerotherapy if close to a mucosal
surface
o Hemorrhagic risk from needle biopsy (no capsule)
o Surgical resection required for symptomatic lesions
Lymphangiomas
o Surgical resection required for symptomatic lesions
I SELECTED REFERENCES
1. Jeung MY, et al: Imaging of cystic masses of the
mediastinum. Radiographies. 22:579-93, 2002
2. Abe K, et al: Venous hemangioma of the mediastinum. Eur
Radial. 11:73-6,2001
3. Charrau L, et al: Mediastinal lymphangioma in adults: CT
and MR imaging features. Eur Radial. 10:1310-4,2000
 HEMANGIOMA LYMPHANGIOMA, MEDIASTINUM
I IMAGE GALLERY 1
57

(Left) Axial CECT shows a

large primarily cystic mass in
the left hemithorax with little
enhancement. Large
rounded calcifications
(arrows) represent
phleboliths in hemangioma.
(Right) Axial CECT shows an
extremely large cystic mass
that fills the entire left
hemithorax, producing
marked shift of the
mediastinum.

(Left) Frontal radiograph

shows lobular abnormality
along the left heart border
extending into the
cardiophrenic region. (Right)
Axial T7WI MR. The mass
contains mixed signal with a
suggestion of tortuous
vascular channels. The mass
encases the descending aorta
(arrows). Hemangioma.

Variant
(Left) Frontal radiograph
shows atypical appearance
of a recurrent lymphangioma
that invaded from the
aorticopulmonic window
region out into the adjacent
left upper lobe of the lung.
(Right) Axial NECT shows a
slightly spiculated peripheral
right lower lobe mass that
was a lymphangioma on
resection.
 NERVE SHEATH TUMORS, MEDIASTINUM
1
58

Transverse graphic at level of great vessels shows Frontal radiograph shows large we/l-circumscribed right
heterogeneous whorled extraparenchymal neural tumor paraspinal mass (arrows) without evidence of rib
(arrows) arising from intercostal nerve just after exiting destruction. Note visible superior interface of mass with
neural foramen (open arrow). lung (curved arrow) confirming posterior location.

May be centered on neural foramen, extend into

ITERMINOlOGY spinal canal
Abbreviations and Synonyms Involvement of phrenic and vagus nerves less
Neurofibroma, schwannoma, peripheral nerve sheath common
tumor, neurilemoma Primary tracheal neoplasm: Rare
Morphology: Round shape and horizontal axis
Definitions
Radiographic Findings
Neurofibroma: Unencapsulated tumor of nerve sheath
orgin Round or oblong sharply marginated mass extending
Schwannoma: Encapsulated tumor of nerve sheath 1-2 rib interspaces
origin o Often centered at neural foramen, widens neural
Malignant tumor of nerve sheath origin: Spindle cell foramen on lateral view
sarcoma of nerve sheath origin Follows axis of involved nerve
o Horizontal extension along intercostal nerves
Incomplete border due to extra-pleural location
I IMAGING FINDINGS Cervicothoracic sign: Air - soft tissue interface
continues above level of clavicle indicating posterior
General Features location
Best diagnostic clue: Round posterior mediastinal mass Manifestations of neurofibromatosis
with widened neural foramen o Cutaneous nodules: Often multiple,
Location well-circumscribed, complete border
o May occur along any peripheral nerve o Skeletal manifestations
Intercostal nerve most common; extend along Well marginated rib erosions due to plexiform
undersurface of rib neurofibromas

DDx: Paraspinal Mass

Paraspinal Abscess Hematoma

 NERVE SHEATH TUMORS, MEDIASTINUM
Key Facts 1
Imaging Findings Neurenteric Cyst 59
Best diagnostic clue: Round posterior mediastinal Paraspinal Abscess
mass with widened neural foramen Pathology
Follows axis of involved nerve
90% of all posterior mediastinal masses neurogenic
Variable enhancement (homogeneous, origin
heterogeneous) with IV contrast
30% neurofibromas associated with von
Local invasion, osseous destruction, and pleural Recklinghausen disease (neurofibromatosis 1)
effusion are features of malignant degeneration Schwannoma: Antoni A (highly cellular) tissue or
Variable signal intensity often isointense to spinal Antoni B (loose myxoid) tissue
cord Neurofibroma: Myelinated and unmyelinated axons,
Intermediate to high T2 signal intensity collagen, reticulin
Protocol advice: Gadolinium helpful for delineating
intradural extent Clinical Issues
Symptoms variable related to mass effect or nerve
Top Differential Diagnoses
entrapment
Sympathetic Ganglion Tumor
Lateral Meningocele

Rib deformity due to associated osseous dysplasia o Tumors may be obscured by high signal intensity of
Short segment, acute angle scoliosis cerebrospinal fluid
Posterior scalloping of vertebral bodies due to o Neurofibroma: May have low central signal due to
dural ectasia collagen deposition
o Pulmonary manifestations Tl C+ FS
Thin walled upper lobe bullae associated with o Enhancement pattern mimics that of CECT
bilateral symmetric basal predominant fibrosis o Neurofibromas may have target appearance
(rare)
Pulmonary nodules from metastasis following
Imaging Recommendations
malignant degeneration of neural tumor Best imaging tool: MR best to assess intraspinal and
extra-dural extension and spinal cord involvement
CT Findings Protocol advice: Gadolinium helpful for delineating
NECT intradural extent
o Dumbbell extension into spinal canal (10%)
o Decreased attenuation at CT due to lipid or cystic
degeneration I DIFFERENTIAL DIAGNOSIS
Calcification in 10% of schwannomas
CECT
Sympathetic Ganglion Tumor
o Decreased attenuation due to lipid, cystic Oval shape and vertical axis; extends 3-5 interspaces
degeneration More often calcified
o Variable enhancement (homogeneous,
Paraganglioma
heterogeneous) with IV contrast
Paraganglioma strongly enhance with contrast
Neurofibroma more commonly homogeneously
enhance Esophageal Duplication Cyst
Neurofibroma may have early central contrast Esophageal duplication cyst more anterior, lower
blush attenuation, fluid characteristics at MRI
Heterogeneity related to regions of cellular and
acellular (myxoid) components Lateral Meningocele
Cellular regions are high attenuation with Fluid attenuation; contiguous with thecal sac
contrast May coexist with neurofibroma in patients with
o Local invasion, osseous destruction, and pleural neurofibromatosis
effusion are features of malignant degeneration
Low attenuation regions due to hemorrhage and Neurenteric Cyst
hyaline degeneration Rare; fluid attenuation
Associated with congenital vertebral body anomalies
MR Findings
Paraspinal Abscess
TlWI
o Variable signal intensity often isointense to spinal Centered on disc rather than neural foramen; may be
cord circumferen tial
o Neurofibromas may have central high signal Paraspinal Hematoma
T2WI Occurs following trauma; associated with spinal
o Intermediate to high T2 signal intensity
fractures
 NERVE SHEATH TUMORS, MEDIASTINUM
I PATHOLOGY
60 General Features
General path comments
o 90% of all posterior mediastinal masses neurogenic
origin
40% of these nerve sheath tumors
3:1 schwannomas to neurofibromas
Genetics
o 30% neurofibromas associated with von
Recklinghausen disease (neurofibromatosis 1)
Deletion on chromosome 17
o Neurofibromatosis 2, chromosome 22q deletion
Epidemiology
o Most common cause of posterior mediastinal mass
o 90% of neurofibromas are solitary
o Solitary neurofibromas and schwannomas rarely
undergo malignant degeneration
o Neurofibromatosis: Malignant degeneration in
approximately 4%
o Neurofibromatosis 1: Prevalence 1 in 3,000
Multiple neurogenic tumors or single plexiform
neurofibroma
Other tumors: Pheochromocytoma, chronic
myelogenous leukemia
o Neurofibromatosis 2: Prevalence 1 in 1,000,000
Gross Pathologic & Surgical Features
Schwannomas
o Encapsulated nerve sheath tumors; grow
eccentrically and compress nerve
o Often undergo cystic degeneration and hemorrhage
Neurofibroma
o Nonencapsulated disorganized proliferation of all
nerve elements, centrally positioned in nerve
o Cystic degeneration and hypocellularity uncommon
o Plexiform neurofibroma involve nerve trunks or
plexuses
Malignant tumor of nerve sheath origin (MTNSO)
o May arise de novo or within preexisting plexiform
neurofibroma
o Rare to arise in preexisting schwan noma
Microscopic Features
Schwannoma: Antoni A (highly cellular) tissue or
Antoni B (loose myxoid) tissue
o Distribution of Antoni A and B tissues responsible
for imaging heterogeneity
Neurofibroma: Myelinated and unmyelinated axons,
collagen, reticulin
MTNSO: Highly cellular with pleomorphic spindle
cells
ICLINICAL ISSUES
Presentation
Most common signs/symptoms: Often asymptomatic
Other signs/symptoms
o Symptoms variable related to mass effect or nerve
entrapment
o Development of pain should raise suspicion of
malignant degeneration
Higher incidence of malignant degeneration in
neurofibromatosis 1
Demographics
Age
o Schwan noma: Average 5th decade of life
o Neurofibroma: Usually 2nd-4th decade of life
Gender: M = F
Natural History & Prognosis
Indolent slow growth
o Recurrence rare following surgical resection
5 year survival malignant lesions 35%
Treatment
Surgical removal for symptomatic or malignant lesions
Radiation not indicated, may induce malignant
degeneration
I DIAGNOSTIC CHECKLIST
Consider
MTNSO when signs of locally aggressive behavior
MTNSO when patient develops new symptom of pain
related to known nerve sheath tumor
I SELECTED REFERENCES
1. Spitzer AL et ai: Anatomic classification system for surgical
management of paraspinal tumors. Arch Surg.
139(3):262-9,2004
2. Takeda S et al: intrathoracic neurogenic tumors--SO years'
experience in a Japanese institution. Eur J Cardiothorac
Surg. 26(4):807-12, 2004
3. Cardona S et al: Evaluation of F18-deoxyglucose positron
emission tomography (FDG-PET) to assess the nature of
neurogenic tumours. Eur J Surg Oncol. 29(6):S36-41, 2003
4. Erasmus JJ et ai: MR imaging of mediastinal masses. Magn
Reson Imaging Clin N Am. 8(1):S9-89, 2000
5. Reeder LB: Neurogenic tumors of the mediastinum. Semin
Thorac Cardiovasc Surg. 12(4):261-7,2000
6. Lee JY et al: Spectrum of neurogenic tumors in the thorax:
CT and pathologic findings. J Com put Assist Tomogr.
23(3):399-406, 1999
7. Marchevsky AM: Mediastinal tumors of peripheral nervous
system origin. Semin Diagn Pathol. 16(1):65-78, 1999
8. Rossi SE et al: Thoracic manifestations of
neurofibromatosis-I. AJR Am J Roentgenol. 173(6):1631-8,
1999
9. Strollo DC et al: Primary mediastinal tumors: part II.
Tumors of the middle and posterior mediastinum. Chest.
112(5):1344-57,1997
10. Sakai F et al: Intrathoracic neurogenic tumors:
MR-pathoiogic correlation. AJR Am J Roentgenol.
159(2):279-83,1992
11. Levine E et al: Malignant nerve-sheath neoplasms in
neurofibromatosis: distinction from benign tumors by
using imaging techniques. AJR Am J Roentgenol.
149(5):1059-64,1987
 NERVE SHEATH TUMORS, MEDIASTINUM
I IMAGE GALLERY 1
61
Typical
(Left) Axial NECT shows
relatively homogeneous low
attenuation right paraspinal
mass (arrows) without rib or
vertebral body invasion
producing mass effect on the
right main bronchus (curved
arrow). Resected specimen
revealed schwannoma.
(Right) Axial CECT shows
homogeneous chest wall
mass (arrow) arising
between anterior ribs
without OSSUS invasion.
Found to be schwannoma of
intercostal nerve at surgery.

Typical
(Left) Coronal T7 C+ FS MR
shows right apical
eXlrapleural mass (arrows)
with peripheral
contrast-enhancement
surrounding a relatively
acellular non-enhancing
central area. Confirmed as
schwannoma at surgery.
(Right) Axial T7 C+ FS MR
shows homogeneously
enhancing neurofibroma
(arrows) that widens
ipsilateral neural foramen
(curved arrow) with
extradural component
producing mass effect on the
spinal cord (open arrow).

Variant
(Left) Frontal radiograph
shows multiple
well-circumscribed
cutaneous nodules (white
arrows) in patient with
neurofibromatosis ,.
Irregular left upper lobe
nodule (black arrow) is
metastasis from malignant
degeneration of plexiform
neurofibroma. (Right) Frontal
radiograph shows symmetric
apical and paraspinal
extrapleural masses (open
arrows) and subtle erosion of
posterior right 4th rib
(curved arrow) due to
plexiform neurofibromas.
 SYMPATHETIC GANGLION TUMORS, MEDIASTINUM

62

Graphic shows large left posterior mediastinal mass Axial T1 C+ F5MR shows large relativelyhomogeneous
(open arrow) originating along sympathetic chain enhancing right paraspinal neuroblastoma (arrows)
(arrows) extending above the thoracic inlet and extending into but not widening the ipsilateralneural
compressing adjacent lung. foramen (open arrow).

I TERMINOlOGY Right vagus nerve descends lateral to trachea;

divides to form esophageal plexus and posterior
Definitions vagal trunk
Neuroblastoma: Malignant neoplasm of neural crest Left vagus nerve descends between left common
cells carotid and subclavian arteries then lateral to
o Age related: Neuroblastoma < 3, aortic arch; divides to form esophageal plexus
ganglioneuroblastoma 3-10, ganglioneuroma> 10 then continues as anterior vagal trunk
Paraganglioma (extraadrenal pheochromocytoma) Size: Variable; may be localized to one level or span
arise from sympathetic ganglia entire thorax
Morphology
o Well-circumscribed or lobular mass
I IMAGING FINDINGS o Oval shape and vertical axis spanning 3 to 5
vertebra
General Features
Radiographic Findings
Best diagnostic clue: Elongated, vertical posterior
mediastinal mass Oval mass extending over several rib interspaces
Location Axis follows sympathetic chain
o Sympathetic chains run vertical along necks of the o Erodes or spreads ribs; may invade vertebral bodies
ribs CT Findings
Covered by parietal pleura except for distal right Neuroblastomas heterogeneous due to hemorrhage,
chain cystic degeneration and necrosis
o Paragangliomas may be located along sympathetic o Malignancy correlates with degree of heterogeneity
chain, vagus nerve or within the heart o Variable enhancement with IV contrast

DDx: Posterior Mediastinal Mass

Neurofibroma Metastatic Disease Discitis

 SYMPATHETIC GANGLION TUMORS, MEDIASTINUM

Key Facts 1
Terminology Top Differential Diagnoses 63
Age related: Neuroblastoma < 3, Nerve Sheath Tumor
ganglioneuroblastoma 3-10, ganglioneuroma> 10 Lateral Meningocele
Metastasis
Imaging Findings
Best diagnostic clue: Elongated, vertical posterior Pathology
mediastinal mass Neuroblastoma: Derived from neural crest cells that
Neuroblastomas heterogeneous due to hemorrhage, form sympathetic nervous system
cystic degeneration and necrosis Paraganglioma: Neuroendocrine tumor of chromaffin
Approximately 85% neuroblastoma have cell origin
calcifications at CT
Paragangliomas strongly and uniformly enhance with Clinical Issues
contrast Neuroblastoma may have paraneoplastic syndrome
MR useful to evaluate intraspinal extension Neuroblastoma may mature to a
Up to 30% neuroblastoma are not MIBG avid ganglioneuroblastoma, then ganglioneuroma
Neuroblastoma primary in the thorax associated with
better prognosis than other sites

o Approximately 85% neuroblastoma have

calcifications at CT
I DIFFERENTIAL DIAGNOSIS
Ganglioneuroblastoma and ganglioneuroma often Nerve Sheath Tumor
homogeneous Neurofibromas may be multiple
Paragangliomas strongly and uniformly enhance with Nerve sheath tumors in older individuals
contrast Horizontal axis, round, centered on neural foramen
MR Findings Esophageal Duplication Cyst
MR useful to evaluate intraspinal extension Esophageal duplication cyst more anterior, lower
Neuroblastoma heterogeneous signal with all attenuation, fluid characteristics at MRI or CT
sequences
o May have regions of increased Tl signal due to Extramedullary Hematopoiesis
hemorrhage or T2 signal due to cystic degeneration Associated with chronic hemolytic disorders
Ganglioneuroblastoma and ganglioneuroma: May be bilateral
Homogeneous intermediate Tl and T2 signal
o Enhance homogeneously with gadolinium Lateral Meningocele
o Ganglioneuroma may have whorled appearance Fluid attenuation; contiguous with thecal sac
with TlWl Metastasis
Paraganglioma strongly enhance with gadolinium
Primary tumor usually evident; often arise within
o Relatively high Tl signal with signal flow voids
vertebral body or pedicle
o High T2 signal particularly with fat suppression
Nuclear Medicine Findings Neurenteric Cyst
Rare; fluid attenuation
PET Associated with congenital vertebral body anomalies
o FOG-PET moderate sensitivity for localizing
paragangliomas Paraspinal Abscess
o Potential new PET agents: 18F-fluorodopamine Usually localized to one disc space, may be
(FDA) and 18F-f1uorohydroxyphenylalanine circumferential
(F-DOPA)
MlBG Scintigraphy Paraspinal Hematoma
o Uptake dependent on catecholamine production Associated with vertebral body fractures and other
o May be useful for following extent of disease trauma
o Up to 30% neuroblastoma are not MlBG avid
o Decreased sensitivity for ganglioneuroma
Imaging Recommendations
I PATHOLOGY
Best imaging tool General Features
o CECT or MR generally sufficient for confirming General path comments: Spectrum from malignancy
location and documenting extent of disease to benign ganglion cells
May need NECT to document presence of Genetics
calcification o Paragangliomas may be associated with familial
Protocol advice: Full staging of neuroblastoma requires syndromes
CT or MR of abdomen and pelvis
 SYMPATHETIC GANGLION TUMORS, MEDIASTINUM
1 Multiple endocrine neoplasias, von Hippel-Lindau
most common
Horner syndrome from mediastinal tumor
o Ptosis, pupillary constriction, ipsilateral facial
64 Etiology anhidrosis, and flushing
o Neuroblastoma: Derived from neural crest cells that Neuroblastoma may have para neoplastic syndrome
form sympathetic nervous system o Vasoactive intestinal peptide (VIP) induced watery
o Paraganglioma: Neuroendocrine tumor of diarrhea, achlorhydria, hypokalemia
chromaffin cell origin o Opsomyoclonus-myoclonus
Epidemiology Paraganglioma
o 20% of neuroblastoma arise in posterior o Blushing, headaches, anxiety and chest pain with
mediastinum catecholamine secretion
o Paraganglioma extremely rare 0.5% mediastinal o Elevated hematocrit due to vasoconstriction and
tumors) volume contraction
10% of extra-adrenal paragangliomas arise in chest
Demographics
Gross Pathologic & Surgical Features Age
Neuroblastoma o Neuroblastoma: Children < 3 years
o Nonencapsulated o Ganglioneuroblastoma: Children < 10 years
o Heterogeneous: Hemorrhage, necrosis, cystic o Ganglioneuroma: Adolescents and young adults
degeneration
Ganglioneuroblastoma Natural History & Prognosis
o More homogeneous in between neuroblastoma and Neuroblastoma may mature to a
ganglioneuroma ganglioneuroblastoma, then ganglioneuroma
Ganglioneuroma o Ganglioneuroma considered benign
o Encapsulated; homogeneous tissue Neuroblastoma primary in the thorax associated with
Paraganglioma better prognosis than other sites
o Highly vascular 90% survival for stage 1 disease decreases to 10% for
o May produce catecholamines stage 4 disease
o 4S: Survival near 100%
Microscopic Features
Neuroblastoma: Small round blue cells arranged in Treatment
sheets Surgical resection
o Shimada classification to separate into favorable and Adjuvant chemotherapy and radiation therapy for
unfavorable histologies advanced disease
Ganglioneuroblastoma: Admixture of neuroblastoma
and ganglioneuroma
Ganglioneuroma: Clustered mature ganglion cells I DIAGNOSTIC CHECKLIST
Paraganglioma: Vascular spaces mixed with
amine-precursor uptake and decarboxylation (APUD)
Consider
cells Neuroblastoma in child with para neoplastic syndrome

Staging, Grading or Classification Criteria Image Interpretation Pearls

Staging: Neuroblastoma (Evans anatomic staging) Calcification useful to distinguish neuroblastoma from
o Stage 1: Confined to organ of interest lymphoma
o Stage 2: Extend beyond organ; does not cross
midline
o Stage3: Extension across midline I SELECTED REFERENCES
o Stage 4: Distant metastases 1. Ilias I et al: New functional imaging modalities for
4S: Age < 1 year, metastatic disease confined to chromaffin tumors, neuroblastomas and ganglioneuromas.
skin, liver, and bone marrow Trends Endocrinol Metab. 16(2):66-72,2005
2. Sahdev A et al: CT and MR imaging of unusual locations of
extra-adrenal paragangliomas (pheochromocytomas). Eur
Radiol. 15(1):85-92, 2005
ICLINICAl ISSUES 3. Lonergan GJ et al: Neuroblastoma, ganglioneuroblastoma,
and ganglioneuroma: radiologic-pathologic correlation.
Presentation Radiographies. 22(4):911-34, 2002
Most common signs/symptoms 4. Aquino SL et al: Nerves of the thorax: atlas of normal and
o Neuroblastoma: Painless abdominal mass pathologic findings. Radiographies. 21(5):1275-81, 2001
o Ganglioneuroblastoma and ganglioneuroma: May be S. Erasmus JJ et al: MR imaging of mediastinal masses. Magn
asymptomatic and detected as incidental mass on Reson Imaging Clin N Am. 8(1):59-89, 2000
chest radiograph 6. Reeder LB: Neurogenic tumors of the mediastinum. Semin
Thorac Cardiovasc Surg. 12(4):261-7,2000
o Paraganglioma: Hypertension due to circulating
7. Lee JY et al: Spectrum of neurogenic tumors in the thorax:
catecholamines CT and pathologic findings. J Com put Assist Tomogr.
Less often hormonally active than adrenal 23(3):399-406, 1999
paragangliomas
Malaise, weight loss
 SYMPATHETIC GANGLION TUMORS, MEDIASTINUM
I IMAGE GALLERY 1
65

(Leh) Frontal radiograph

shows large right posterior
mediastinal mass (arrows)
extending along the expected
course of the sympathetic
chain. Biopsy revealed
neuroblastoma. (Right) Axial
CECT in same patient shows
homogeneously enhancing
right paraspinal mass
(arrows) with tiny punctate
calcifications (open arrows)
and mass effect on trachea
and esophagus (curved
arrows).

(Leh) Sagittal T7 WI MR
shows elongated
heterogeneous paraspinal
mass with area of cystic
degeneration (curved arrow)
and punctate signal voids
due to calcification (arrows).
Canglioneuroblastoma.
(Right) Coronal T2WI FS MR
shows homogeneous, slightly
elongated left paraspinal
mass (arrows) without
calciFication or cystic
degeneration.
Ganglioneuroma confirmed
at histology.

Variant
(Left) Axial T7WI MR shows
homogeneous mediastinal
mass compressing the
superior vena cava and
insinuating between
ascending and descending
aorta (open arrows). Note
prominent flow void (arrow).
Paraganglioma. (Right)
Sagittal T7WI MR in same
patient shows homogeneous
mass due to paraganglioma
arising inferior to transverse
aortic arch (arrow) that
compresses but does not
invade superior vena cava
(curved arrow).
 EXTRAMEDULLARY HEMATOPOIESIS, MEDIASTINUM

66

Coronal scoul shows bilateral/ower thoracic paraspinal Axial CECT shows mild variable contrast-enhancemenl
masses (arrows)and an enlarged spleen (curved arrow) of the paraspinal extramedullary hematopoiesis
in patient with thalassemia intermedia and suspected (arrows). No bone erosion.
extramedullary hematopoiesis.

o Sharply demarcated, lobulated, centered on vertebral

ITERMINOlOGY bodies
Definitions o No vertebral body erosion
Compensatory mechanism for chronic anemias due to o No calcification with mass
bone marrow dysfunction o Very slow growth
Ribs
o Marrow expansion with widening of the ribs, most
IIMAGING FINDINGS marked at the vertebral end
o Prominent trabeculae
General Features o Ribs may be normal
Best diagnostic clue: Multiple lobulated posterior Subpleural para costal masses
mediastinal masses with vertebral bodies which have o Separate or contiguous with paraspinal mass
prominent trabeculae o No rib erosion
Location: Paras pinal region caudal to the 6th thoracic Associated hematopoiesis: Hepatosplenomegaly may
vertebra also be found
Size: Microscopic to masses> 5 cm in diameter o Spleen, however, will be small in sickle cell disease
Complications from hemorrhage
Radiographic Findings o Pleural effusion, may be massive
Posterior mediastinal mass
o Unilateral or bilateral CT Findings
o Located anywhere along spine, most common NECT
caudal to the 6th thoracic vertebra o Usually contain fat
o May extend the entire length of spine o Calcification absent
o No bone erosion

DDx: Enhancing Posterior Mediastinal Masses

Neuroblastoma Paraganglioma Esophageal Varices

 EXTRAMEDULLARY HEMATOPOIESIS, MEDIASTINUM

Key Facts 1
Terminology Top Differential Diagnoses 67
Compensatory mechanism for chronic anemias due Nerve Sheath Tumor
to bone marrow dysfunction Paragangliomas
Esophageal Varices
Imaging Findings
Sympathetic Ganglion Tumors
Best diagnostic clue: Multiple lobulated posterior Lymphoma
mediastinal masses with vertebral bodies which have Pleural Metastases
prominent trabeculae Mesothelioma
Location: Paraspinal region caudal to the 6th thoracic Lateral Meningocele
vertebra
May extend the entire length of spine Clinical Issues
Usually contain fat May rarely cause cord compression either due to
Calcification absent extension of paravertebral masses or intraspinal
No bone erosion hematopoiesis
CECT: Will enhance with contrast administration, Options, risks, complications: Transthoracic needle
often inhomogeneous biopsy risk of hemorrhage from tumor

o Size 5 mm to > 5 cm
o Very slow growth Esophageal Varices
o Most common location along costovertebral Multiple small vessels enhance, no marrow expansion
junction Liver small, (may be enlarged from hematopoiesis)
o May extend into spinal canal Sympathetic Ganglion Tumors
CECT: Will enhance with contrast administration,
Oval shape and vertical axis spanning 3 to 5 vertebra,
often inhomogeneous
similar to hematopoiesis
MR Findings Malignant tumors usually calcified
Fat signal on T1 and T2 weighted imaging May have paraneoplastic syndromes
Useful for epidural evaluation in those with symptoms Age related: Neuroblastoma < 3, ganglioneuroblastoma
of spinal cord com pression 3-10, ganglioneuroma> 10 years of age
Heterogeneous content at CT due to hemorrhage,
Other Modality Findings cystic degeneration, and necrosis
Nuclear medicine findings Erode bone
o Uptake Tc-99m sulfur colloid
o Radionuclide scans may be normal Lymphoma
Usually multiple lymph node groups throughout the
Imaging Recommendations mediastinal and retroperitoneum
Best imaging tool Faster growth than extramedullary hematopoiesis
o CT in patient with appropriate history usually
sufficient for diagnosis Pleural Metastases
o MRI procedure of choice for evaluation of spinal History of primary tumor, commonly from
cord compression adenocarcinomas
Bone destruction of ribs or vertebral bodies
Mesothelioma
I DIFFERENTIAL DIAGNOSIS Encases the entire hemithorax, not restricted to the
Nerve Sheath Tumor paravertebral region
Extent usually less than 4 vertebral bodies, different Calcified asbestos plaques may be present
from the long mass of extramedullary hematopiesis Lateral Meningocele
Horizontal axis if elliptical, round, centered on neural Unilateral
foramen Widen neural foramen, scoliosis common
Pressure erosion on adjacent vertebral body More common in neurofibromatosis
No marrow expansion Fluid density, no fat
Usually unilateral, may be bilateral in
neurofibromatosis
Paragangliomas I PATHOLOGY
Intense enhancement with contrast, extramedullary General Features
hematopoiesis has mottled enhancement
General path comments: Benign marrow elements
No marrow expansion outside the marrow in patients with severe anemia
Unilateral
Etiology
 EXTRAMEDULLARY HEMATOPOIESIS, MEDIASTINUM
1 o Proposed mechanisms
Extrusion of marrow through vertebral cortical Treatment
Options, risks, complications: Transthoracic needle
68 defects
Growth of heterotopic or multipotential stem cells biopsy risk of hemorrhage from tumor
Embolic phenomena from other areas of Treatment directed at underlying anemia
hematopoiesis Spinal cord compression
Epidemiology o Responds to small doses of radiation
o Most common in patients who are transfusion Radiation in large doses will suppress marrow,
independent worsening the stimulus for extramedullary
Compensatory bone marrow mechanisms hematopoiesis
adequate, in those requiring transfusions no o Blood transfusion: Decrease the stimulus for
stimulus for hematopoiesis extramedullary hematopoiesis
o Most common anemias o Hydroxyurea therapy for temporary bone marrow
Thalassemia intermedia or major suppression of hematopoietic tissue
Congenital spherocytosis o Surgical decompression less common but may be
Congenital hemolytic anemia required for severe neurologic deterioration
Sickle cell anemia High incidence of recurrence after surgical
o Less common causes resection
Myelofibrosis Removal of extramedullary hematopoietic masses
Lymphoma and leukemia may lead to anemic crisis
Gaucher disease
Paget disease
Rickets I DIAGNOSTIC CHECKLIST
Hyperparathyroidism
Consider
Pernicious anemia
Associated abnormalities Extramedullary hematopoiesis in chronically anemic
o Other sites of hematopoiesis patient with bilateral posterior mediastinal mass
Liver, spleen, lymph nodes
Retroperitoneum
Kidney
I SELECTED REFERENCES
Adrenal gland 1. Ghosh AKet al: Primary extramedullary hematopoiesis
Breasts manifesting as massive bilateral chylothorax. Ann Thorac
Thymus 5urg. 80(4):1515-7, 2005
Prostate
2. Castelli Ret al: Intrathoracic masses due to extramedullary
hematopoiesis. Am J Med 5ci. 328(5):299-303, 2004
Spinal cord 3. Koch CA et al: Nonhepatosplenic extramedullary
Pericardium hematopoiesis: associated diseases, pathology, clinical
Intracranial dura matter course, and treatment. Mayo Clin Proc. 78(10):1223-33,
2003
Gross Pathologic & Surgical Features 4. Xiros N et al: Massive hemothorax due to intrathoracic
Lobulated masses of hematopoietic marrow extramedullary hematopoiesis in a patient with hereditary
spherocytosis. Ann Hematol. 80(1):38-40, 2001
5. Kwak H5 et al: CT findings of extramedullary
I CLINICAL ISSUES hematopoiesis in the thorax, liver and kidneys, in a patient
with idiopathic myelofibrosis. J Korean Med 5ci.
Presentation 15(4):460-2,2000
Most common signs/symptoms 6. Moran CA et al: Extramedullary hematopoiesis presenting
as posterior mediastinal mass: a study of four cases. Mod
o Asymptomatic Pathol. 8(3):249-5I, 1995
No treatment required 7. De Klippel Net al: Progressive paraparesis due to thoracic
o Development of symptoms depends on duration of extramedullary hematopoiesis in myelofibrosis. Case
disease report. J Neurosurg. 79(1):125-7, 1993
o May rarely cause cord compression either due to 8. Martin J et al: Fatty transformation of thoracic
extension of paravertebral masses or intraspinal extramedullary hematopoiesis following splenectomy: CT
hematopoiesis features. J Comput Assist Tomogr. 14(3):477-8, 1990
9. Papavasiliou C et al: The marrow heterotopia in
Demographics thalassemia. EurJ Radiol. 6:92-6, 1986
Age: Clinical presentation most frequent during the 10. Long JA et al: Computed tomographic studies of thoracic
third and fourth decades extramedullary hematopoiesis. J Com put AssistTomogr.
4(1):67-70,1980
Ethnicity 11. Mulder H et al: Extramedullary hematopoiesis in the
o Thalassemia most common in Mediterranean posterior mediastinum. Radiol Clin (Basel).44(6):550-6,
countries 1975
o Sickle cell disease in African-Americans 12. Korsten J et al: Extramedullary hematopoiesis in patients
with thalassemia anemia. Radiology. 95:257-63, 1970
Natural History & Prognosis
Related to anemia
 EXTRAMEDULLARY HEMATOPOIESIS, MEDIASTINUM
IIMAGE GALLERY 1
69
Typical
(Leh) Axial NECT shows the
largest mass contains areas
of low attenuation (arrow)
with Hounsfjeld numbers
consistent with fat.
Extramedullary
hematopoiesis often contains
fat and is a helpful
distinguishing characteristic.
(Right) Axial CECT shows in
a 25 year old woman with
sickle cell disease. Right
paraspinal oval soft tissue
mass is homogeneous and
enhances slighlly with
contrast (arrow). No bone
erosion. Extramedullary
hematopoiesis.

(Left) Axial CECT shows

bilateral paraspinal masses in
the lower thoracic spine, the
largest faintly enhances with
contrast (arrows). No
calcification or bone erosion.
Thalassemia inlermedia and
extramedullary
hematopoiesis. (Right) Axial
CECT at bone windows
shows prominent trabecula
within the vertebral body
(arrow). Ribs are normal.

(Left) Axial CECT at bone

windows. Oval-shaped
bilateral paraspinal masses in
upper thoracic spine.
Generalized marrow
expansion with widening of
ribs (arrows). Thalassemia
intermedia and
extramedullary
hematopoiesis. (Right) Axial
NECT through upper
abdomen in same patient.
Generalized splenomegaly
(arrows). Accessory sites
include liver, spleen,
retroperitoneal lymph nodes
and even thymus.
 VARICES, MEDIASTINUM
1
70

Graphic shows varices within the wall of the esophagus Axial CECT shows multiple dilated, enhancing
(arrow) as well as paraesophageal varices surrounding paraesophageal varices to right of esophagus (arrows),
the lower 1/3 of the esophagus (open arrows). filling in the azygoesophageal recess and extending
anterior and posterior to the esophagus.

o Serpiginous enhancing vessels adjacent to or in

ITERMINOlOGY esophageal wall
Definitions Location: Most commonly around lower one-third of
Abnormal dilation of veins within the mediastinum esophagus
o Esophageal varices: Collateral vessels within the wall Radiographic Findings
of the esophagus Radiography
Prone to hemorrhage o Lateral displacement or inferior obliteration of
o Paraesophageal varices: Collateral vessels in azygoesophageal recess
mediastinum adjacent to esophagus o Visible in approximately 50% with known varices
May coexist with esophageal varices; connect via
perforating veins CT Findings
o Downhill varices: Dilated veins in the upper NECT
mediastinum related to obstruction of superior vena o Asymmetric apparent thickening or esophageal wall
cava May be confused with hiatal. hernia
Azygoesophageal recess: Posterior reflection of right o Dilated tubular soft tissue structures surrounding
lung with mediastinum, inferior to azygos arch esophagus
CECT
o Dilated, contrast filled vessels adjacent to or in
I IMAGING FINDINGS esophageal wall
May be unopacified on arterial phase imaging
General Features o Increased number or tortuosity of mediastinal veins
Best diagnostic clue Dilated azygos, hem i-azygos, bronchial or
o Lobular contour abnormality in lower mediastinal veins
azygoesophageal recess

DDx: Lower Azygoesophageal Recess Abnormality

Hiatal Hernia Lipomatosis Gastrointestinal Stromal Tumor

 VARICES, MEDIASTINUM

Key Facts 1
Terminology Top Differential Diagnoses 71
Esophageal varices: Collateral vessels within the wall Hiatal Hernia
of the esophagus Esophageal Carcinoma
Paraesophageal varices: Collateral vessels in Mediastinal Adenopathy
mediastinum adjacent to esophagus
Downhill varices: Dilated veins in the upper Pathology
mediastinum related to obstruction of superior vena Uphill varices due to portal hypertension
cava Cirrhosis due to alcoholic liver disease most frequent
cause in US
Imaging Findings Cirrhosis due to hepatitis Band C most common in
Dilated, contrast filled vessels adjacent to or in developing nations
esophageal wall
Well-defined and serpiginous flow voids on T1 Clinical Issues
imaging Hemorrhage occurs in up to 1/3 of patients with
Best imaging tool: CECT best radiographic technique esophageal varices
for detection Mortality for bleeding episode approximately 30%

o Accessory drainage via thoracoepigastric vein,

internal mammary vein, pericardiophrenic vein Esophageal Carcinoma
Irregular or asymmetric wall thickening, stricture,
Angiographic Findings luminal obstruction
Downhill varices: Multiple small collateral vessels in
the thorax with upper extremity venography Gastrointestinal Stromal Tumor
Uphill varices: May be detected during transjugular Submucosal lobular mass
intrahepatic portosystemic shunt placement Mediastinal Adenopathy
o Associated with splenic and gastric varices
Oblong or triangular shape; may have fatty hilum
MR Findings
Mediastinal Mass
T1WI Often large without tubular morphology; may have
o Well-defined and serpiginous flow voids on T1
fat, calcium or necrosis
imaging
Signal voids may be absent if flow is diminished
T2WI: Similar to T1 imaging
I PATHOLOGY
MRV
o Can use gradient echo, phase contrast or time of General Features
flight technique Etiology
Best visualized on portal venous phase o Uphill varices due to portal hypertension
Fluoroscopic Findings Reversal of flow diverts blood through left gastric
vein to esophageal venous plexus
Esophagram
o Tortuous, longitudinal filling defects projecting into Late com plication; hepatic venous pressure
lumen gradient above 12 mm Hg
Detection may be enhanced by Valsalva maneuver o Uphill varices may be pre-sinusoidal, sinusoidal or
and TrendeIenburg position post-sinusoidal
o Scalloped border of barium filled esophagus Pre-sinusoidal causes occur at level of portal vein;
e.g., portal vein thrombosis
Ultrasonographic Findings Sinusoidal causes occur within hepatic
Hypoechoic or anechoic tubular structures at parenchyma; e.g., Cirrhosis
endoscopic ultrasonography Post-sinusoidal causes occur at level of hepatic
o Color Doppler useful to confirm flow veins; e.g., Budd-Chiari syndrome
o Downhill varices due to superior vena cava
Imaging Recommendations obstruction
Best imaging tool: CECT best radiographic technique May be due to malignancy, central venous
for detection catheter, mediastinal fibrosis or mediastinal mass
Protocol advice: Image in portal venous phase Epidemiology
o Cirrhosis due to alcoholic liver disease most
frequent cause in US
I DIFFERENTIAL DIAGNOSIS 50% lifetime risk for development of varices
o Cirrhosis due to hepatitis Band C most common in
Hiatal Hernia developing nations
Confirmed by presence of gas fluid level
 VARICES, MEDIASTINUM
1 o Periportal fibrosis due to schistosomiasis
japonicum important cause worldwide
mansoni or
I DIAGNOSTIC CHECKLIST
72 Associated abnormalities Consider
o Cirrhosis, splenomegaly, recanalized umbilical vein, Endoscopy as first line test for detection of esophageal
internal hemorrhoids varices
o Spontaneous spleno-renal shunt
Image Interpretation Pearls
Staging, Grading or Classification Criteria
Do not overdistend esophagus during esophagram
Endoscopic grading Continuous swallows may collapse varices during
o Grade 1: Small straight varices peristalsis
o Grade 2: Tortuous varices involving less than 1/3 of
lumen
o Grade 3: Large varices occupying greater than 1/3 of I SELECTED REFERENCES
lumen
1. TesdallK et al: Transjugular intrahepatic portosystemic
shunts: adjunctive embolotherapy of gastroesophageal
collateral vessels in the prevention of variceal rebleeding.
IClINICAL ISSUES Radiology. 236(1):360-7, 2005
2. Bhasin DK et al: Variceal bleeding and portal hypertension:
Presentation new lights on old horizon. Endoscopy. 36(2):120-9, 2004
Most common signs/symptoms 3. De Franchis R: Incidental esophageal varices.
o Asymptomatic until disease is advanced Gastroenterology. 126(7):1860-7,2004
o Presence often heralded by hematemesis 4. Lo GH et al: The characteristics and the prognosis for
Other signs/symptoms patients presenting with actively bleeding esophageal
o Signs of cirrhosis varices at endoscopy. Gastrointest Endosc. 60(5):714-20,
2004
Ascites
5. Tamano M et al: Evaluation of esophageal varices using
Abnormal liver function tests and low albumin
contrast-enhanced coded harmonic ultrasonography. J
Gynecomastia, testicular atrophy, and spider Gastroenterol Hepatol. 19(5):572-5,2004
angiomas due to inability to metabolize estrogens 6. Matsuo M et al: Esophageal varices: diagnosis with
o Splenomegaly due to portal hypertension gadolinium-enhanced MR imaging of the liver for patients
Thrombocytopenia due to platelet sequestration with chronic liver damage. AJR Am J Roentgenol.
o Jaundice, coagulopathy and encephalopathy due to 180(2):461-6,2003
liver failure 7. Miller Let al: Risk of esophageal variceal bleeding based on
o Facial or upper extremity edema due to venous endoscopic ultrasound evaluation of the sum of esophageal
variceal cross-sectional surface area. Am J Gastroenterol.
obstruction in superior mediastinum
98(2):454-9,2003
o Arm claudication (rare) due to venous insufficiency 8. Irisawa A et al: Collateral vessels around the esophageal
wall in patients with portal hypertension: comparison of
Demographics EU5 imaging and microscopic findings at autopsy.
Age: Varies depending on underlying etiology Gastrointest Endosc. 56(2):249-53, 2002
9. Cihangiroglu M et al: Collateral pathways in superior vena
Natural History & Prognosis caval obstruction as seen on CT. J Com put Assist Tomogr.
Hemorrhage occurs in up to 1/3 of patients with 25(1):1-8,2001
esophageal varices 10. Lawler LP et al: Pericardial varices: depiction on
o Risk of bleeding highest in first year following three-dimensional CT angiography. AJR Am J Roentgenol.
diagnosis 177(1):202-4,2001
o Risk increases significantly with increasing portal 11. Chalasani Net al: Predictors of large esophageal varices in
venous pressure and grade of varices patients with cirrhosis. Am J Gastroenterol.
94(11):3285-91,1999
Red wheals: Endoscopic finding of dilated
12. 5himizu T et al: Esophageal varices before and after
intra-epithelial veins under tension endoscopic variceal ligation: evaluation using helical CT.
o Mortality for bleeding episode approximately 30% Eur Radiol. 9(8): 1546-9, 1999
Downhill varices: Prognosis based on underlying cause 13. Lee 5J et al: Computed radiography of the chest in patients
o Poor prognosis when due to obstructing neoplasm with paraesophageal varices: diagnostic accuracy and
o Bleeding rare with downhill varices characteristic findings. AJR Am J Roentgenol.
170(6):1527-31,1998
Treatment 14. Cho KC et al: Varices in portal hypertension: evaluation
Pharmacologic management to prevent bleeding with CT. Radiographics. 15(3):609-22, 1995
o Beta-blockers, nitrates 15. Ishikawa T et al: Detection of paraesophageal varices by
plain films. AJR AmJ Roentgenol. 144(4):701-4, 1985
Correction of underlying cause
16. Hirose Jet al: "Downhill" esophageal varices demonstrated
Transjugular intrahepatic porto systemic shunt by dynamic computed tomography. J Com put Assist
o Can embolize varices in same setting Tomogr. 8(5):1007-9, 1984
Sclerotherapy for bleeding
Variceal ligation
 VARICES, MEDIASTINUM
[IMAGE GALLERY 1
73
Typical
(Left) Frontal radiograph
shows subtle con lOur
abnormality of the lower
azygoesophageal recess
(arrows) without evidence of
gas-fluid level. Confirmed as
paraesophageal varices at
CECT. (Right) Axial CECT
shows downhill mediastinal
varices (curved arrow) and
dilated azygos vein (open
arrow) with reversal of flow
due to catheter related
stricture of superior vena
cava (arrow).

(Left) Axial CECT prior to

portal venous phase shows
multiple nonenhancing
serpiginous paraesophageal
varices to the left of the
esophagus and anterior to
the aorta (arrows). (Right)
Axial CECT during portal
venous phase shows dilated
enhancing varices within the
wall of the esophagus
(arrow) resulting in smooth
asymmetric thickness of the
esophageal wall.

Typical
(Left) Axial TI C+ FS MR
shows smooth asymmetric
thickening of lower
esophagus with serpiginous
enhancing esophageal
varices (arrow). (Right)
Anteroposterior DSA
following transjugular
intrahepatic portosyslemic
shunt (open arrows) shows
extensive tangle of varices
ascending from lesser
curvature of stomach along
the esophagus (arrows).
 Congenital
Right Aortic Arch 11-2-2
Aberrant Subclavian 11-2-6
Aortic Coarctation 11-2-10
Intralobar Sequestration 11-2-14
Left Superior Vena Cava 11-2-18
Azygos Continuation of IVC 11-2-22
Azygos Fissure 11-2-26

Inflammatory - Degenerative
Aortic Atherosclerosis 11-2-28
Marfan Syndrome 11-2-32
Aortic Dissection 11-2-36
Aortic Aneurysm 11-2-40
Takayasu Disease 11-2-44
SVC Obstruction 11-2-48
 RIGHT AORTIC ARCH

2
2

Axial CECT shows a right aortic arch (open arrow) and Axial CECT shows right aortic arch (open arrow) with a
the retroesophagealleft subclavian artery (arrow). large retroesophageal vessel (arrow) representing a
diverticulum of Kommerell.

ITERMINOlOGY IIMAGING FINDINGS

Abbreviations and Synonyms General Features
Right arch Best diagnostic clue: Right paratracheal density on
radiography with leftward tracheal deviation
Definitions Location: Right paratracheal region
Right aortic arch due to partial regression of left fourth Size: Usually 2.5-4 cm
aortic arch - two major types Morphology: Soft tissue density often convex in the
Right aortic arch with mirror imaging branching right paratracheal region enhances on CT
usually associated with congenital heart disease
o Left arch interruption is posterior to the left Radiographic Findings
subclavian artery Radiography
Right aortic arch with aberrant left subclavian artery: o Right aortic arch
Minimal increased incidence congenital heart disease Right paratracheal density
o Left arch interruption is between carotid and Right paraspinal shadow representing the
subclavian arteries descending aorta
o Potential vascular ring formed with a left ductus Absence of normal left aortic arch
arteriosus Absence of normal descending aortic shadow
o Sometimes associated with diverticulum of Leftward tracheal deviation
Kommerell and dysphagia Retroesophageal density on lateral view common
Other right arch variants occur including in adults
retroesophageal innominate artery and right arch with Leftward displacement of barium column on
isolated left subclavian artery often arising from a esophagram
ductus arteriosus

DDx: Double Arch

Double Arch CT Double Arch MRI Double Arch MRI

 RIGHT AORTIC ARCH
Key Facts
Terminology
Right aortic arch due to partial regression of left
fourth aortic arch - two major types
Right aortic arch with mirror imaging branching
usually associated with congenital heart disease
Right aortic arch with aberrant left subclavian artery:
Minimal increased incidence congenital heart disease
Other right arch variants occur including
retroesophageal innominate artery and right arch
with isolated left subclavian artery often arising from
a ductus arteriosus
Imaging Findings
Best diagnostic clue: Right paratracheal density on
radiography with leftward tracheal deviation
Right aortic arch with aberrant left subclavian artery
Arch passes to right of trachea
CT Findings
CTA
o Right aortic arch with aberrant left subclavian artery
Arch passes to right of trachea
Four branches: Left carotid, right carotid, right
subclavian, left subclavian arteries
Retroesophageal vessel
Dilated subclavian artery (diverticulum of
Kommerell) may cause esophageal compression
Aortic arch usually descends on right
Coronal reconstructions show aortic arch
rightward of trachea
Sagittal reconstructions show retroesophagealleft
subclavian artery
o Right aortic arch with mirror imaging branching
Arch passes to right of trachea
Three branch vessels: Left innominate, right
carotid, right subclavian arteries
No retroesophageal vessel
Mirror imaging branching usually associated with
congenital heart disease in children
No tracheal or esophageal compression typically Imaging Recommendations
Ancillary findings: Stigmata of congenital heart
disease
Coronal reconstructions show aortic arch
rightward of trachea
MR Findings
TlWI
o Right aortic arch with aberrant left subclavian artery
Arch passes to right of trachea
Four branches: Left carotid, right carotid, right Mediastinal
subclavian, left subclavian arteries
Retroesophageal vessel
Dilated subclavian artery (diverticulum of
Kommerell) may cause esophageal compression
Aortic arch usually descends on right
Coronal images show aortic arch rightward of
trachea
Sagittal images show retroesophagealleft
subclavian artery
o Right aortic arch with mirror imaging branching
Four branches: Left carotid, right carotid, right
subclavian, left subclavian arteries
Retroesophageal vessel
Dilated subclavian artery (diverticulum of
Kommerell) may cause esophageal compression
Aortic arch usually descends on right 2
Right aortic arch with mirror imaging branching
Arch passes to right of trachea 3
Three branch vessels: Left innominate, right carotid,
right subclavian arteries
No retroesophageal vessel
Best imaging tool: CT
Top Differential Diagnoses
Mediastinal Mass Any Compartment
Double Aortic Arch
Arch passes to right of trachea
Three branch vessels: Left innominate, right
carotid, right subclavian arteries
No retroesophageal vessel
Mirror imaging branching usually associated with
congenital heart disease
No tracheal or esophageal compression
Ancillary findings: Stigmata of congenital heart
disease
T2WI: Not commonly used
PO/Intermediate: Not commonly used
T2* GRE
o Bright blood
o Right aortic arch with aberrant left subclavian artery
o Right aortic arch with mirror imaging branching
MRA: Gadolinium-enhanced
Advantages
o No radiation
o Multiplanar capabilities
o Valvular morphology and function
o Intracardiac morphology assessment
Best imaging tool: CT
Protocol advice
o Contrast-enhancement
o I mm thick sections permit best off-axial
reconstructions
I DIFFERENTIAL DIAGNOSIS
Mass Any Compartment
All mediastinal masses should be considered vascular
until proven otherwise especially if
o Adjacent to known vascular structures
o Mural calcification
o Oval or round shape with smooth contour
o Poor visualization in orthogonal view
Double Aortic Arch
Right arch typically higher and larger than left arch
Difficult to distinguish if left arch is atretic
 RIGHT AORTIC ARCH
I PATHOLOGY
General Features
General path comments: Anomalies common
anatomic variants
Genetics Consider
2 a Right aortic arch with aberrant left subclavian
artery: Interruption of embryonic double arch
between the left common carotid artery and the left
4
subclavian artery
a Right aortic arch with mirror imaging branching:
Interruption of embryonic double arch after the left
subclavian artery
Epidemiology: Occurs in 1:100
Associated abnormalities
a Right aortic arch with mirror imaging branching
Congenital heart disease: 98%
90% are tetralogy of Fallot (TOF): 25% of TOF
have right arch
2.5% are truncus arteriosus (TA): Approximately 2.
35% of TA have right arch
1.5% are transposition of great vessels (TGV): 10% 3.
of TGV have right arch
Gross Pathologic & Surgical Features
Aorta passes to right of trachea
IClINICAllSSUES
Presentation
Most common signs/symptoms: Usually asymptomatic
Other signs/symptoms 7.
a Dysphagia lusoria due to aberrant left subclavian
artery
a Dysphagia or stridor due to vascular ring
Right aortic arch
a Mirror imaging branching
a Associated with congenital heart disease
Tetralogy of Fallot (25% frequency of right arch 10.
but most common anomaly due to prevalence of
tetralogy)
Transposition of great vessels (5%) 11.
Truncus arteriosus (35%)
Pulmonary atresia with ventricular septal defect
Demographics
Age
a Mirror imaging branching form usually in neonate
or child
a Aberrant subclavian form usually in adult
Gender: No gender predominance
Ethnicity: No ethnic predominance is reported
Natural History & Prognosis
Dysphagia may worsen with time due to tightening of
vascular ring or atherosclerosis/ectasia of
retroesophageal vessel and compression of esophagus
Morbidity and mortality of surgical repair
Treatment
None for anomalies unless symptomatic
Surgery for relief of dysphagia or stridor
Surgery for aneurysmal dilatation of diverticulum of
Kommerell
I DIAGNOSTIC CHECKLIST
Volumetric reconstructions for 3D orientation of right
arch
Image Interpretation Pearls
For right arch passing posterior to esophagus and
descending on left, consider double arch with atretic
left segment
I SElECTED REFERENCES
1. Craatz S et al: Right-sided aortic arch and tetralogy of Fallot
in humans--a morphological study of 10 cases. Cardiovasc
Pathol. 12(4):226-32,2003
Singh B et al: Right aortic arch with isolated left
brachiocephalic artery. Clin Anat. 14(1):47-51,2001
Grathwohl KW et al: Vascular rings of the thoracic aorta in
adults. Am 5urg. 65(11):1077-83, 1999
4. Caus T et al: Right-sided aortic arch: surgical treatment of
an aneurysm arising from a Kommerell's diverticulum and
extending to the descending thoracic aorta with an
aberrant ieft subclavian artery. Cardiovasc Surg. 2(1):110-3,
1994
5. Moes CA et al: Rare types of aortic arch anomalies. Pediatr
Cardiol. 14(2):93-101,1993
6. van Son JA et al: Surgical treatment of vascular rings: the
Mayo Clinic experience. Mayo Clin Proc. 68(11):1056-63,
1993
Jaffe RB: Radiographic manifestations of congenital
anomalies of the aortic arch. Radiol Clin North Am.
29(2):319-34, 1991
8. Lowe GM et al: Vascular rings: 1O-year review of imaging.
Radiographies. 11(4):637-46, 1991
9. Luetmer PH et al: Right aortic arch with isolation of the left
subclavian artery: case report and review of the literature.
Mayo Clin Proc. 65(3):407-13,1990
Backer CL et al: Vascular anomalies causing
tracheoesophageal compression. Review of experience in
children. J Thorac Cardiovasc Surg. 97(5):725-31,1989
Gomes AS: MR imaging of congenital anomalies of the
thoracic aorta and pulmonary arteries. Radial Clin North
Am. 27(6):1171-81, 1989
12. Salomonowitz E et al: The three types of aortic diverticula.
AjR. 142:673-9, 1984
13. Hastreiter AR et al: Right-sided aorta. I. Occurrence of right
aortic arch in various types of congenital heart disease. II.
Right aortic arch, right descending aorta, and associated
anomalies. Br Heart J. 28(6):722-39, 1966
14. FELSONB et al: The Two Types of Right Aortic Arch.
Radiology. 81:745-59,1963
 RIGHT AORTIC ARCH
I IMAGE GAllERY

(Left) Frontal radiograph

shows right aortic arch (open
arrow) as a paralracheal
density. Note shadow from 2
right-sided descending aorta
(curved arrow). (Right) Axial 5
MR cine shows right arch
with aberrant subclavian
artery and a diverticulum of
Kommerell (arrow).

Typical
(Left) Coronal T7 WI MR
shows a right aortic arch
(open arrow) with an
aberrant left subclavian
artery (curved arrow). Note
aorta descending on right
(arrow). (Right) Coronal MR
shows right aortic arch
descending on the right
(open arrow) and aberrant
left subclavian artery
(arrow).

Typical
(Left) Axial MR cine shows
right arch with mirror image
branching. No
retroesophageal vessel is
present. (Right) Axial MR
cine shows mirror imaging
branching with left
innominate (open arrow),
right common carotid
(curved arrow), and right
subclavian (arrow) arteries.
 ABERRANT SUBCLAVIAN

2
6

Graphic shows left aortic arch with aberrant right Axial CECT shows the aberrant right subclavian artery
subclavian artery (arrow) that crosses to the right side (arrow) coursing posterior to the trachea and
posterior to the esophagus. esophagus.

ITERMINOLOGY I IMAGING FINDINGS

Abbreviations and Synonyms General Features
Aberrant right subclavian artery (ARSA) Best diagnostic clue: Tubular enhancing structure
posterior to esophagus
Definitions
Location: Retroesophageal and anterior to the spine
ARSA most common major aortic anomaly (not Size: Ranges from normal subclavian size (1 em) to
including bovine arch) aneurysmal (> 4 em)
Aberrant right subclavian artery occurs in 0.5% of Morphology
individuals o Tubular when not associated with diverticulum of
ARSA is last branch of a four branch vessel aortic arch Kommerell
ARSA traverses posterior to the esophagus and anterior o Tapering from the aorta when associated with
to the spine diverticulum of Kommerell
Aberrant artery origin often dilated (diverticulum of o Rounded when aneurysmal
Kommerell)
Diverticulum of Kommerell may become Radiographic Findings
atherosclerotic and aneurysmal Radiography: Often normal
Compression by ARSA on the posterior esophagus may Aberrant right subclavian artery
occasionally cause "dysphagia lusoria" o Frontal radiograph
Mediastinal widening occasionally
Oblique edge extending to the right arising from
the aortic arch (60%), often seen through tracheal
air column, edge may be sharp or indistinct

DDx: ARSA Mimics

Right Arch CXR Right Arch Azygos Cont

 ABERRANT SUBCLAVIAN
Key Facts
Terminology Chest radiograph often normal
Esophagram: Oblique posterior impression on
ARSA most common major aortic anomaly (not
esophagram directed superiorly to right shoulder
including bovine arch)
ARSAis last branch of a four branch vessel aortic arch Top Differential Diagnoses
ARSAtraverses posterior to the esophagus and
anterior to the spine
Mediastinal Mass any Compartment 2
Aberrant artery origin often dilated (diverticulum of Pathology
7
Kommerell) Frequency approximately 0.5% (1 in 200)
Compression by ARSA on the posterior esophagus Thoracic duct may terminate on the right
may occasionally cause "dysphagia lusoria"
Diagnostic Checklist
Imaging Findings All mediastinal masses should be considered vascular
Best diagnostic clue: Tubular enhancing structure until proven otherwise
posterior to esophagus Important anomaly for ENT surgeon who must be
Oblique edge extending to the right arising from the aware of nonrecurrent laryngeal nerve
aortic arch (60%), often seen through tracheal air Arterial catheter introduced in right arm will enter
column, edge may be sharp or indistinct descending aorta directly

Ill-defined mass in the right medial clavicular area May contain thrombus or calcification
(30%) T2WI: Not typically used
o Lateral radiograph: Mass effect posterior to trachea T2* GRE: See discussion for Tl WI images
in Raider triangle T1 C+: Not typically used
Raider triangle: Clear space posterior to trachea, MRA
anterior to vertebral bodies, and superior to aortic o Complements dark and bright blood images
arch o ARSA arises as posterior branch from superolateral
Obscuration of the aortic arch (60%) surface
Imprint on posterior tracheal wall (50%) Advantages
o Chest radiograph often normal o No radiation
o In coexistent coarctation o Multiplanar capabilities
Unilateral left rib notching o Noniodine based contrast (gadolinium)
o Assess intracardiac morphology
CT Findings
Aberrant right subclavian artery Fluoroscopic Findings
o Arch vessel order from left arch Esophagram: Oblique posterior impression on
Right common carotid artery esophagram directed superiorly to right shoulder
Left common carotid artery
Left subclavian artery
Imaging Recommendations
Aberrant right subclavian artery Best imaging tool: Contrast-enhanced CT
o Courses posterior to trachea and esophagus Protocol advice: Thin-section acquisition allows
Extends superiorly from left to right optimal reformatted images
Esophageal compression frequently evident
o No brachiocephalic
o Diverticulum of Kommerell I DIFFERENTIAL DIAGNOSIS
Visible as tapering tubular structure arising from Mediastinal Mass any Compartment
the posterior aortic arch
All mediastinal masses should be considered vascular
May contain thrombus or calcification
until proven otherwise especially if
MR Findings o Adjacent to known vascular structures
TlWI o Mural calcification
o Aberrant right subclavian artery o Oval or round shape with smooth contour
Signal is absent in vessel lumen but walls show o Poor visualization in orthogonal view
medium signal intensity Other mediastinal lesions
Arch vessel order from left arch o Posterior lymph nodes: Lymphoma
Right common carotid artery o Esophageal tumor carcinoma, leiomyoma
Left common carotid artery Transection Aorta
Left subclavian artery
Blunt chest trauma
Aberrant right subclavian artery
Aortic isthmus
o Courses posterior to trachea and esophagus
No aberrant artery
o Diverticulum of Kommerell
Visible as tapering tubular structure arising from Mass in Retrotracheal Triangle
posterior aortic arch Vascular
 ABERRANT SUBCLAVIAN
o Aberrant subclavian artery
o Right or double aortic arch
I CLINICAL ISSUES
Thoracic duct: Cyst Presentation
Esophagus Most common signs/symptoms: Most patients
o Duplication cysts asymptomatic
o Achalasia Other signs/symptoms
o Foreign body
2 o Neoplasm: Benign or malignant
o Dysphagia (lusoria) due to esophageal compression
o Dyspnea, cough from tracheal compression
Substernal thyroid Often occurs in early childhood prior to two years
8 Bronchogenic cyst of age
o Chest pain from aneurysm rupture
o Brainstem infarction from dissection
I PATHOLOGY o Fistulization to esophagus (rare)
General Features o Venous compression (rare)
General path comments Demographics
o Anomalies common anatomic variants
Age
o ARSA: Interruption of embryonic double arch o Symptomatic patients may present in childhood or
between the right common carotid artery and the adulthood depending on etiology
right subclavian artery o Asymptomatic patients usually recognized on CT
Genetics Gender: No known predilection
o No known genetic linkage
o Higher association with other conditions Natural History & Prognosis
Etiology: Involution of embryonic right fourth aortic Morbidity and mortality of surgical repair
arch between left carotid and left subclavian artery
Epidemiology
Treatment
o Frequency approximately 0.5% (1 in 200) None for anomalies unless symptomatic
o Most common congenital anomaly of the aortic Mild symptoms of dysphagia may respond to dietary
arch modification
Associated abnormalities Major symptoms may require surgery
o Congenital heart disease o Division of ARSA with reattachment of distal
Conotruncal anomalies subclavian to aorta proximal to right carotid artery
Left and right heart anomalies o Division of ARSA with reattachment of distal
Ventricular septal defects subclavian to right common carotid artery
o Down syndrome
With congenital heart disease, 37% have ARSA
o Also Edward, DiGeorge, and Dubowitz syndromes I DIAGNOSTIC CHECKLIST
o Possible higher association with a conjoined carotid
Consider
trunk
Other aortic arch abnormalities
o Anomalous recurrent laryngeal nerve (nonrecurrent
o Right aortic arch
laryngeal nerve)
o Double aortic arch
Important for surgeon to recognize that possibility
for aberrant nerve All mediastinal masses should be considered vascular
until proven otherwise
o Thoracic duct may terminate on the right
Important anomaly for ENT surgeon who must be
Important for surgeon
aware of nonrecurrent laryngeal nerve
Gross Pathologic & Surgical Features
Image Interpretation Pearls
Aberrant right subclavian artery course on pathology
series Arterial catheter introduced in right arm will enter
descending aorta directly
o Retroesophageal 50%
o Retrotracheal, between trachea and esophagus 12%
o Pre-tracheal < 2%
Diverticulum of Kommerell I SELECTED REFERENCES
o Remnant primitive distal right aortic arch 1. Carrizo G) et al: Dysphagia lusoria caused by an aberrant
o Seen in 60% of ARSA right subclavian artery. Tex Heart Inst). 31(2):168-71, 2004
Aberrant left subclavian artery 2. Donnelly LF et al: Aberrant subclavian arteries:
cross-sectional imaging findings in infants and children
o Right aortic arch
referred for evaluation of extrinsic airway compression. A)R
o Last branch off aorta, no increased incidence of Am) Roentgenol. 178(5):1269-74,2002
congenital heart disease 3. Katz M et al: Spiral CT and 3D image reconstruction of
o First branch off aorta (mirror image branching), vascular rings and associated tracheobronchial anomalies. J
high incidence of congenital heart disease Comput Assist Tomogr. 19(4):564-8, 1995
Most commonly Tetralogy of Fallot, ventricular 4. Proto AV et al: Aberrant right subclavian artery: Further
septal defect, and truncus arteriosus observations. A)R. 148:253-7, 1987
 ABERRANT SUBCLAVIAN
I IMAGE GALLERY
Typical
(Left) Frontal radiograph
shows a vascular structure
(arrow) corresponding to a
dilated aberrant right
2
subclavian. (Right) Sagittal
oblique esophagram shows a 9
posterior impression on the
esophagus (arrow) caused
by the aberrant right
subclavian artery.

Typical
(Left) Sagittal MR cine shows
the aberrant right subcfavian
artery (arrow) coursing
posterior to the trachea and
esophagus. (Right) Coronal
oblique CECT shows a
volume-rendered image of
the aortic arch with an
aberrant right subclavian
artery (arrow).

Variant
(Left) Frontal radiograph
shows a right paratracheal
mass (arrow) corresponding
to an aneurysm of aberrant
right subclavian artery.
(Right) Axial CECT shows an
aneurysm of the aberrant
right subclavian artery
(arrow).
 AORTIC COARCTATION

2
10

Sagittal graphic shows high grade, short segmental Frontal radiograph on barium assessment shows "figure
narrowing of the thoracic aorta distal to the ductus ]" sign (open arrow) in a patient diagnosed with aortic
arteriosis. coarctation.

Unilateral right rib notching: Coarctation located

I TERMI NOLOGY between left common carotid artery and left
Abbreviations and Synonyms subclavian artery
Coarctation, coarctation syndrome, tubular hypoplasia o "Figure 3" sign
Up to 1/3 to 1/2, less common in children
Definitions Proximal bulge dilated left subclavian artery
Narrowing of distal aortic arch and/or proximal Indentation at coarctation, lower bulge
descending aorta with obstruction to blood flow post-stenotic dilatation.descending aorta
o Heart: Rounded apex from left ventricular
hypertrophy
IIMAGING FINDINGS Calcified bicuspid aortic valve
Esophagram
General Features o Compression from the dilated left subclavian artery
Best diagnostic clue: Inferior rib notching and poststenotic dilatation of the descending aorta
(reverse 3 or E sign)
Radiographic Findings
Osseous findings Turner syndrome: Dwarfism, short
Chest radiograph fourth metacarpal, overgrowth medial femoral condyle
o Inferior rib notching from enlarged tortuous
intercostal arteries serving as collateral vessels; may CT Findings
regress post repair CT angiography with multiplanar reformations
Notching occurs in high pressure circuit, not seen (oblique sagittal) plane
before 6 years of age
Ribs 3 through 8; 1st and 2nd intercostals arise Angiographic Findings
from costocervical trunk and do not serve aorta Direct measurement of gradient
o < 20 mm Hg, coarctation is mild

DDx: Aortic Coarctation

Takayasu Arteritis Pseudocoarctation Pseudoaneurysm

 AORTIC COARCTATION
Key Facts
Terminology Pathology
Narrowing of distal aortic arch and/or proximal Genetics: Associated with Turner syndrome (20%
descending aorta with obstruction to blood flow have coarctation)
Associated with bicuspid aortic valve (75%) and
Imaging Findings cerebral aneurysms (5-10%)
Best diagnostic clue: Inferior rib notching 2
"Figure 3" sign Clinical Issues
Length of systolic (dark) flow jet proportional to Gender: More common in males (2:1) 11
hemodynamic significance of coarctation Uncorrected, average age of death 42
> 20 mm Hg, suggests that intervention required Re-coarctation (2-5%)
Long term survival decreased, must be considered
Top Differential Diagnoses long term disease "repaired but not corrected"
Pseudocoarctation
Takayasu Arteritis Diagnostic Checklist
Chronic Traumatic Pseudoaneurysm Search for subtle signs of coarctation in any young
patient with hypertension

a > 20 mm Hg, suggests that intervention required

Chronic Traumatic Pseudoaneurysm
MR Findings History of trauma, healed rib and other skeletal
T2* GRE fractures
a Cine (white blood) Pseudoaneurysm may calcify
Length of systolic (dark) flow jet proportional to
hemodynamic significance of coarctation
Inferior Rib Notching Differential
MRA: Phase-contrast to estimate pressure gradient Neurofibromatosis
T1WI cardiac-gated (black blood) and 3D gadolinium Venous collaterals (SVC obstruction)
sequences for morphology Unilateral pulmonary obstruction
Idiopathic
Echocardiographic Findings
Echocardiogram
a Suprasternal long axis view I PATHOLOGY
a Color Doppler estimates gradient across coarctation
a Classic findings: Narrowing of the isthmus and General Features
posterior indentation or shelf General path comments
a Longer segment stenosis referred to as tubular
Imaging Recommendations hypoplasia
Best imaging tool a May occur at multiple sites within aorta, more often
a Echocardiography in infancy observed in Turner syndrome
a MR in older child or adult Genetics: Associated with Turner syndrome (20% have
a Angiography for treatment and measurement of coarctation)
gradient Etiology
Protocol advice: MR include sagittal-oblique plane a Muscular theory
through aortic arch and perpendicular plane through Migration of ductal tissue from the ductus
coarctation for measurement cross-sectional diameter arteriosus into aorta, when ductus contracts aortic
lumen narrowed
a Hemodynamic theory
I DIFFERENTIAL DIAGNOSIS During fetal development, decrease in aortic blood
flow may not allow proper growth of aorta
Pseudocoarctation Increased incidence of coarctation in disorders
Aging and atherosclerosis elongation and kinking of where left ventricular outflow tract obstruction
aorta without obstruction to blood flow reduces aortic blood flow, conversely decreased
a No collateral vessels incidence of coarctation in disorders where
a Older adult decreased ductal flow is present (e.g., Tetralogy of
Takayasu Arteritis Fallot)
Epidemiology
Inflammatory narrowing of unknown etiology
a Incidence: 2-6 per 10,000 births
Narrowing and or occlusion of aorta and branch
a Comprises 5-10% of cases of congenital heart disease
vessels, rarely isolated to aortic isthmus
Associated abnormalities
a Ventricular septal defect (VSD) (33%)
 AORTIC COARCTATION
o Shone syndrome: Aortic coarctation, subaortic
stenosis, parachute mitral valve, supravalvular mitral
ring
Gross Pathologic & Surgical Features
Obstructing membrane or ridge or tissue at the level of
aortic isthmus
2 May develop cystic medial necrosis in aorta adjacent
to coarctation site: Locus for late aneurysm or
12 dissection
Staging, Grading or Classification Criteria
Adult type
o More common
o Short-segment stenosis distal to ductus
o Collateral vessels supply flow to distal aorta
o Associated with bicuspid aortic valve (75%) and
cerebral aneurysms (5-10%)
Infantile type
o Long-segment narrowing proximal to the ductus
arteriosus
o Patent ductus arteriosus (PDA) supplies flow to distal
aorta
When PDA closes, heart failure ensues (one of the
common causes of congestive heart failure first
week of life)
o Associated anomalies more common particularly
VSD, atrial septal defect (ASD) and mitral valve
pathology (50%)
ICLINICAllSSUES
Presentation
Most common signs/symptoms
o Frequently asymptomatic except for incidental
hypertension
o If symptomatic: Headache, nosebleeds, leg cramps,
cold feet
o Differential hypertension between upper and lower
extremities, diminished femoral pulses
o Systolic murmur over thoracic spine, murmur may
also be due to associated bicuspid aortic valve
Other signs/symptoms: Turner syndrome: Short
webbed neck, broad chest, pigmented facial nevi, short
4th metacarpals
Demographics
Gender: More common in males (2:1)
Natural History & Prognosis
Uncorrected, average age of death 42
o Due to spontaneous aortic rupture, bacterial
endocarditis, and cerebral hemorrhage
o 25% dead by the age of 20
o 50% dead by the age of 30
o > 90% dead by the age of 58
Repaired below age 14 years, 20 year survival 90%;
after age 14 years, 20 year survival 80%
Vigorous treatment of unsuspected hypertension in
patients with coarctation may lead to renal failure
Re-coarctation (2-5%)
o Associated with younger age surgery, small patient
size and type of surgical repair
Post-operative aneurysms (25% after patch
angioplasty)
Long term survival decreased, must be considered long
term disease "repaired but not corrected"
o Hypertension and coronary artery disease
o Long term risk for dissection
Pregnancy related issues
o Untreated coarctation has high mortality (5%)
a Treated coarctation at risk for aortic dissection and
cerebral aneurysm rupture in third trimester
o Significant stenosis contraindication to pregnancy
Treatment
Indication for treatment
o Long-standing hypertension with or without
symptoms
o Hemodynamically significant aortic stenosis
o Female patient contemplating pregnancy
Surgical correction
o End to end anastomosis
Higher risk of spinal artery injury and restenosis
(from circumferential suture)
o Left subclavian flap aortoplasty
Sacrifice vertebral (to avoid subclavian steal
phenomena) and left subclavian artery
o Prosthetic patch or interposition graft
Higher long term risk of infection or aneurysm
with prosthetic material
Acute complications
o Paradoxic hypertension from reactivation
renin -angiotensin system
o Postcoarctectomy syndrome
Abdominal pain and distension (20%) due to
increased pressure in mesenteric arteries, reflex
vasoconstriction may progress to intestinal wall
hemorrhage or even perforation
a Paraplegia
Damage to spinal artery, less common in
neonates, up to 2% adults
Paralysis uncommon when well-developed
collaterals present
o Recurrent laryngeal or phrenic nerve injury
Balloon angioplasty most often used for r.estenosis but
increasing applied for primary treatment
Late complications
o Recoarctation < 5%
o Aneurysm post repair 5%
o Endocarditis 33%
More common in aortic valve prosthesis than at
coarctation site
I DIAGNOSTIC CHECKLIST
Consider
Search for subtle signs of coarctation in any young
patient with hypertension
I SELECTED REFERENCES
1. de Bono JP et al: Long term follow up of patients with
repaired aortic coarctations. Heart. 91(4):537-8, 2005
 AORTIC COARCTATION

I IMAGE GALLERY

(Left) Frontal radiograph

(coned down view) shows
left-sided rib notching at
multiple levels (curved
2
arrows). Patient was
subsequently diagnosed with 13
coarctation of the aorta.
(Right) Sagittal oblique CECT
(magnified image) shows
short segment, high grade
coarctation involving the
proximal descending aorta
(arrow). This patient had left
upper extremity
hypertension.

Typical
(Left) Sagittal CECT shows
circumscribed, high grade
narrowing of the proximal
descending thoracic aorta
(arrow). (Right) Sagittal
NECT shows high grade
stenosis of the proximal
descending aorta in a 27
year old with coarctation.
Elongation of the supraaortic
vessels a/50 is visible
(arrows).

Typical
(Left) Sagittal oblique T1 C +
FS MR shows focal
narrowing of the proximal
descending thoracic aorta
(open arrow). Turbid ffow is
seen with hypointensity
distal to the narrowing
(arrow). (Right) Sagittal
CECT
maximum-intensil y-projection
reconstruction shows
endovascular stent (arrows)
in the proximal descending
aorta. 19 year old man after
surgical repair of aortic
coarctation.
 INTRALOBAR SEQUESTRATION

2
14

Graphic shows typical features of intralobar Coronal oblique CECT volume rendered image displays
sequestradon. Focal mass or opacity in left a muldcysdc left lower lobe sequestradon in a 43 year
costovertebral angle. Area supplied by arterial branch old male. The systemic supplying artery (arrow) arises
off of the aorta (arrow) . from the distal thoracic aorta.

Posterior basal segmental> medial basal

ITERMINOlOGY segmental region
Definitions Size: Variable, but cystic lesions often quite large
Pulmonary sequestration represents nonfunctioning Radiographic Findings
lung tissue separated from normal lung Inferior paraspinal mass or opacity located in posterior
o Receives its blood supply from a systemic artery basal segment adjacent to diaphragm
o Lacks normal communication with bronchi o Less common manifestation as predominantly cystic
Two major forms mass
o Intralobar sequestration (75%) Margins may be either sharp, lobulated or ill-defined
Shares visceral pleura of normal lung o Concurrent volume loss and mediastinal shift may
o Extralobar sequestration (25%) be present
Has separate pleura from normal lung 1/3 of cystic sequestrations contain air or air-fluid
o Communicating bronchopulmonary foregut levels
malformation is an uncommon form of o Localized emphysema without consolidation or fluid
sequestration, usually seen with extra lobar type is a well-described, but uncommon manifestation
Chronic or recurrent bacterial pneumonia
o May decrease in size with antibiotic therapy, but will
IIMAGING FINDINGS not resolve
General Features Pleural effusion (4%) and calcifications rare
Best diagnostic clue: Persistent left-sided inferior CT Findings
paraspinal mass with history of recurrent pneumonia Complex lesion containing solid, fluid and cystic
Location components
o Left lower lobe (65%), right lower lobe (55%) o Cysts may be single or multiple

DDx: Intralobar Sequestration

Lipoid Pneumonia Contusion/Traumatic Lung Cysts

 INTRALOBAR SEQUESTRATION
Key Facts
Terminology
Pulmonary sequestration represents nonfunctioning
lung tissue separated from normal lung
Receives its blood supply from a systemic artery
Lacks normal communication with bronchi
Imaging Findings
Best diagnostic clue: Persistent left-sided inferior
paraspinal mass with history of recurrent pneumonia
Margins may be either sharp, lobulated or ill-defined
Chronic or recurrent bacterial pneumonia
May decrease in size with antibiotic therapy, but will
not resolve
Complex lesion containing solid, fluid and cystic
components
Multicystic form often quite large
Systemic artery identification from aorta is diagnostic
Fluid, air-fluid levels or only air may be present
o Heterogeneous enhancement
o Lung bordering sequestration maybe hyperinflated
or emphysematous tissue
Emphysematous border has appearance of
air-trapping
Multicystic form often quite large
Systemic artery identification from aorta is diagnostic
o Seen in vast majority of cases on CECT or CT
angiography
o Non-visualization of systemic artery does not
exclude diagnosis
Occasionally, multiple small arteries supply
sequestration
Calcification and effusions are uncommon
Angiographic Findings
Traditional method of diagnosis
o Essentially replaced by CT angiography
Origin of feeding artery
o Thoracic aorta 75%
o Abdominal aorta 20%
o Intercostal artery 5%
o Multiple 16%
A vessel less < 3 mm is likely one of multiple
supplying arteries
95% have pulmonary venous drainage
o 5% systemic venous drainage, usually via azygos,
hemiazygos, superior vena cava or intercostal routes
Role has decreased with multidetector CT, although
used when there is no identifiable systemic vessel
o Usually due to multiple small arteries
MR Findings
Excellent depiction of complex cystic, solid and
fibrotic components
o Cystic portions have variable signal manifestations
depending on fluid characteristics
Often higher signal on T2Wl sequences
Hemorrhage within lesion represented by higher
signal on both 1'1WI and T2Wl sequences
95% have pulmonary venous drainage
Pathology
The vast majority are likely an acquired abnormality
Chronic inflammation induces hypertrophy of
arterial vessels, resulting in a systemic vascular supply
to maintain parenchymal viability
2
Clinical Issues 15
Often presents with chronic productive cough and
recurrent lower lobe bacterial pneumonia
Hemoptysis common presenting sign
Most common in young adults, 50% < 20 years old
Diagnostic Checklist
Recurrent or persistent pneumonia localized to the
same region of the lower lobe
Imaging Recommendations
Best imaging tool: Multidetector CT angiography
Protocol advice: Thin collimation for multi planar
volume post processing
I DIFFERENTIAL DIAGNOSIS
Extralobar Sequestration
Congential lesion, often presents in first 6 months
o Completely distinct entity from intralobar form
Associated with other congential anomalies
Systemic arterial supply from aorta
o Drainage into systemic veins (80%), not pulmonary
o Invested in own pleural lining, separated from
normal lung
Essentially an accessory lung
Located on left in 90%, although may lie within or
below diaphragm
Chronic Pneumonia/Lipoid Pneumonia
Chronic consolidative process in the lower lobe such
as lipoid pneumonia or cryptogenic organizing
pneumonia (COP)
o No feeding vessel will be present
Fat density may be seen in lipoid pneumonia
COP often resolves with therapy
Necrotizing Pneumonia/Abscess
Similar imaging appearance if in lower lobe
o Tends to essentially resolve with therapy
No feeding artery
Congential Cystic Adenomatoid
Malformation
Hamartomatous lesion often diagnosed in neonatal
period or childhood
o No systemic feeding vessel
Most have some cystic regions
Contusion/Traumatic Pneumatoceles
Known trauma with consolidation and "cystic" areas
 INTRALOBAR SEQUESTRATION
o Rib fractures may be present
Often acute and resolves over weeks to months
Post Obstructive Pneumonia/Central
Bronchial Neoplasm
Ill-defined or consolidative and atelectatic area
o Concurrent volume loss common
2 No dominant vessel arising from aorta
Central obstructing lesion often seen
16
I PATHOLOGY
General Features
Etiology
o Originally, all considered congenital anomaly
A few cases are definitely congential in origin,
although they are believed to be less common
o The vast majority are likely an acquired abnormality
Proposed bronchial obstruction from recurrent or
chronic pneumonia
Inflammation compromises pulmonary arterial
supply to lung
o Chronic inflammation induces hypertrophy of
arterial vessels, resulting in a systemic vascular
supply to maintain parenchymal viability
Inferior pulmonary ligament artery supplies the
medial inferior visceral pleura and may explain
why it is a common parasitized systemic artery
Phrenic vessels from the celiac supply
diaphragmatic pleural surface also may be
recruited
o This proposed etiology better explains the older age
of presentation, consistent lower and medial
distribution and rare incidence of associated
anomalies
Epidemiology: Uncommon: 1-6% of all pulmonary
malformations
Gross Pathologic & Surgical Features
Contiguous with normal lung and a thick fibrinous
visceral pleural lining
o Numerous thick adhesions from visceral pleura to
diaphragm, mediastinum and parietal pleural
Systemic arterial supply
o Venous drainage via pulmonary veins
Cystic and scarred lung tissue: Usually no bronchial
communication
o Air present in sequestration may relate to
incomplete bronchial obstruction or partial
recanalization
Thick fibrous and consolidative lung usually surrounds
fluid-filled cysts
o Cysts contain blood, purulent or gelatinous material
Cystic spaces resemble ectatic bronchi, potentially
from accumulated secretions in obstructed airways
Microscopic Features
Chronic inflammation, cysts and extensive fibrosis
No communication with bronchi
o Bronchial and bronchiolar segments are surrounded
by fibrosis and inflammatory cells
Focal areas of isolated bronchopneumonia
common
Atherosclerotic changes in arterial supply from aorta
are common, even in the young
o These systemic vessels are predominantly composed
of elastic rather than muscular components, thus
resembling pulmonary arteries
Rare foci of squamous or adenocarcinoma
ICLINICALISSUES
Presentation
Most common signs/symptoms
o Often presents with chronic productive cough and
recurrent lower lobe bacterial pneumonia
Hemoptysis common presenting sign
Chest and/or pleuritic pain
o Persistent opacification in same portion of lower
lobe supports diagnosis
15-20% are asymptomatic
High output failure from left-to-right shunting in
infants a rare but well-described presentation
Demographics
Age
o Most common in young adults, 50% < 20 years old
May occur at any age
Gender: M = F
Natural History & Prognosis
Excellent prognosis following surgical excision
Treatment
SurgIcal resection for symptomatic lesions
o Lobectomy rather than segmentectomy since it
usually crosses segmental planes
Surgical removal for chronic infection or hemoptysis
o Imaging should always search for multiple vessels,
which is vital to know prior to surgery
Embolization has been used to treat hemodynamic
shunts, although resection still required because of
persistent infections
I DIAGNOSTIC CHECKLIST
Consider
Recurrent or persistent pneumonia localized to the
same region of the lower lobe
I SELECTED REFERENCES
l. Corbett H] et al: Pulmonary sequestration. Paediatr Respir
Rev.5(1):59-68, 2004
2. Zylak CJ et al: Developmental lung anomalies in the adult:
radiologic-pathologic correlation. Radiographies. 22 5pec
No:525-43, 2002
3. Bratu I et al: The multiple facets of pulmonary
sequestration.] Pediatr 5urg. 36(5):784-90, 2001
4. 5aygi A: Intralobar puimonary sequestration. Chest.
119(3):990-2, 2001
5. Frazier AAet al: Intralobar sequestration:
Radiologic-pathologic correlation. Radiographies.
17:725-45, 1997
 INTRALOBAR SEQUESTRATION

IIMAGE GALLERY

(Left) Axial CECT in a 7 year

old boy with recurrent
pneumonia. Left lower lobe
posterior and medial basal 2
segmental conso/idative
mass also has cystic areas. Its 17
anterior margin is ill-defined.
(Right) Axial CECT shows a
dominant branching artery
(arrow), which originates
from the descending thoracic
aorta. Surgical resection
confirmed an intralobar
sequestration.

Typical
(Left) Frontal radiograph in
this 37 year old male with
chronic cough, but otherwise
asymptomatic. There is a
large lobulated mass in the
left lower lobe, no evidence
of cavitation. (Right) Axial
CECT shows the large
multicyslic mass with
heterogeneous
enhancement The dominant
vessel medially (arrow)
arises form the thoracic
aorta. Venous drainage was
pulmonary (not shown).

Variant
(Left) Axial CECT in an
asymptomatic 64 year old
male with an incidental
radiographic finding shows
an ill-defined consolidative
mass with systemic arterial
supply (arrow) and
pulmonary venous drainage.
(Right) Axial CECT in a 46
year old asymptomatic
female. A left lower lobe
"emphysematousll region
had a dominant systemic
arterial vessel (arrow).
Surgery removed an air-filled
intralobar sequestration.
 LEFT SUPERIOR VENA CAVA

2
18

Graphic shows typical location of left superior vena Axial CECT shows a large vessel lateral to the aorta
cava. Anteriorly located in a similar plane to the right (arrow) consistent with a left superior vena cava. Note
superior vena cava. Usually not border forming as the absence of the right superior vena cava.
vein is media! to the aortic arch (insert).

Location: Left lateral mediastinum

ITERMINOlOGY Size: Variable and usually inversely related to the size
Abbreviations and Synonyms of the right SVC
Left superior vena cava (LSVC) Morphology: Tubular structure
Persistent left superior vena cava Radiographic Findings
Left SVC Radiography
LSVC o Occasionally visualized as straight border forming
Definitions left-sided upper mediastinal structure
Represents persistence of the left common cardinal o Prominent appearing right-sided aorta may be due
vein to absence of right SVC
Courses along left side of mediastinum o Left-sided central catheter of pacemaker/defibrillator
Usually drains into coronary sinus may enter left SVC (particular if left brachiocephalic
Majority associated with absent left brachiocephalic vein is absent)
vein Catheter passes inferiorly along left mediastinal
Usually associated with normal to decreased right SVC border
Minority associated with absent right SVC CT Findings
CECT
o Tubular structure along left superior mediastinum
I IMAGING FINDINGS o Originates from junction of left internal jugular and
General Features subclavian veins
Receives drainage from the left superior intercostal
Best diagnostic clue: Tubular left mediastinal structure
vein
with enhancement
o Courses inferiorly in prevascular space

DDx: Periaortic Vascular Structures

Partial APVR Partial APVR LSIV

 LEFT SUPERIOR VENA CAVA
Key Facts
Terminology
Represents persistence of the left common cardinal
vein
Courses along left side of mediastinum
Usually drains into coronary sinus
Majority associated with absent left brachiocephalic
vein
Usually associated with normal to decreased right
SVC
Minority associated with absent right SVC
Imaging Findings
Best diagnostic clue: Tubular left mediastinal
structure with enhancement
Tubular structure along left superior mediastinum
Originates from junction of left internal jugular and
subclavian veins
a Passes lateral to aorta
a Passes anterior to left main bronchus
a May be difficult to identify as it courses posterior to
left atrium
a No feeding vessels from lung
a Usually drains to enlarged coronary sinus
Minority drain to left atrium (8%) and have a high
association with congenital heart disease
a Note small or absent right SVC
a Note absence of left brachiocephalic vein
a May receive drainage from left hemiazygous system,
particularly with hemiazygous continuation
Angiographic Findings
DSA: Injection often through a left-sided catheter will
show contrast coursing inferiorly into the coronary
sinus and right atrium
MR Findings
TlWl
a Tubular structure along left superior mediastinum
a Courses in prevascular space
a Passes anterior to left main bronchus
a No feeding vessels from lung
a Usually drains to enlarged coronary sinus
Minority drain to left atrium - high association
with congenital heart disease
a Note small or absent right SVC
a Note absence of left brachiocephalic vein
MRV
a Tubular structure along left superior mediastinum
a Signal intensity often different than adjacent
structures
a Often can be followed along entire course
a Note large coronary sinus
a Phase contrast imaging shows inferior flow towards
coronary sinus
a Note small or absent right SVC
a Note absence of left brachiocephalic vein
Imaging Recommendations
Best imaging tool: Contrast-enhanced CT scan
Courses inferiorly in prevascular space
Passes anterior to left main bronchus
No feeding vessels from lung
Usually drains to enlarged coronary sinus
Tubular structure along left superior mediastinum
Phase contrast imaging shows inferior flow towards 2
coronary sinus
Best imaging tool: Contrast-enhanced CT scan 19
Top Differential Diagnoses
Partial Anomalous Pulmonary Venous Return (Partial
APVR) from Left Upper Lobe
Enlarged Left Superior Intercostal Vein (LSIV)
Lymph Node
Pathology
Congenital heart disease
Protocol advice: Inject L arm for maximum
opacification of vessel
I DIFFERENTIAL DIAGNOSIS
Partial Anomalous Pulmonary Venous Return
(Partial APVR) from Left Upper Lobe
Properly termed: Vertical vein
Like left SVC, courses in left mediastinum in
prevascular space
Located lateral to aortic arch
Anomalous veins visible entering from left lung to join
vertical vein
At level of left main bronchus, normal left superior
pulmonary vein typically absent
No enlargement of coronary sinus
Normal to enlarged left brachiocephalic vein and right
SVC
On MRV, flow direction is cephalic not caudal
Enlarged Left Superior Intercostal Vein (LSIV)
Courses along lateral margin of aorta
Typically smaller than left SVC
Provides anastomotic connection between left
brachiocephalic vein and accessory hemiazygous vein
Lymph Node
Typically does not extend on multiple sections in left
mediastinum
I PATHOLOGY
General Features
Genetics
a No known genetic predisposition
a Higher prevalence in patients with congenital heart
disease
Etiology
a Persistence of left common cardinal vein and sinus
horn
 LEFT SUPERIOR VENA CAVA
o Sometimes associated with involution of right
cardinal vein system Image Interpretation Pearls
Epidemiology Consider diagnosis in patient with catheter or
o Left superior vena cava occurs in 0.2-0.4% of all pacemaker that courses inferiorly along the lateral left
patients mediastinum
o Prevalence is 3-10% in children with congenital
heart disease
2 In congenital heart disease, left SVC often drains I SELECTED REFERENCES
directly into left atrium (Raghib syndrome) 1. Gonzalez-Juanatey C et al: Persistent left superior vena
20 Drainage is into the top of the left atrium usually cava draining into the coronary sinus: report of 10 cases
between the left atrial appendage and pulmonary and literature review. Clin Cardiol. 27(9):515-8, 2004
veins 2. Eckart REet al: Utility of magnetic resonance imaging in
cardiac venous anatomic variants. Cardiovasc Intervent
Coronary sinus often absent or unroofed, Radiol. 26(3):309-11, 2003
producing an intraatrial communication 3. Haramati LBet al: Computed tomography of partial
Associated abnormalities anomalous pulmonary venous connection in adults. J
o Congenital heart disease Comput AssistTomogr. 27(5):743-9, 2003
Atrial septal defect 4. Hahm JK et al: Magnetic resonance imaging of unroofed
Conotruncal abnormalities coronary sinus: three cases. Pediatr Cardiol. 21(4):382-7,
Heterotaxy syndrome especially asplenia 2000
Mitral atresia 5. Raptopoulos V: Computed tomography of the superior
vena cava. Crit Rev Diagn Imaging. 25(4):373-429, 1986
Cor triatriatum 6. Brown KTet al: Pseudoprominent aorta: radiographic
Rarely, may produce a right to left shunt with findings and CT correlation. Radiology. 155(2):299-301,
cyanosis 1985
7. Fisher MR et al: Magnetic resonance imaging of
Gross Pathologic & Surgical Features developmental venous anomalies. AJRAm J Roentgenol.
May encounter vessel unexpectedly if performing left 145(4):705-9, 1985
mediastinal surgery 8. Huggins TJ et al: CT appearance of persistent left superior
vena cava. J Comput AssistTomogr. 6(2):294-7, 1982
9. Baron RLet al: CT of anomalies of the mediastinal vessels.
IClINICALISSUES AJRAm J Roentgenol. 137(3):571-6, 1981
10. Cha EM et aI: Persistent left superior vena cava. Radiologic
Presentation and clinical significance. Radiology. 103(2):375-81, 1972
11. Campbell M et al: The left-sided superior vena cava. Br
Most common signs/symptoms: Usually asymptomatic
HeartJ. 16(4):423-39, 1954
Other signs/symptoms 12. Winter FS:Persistent left superior vena cava; survey of
o Rarely, cardiac arrhythmias due to atrioventricular world literature and report of thirty additional cases.
nodal stretching in the setting of catheter placement Angiology. 5(2):90-132, 1954
o Rarely, left ventricular outflow obstruction due to
incomplete occlusion of mitral valve
o Congenital heart disease
May have symptoms related to atrial septal defect
or heterotaxy syndrome
Demographics
Age
o Asymptomatic patients are diagnosed at any age,
usually on CT performed for another reason
o Patients with complex associated congenital
anomalies may present early in life
Gender: No predilection
Natural History & Prognosis
Related to associated congenital anomalies
Treatment
None if isolated

I DIAGNOSTIC CHECKLIST
Consider
Injection in left arm for optimal opacification
Associated congenital anomalies
 LEFT SUPERIOR VENA CAVA

I IMAGE GALLERY
Typical
(Left) Axial CECT shows the
left superior vena cava
(arrow) lateral to the aorta.
Note the small right SVC
2
(open arrow). (Right) Axial
CECT shows the left superior 21
vena cava (arrow) anterior
to the left superior
pulmonary vein at level of
the left main bronchus.

Typical
(Left) Axial CECT shows the
markedly enlarged coronary
sinus, drainage site of the left
superior vena cava (arrow).
(Right) Coronal CECT shows
the left superior vena cava
(arrow) coursing inferiorly to
join the coronary sinus with
drainage to the right atrium.

(Left) Frontal radiograph

shows a central venous
catheter extending inferiorly
along the left lateral
mediastinal border within a
left superior vena cava.
(Right) Frontal DSA shows a
catheter within a left superior
vena cava. Contrast injected
through the catheter courses
through the coronary sinus
into the right atrium.
 AZYGOS CONTINUATION OF IVC

2
22

Sagittal graphic shows characterisUc features of azygos Axial CECT shows a dilated azygos arch (arrow) in a
continuation. IVC is absent. Hepatic veins drain directly patient with azygos continuation.
into right atrium. Azygos vein is enlarged and serves as
the main venous drainage below the diaphragm.

I TERMINOLOGY I IMAGING FINDINGS

Abbreviations and Synonyms General Features
Azygos continuation of the inferior vena cava (lVC) Best diagnostic clue: Absence of intrahepatic segment
Interruption of the IVC of IVC with dilated azygos or hemiazygos vein on
Absence of the hepatic segment of the IVC with contrast-enhanced CT
azygos continuation Size
o Azygos vein in right tracheobronchial angle
Definitions Dilated: > 10 mm short-axis diameter in erect
IVC interrupted above the renal veins position
Hepatic veins drain directly into right atrium Dilated: > 15 mm short-axis diameter in supine
Large azygos vein carries venous return from lower position
extremity
o Occasionally, a large hemiazygos vein carries venous Radiographic Findings
return Posteroanterior
Caused by persistence of the embryonic right o Focal enlargement of azygos arch in the right
supracardinal vein and lack of development of the tracheobronchial angle
suprarenal part of the subcardinal vein Round or oval shape
Associated with congenital heart disease and situs Considered dilated when> 10 mm diameter in
abnormalities, especially polysplenia (heterotaxy erect position
syndrome) Considered dilated when> 15 mm diameter in
supine position
o Prominence of retroesophageal stri pe

DDx: Azygos Continuation Mimics

Right Arch Right Arch SVC Syndrome

 AZYGOS CONTINUATION
Key Facts
Terminology
IVC interrupted above the renal veins
Hepatic veins drain directly into right atrium
Large azygos vein carries venous return from lower
extremity
Caused by persistence of the embryonic right
supracardinal vein and lack of development of the
suprarenal part of the subcardinal vein
Associated with congenital heart disease and situs
abnormalities, especially polysplenia (heterotaxy
syndrome)
Imaging Findings
Best diagnostic clue: Absence of intrahepatic segment
of IVC with dilated azygos or hemiazygos vein on
contrast-enhanced CT
o Prominence of aortic nipple may occur with
hemiazygos continuation
o Bilateral left lungs and bronchi if associated with
polysplenia
o Transverse or transposed liver if associated with
polysplenia
Lateral
o Absence of retrocardiac shadow of the IVC
(sometimes)
o However, suprahepatic portion of IVC may be
present
CT Findings
CECT
o Absent suprarenal and intrahepatic portion of IVC
o Hepatic veins enter directly into right atrium
o Large posterior, paraspinal vessel corresponding to
the azygos (right) or hemiazygos (left) continuation
o Dilated azygos courses upward and drains to the
SVC
Look for dilated azygos arch
o Dilated hemiazygos courses upward
Typically drains to a left SVC with dilated
coronary sinus
May cross midline and join azygos
Rarely drains to accessory hemiazygos vein, left
superior intercostal vein, and left brachiocephalic
vein
Look for dilated left-sided venous arch lateral to
aorta in typical drainage
o Polysplenia findings (heterotaxy)
Multiple spleens
Situs ambiguus
Bilateral bilobed (left-sided morphology) lungs
Bilateral hyparterial (left-sided morphology)
bronchi
Congenital heart disease (especially atrial septal
defect, ventricular septal defect)
Midline or transposed abdominal viscera,
intestinal malrotation, preduodenal portal vein,
truncated pancreas
OF IVC
Focal enlargement of azygos arch in the right
tracheobronchial angle
Prominence of aortic nipple may occur with
hemiazygos continuation
Hepatic veins enter directly into right atrium
Large posterior, paraspinal vessel corresponding to 2
the azygos (right) or hemiazygos (left) continuation
23
Top Differential Diagnoses
Enlargement of Azygos Arch and Vein due to Superior
Vena Cava (SVC) Obstruction
Enlargement of Azygos Arch due to High Volume
States
Enlargement of Azygos-Region Lymph Node
Clinical Issues
If inadvertently ligated at surgery may be lethal
MR Findings
TlWI
o Absent suprarenal and intrahepatic portion of IVC
o Hepatic veins enter directly into right atrium
o Large posterior, paraspinal vessel corresponding to
the azygos (right) or hemiazygos (left) continuation
o Dilated azygos courses upward and drains to the
SVC
Look for dilated azygos arch
o Dilated hemiazygos courses upward along left spine
Typically drains to a left SVC with dilated
coronary sinus
May cross midline and join azygos
Rarely drains to accessory hemiazygos vein, left
superior intercostal vein, and left brachiocephalic
vein
Look for dilated left-sided venous arch lateral to
aorta in typical drainage
o Polysplenia findings identical to CT findings
Multiple spleens
Situs ambiguus
MRA: Azygos continuation, interrupted IVC observed
on venous phase
Imaging Recommendations
Best imaging tool: Contrast-enhanced CT
I DIFFERENTIAL DIAGNOSIS
Enlargement of Azygos Arch and Vein due to
Superior Vena Cava (SVC) Obstruction
Note distal occlusion of SVC by mass or thrombosis
Azygos serves as collateral pathway
NormallVC
Enlargement of Azygos Arch due to
Pulmonary Artery Hypertension
Note dilated right heart chambers and SVC
Enlarged central pulmonary arteries
NormallVC
 AZYGOS CONTINUATION OF IVC
o Early in life if associated with severe congenital
Enlargement of Azygos Arch due to High heart disease
Volume States Gender: No predilection
Note enlarged heart and prominent pulmonary vessels
Natural History & Prognosis
Normal or dilated IVC
Seen with pregnancy, sickle cell disease, renal disease Related to associated anomalies, particularly
congenital heart disease
2 Enlargement of Azygos-Region Lymph Node
Azygos arch and vein normal and separate from node
If inadvertently ligated at surgery may be lethal
Treatment
24 NormallVC
Related to associated anomalies, particularly
Occlusion of Intrahepatic IVC due to Tumor congenital heart disease
or Thrombosis May complicate surgical procedures such as liver
transplantation
Liver mass, especially hepatocellular carcinoma which
grows intravascularly
Infrahepatic IVC normal
I DIAGNOSTIC CHECKLIST
Right or Double Aortic Arch
Azygos arch and vein normal
Consider
IVC normal Difficulties may arise during catheter-based
intervention through the IVC such as with right heart
catheterization
I PATHOLOGY Image Interpretation Pearls
General Features Lateral chest radiograph may not show absence of the
retrocardiac IVC shadow due to drainage of the
Genetics: Sporadic
hepatic veins in that location
Etiology
o Persistence of the embryonic right supracardinal
vein
o Lack of development of the suprarenal part of the I SELECTED REFERENCES
subcardinal vein 1. Demos TC et al: Venous anomalies of the thorax. AJR Am J
Epidemiology Roentgenol. 182(5):1139-50, 2004
o Prevalence less than 0.6% 2. Yilmaz E et al: Interruption of the inferior vena cava with
azygos/hemiazygos continuation accompanied by distinct
o 0.2-4.3% of cardiac catheterizations for congenital
renal vein anomalies: MRA and CT assessment. Abdom
heart disease Imaging. 28(3):392-4, 2003
Associated abnormalities 3. Bass JE et al: Spectrum of congenital anomalies of the
o Polysplenia inferior vena cava: cross-sectional imaging findings.
Bilateral hyparterial bronchi and bilobed lungs Radiographics. 20(3):639-52, 2000
Midline liver 4. Gayer G et al: Polysplenia syndrome detected in
Multiple spleens adulthood: report of eight cases and review of the
Congenital heart disease: Atrial and ventricular literature. Abdom Imaging. 24(2):178-84,1999
5. Jelinek JS et al: MRI of polysplenia syndrome. Magn Reson
septal defects
Imaging. 7(6):681-6, 1989
o Rare in asplenia 6. Munechika H et al: Hemiazygos continuation of a left
Gross Pathologic & Surgical Features inferior vena cava: CT appearance. J Com put Assist
Tomogr. 12(2):328-30, 1988
Complicates surgical planning for 7. Cohen MI et aI: Accessory hemiazygos continuation of left
o Esophagectomy inferior vena cava: cr demonstration. J Com put Assist
o Liver transplantation Tomogr. 8(4):777-9,1984
o IVC filter placement 8. Schultz CL et aI: Azygos continuation of the inferior vena
o Abdominal aortic aneurysm repair cava: demonstration by NMR imaging. J Com put Assist
Tomogr. 8(4):774-6, 1984
9. Siegfried MS et al: Diagnosis of inferior vena cava
anomalies by computerized tomography. Comput Radiol.
IClINICAllSSUES 7(2):119-23,1983
10. Allen HA et al: Case report. Left-sided inferior vena cava
Presentation with hemiazygos continuation. J Comput Assist Tomogr.
Most common signs/symptoms: Often asymptomatic 5(6):917-20, 1981
Other signs/symptoms 11. Ginaldi S et al: Absence of hepatic segment of the inferior
o Symptoms related to congenital heart disease vena cava with azygous continuation. J Com put Assist
o May be associated with sick sinus syndrome Tomogr. 4(1):112-4,1980
12. O'Reilly RJ et al: The lateral chest film as an unreliable
Demographics indicator of azygos continuation of the inferior vena cava.
Age Circulation. 53(5):891-5, 1976
o Variable, often discovered incidentally
 AZYGOS CONTINUATION OF IVC

I IMAGE GALLERY

(Left) Axial CECT shows the

dilated azygos vein (arrow)
adjacent to the aorta. (Right)
Axial CECT shows the dilated
azygos vein (arrow) in a
2
retrocrurallocation. Note
25
absence of the intrahepatic
IVC.

Variant
(Left) Frontal radiograph
shows a focal bulge in the
lower right pararracheal
region (arrow),
corresponding to the dilated
azygos arch. (Right) Axial
CECT shows azygos
continuation (open arrow)
and multiple small spleens
(arrows) consistent with
polysplenia.

Variant
(Left) Axial MR cine shows
an enlarged hemiazygos vein
(arrow) in a patient with
hemiazygos continuation.
(Right) Axial MR cine shows
a hemiazygos arch (arrow)
connecting the hemiazygos
vein to a left SVC.
 AZYGOS FISSURE

2
26

Sagittal multiplanar reconsuuction shows che azygos Frontal radiograph shows a curvilinear shadow (arrows)
vein ascending in the posterior mediastinum on the right extending obliquely across che upper portion of che right
anterior aspect of the vertebral bodies (arrow). Azygos lung terminating in a "teardrop" shadow caused by che
arch is a/so seen (curved arrow). vein itself.

o Azygos fissure: Curvilinear shadow convex toward

ITERMINOLOGY the chest wall extending from the right
Definitions tracheobronchial angle to the apex of the right lung
A portion of the right upper lobe (RUL) limited by an o Lung limited by the fissure normally aerated
accessory fissure and supplied by branches of the o Increased density of azygos lobe may be seen
apical segment bronchus CT Findings
Variable in size o Arcuate linear opacity extending from the
Occasionally, azygos vein may have an posterolateral aspect of upper thoracic spine to the
intrapulmonary route creating an accessory fissure superior vena cava (SVC)
o Four layers of pleura invaginating into the lung apex o Traverse the lung before entering the SVC
o Trigone o Useful in excluding pathologic changes
Most cranial part of the fissure (triangular form) o Increased density azygos lobe
Determines the size of the lobe Overlapping tortuous supraaortic vessels
o Contains the azygos vein within its lower margin Increase in the depth of the soft tissues of the
upper mediastinum
In young infants: Thymus
I IMAGING FINDINGS
General Features I DIFFERENTIAL DIAGNOSIS
Radiographic findings
o Azygos vein: Oval shadow occupying the right Enlarged Paratracheal Nodes
tracheobronchial angle Widening of right para tracheal stripe
Tortuous Supraaortic Vessels
Widening mediastinum without tracheal displacement

DDx: Paratracheal Density

Calcified Node Lymph Node (CXR) Lymph Node (Cn

 AZYGOS FISSURE

Key Facts
Terminology Increased density of azygos lobe may be seen
A portion of the right upper lobe (RUL) limited by an Top Differential Diagnoses
accessory fissure and supplied by branches of the
Enlarged Paratracheal Nodes
apical segment bronchus
Right Upper Lobe Collapse
Imaging Findings Pathology 2
Azygos fissure: Curvilinear shadow convex toward the Azygos lobe: Normal variant in 0.4% of the
chest wall extending from the right tracheobronchial population
27
angle to the apex of the right lung
Lung limited by the fissure normally aerated

Right Upper lobe Collapse IClINICALISSUES

Elevated minor fissure
Secondary signs of volume loss: Tracheal shift,
Presentation
elevation right hilum, elevated right hemidiaphragm Most common signs/symptoms: None, usually
(often with juxtaphrenic peak) incidental radiographic finding

I PATHOLOGY I DIAGNOSTIC CHECKLIST

General Features Image Interpretation Pearls
Etiology: Failure of normal migration of the azygos Dense azygos lobe: Does not signify underlying disease
vein over the apex of the right lung Enlarged azygos vein: Rule out elevated central venous
pressure, congenital malformations (polysplenia
Epidemiology
o Azygos lobe: Normal variant in 0.4% of the syndrome & azygos continuation), or obstruction of
population the vena cava
o Azygos fissure: Seen in 10/0 of individuals
Associated abnormalities
o Dense azygos lobe I SELECTED REFERENCES
Potential diagnostic pitfall 1. Demos TC et al: Venous anomalies of the thorax. AJRAmJ
Can simulate pathology Roentgenol. 182:1139-50,2004
o Azygos vein dilatation 2. Lawler LP et al: Thoracic venous anatomy. Multidetector
Elevated central venous pressure: Cardiac row CT evaluation. Radiol Clin N Am. 41:545-560,2003
3. Caceres J et al: The azygos lobe: normal variants that may
decompensation, tricuspid stenosis, acute simulate disease. Eur] Radiol. 27:15-20, 1998
pericardial tamponade, constrictive pericarditis 4. Caceres] et al: Increased density of the azygos lobe on
Intrahepatic and extrahepatic portal vein frontal chest radiographs simulating disease: CT findings in
obstruction seven patients A]RAm] Roentgenol. 160:245-248, 1993
Anomalous pulmonary venous drainage 5. Dudiak CM et al: CT evaluation of congenital and acquired
Azygos continuation: Polysplenia syndrome abnormalities of the azygos system. RadioGraphies.
Acquired occlusion of SVC or inferior vena cava 11:233-246, 1991
(lVe)

I IMAGE GAllERY

(Left) Axial NECT shows the azygos fissure (arrows). This fissure is formed by four pleural layers (two parietal and two viscera/). (Center) Axial
NECT shows a moderately dilated azygos vein. (Right) Frontal radiograph shows increased density of azygos lobe due to overlapping tortuous
supraaorlic vessels (arrows). (Courtesy j. Caceres, MOJ.
 AORTIC ATHEROSCLEROSIS

2
28

Advanced pathologic stages of atheroscferosis. (IV) Frontal radiograph shows an ectatic aorta and
atheroma, (V) fibroatheroma, (VI) complicated lesion, atheroscferotic calcification (arrow). With normal aging
(VII) calcific lesion, (VIII) fibrotic lesion. the aorta looses elasticity and elongates. Because the
aorta is fixed, the aorta buckles with a tortuous course.

o Ascending aorta more often involved in diabetes

I TERMINOLOGY and familial hypercholesterolemia
Abbreviations and Synonyms o The aortic root may be involved in familial
Hardening of the arteries hypercholesterolemia
o Syphilis causes ascending aortic atherosclerosis
Definitions Size: Aortic caliber remains normal for many years but
Aortic atherosclerosis a form of degenerative disease of ultimately an aneurysm may occur
the arteries Morphology: Mural thickening and irregularity are
Begins in adolescence and progresses with age common
Causes progressive intimal thickening, leading to
Radiographic Findings
stenosis
Ultimately may cause mural thrombus and aneurysm Radiography
o Aorta may appear tortuous and contain mural
formation
calcification
o Calcification most often visualized in the aortic arch
and descending aorta
I IMAGING FINDINGS
CT Findings
General Features
CECT
Best diagnostic clue: Irregular mural thickening with
o Mural thickening due to thrombus formation
calcium on CT
o Mural thrombus typical
Location o Calcification common within mural thrombus
o Most common in descending aorta
o Visualized complications include aneurysm, rupture,
o Predilection for vessel branch points
dissection, penetrating ulcer

DDx: Atherosclerotic Mimics

Mural Dissection Takayasu Disease Aortic Metastasis

 AORTIC ATHEROSCLEROSIS
,
Key Facts
Terminology o Protruding atheroma and plaque> 4 mm is a risk
o Aortic atherosclerosis a form of degenerative disease factor for embolism
of the arteries Top Differential Diagnoses
o Begins in adolescence and progresses with age o Aortic Dissection
Causes progressive intimal thickening, leading to
o
stenosis
o
o
Thoracic Aortic Aneurysm
Takayasu Disease
2
o Ultimately may cause mural thrombus and aneurysm
formation Pathology 29
o Genetics: Familial hypercholesteremia a major risk
Imaging Findings
factor
o Best diagnostic clue: Irregular mural thickening with o Common in Western cultures
calcium on CT
o Most common in descending aorta Diagnostic Checklist
o Predilection for vessel branch points o Coronary artery disease in the setting of substantial
o Assessment of amount of ascending aortic aortic atherosclerotic. disease
atherosclerosis may be important for planning bypass o Distinction of aortic atherosclerosis from type B
surgery intramural dissection can be challenging

o In patients with atheroembolic phenomena, CT o Brachial artery reactivity is being investigated as a

useful to look for a source and provide an
assessment of the extent of atherosclerosis
o Assessment of amount of ascending aortic
atherosclerosis may be important for planning
bypass surgery
Angiographic Findings
o DSA
o Mural thrombus encroaches into aortic lumen
causing irregularity
o Mural thickening is difficult to evaluate
MR Findings
o TlWI
o Mural thrombus appears as medium to low signal
intensity protruding into aortic lumen
o Calcification difficult to visualize
o Visualize complications include aneurysm, rupture,
dissection
o T2* GRE
o Mural thrombus appears as low signal intensity
encroaching on aortic lumen
o Visualize complications include aneurysm, rupture,
dissection
oMRA
o Mural thrombus causes irregularity of aorta
o Visualize complications include aneurysm,
dissection, and sometimes rupture
o Investigational work ongoing to evaluate plaque
morphology based on tissue characteristics such as
lipid component and fibrin cap to stratify plaque
rupture risk
Ultrasonographic Findings
o Grayscale Ultrasound
o Mural thrombus causes wall thickening and
irregularity
o Protruding atheroma and plaque> 4 mm is a risk
factor for embolism
Used as a marker for stroke risk in the ascending
aorta
marker for vascular impairment by atherosclerosis
Imaging Recommendations
o Best imaging tool: CECT scan to determine extent of
plaque, evaluate aortic wall, and technique of choice
for complications of aneurysm and dissection
o Protocol advice: Intravenous contrast material
necessary to define extent of mural thrombus
I DIFFERENTIAL DIAGNOSIS
Aortic Dissection
o Intimal calcification displaced from aortic wall
o Hypertension main predisposing factor
Thoracic Aortic Aneurysm
o Saccular (20%) or fusiform dilatation of aorta
o Predisposing conditions: Atherosclerosis, trauma,
mycotic, cystic medial necrosis, bicuspid aortic valve,
hypertension, smoking
Takayasu Disease
o Granulomatous inflammatory vasculitis
o Wall-thickening of large and medium sized artery that
narrows the lumen
Aortic Sarcoma or Metastases
o Extremely rare
o Non-calcified discrete mass of aortic wall
o Hematogenous metastases common
I PATHOLOGY
General Features
o Genetics: Familial hypercholesteremia a major risk
factor
o Etiology
o Hypotheses include response to injury,
accumulation of excess lipid, and a monoclonal
tumor-like propagation
o Response to injury the leading theory
 AORTIC ATHEROSCLEROSIS
Injury caused by toxic agents such as low density o Ischemic symptoms of extremities or viscera due to
lipoprotein, products of smoking, and elevated branch stenoses or emboli
glucose Clinical Profile
In reaction, macrophages respond and the process o Hypertensive
causes decreased nitrous oxide production leading o Smoker
to a prothrombotic state o Diabetic
o Pathophysiologic correlates (Virchow triad) o Family history
2 Local vessel wall substrates such as atherosclerotic o Obese
plaques
30 Blood flow characteristics such as shear and stasis
Demographics
Circulating blood factors including hormonal Age: Correlates with advancing age, very common in
factors and blood elements elderly
o Risk factors Gender: M > F
Hypertension Natural History & Prognosis
Smoking Atherosclerosis progresses with age
Hypercholesterolemia Natural history and prognosis related to onset of
Diabetes mellitus complications
Obesity
Epidemiology Treatment
o Common in Western cultures Modification of risk factors
o Less common in Asia and Africa: Diet or genetic o Smoking cessation
factors may playa role o Diet modification and exercise
Associated abnormalities o Medical therapy especially with lipid lowering
o Aortic aneurysm agents
o Spontaneous aortic rupture o Results of trials using antioxidants to treat
o Aortic atheroembolic phenomena atherosclerosis have been inconclusive
o Aortic branch stenosis Medical or surgical therapy for complications
o Penetrating ulcer
o Predictive of coronary artery disease
Gross Pathologic & Surgical Features I DIAGNOSTIC CHECKLIST
Fatty streak: Deposition of intracellular fat in the Consider
intima Coronary artery disease in the setting of substantial
Fibrous plaque: Scar aortic atherosclerotic disease
Atheroma
o Involves intima and sometimes media Image Interpretation Pearls
o Common at branch sites Distinction of aortic atherosclerosis from type B
o May be complicated by thrombus, calcification, intramural dissection can be challenging
hemorrhage Type B intramural dissection typically begins
immediately distal to the subclavian artery and has a
Microscopic Features smooth interface with the aortic lumen
Fatty streak includes macrophages, T-lymphocytes, Atherosclerosis has a variable proximal point of origin
and smooth muscle cells and typically has an irregular interface with the aortic
Fibrous scar due to proliferation of smooth muscle lumen
cells
Atheroma has three components
o Myofibroblasts
o Lipid
I SELECTED REFERENCES
o Fibrin cap 1. Fayad ZA et al: Magnetic resonance imaging and computed
tomography in assessment of atherosclerotic plaque. CUff
o Plaque rupture leads to a thrombogenic state ~ Atheroscler Rep. 6(3):232-42, 2004
Interferon and metalloproteinase are produced 2. Yuan C et al: MRI of atherosclerosis. J Magn Reson
which impair collagen synthesis and cause its Imaging. 19(6):710-9,2004
degradation 3. Takasu J et aI: Aortic atherosclerosis detected with
electron-beam CT as a predictor of obstructive coronary
artery disease. Acad Radiol. 10(6):631-7,2003
ICLINICAllSSUES 4. Miller WT: Thoracic aortic aneurysms: plain film findings.
Semin Roentgenol. 36(4):288-94, 2001
Presentation 5. Rauch U et al: Thrombus formation on atherosclerotic
plaques: pathogenesis and clinical consequences. Ann
Most common signs/symptoms: Asymptomatic unless
Intern Med. 134(3):224-38,2001
complication occurs 6. Libby P: Changing concepts of atherogenesis. J Intern Med.
Other signs/symptoms 247(3):349-58,2000
o Chest or back pain due to aortic ulceration or 7. Tunick PA et al: Atheromas of the thoracic aorta: clinical
rupture and therapeutic update. J Am Coll Cardiol. 35(3):545-54,
2000
 AORTIC ATHEROSCLEROSIS

I IMAGE GALLERY
Typical
(Left) Axial CECT shows
calcified thrombus in the
descending aorta (arrow) 2
consistent with
atherosclerosis. (Right)
Sagittal oblique CECT shows 31
multiple discrete calcified
thrombus in the descending
aorta consistent with
atherosclerosis. Typically
ascending aorta is usually
spared

(Left) Axial CECT shows

protruding thrombus (arrow)
within the descending aorta
near the aortic hiatus. Large
plaques more likely to lead
to embolic complications.
(Right) Axial TlWI MR
shows irregularity of the wall
of the descending aorta
(arrow) consistent with
atherosclerosis. Calcification
is poorly depicted on MRI

Typical
(Left) Axial MR cine shows
mural irregularity of the
descending aorta (arrow)
consistent with
atherosclerosis. (Right)
Sagittal oblique angiography
shows marked irregularity of
the descending aorta with
ulceration (arrow) consistent
with extensive
atherosclerosis.
 MARFAN SYNDROME

2
32

Anteroposterior radiographshows prominent ascending Coronal CT reconstruction shows typical annuloaortic

aorta (arrows) and mild cardiomegaly in patient with ectasia in Marian syndrome. Sinocubular ridge is
known Marfan syndrome. obliterated and dilatation affects both the sinuses of
Valsalvaand ascending aorta.

Mitral valve prolapse (most prevalent valvular

ITERMINOLOGY abnormality seen in 35-100% of patients)
Definitions Mitral annulus calcification (in patients < 40 years
Inherited autosomal dominant disorder of connective of age)
tissue characterized by skeletal, cardiovascular and Main pulmonary artery dilatation (pulmonary
ocular abnormalities valve normal)
Dilatation/dissection of descending thoracic or
abdominal aorta
jlMAGING FINDINGS o Evidence of mild, but definite left ventricular
impairment, not related to valvular heart disease
General Features also reported
Best diagnostic clue o Skeletal abnormalities
o Diagnosis of Marfan syndrome based on Ghent o Major criteria
criteria which includes cardiovascular (major Pectus excavatum or carinatum severe enough to
criteria), ocular (major criteria) and pulmonary warrant surgery
(minor criteria) abnormalities Arm span to height ratio> 1.05
o Cardiovascular and valvular disease: Seen in Scoliosis> 20 or spondylolisthesis (60%)
majority (> 90%) of patients and dominant cause of Limited elbow extension 170)
mortality Pes planus
o Major criteria Protrusio ace tabu Ii
Annuloaortic ectasia: Prevalence 75% Dural ectasia
Ascending aortic dissection o Minor criteria
o Minor criteria Joint hypermobility

DDx: Syndromes Associated with Aortic Disease

Neurofibromatosis Ankylosing Spondylitis Relapsing Polychondritis

 MARFAN SYNDROME

Key Facts
Terminology Top Differential Diagnoses
Inherited autosomal dominant disorder of connective Neurofibromatosis Type 1
tissue characterized by skeletal, cardiovascular and Ankylosing Spondylitis
ocular abnormalities Relapsing Polychondritis
Imaging Findings Pathology 2
Cardiovascular and valvular disease: Seen in Autosomal dominant inheritance with complete
majority (> 90%) of patients and dominant cause of penetrance but variable expression 33
mortality Prevalence 1 in 3-5000 population
Annuloaortic ectasia: Prevalence 7S% Annuloaortic ectasia: Uniform dilatation all 3 sinuses
Mitral annulus calcification (in patieqts < 40 years of of Valsalva extending into the ascending aorta
age) obliterating the normal sino tubular ridge
Ectopia lentis (50%)
Annuloaortic ectasia: Pear-shaped dilatation of the Diagnostic Checklist
sinuses of Valsalva extending into the ascending Long term surveillance following aortic graft repair
aorta paramount; nearly 50% of late deaths due to
anastomotic pseudoaneurysms

Pectus excavatum or carinatum of moderate Dissecting aortic aneurysm

severity
High arched palate
o Other skeletal features include: Arachnodactyly,
long toes, thoracic lordosis
o Pulmonary disease
o Minor criteria
Apical blebs
Pneumothorax (recurrent and bilateral):
Prevalence 5%
o Other reported pulmonary problems include:
Emphysema, recurrent lower respiratory tract
infections, upper lobe fibrosis (rare)
o Ocular abnormalities
o Major criteria
Ectopia lentis (50%)
o Other features include: Myopia, flattening of cornea,
iris or ciliary muscle hypoplasia
o Other diagnostic criteria include
Family history of first-degree relative who
independently fulfills diagnostic criteria
Lumbosacral dural ectasia (major criterion)
Recurrent/incisional hernias (minor criteria)
Striae atrophicae but not in context of weight
change or pregnancy (minor criterion)
Radiographic Findings
Radiography Neurofibromatosis
o Chest radiograph
Long elongated thorax with large-volume lungs
Dilatation ascending aorta
Cardiomegaly (due to aortic or mitral
regurgitation, or spurious from pectus deformity)
Pectus deformity, either excavatum or carinatum
Apical blebs
Scoliosis and scalloping vertebral bodies
CT Findings
CTA
o More sensitive than chest radiography for
Annuloaortic ectasia: Pear-shaped dilatation of the Ankylosing Spondylitis
sinuses of Valsalva extending into the ascending
aorta
Subpleural blebs
Dural ectasia
MR Findings
Similar to CT in sensitivity for major cardiovascular
complications
Better than CT for valve assessment
Nonionizing radiation major advantage in following
aortic root dilatation in young individuals
Echocardiographic Findings
Standard surveillance for aortic root dilatation and
assessment of aortic and mitral valve function
Imaging Recommendations
Best imaging tool
o Plain radiography for detection of skeletal anomalies
o Serial chest radiography for demonstration of
progressive aortic dilatation
o 2D echocardiography for early diagnosis and
monitoring of ascending aortic dilatation
o Multi-detector row CT or magnetic resonance
angiography for evaluation of aortic disease
I DIFFERENTIAL DIAGNOSIS
Type 1
Inherited autosomal dominant neurocutaneous
syndrome
Also have dural ectasia and gibbus deformity (typically
at thoracolumbar junction), lateral meningocele
Essential hypertension (or rarely from
pheochromocytomas), coarctation of the aorta
Pulmonary valve stenosis
Optic gliomas
Ribbon ribs, rib notching
Basilar interstitial lung disease concomitant with
upper lobe bullous lung disease (rare)
Seronegative arthritis possibly genetically related with
predilection for the axial skeleton
 MARFAN SYNDROME
Syndesmophyte spinal fusion
Aortitis may lead to aortic regurgitation
Fibrocystic upper lobe disease 2%)
Relapsing Polychondritis
Rare autoimmune inflammatory condition involving
cartilage of the ear, tracheobronchial tree, eye,
2 cardiovascular system and peripheral joints
Tracheal involvement in 50%
Also associated with aortic dissection and aortic or
34
mitral regurgitation (25%)
Ehlers-Danlos Syndrome
Genetic defect in collagen and connective tissue,
much rarer than Marfan
Affects skin, joints and blood vessels
May also have aortic aneurysm, dissection and mitral
valve prolapse
I PATHOLOGY
General Features
Genetics
o Autosomal dominant inheritance with complete
penetrance but variable expression
No family history in 25% of patients
o Mutation in FBN1 gene (encoding for large
[approximately 350 kDa] glycoprotein called
fibrillin-1, a major component of microfibrils)
o Animal studies demonstrate dysregulation of
transforming growth factor-beta (TGF-~) activation
and signaling, leading to apoptosis in developing
lung
Epidemiology
o Prevalence 1 in 3-5000 population
Common genetic malformation
Gross Pathologic & Surgical Features
Annuloaortic ectasia: Uniform dilatation all 3 sinuses
of Valsalva extending into the ascending aorta
obliterating the normal sinotubular ridge
o Most aortic diseases do not cross the sinotubular
ridge but involve either the sinuses or the ascending
aorta
o Also seen with Ehlers-Danlos and homocystinuria
Microscopic Features
Cystic medial necrosis
ICLINICAllSSUES
Presentation
Most common signs/symptoms
o Cardiovascular disease
Acute onset chest pain (aortic dissection)
o Pulmonary disease
Abrupt onset shortness of breath (spontaneous
pneumothorax)
Dyspnea on exertion, substernal chest pain (severe
pectus excavatum)
o Ocular
Loss of vision (lens dislocation)
Demographics
Gender
o None
However, aortic root dilatation more common in
men
Natural History & Prognosis
Morbidity and mortality due to cardiovascular disease
(aortic dissection)
o Poor outlook before modern advances in cardiac
surgery
o Average age death untreated 35 years of age
Prognosis improved with increased vigilance, plus
current medical and surgical intervention
o Average life expectancy up to 70 years
Pregnant women at significant risk for aortic
dissection
o Surveillance echocardiography or MRI every 6-10
weeks during pregnancy
Treatment
Screening important for early detection of aortic
vascular complications
B-adrenergic blocking drugs (valuable in some
patients)
o Slows the rate of aortic dilatation
o Lowers incidence of aortic regurgitation, dissection
or need for surgery, congestive cardiac failure
o Improves survival
Exercise restriction
o Avoidance of contact sports and isometric exercises
Surgical intervention
o Prophylactic aortic root surgery recommended when
aortic diameter reaches 5 cm because of increased
risk of ru pture or dissection
o In subjects with smaller aortic diameters, surgery
still indicated if rapid growth (> 1 cm/year), a family
history of dissection and moderate to severe aortic
regurgitation
o Surgical options include
Composite valve graft repair
Valve-sparing aortic root replacement
o Genetic counseling: 50% offspring will have disease
I DIAGNOSTIC CHECKLIST
Consider
Diagnosis to be considered in patients with positive
family history and combinations of cardiovascular,
skeletal, ocular and possibly pulmonary disease
Image Interpretation Pearls
Long term surveillance following aortic graft repair
paramount; nearly 50% of late deaths due to
anastomotic pseudoaneurysms
I SElECTED REFERENCES
1. Boileau C et al: Molecular genetics of Marfan syndrome.
Curr Opin Cardiol. 20(3):194-200, 200S
2. De Backer JF et al: Primary impairment of left ventricular
function in Marfan syndrome. Int J Cardiol. 200S
 MARFAN SYNDROME

I IMAGE GALLERY

(Left) Frontal radiograph

shows prominent ascending
(arrows) and descending 2
aorta (open arrows) in
patient with Madan
syndrome. Thorax is 35
elongated. (Right) Lateral
radiograph shows marked
dilatation of ascending aorta
(arrows) from annuloaortic
ectasia.

Typical
(Left) Coronal CECT
muftipfanar reconstruction
shows annuloaorUc ectasia
(arrows) and an ascending
aortic dissection with an

/.'~'"
..
intimal flap (curved arrow)
in a patient with Madan
syndrome. (Right) Axial
(/ - ~ CECT shows intimal flap

o . ~. "-' . Q from dissection

ascending
in the
aorta (arrow).
Note the dural ectasia with

" Ae .. PI scalloping of the vertebral

body (open arrow) in patient
"----- ~? ~./ with Madan syndrome.

Typical
(Left) Axial CECT shows
severe pectus (open arrow)
markedly displacing the
heart and compressing the
right atrium in patient with
Madan syndrome. (Right)
Coronal oblique CECT
reconstruction shows aortic
graft repair (arrows) of
ascending aortic aneurysm in
Madan syndrome.
Surveillance for
pseudoaneurysm important
in Madan patients.
 AORTIC DISSECTION

2
36

Stanford type A aorUc dissecUon (OeBakey types I and Coronal CECT shows nearly thrombosed false lumen in
II) involves ascending aorta and is surgically repaired. descending dissecUon (Stanford B). True lumen is
Type B (or type III) involves the descending aorta and is smaller than false lumen. Intimal flap (arrow). No
treated medically. Intimal flap (arrow). pleural effusion to suggest rupture.

o Widened superior mediastinum 75%

ITERMINOlOGY o Sensitivity 80%, specificity 80%
Abbreviations and Synonyms o Nonspecific
Aortic tear, aortic intramural hematoma Double aortic knob sign (40%)
Disparity in size between ascending and
Definitions descending aorta
Spontaneous intimal tear with propagation of Mediastinal mass effect, tracheal shift, depression
subintimal hematoma left main bronchus
Chronic dissection after 2 weeks Progressive aortic enlargement on serial chest
Aortic intramural hematoma: No intimal flap, radiographs
spontaneous medial hematoma secondary to Left apical cap
infarction of the vasa va sorum Cardiomegaly
Left pleural effusion suggests aortic rupture
o Most specific finding
I IMAGING FINDINGS Ring sign: Displaced intimal calcification from
aortic wall> 1 cm in SOlo
General Features False positives due to projection of intimal
Best diagnostic clue: Chest radiograph: Displaced calcification over aorta at a different location;
intimal calcification from aortic wall other processes such as fat may create false aortic
Location: Ascending aorta integrity primary question wall separated from calcified intima; or aortic wall
may be thickened from aortitis
Radiographic Findings
Radiography CT Findings
a Normal in 25% NECT

DDx: Aortic Dissection

Thoracic Aneurysm Atherosclerosis Aortic Transection

 AORTIC DISSECTION
,-------------------,- ,------------------------------.
Key Facts
Terminology Post-Stenotic Dilatation from Aortic Valve Stenosis
Spontaneous intimal tear with propagation of Lymphoma
subintimal hematoma Mediastinal Germ-Cell Tumors
Aortic intramural hematoma: No intimal flap, Pathology
spontaneous medial hematoma secondary to
infarction of the vasa vasorum
Greatest hydraulic stress right lateral wall ascending
aorta or descending aorta in proximity of
2
Imaging Findings ligamentum arteriosum 37
Ring sign: Displaced intimal calcification from aortic Intimal tear spirals with false lumen lying anterior
wall> 1 em in 5% and right in the ascending aorta and posterior and
Best imaging tool: Cross-sectional imaging now left in the descending aorta
accepted as primary acute imaging modality Stanford classification (preferred classification)

Top Differential Diagnoses Clinical Issues

Without treatment mortality rate 1-2% per hour first
Thoracic Aneurysm
Tortuosity (Aging) of the Aorta 48 hours
Aortic Transection

o Displacement of calcified intima o Good for extent of dissection and involvement of

o Acute intramural hematoma: Crescentic
high-attenuation clot in aortic wall
o IV contrast required to definitively characterize flap
CECT
o 95% accurate
o "Double barrel" spirals down the aorta with true and
false lumen
o Branch vessel involvement well demonstrated
o Delayed contrast passage through false lumen
o Which is true and which is false lumen?
Connect true lumen with non-dissected portion
on sequential images
o False lumen
Beak sign: Acute angle between the dissected flap
and the outer wall, angle may contain thrombus
Cobwebs: Thin strands crossing lumen
Intraluminal thrombus: Entire lumen may be
thrombosed
Largest lumen usually the false lumen
o Complete circumferential stripping (360) may result
in intimo-intimo intussusception
o Obstruction aortic branch vessels (left renal artery
most common - 25%)
o Pericardial effusion ominous finding suggesting
dissection rupture into pericardial sac
o Pitfalls
False negatives: Poor contrast-enhancement
False positives: Streak artifacts
Angiographic Findings
Intimal flap detected in 90%
Delayed filling of false lumen, flow typically slower in
false lumen
Displacement of catheter from apparent aortic wall by
false lumen
Highly accurate for entry and exit sites
MR Findings
TlWI: Cardiac-gated black blood sequence> 95%
accurate
MRA
branch vessels
o Useful to quantitate aortic regurgitation
Echocardiographic Findings
Transthoracic echo 60-85% sensitive
Transesophageal echo> 95% accurate
Highly accurate for demonstrating aortic valve
involvement
Imaging Recommendations
Best imaging tool: Cross-sectional imaging now
accepted as primary acute imaging modality
Protocol advice: Multidetector CT 1-2.5 collimation,
initial noncontrast scan to evaluate for wall
hemorrhage
I DIFFERENTIAL DIAGNOSIS
Thoracic Aneurysm
Saccular (20%) or fusiform (80%) dilatation of aorta
Tortuosity (Aging) of the Aorta
Normal aging: Loss of elasticity elongates aorta;
because aorta fixed, the aorta buckles with a tortuous
course
No displacement intimal calcification
Aorta not dilated
Aortic Transection
Chronic pseudoaneurysm seen in 5% of aortic
transections
Usual location at aortic isthmus
Calcified mass aortopulmonary window
Post-Stenotic Dilatation from Aortic Valve
Stenosis
Involves ascending aorta
Aortic valve may be calcified
Lymphoma
Lobulated mediastinal contours
Not calcified prior to treatment
 AORTIC DISSECTION
a Type 3: Descending only (40-50%) A: Extends to
Mediastinal Germ-Cell Tumors diaphragm, B: Descends below diaphragm
Rapid enlargement from spontaneous hemorrhage
mimics dissection or aneurysm
Calcification focal, not curvilinear IClINICAllSSUES
Presentation
2 I PATHOLOGY Most common signs/symptoms
o Sudden onset chest or back pain 80-90% often
38
General Features described as "ripping"
General path comments: Transverse tear in weakened Ischemic heart disease 1000x more common
intima 95%, no intimal tear in 5% o Murmur in 65% secondary to aortic regurgitation
Genetics: Autosomal dominant Marfan syndrome Other signs/symptoms
Etiology a Discrepancy between extremity pulses
o Hypertension: 60-90% of dissections have elevated a Neurologic deficits (20%)
blood pressure Syncope and altered mental status
o Collagen disorders a Silent dissections uncommon (10%), more common
Marfan autosomal dominant in Marfan syndrome
Ehlers-Danlos a Hypotension ominous finding, suggests
o Pregnancy Cardiac tamponade or hypovolemia from rupture
50% of dissections in women occur during a Occlusion aortic branch vessels
pregnancy Renal failure, mesenteric ischemia; lower
a Congenital extremity ischemia
Bicuspid aortic valve
Aortic coarctation Demographics
Polycystic kidney disease Age: Peak age 60 years
Turner syndrome, Noonan syndrome Gender: M:F = 3:1
Osteogenesis im perfecta Ethnicity: More common in blacks
Hypercholesterolemia, homocystinuria
o Crack cocaine use Natural History & Prognosis
o Trauma Without treatment mortality rate 1-2% per hour first
Rare cause of dissection 48 hours
Epidemiology: Incidence 6 cases per 100,000 Long term survival in patients with operative
person-years management 50%
Pathogenesis Treatment
a Greatest hydraulic stress right lateral wall ascending
Type A: Surgical placement of tubular interposition
aorta or descending aorta in proximity of
graft
ligamentum arteriosum
a Aortic regurgitation (50%) may require valve
Gross Pathologic & Surgical Features replacement
Transverse tear in weakened intima 95%, no intimal a Operative mortality < 10%
tear in 5% o Medical treatment mortality rate 60%, surgical
Intimal tear spirals with false lumen lying anterior and treatment mortality 30%
right in the ascending aorta and posterior and left in Type B
the descending aorta a Control hypertension
Dissection usually stops at an aortic branch vessel or at a Surgery if dissecting aneurysm larger than 5 cm or
the level of an atherosclerotic plaque increasing in size by > 1.0 cm per year
a Medical treatment mortality 10%, surgical mortality
Microscopic Features 30%
Cystic medial necrosis from aging, atherosclerosis or Aortic intravascular fenestration for end organ
inherited disorders (Marfan) ischemia
o Breakdown of collagen, elastin, and smooth muscle a Needle advanced from the true lumen to the false
with an increase in basophilic ground substance lumen with balloon dilatation of tract
o Site chosen as close as possible to compromised
Staging, Grading or Classification Criteria arteries
Stanford classification (preferred claSSification) Follow-up examinations
a Type A: Originates in ascending thoracic aorta o 3-6 months for 2 years and then annually
(60-70%)
o Type B: Originates distal to left subclavian artery
(30-40%) I SElECTED REFERENCES
DeBakey classification
o Type 1: Ascending and descending thoracic aorta 1. Willoteaux S et al: Imaging of aortic dissection by helical
computed tomography (CT). Eur Radial. 14(11):1999-2008,
(30-40%)
2004
o Type 2: Ascending only (10-20%)
 AORTIC DISSECTION

I IMAGE GALLERY

(Left) Frontal radiograph

shows widened
mediastinum. Aortic intimal
calcification (arrow) is
displaced more than I cm
2
from the outer aortic wall in
39
this dissection. Opacity at
the left base atelectatic lung.
(Right) Axial CECT shows
intimal flap in ascending
aorta. Contrast extravasation
into pericardium (arrows).
Middle mediastinum is
diffusely infiltrated by blood.
Patienl expired.

(Left) Fronlal radiograph

shows in patienl with sudden
anterior tearing chest pain.
Ascending aorta is enlarged
(arrows). Differential
includes aortic valve stenosis
or aortic dissection. (Right)
Coronal CECT shows intimal
flap in ascending aorta
(Slanford A). BOlh the false
and true lumen opacified
with contrast. No pericardia I
effusion. Flap slarls a couple
of em above aortic valve
(arrow), a Iypicallocation.

(Left) Axial CECT shows

descending aortic aneurysm
with aneurysm difatation of
false lumen (arrows) althe
aortic hiatus. False lumen
larger, less opacified and
conlains Ihrombus. (Right)
Axial CECT shows intimal
flap in dissection (Slanford
B) extending into an aberrant
right subclavian artery
(arrow). Dislalsubclavian is
Ihrombosed. Dissection
often stop at branch vessel
or plaque.
 AORTIC ANEURYSM

2
40

Crawford classification. (I) descending thoracic and Sagittal CTA shows fusiform aneurysm of the
proximal abdominal aorta, (II) entire descending descending aorta. Large thrombus is eccentric in the
thoracic and abdominal aorta, (III) distal descending lumen (arrows). Atherosclerotic calcified plaque
and abdominal aorta, (IV) abdominal aorta. (curved arrows).

Eccentric focal dilatation of the aorta often filled

ITERMINOlOGY with thrombus
Abbreviations and Synonyms o Fusiform (80%)
Thoracic aneurysm Circumferential long segment spindle-shape
dilatation of the aorta
Definitions
Localized or diffuse dilation of aorta with a diameter at
Radiographic Findings
least 50% greater then the normal artery size Radiography
Classification by type (true or false), location, o Normal
morphology, and etiology Ascending aorta < 4 cm in diameter
Descending aorta < 3 cm in diameter
Aging: Loss of elasticity elongates aorta; because
I IMAGING FINDINGS aorta fixed, the aorta buckles with a tortuous
course
General Features o Curvilinear calcification clue to vascular origin
Best diagnostic clue: Mediastinal mass with curvilinear o Left pleural effusion suggests rupture
rim calcification o Calcification ascending aorta
Location: 3 groups: Ascending aorta, aortic arch, Typical atherosclerotic plaque uncommon
descending aorta Syphilis or type II hyperlipidemia
Size: Saccular aneurysm> 6.5 cm significant risk for Presents embolic risk at coronary artery bypass
rupture surgery
Morphology o Ascending aorta aneurysm
o Saccular (20%) Right para mediastinal anterior mass with convex
border

DDx: Thoracic Aneurysm

Aging Aortic Stenosis Patent Ductus Arteriosus

 AORTIC ANEURYSM

Key Facts
Terminology Pathology
Localized or diffuse dilation of aorta with a diameter General path comments: True aneurysm composed of
at least 50% greater then the normal artery size all layers of aortic wall, false aneurysm represents a
contained perforation of wall
Imaging Findings Greatest hydraulic stress right lateral wall ascending
Size: Saccular aneurysm> 6.5 cm significant risk for aorta or descending aorta in proximity of 2
rupture ligamentum arteriosum
NECT: Crescent sign: Peripheral high mural 41
Marfan: Ascending aortic aneurysm that involves the
attenuation indicating impending rupture due to aortic root (annuloaortic ectasia), aortic valve
acute intramural hematoma insufficiency common when aortic root diameter
Top Differential Diagnoses exceed 5 cm
Prevalence 3-4% in those older than 65 years
Tortuosity (Aging) of the aorta
Post-Stenotic Dilatation from Aortic Valve Stenosis Diagnostic Checklist
Patent Ductus Arteriosus Any mediastinal mass should be considered as a
Achalasia vascular aneurysm, a needle or scalpel could find a
surprise

a Aortic arch aneurysm

Enlarged or obscuration aortic arch
I DIFFERENTIAL DIAGNOSIS
Focal left paramediastinal mass obliterating Tortuosity (Aging) of the aorta
aorticopulmonary window No displacement intimal calcification, not dilated
Rightward tracheal deviation
a Descending aorta aneurysm Post-Stenotic Dilatation from Aortic Valve
Focal or diffuse left paramediastinal or posterior Stenosis
mediastinal mass Involves ascending aorta
CT Findings Aortic valve may be calcified
NECT: Crescent sign: Peripheral high mural Patent Ductus Arteriosus
attenuation indicating impending rupture due to acute Shunt vascularity or pulmonary artery hypertension
intramural hematoma Enlarged aortic arch and obliteration aorto-pulmonary
CECT window
a Annuloaortic ectasia
Classic pear-shape due to dilatation of sinuses of Achalasia
Valsalva and proximal ascending aorta Air-fluid level upper mediastinum
Normally sharp demarcation between normal and Absent gastric air bubble
abnormal aorta
Angiographic Findings
I PATHOLOGY
DSA
a Images the aortic lumen, may underestimate true General Features
size of aorta with circumferential thrombus General path comments: True aneurysm composed of
a Largely replaced by CT or MR which are noninvasive all layers of aortic wall, false aneurysm represents a
MR Findings contained perforation of wall
Can be used to evaluate for aortic valvular Genetics
a Inherited connective tissue disorders
regurgitation
Marfan autosomal dominant
More sensitive to detect hematoma in penetrating
a Usually inheritance unrelated to specific syndrome,
ulcer
however, of first degree relatives 15% will have an
Useful for serial surveillance of those at risk, (e.g.,
aneurysm
Marfan syndrome)
Etiology
Imaging Recommendations a Greatest hydraulic stress right lateral wall ascending
Best imaging tool: CECT to determine the location, aorta or descending aorta in proximity of
extent, and size of aneurysm, its relationship to major ligamentum arteriosum
branch vessels, and detect complications of dissection, Medial layer responsible for tensile strength and
mural thrombus, intramural hematoma, free rupture elasticity
Laplace law: Arterial wall tension (T) = pressure (P)
x radius (R)
Vicious circle: As diameter increases, wall tension
increases which leads to further increase in
diameter
 AORTIC ANEURYSM
o Predisposing conditions: Atherosclerosis, trauma,
mycotic, cystic medial necrosis, bicuspid aortic
valve, hypertension, smoking
o Atherosclerosis most common etiology (75%)
Usually does not involve ascending aorta
o Trauma
False aneurysm aortic isthmus, 5% of aortic
2 transections present late as false aneurysms
o Mycotic
42 Syphilis (luetic), ascending aortic aneurysm that
spares aortic root, occurs in 10% of untreated
patients
Bacterial: Saccular eccentric rapidly enlarging
aneurysm, usually false aneurysm
o Connective tissue disorder and cystic medial
degeneration
Marfan: Ascending aortic aneurysm that involves
the aortic root (annuloaortic ectasia), aortic valve
insufficiency common when aortic root diameter
exceed 5 cm
Ehlers-Danlos syndrome
o Vasculitis
Aneurysm unusual
o Annuloaortic ectasia
Classically Marfan, also seen in ankylosing
spondylitis, and Reiter syndrome
Epidemiology
o Prevalence 3-4% in those older than 65 years
o Incidence 6 cases per 100,000 person-years
Gross Pathologic & Surgical Features
Cystic medial degeneration most common cause of
thoracic aneurysms
Microscopic Features
Diseased intima with secondary degeneration and
fibrous replacement of media
Common organisms in mycotic aneurysms:
Staphylococcus aureus, Salmonella and Streptococq.Js
Staging, Grading or Classification Criteria
Crawford classification
a Type 1: Aneurysm from left subclavian artery to
renal arteries
o Type II: Aneurysm from left subclavian artery to
aortic bifurcation
a Type III: Aneurysm from mid-descending aorta to
aortic bifurcation
o Type IV: Aneurysm from upper abdominal aorta and
all or none of the infrarenal aorta
I CLINICAL ISSUES
Presentation
Most common signs/symptoms
o Asymptomatic and accidentally discovered on
routine chest radiographs
o Most common presenting symptom: Pain
May be localizing, anterior from ascending
aneurysm, neck pain from arch aneurysm, and
mid-scapular pain from descending aorta
o Most common complication: Life-threatening
hemorrhage
Other signs/symptoms
o Large aneurysms may compress mediastinal
structures: Airways, superior vena cava, esophagus,
pulmonary arteries, nerves
o Rupture
Into pericardium (ascending aorta)
Left hemithorax (descending aorta)
o Peripheral embolization
Central nervous system stroke or transient
ischemic attacks, organ or bowel ischemia,
extremity ischemia
Demographics
Age: Increased prevalence in each decade of life
Gender: Men greater than women
Natural History & Prognosis
Growth rates average 0.07 cm/year in ascending aorta
Growth rates average 0.19 cm/year in descending aorta
5 year survival of untreated aneurysms 20%
Survival less than 20% for aneurysms that rupture
outside the hospital
Treatment
Options, risks, complications
o Stroke risk higher for ascending aorta or arch repair
o Spinal cord injury from descending aorta repair
Risk reduction
o Control hypertension
o Smoking cessation
Indications for surgery
o Based on size, growth rate, or symptoms
Size criteria
a Repair ascending aneurysms> 5.5 cm (5 cm for
familial or Marfan syndrome)
o Repair descending aneurysms> 6.5 cm (6 cm for
familial or Marfan syndrome)
Growth rate criteria> 1 cm per year
Symptomatic patients repaired regardless of size
Surgical repair
o Surgical repair usually involve synthetic grafts
o May have to replace aortic valve for aneurysms that
involve the sino-tubular ridge
o Mortality rates: Ascending aneurysms 5-10%, aortic
arch 25%, descending aorta 5-15%
Intravascular stents promising
Yearly serial CT or MR evaluation of the aorta for
recurrent aneurysms
I DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Any mediastinal mass should be considered as a
vascular aneurysm, a needle or scalpel could find a
surprise
I SELECTED REFERENCES
1. LePage MA et al: Aortic dissection: CT features that
distinguish true lumen from false lumen. A)R. 177:207-11,
2001
2. Pasniak HV et al: CT of thoracic aortic aneurysms.
Radiographies 10:839-55, 1990
 AORTIC ANEURYSM

I IMAGE GALLERY

(Left) Frontal radiograph

shows focal left mediastinal
mass. No definite
calcification. Proximal edge
of descending aorta
2
silhouetted. Differential must
43
include aneurysm. (Right)
Anteroposterior angiography
shows that the mass is a
saccular aneurysm. Etiology
would include
atherosclerosis,
post-traumatic, and mycotic.

fLeft) Anteroposterior
radiograph shows large left
mediastinal rounded mass
with sharp border.
Differential would include
aneurysm. (Right) Axial
CECT shows that the mass is
a ductus aneurysm
containing thrombus. Small
left pleural effusion raises the
possibility of leakage
(arrow).

(Left) Frontal radiograph

shows enlarged ascending
aorta with thick rim of
curvilinear calcification
(arrows). Old right rib
fractures. (Right) Lateral
radiograph better
demonstrates the extensive
calcification of the ascending
aorta (arrows). Descending
aorta normal. Syphilis (luetic
aneurysm). Differential
includes type"
hyperlipidemia.
 TAKAYASU DISEASE

2
44

Axial T1WI MR shows abnormal high signal within the Sagittal oblique DSA shows patency only of the
left common carotid artery (arrow) consistent with subclavian artery with occlusion of the innominate and
occlusion. left subclavian arteries (arrows).

o Distribution usually patchy, symmetric great vessel

ITERMINOlOGY distribution common
Abbreviations and Synonyms Size: Essentially only vasculitis to involve aorta and
Takayasu arteritis, pulseless disease, aorta arch major vessels
syndrome, reverse coarctation, young female arteritis Morphology: Wall thickening of large and medium
vessels
Definitions
Radiographic Findings
Granulomatous inflammatory vasculitis affects walls
of medium and large vessels, especially aorta and Radiography: Chest radiograph may show premature
branches calcification of the aorta and rib notching due to
o Described in 1908 by a Japanese ophthalmologist formation of collateral vessels
Diagnosis may be problematic and delayed due to CT Findings
smoldering nature of disease CTA: Multiplanar reconstruction useful for evaluation
stenosis
CECT
I IMAGING FINDINGS o Great vessel changes
General Features Stenosis or coarctation most common
Wall thickening, often concentric
Best diagnostic clue: Smooth narrowing of aorta and
Wall may enhance
ma jor vessels
Aneurysm
Location Calcification (dystrophic) of wall, different than
o Aorta and branches
arteriosclerotic plaque
Left subclavian most common
o Location: Aorta, subclavian arteries, carotid arteries,
o Occasional pulmonary artery involvement
and renal arteries

DDx: Takayasu Mimics

Coarctation Aortoocclusive Disease Aortic Aneurysm

 TAKAYASU DISEASE

Key Facts
Terminology Aortic Aneurysm
Granulomatous inflammatory vasculitis affects walls Fibromuscular Dysplasia
of medium and large vessels, especially aorta and Middle Aortic Syndrome
branches Pathology
Imaging Findings Most common in Asian countries
Thickening of the large and medium size vessels
2
Best diagnostic clue: Smooth narrowing of aorta and
major vessels Clinical Issues 45
Occasional pulmonary artery involvement Early phase inflammatory or prepulseless phase
Distribution usually patchy, symmetric great vessel Late phase occlusive or pulseless
distribution common Age < 30 in 90% of patients
Size: Essentially only vasculitis to involve aorta and Gender: M:F = 1:8, but may be less female
major vessels predominant in non-Asian countries
Top Differential Diagnoses Morbidity and mortality are due to hypertension and
Aortic Coarctation stroke
Other Vasculitis Corticosteroids are mainstay of therapy

a Distribution patchy, symmetrical involvement of

great vessels common
Imaging Recommendations
Best imaging tool
Angiographic Findings a CT and MR have generally replaced angiography for
Conventional diagnosis
a Focal areas of narrowing and occasionally dilatation Angiography higher morbidity in pediatric
Aortic involvement in 75%; abdominal aorta in patients
53% Protocol advice: Multiplanar and volumetric
Left subclavian in 55% reconstructions may be valuable to assess wall
Right subclavian in 38% thickness and occlusions
Left common carotid in 30%
Right common carotid in 15%
Renal artery in 38% I DIFFERENTIAL DIAGNOSIS
Coronary artery in 15%
Pulmonary arteries may be involved (15-70% Aortic Coarctation
depending on series) Characteristic location in post-ductal variety is at the
Mesenteric involvement occasionally, especially ligamentum arteriosus
superior mesenteric artery Rib notching common
a Stenotic areas focal, smooth More common in males
a Lesions often symmetric in great vessels
Other Vasculitis
a Distribution of lesion often patchy
Giant cell or temporal arteritis typically affects
MR Findings medium size cranial vessels in older patients
TlWI Aortic Aneurysm
a Great vessel changes similar to CT
Occurs in men more than women, most> 40 years
Calcification less well demonstrated than CT
a Enhancement of wall on fat-suppressed Mural calcification common
Wall thickening eccentric and due to thrombus
post-contrast images suggests disease activity
MRA: Areas of focal stenosis or occlusion involving the Fibromuscular Dysplasia
aorta and great vessels Artery is beaded
Nuclear Medicine Findings Spares aorta
PET Middle Aortic Syndrome
a May show increased activity in areas of active Neurofibromatosis type 1: Dural ectasia, ribbon ribs
inflammation William syndrome: genetic disorder: Elfin facial
a Decreasing activity may be useful to monitor features, neonatal hypercalcemia, supravalvular aortic
treatment response stenosis, behavioral disorder
Ultrasonographic Findings Rubella more often affect middle and distal aorta
Grayscale Ultrasound Syphilitic Aortitis
a Shows thickening of carotid artery walls Rare condition that affects ascending aorta
a May distinguish from atherosclerosis by paucity of predominantly
plaque formation Spares aortic root
 TAKAYASU DISEASE
10% untreated patients
Kawasaki Disease
Typically younger age with predominance in coronary
arteries
Mucocutaneous lymph node syndrome: vasculitis of
unknown etiology
2 Difference of at least 10 mm Hg in systolic blood
46 I PATHOLOGY
General Features
Genetics
o May have a hereditary component but not
confirmed
Postulated link with various human leukocyte
antigens (HLA) subtypes, HLA-B22
Etiology: Unknown but may be CD4 T-cell mediated
Epidemiology
o Most common in Asian countries
6 per 1000 persons
o Japanese: Higher incidence of aortic arch
involvement
o India: Higher incidence of thoracic and abdominal
involvement
o US: Higher incidence of great vessel involvement
o Also common in developing countries
1 person per 1000 in United States
Associated abnormalities: Tuberculosis has been
observed in developing countries
Gross Pathologic & Surgical Features
Thickening of the large and medium size vessels
Ridged, tree-bark appearance to intima
Patchy distribution
Microscopic Features
Mononuclear infiltration of the adventitia early
Cuffing of vaso vasorum
Granulomatous changes in the tunica media
Thickening and fibrosis of intima and media late
Claudication
Congestive heart failure
o American College of Rheumatology (3 of 6 needed
for diagnosis)
Age less than 40
Claudication of extremities
Decreased pulses of either brachial artery
pressure between arms
Bruit over one or both subclavian arteries or
abdominal aorta
Radiographic narrowing or occlusion of aorta,
great vessels, or large arteries in upper or lower
extremities
Other signs/symptoms
o Often asymmetric pulses rather than truly puIseless
o Retinopathy
o Pulmonary hypertension in patients with
pulmonary artery involvement
Demographics
Age
o Age < 30 in 90% of patients
o Most common in 2nd and 3rd decade of life
Gender: M:F = 1:8, but may be less female
predominant in non-Asian countries
Natural History & Prognosis
Morbidity and mortality are due to hypertension and
stroke
o Congestive heart failure primary cause of death
15 year survival 90-95%
Minority have self-limited symptoms
o 20% have monophasic episode and remit
Treatment
Corticosteroids are mainstay of therapy
o Cyclophosphamide and methotrexate are
second-line
Angioplasty or surgical bypass are options for
narrowing or occlusion
Stents have high failure rate for unknown reasons

ICLINICAL ISSUES
Presentation
I DIAGNOSTIC CHECKLIST
Most common signs/symptoms Consider
o Early phase inflammatory or prepulseless phase Takayasu arteritis in a young woman with apparent
Fever, tachycardia, fatigue (40%) atherosclerosis
Pain of involved vessels (e.g., carotodynia) Takayasu arteritis in a young women with apparent
Bruits coarctation at an unusual site
Hypertension
Aortic insufficiency from dilated aortic root Image Interpretation Pearls
o Late phase occlusive or pulseless Gadolinium-enhancement of the vessel wall may
Follows early phase by 5-20 years indicate the extent of disease activity
Type 1: Involves arch vessels and is classic PET scanning may be useful to determine disease
pulseless disease activity
Type 2: Involves aorta and arch vessels
Type 3: Involves aorta and may produce
coarctation I SELECTED REFERENCES
Type 4: Involves aortic dilatation 1. Kobayashi Y et al: Aortic wall inflammation due to
Type 3 is most common (65% of patients) Takayasu arteritis imaged with 18F-FDG PET coregistered
Stroke with enhanced CT. J Nucl Med. 46(6):917-22, 200S
Mesenteric ischemia
 TAKAYASU DISEASE
I IMAGE GALLERY

(Left) Axial CECT shows low

attenuation within the left
subclavian artery (arrow)
consistent with occlusion. 2
(Right) Axial CECT shows
marked thickening of the 47
waif of aortic arch with
mural irregularity after
attempted angioplasty.

(Left) Sagittal oblique CECT

shows marked thickening of
the waif of aortic arch with
mural irregularity after
attempted angioplasty. Note
occlusion of left subclavian
artery (arrow). (Right) Axial
T1 WI MR shows absence of
flow within the left
subclavian artery (arrow)
consistent with occlusion.

Variant
(Left) Sagittal oblique DSA
shows occlusion of the left
subclavian artery (arrow).
Note conjoined innominate
and left common carotid
branches. (Right) Axial CECT
shows an aortic dissection
(arrow) with marked mural
thickening of the false
lumen. Takayasu confirmed
on pathological examination
of the resected specimen.
 SVC OBSTRUCTION

2
48

Coronal graphic shows a middle mediastinal mass Frontal radiograph shows a large right hilar mass
causing marked SVC narrowing (and proximal dilatation extending into right upper lobe (arrow). Mediastinum is
of the innominate vessels). There are also prominent widened. Patient had SVC syndrome.
right intercostal vein collatera/s.

o CT is the ideal examination to demonstrate location,

ITERMINOlOGY cause of obstruction and collateral vessels
Abbreviations and Synonyms Radiographic Findings
Superior vena cava syndrome (SVCS)
Most patients have abnormal chest radiographs
Definitions o Greater than 80% abnormal
Com plete or near total obstruction of flow in the Often nonspecific signs are present that indicate
superior vena cava underlying cause (namely malignancy)
Can be a result of external compression, intra-vascular Most common diagnostic clue: Superior mediastinal
growth, thrombus formation, or a combination of widening
these factors Other common clues include
Most common cause of SVC Obstruction: o Pleural effusion
Bronchogenic carcinoma o Hilar or mediastinal mass
o Calcifications

I IMAGING FINDINGS
CT Findings
Narrowing or obstruction of SVC accurately
General Features demonstrated
CT with intravenous contrast indicates level of
Best diagnostic clue: Mediastinal widening with
obstruction
enlarged azygos vein and aortic nipple
CT often demonstrates cause of obstruction
Location: Superior mediastinum
o Most common cause is malignancy
Size: Variable
o Can help with treatment and management
Best imaging study
Localization for biopsy of lesion
Radiotherapy planning

DDx: SVC Narrowing

Fibrosing Mediastinitis Pericarditis

 SVC OBSTRUCTION

Key Facts
Terminology
Complete or near total obstruction of flow in the
superior vena cava
Most common cause of SVC Obstruction:
Bronchogenic carcinoma
Imaging Findings
Best diagnostic clue: Mediastinal widening with
enlarged azygos vein and aortic nipple
Most patients have abnormal chest radiographs
Most common diagnostic clue: Superior mediastinal
widening
CT with intravenous contrast indicates level of
obstruction
CT often demonstrates cause of obstruction
Best imaging tool: CT recommended to diagnose SVC
obstruction as well as determine cause and define
venous anatomy
Pathology
80-9S% caused by malignant neoplasms
Biopsy and histological confirmation necessary prior
2
to treatment 49
Small cell lung cancer is the most common histology
Clinical Issues
Dyspnea (50%)
Thoracic vein distention (70%)
Cervical vein distention (60%)
SVCS is most common over the age of 40
More common in males
Treatment depends on cause of obstruction

Visualization of collateral pathways with contrast

imaging
I DIFFERENTIAL DIAGNOSIS
o Posterior collateral system Pseudocollaterals
Azygous-hemiazygous system Hyperabduction of arm may narrow subclavian vein
Paravertebral system normally
o Superior collateral system o Contrast injection may then opacify periscapular
Periscapular collaterals veins normally
Anterior jugular venous system
External jugular vein Interruption of the Inferior Vena Cava with
Horizontal vein Azygos Continuation
Transverse arch No collaterals
o Anterolateral collateral system
No obstructing mass
Anterior intercostal veins
Internal mammary veins
Long thoracic vein I PATHOLOGY
MR Findings
General Features
Similar to CT
Poor demonstration of calcification Etiology
o Malignant neoplasms most common cause
Does not require contrast administration
Bronchogenic carcinoma accounts for an
Useful in patients with contrast allergy
overwhelming majority
o Also helpful in patients with no venous access
Obstructing mass often attributed to mediastinal
Other Modality Findings lymph node involvement
Bilateral upper extremity venography can be used to Other malignancies causing SVCS include
reliably demonstrate obstruction lymphoma, metastases (especially breast), and
o Also indicates collateral pathways thymoma
o May overestimate obstruction size secondary to o Non-malignant causes
collateral shunting Fibrosing mediastinitis (usually secondary to
o Poor choice for imaging cause of obstruction (such Histoplasma)
as tumor) Sarcoidosis
Infection
Imaging Recommendations Radiation fibrosis
Best imaging tool: CT recommended to diagnose SVC Syphilitic aneurysms and tuberculosis used to
obstruction as well as determine cause and define account for 40%, now rare
venous anatomy Thrombosis secondary to venous catheters or
Protocol advice: With intravenous contrast pacemaker wires increasing in frequency
Epidemiology
o 80-95% caused by malignant neoplasms
Right-sided mass four times more common than
left
SVCS seen in approximately 2-10 percent of
patients with bronchogenic carcinoma
 SVC OBSTRUCTION
Associated abnormalities Prognosis for patients with malignancy is generally
o Since strongly associated with bronchogenic less than 6 months
carcinoma, other signs of malignancy common SVCS is rarely, if ever, fatal by itself
Pleural effusion
Hilar or mediastinal mass Treatment
Treatment depends on cause of obstruction
Gross Pathologic & Surgical Features Obstruction caused by compressive masses
2 Whether malignant or benign, obstructing mass is
usually not resectable
o Radiation therapy
Tissue diagnosis essential for emergent
50 Biopsy and histological confirmation necessary prior radiotherapy
to treatment Adjuvant chemotherapy and steroid
Tumor can infiltrate superior vena cava administration may also be helpful
o Intraluminal metallic venous stents
Microscopic Features Provides more rapid relief than other modalities
Differs according to etiology of obstruction Often more successful (95%) than radiation or
Small cell lung cancer is the most common histology chemotherapy
Tissue diagnosis can be obtained in multiple ways Much lower rate of SVCS recurrence compared to
o Sputum cytology radiation or chemotherapy
o Bronchoscopy Unclear role for thrombolytics in these patients
o Mediastinoscopy Stents also beneficial in patients with
o Thoracotomy non-malignant lesions (such as fibrosis)
Thrombosis-related SVCS
o Thrombolytics effective for clots five or fewer days
ICLINICAL ISSUES old
o Percutaneous transluminal angioplasty also may be
Presentation used
Most common signs/symptoms
o Long term therapy with heparin or Coumadin may
o Symptoms
be necessary to avoid recurrence
Dyspnea (50%) Surgical bypass difficult, rarely performed
Head fullness
Cough
o Signs
Thoracic vein distention (70%)
I SElECTED REFERENCES
Cervical vein distention (60%) 1. Bolad let al: Percutaneous treatment of superior vena cava
Facial swelling/plethora obstruction following transvenous device implantation.
Catheter Cardiovasc Interv. 65(1):54-9, 2005
Upper extremity edema 2. Kentos A et aI: Long-term remission with surgery for
Other signs/symptoms recurrent localized Hodgkin lymphoma. J Thorac
o Chest pain Cardiovasc Surg. 129(5):1172,2005
o Dysphagia 3. Schifferdecker Bet al: Nonmalignant superior vena cava
o Cyanosis syndrome: Pathophysiology and management. Catheter
Presentation can range from asymptomatic to Cardiovasc Interv. 65(3):416-423, 2005
dyspnea, choking, neurologic impairment 4. Lee-ElliottCE et al: Fast-track management of malignant
Symptoms depend on time course superior vena cava syndrome. Cardiovasc Intervent Radiol.
27(5):470-3,2004
o Slowly developing obstruction allows time for
5. Young N et al: Use of endovascular metal stents to alleviate
collateral development with few or no symptoms malignant superior vena cava syndrome. Intern Med J.
Demographics 33(11):542-4, 2003
6. Markman M: Diagnosis and management of superior vena
Age cava syndrome. Cleve Clin J Med. 66: 59-61, 1999
o Extremely rare in pediatric population 7. Gosselin MV et al: Altered intravascular contrast material
o SVCS is most common over the age of 40 flow dynamics: Clues for refining thoracic CT diagnosis.
Due to correlation with lung cancer AJR.169:1597-603, 1997
o In adults under the age of 40, benign etiologies are 8. Ostler PJ:Superior Vena Cava Obstruction: A Modern
the most common cause Management Strategy. Clin Oncol (R Coil Radiol). 9: 83-89,
1997
Gender 9. Wurschmidt F et al: Small cell lung cancer with and
o More common in males without superior vena cava syndrome: a multivariate
Due to increased frequency of bronchogenic analysis of prognostic factors in 408 cases. IntJ Radiat
carcinoma in this population Oncol Bioi Phys 22: 77-82, 1995
o With benign etiologies, there is no gender 10. Finn JP et al: Central Venous Occlusion: MRAngiography.
predominance Radiology. 187: 245-251,1993
11. Baker GL et al: Superior Vena Cava Syndrome: Etiology,
Natural History & Prognosis Diagnosis, and Treatment. AmJ Crit Care. 1:54-64, 1992
Depends on underlying etiology 12. Standford W et al: Superior vena cava obstruction: A
SVC syndrome a positive prognostic indicator for venographic classification. AJR.148: 259-62, 1987
small cell lung cancer 13. Parish JM et al: Etiologic considerations in superior vena
cava syndrome. Mayo Clin Proc 56: 407-13, 1981
 SVC OBSTRUCTION
I IMAGE GAllERY
Typical
(Left) Axial CECT shows
large right lung mass
invading right para tracheal
mediastinum and obliterating 2
svc (open arrows).
Collateral (Jaw in enlarged 51
left anterior chest wall and
internal mammary veins is
seen (curved arrows).
(Right) Axial CECT shows
partially calci(ied soft tissue
mass in the prevascular
space (arrow) with poor flow
in the superior vena cava
and collateral vessels (open
arrow) in the mediastinum.

Typical
(Left) Axial CECT shows
numerous small eol/alerals
(open arrows) throughout
mediastinum, internal
mammary, left chest wall.
Mediastinal mass causing
SVC occlusion. Note
paraesophageal collaterals
(curved arrow). (Right) Axial
CECT shows large anterior
mediastinal mass encasing
the great vessels and
occluding the SVC (arrow).
Prominent collateral vessels
(open arrow) are seen in the
right anterior chest wall.

Typical
(Left) Transverse CECT
shows soft tissue mass
obstructing SVC (open
arrow). Note the left chest
wall and left superior
intercostal eol/alerals
(curved arrows). (Right)
Axial CECT shows
diaphragmatic collateral to
inferior vena cava (open
arrow) and increased flow in
azygous and hemiazygous
veins (curved arrows).
 Congenital
Partial Absence Pericardium 11-3-2
Heterotaxy Syndrome 11-3-4
Pericardial Cyst 11-3-8

Inflammatory - Degenerative
Coronary Artery Calcification 11-3-12
Left Atrial Calcification 11-3-16
Ventricular Calcification 11-3-20
Valve and Annular Calcification 11-3-24
Aortic Valve Dysfunction 11-3-28
Mitral Valve Dysfunction 11-3-32
Constrictive Pericarditis 11-3-36
Postcardiac Injury Syndrome 11-3-40

Toxic - Metabolic
Myxedema 11-3-44

Neoplastic
Left Atrial Myxoma 11-3-46
Metastases Pericardium 11-3-50

 PARTIAL ABSENCE PERICARDIUM

3
2

Sagittal graphic shows left atrial appendage has Frontal radiograph shows the cardiac silhouette shifted
herniated through a partial pericardial defect. While to the left (Snoopy's nose). Air interposed between the
usually an incidental finding, the herniated structures aortic arch and pulmonary artery (Snoopy's ear) in
may strangulate. partial pericardial absence.

I TERMINOlOGY CT Findings
NECT
Definitions o Interpositioning of lung parenchyma between main
Congenital or acquired absence of portion of pulmonary artery and aortic arch
pericardium o Rotation of the heart toward the left
MR Findings
I IMAGING FINDINGS Absence of low signal pericardialline
General Features Imaging Recommendations
Location: Most pericardial defects are partial on the Best imaging tool: Echocardiography primary tool to
left investigate pericardium
Radiographic Findings
Absence of left pericardium I DIFFERENTIALDIAGNOSIS
o "Snoopy dog" appearance
Left cardiac shift (Snoopy's nose) Pericardial/Epicardial Fat Pad or Cyst
Air interposed between aortic arch and main Can occur at either cardiophrenic angle
pulmonary artery Rounded, hemispherical shape
Prominent left atrial appendage (Snoopy's ear) Fat or fluid density at CT
Air interposed between left hemidiaphragm and
inferior heart border Morgagni Hernia
Bowel or mesenteric fat in anterior hernia sac
Usually develops on right

DDx: Abnormal Cardiac Contour

Morgagni Hernia Pericardia! Cyst Fibrosarcoma

 PARTIAL ABSENCE PERICARDIUM

Key Facts
Terminology Thymic Cysts or Thymolipoma
Congenital or acquired absence of portion of Loculated Pleural Effusion
pericardium Pericardial Effusion
Left Ventricular Aneurysm
Imaging Findings
"Snoopy dog" appearance Clinical Issues
Usually asymptomatic incidental finding
Top Differential Diagnoses Non-exertional paroxysmal stabbing chest pain
Pericardial/Epicardial Fat Pad or Cyst Can be lethal complication if not detected
Morgagni Hernia

Thymic Cysts or Thymolipoma Associated abnormalities: Patients with pericardial

defects also may have one or more associated
3
Thymolipomas contain fat
congenital abnormalities: Atrial septal defect, patent 3
Cysts will have fluid density/intensity on CT or MRI
ductus arteriosus, mitral valve stenosis, and tetralogy
Loculated Pleural Effusion of Fallot
Fluid density on CT
Usually can be separated from uninvolved pericardium
ICLINICAl ISSUES
Pericardial or Lung Neoplasm
Commonly bronchogenic carcinoma Presentation
Primary pericardial tumors rare Most common signs/symptoms
Soft tissue mass at CT, separate from normal a Usually asymptomatic incidental finding
pericardium a Non-exertional paroxysmal stabbing chest pain
Caused by herniation of ventricular base through
Pericardial Effusion the defect with compression of the left coronary
Water bottle shaped cardiac silhouette on radiograph artery branches by the rim of the defect
Can have widened pericardial stripe on lateral
examination Natural History & Prognosis
Herniation and entrapment of a cardiac chamber,
Left Ventricular Aneurysm especially left atrial appendage
Rare complication of myocardial infarction Can be lethal complication if not detected
May be calcified
Treatment
Surgical goal: Close the pericardial defect or enlarge
I PATHOLOGY the pericardial defect to prevent strangulation of the
heart
General Features
Etiology: Interruption of vascular supply to developing
pericardium during embryogenesis I SELECTED REFERENCES
1. Abbas AE et al: Congenital absence of the pericardium: case
presentation and review of literature. Int J Cardial.
98(1):21-5, 2005

I IMAGE GALLERY

(Left) Frontal radiograph shows lehward displacement of the heart in a patient with partial pericardia I absence. Air deeply invaginates between
aortic arch and pulmonary artery (arrow). (Center) Transverse NECT shows herniation o( the right ventricle (arrows) through a partial defect of
the pericardium. Right ventricle contacts the left chest wall. (Right) Coronal CECT reconstruction shows superior displacement of the left atrium
(arrow) in a patient with partial pericardia! absence. Note also the shirt of the heart to the left chest wall.
 HETEROTAXY SYNDROME

3
4

Frontal radiograph shows total situs inversus with right Axial CECT shows midline liver and midline aortic arch.
aortic arch, dextrocardia and right stomach bubble Multiple spleens (arrow) in the right upper quadrant.
(arrow). Left marker (open arrow) was placed correctly. Polysplenia heterotaxy.

I TERMINOlOGY I IMAGING FINDINGS

Abbreviations and Synonyms
Situs inversus totalis, situs inversus with dextrocardia,
situs inversus with levocardia, Kartagener syndrome,
immotile cilia syndrome, dysmotile cilia syndrome
Definitions
Heterotaxy syndrome characterized by situs ambiguus, Radiographic Findings
congenital heart malformations, and splenic
malformations
Situs describes the position of the cardiac atria and
viscera
Situs solitus is the normal position and situs inversus
is the mirror image
o Situs is independent of the cardiac apical position
Levocardia and dextrocardia indicate only the
direction of the cardiac apex
Dextroversion: Situs solitus with dextrocardia
o Cardiac apex points to the right but visceral situs
normal
Levoversion: Situs inversus with levocardia
General Features
Best diagnostic clue
o Cardiac apex usually ipsilateral to stomach bubble, if
not suspect heterotaxy syndrome
o Atrial situs best determined by location of the liver
Radiography
o First analyze situs
o Situs solitus
Left aortic arch, levocardia, stomach bubble
left-sided, liver right-sided, trilobed right lung
Levocardia not synonymous with situs solitus
o Situs inversus totalis
Right aortic arch, dextrocardia, stomach bubble
right-sided, liver left-sided, trilobed left lung
o Visceroatrial concordance rule
Site of the liver correlates with situs of right
atrium
Site of the stomach also correlates with situs of left
atrium but not to the degree seen with the liver
and right atrium

DDx: Dextrocardia

Scimitar Syndrome Cardiac Volvulus

 HETEROTAXY SYNDROME

Key Facts
Terminology Atrial morphology cannot be determined on
Situs inversus totalis, situs inversus with dextrocardia, radiographs but can also be inferred by bronchial
situs in versus with levocardia, Kartagener syndrome, branching pattern or by location of liver and stomach
immotile cilia syndrome, dysmotile cilia syndrome Bronchiectasis suggests Kartagener syndrome
Heterotaxy syndrome characterized by situs Top Differential Diagnoses
ambiguus, congenital heart malformations, and
Mislabeled Films
splenic malformations
Kartagener Syndrome
Situs describes the position of the cardiac atria and
Scimitar Syndrome
viscera
Cardiac Volvulus
Situs solitus is the normal position and situs inversus
is the mirror image Diagnostic Checklist
Situs is independent of the cardiac apical position Abdominal situs in any patient with dextrocardia
Discordance between cardiac apex and abdominal
3
Imaging Findings
situs (stomach bubble and liver) suggests congenital 5
Cardiac apex usually ipsilateral to stomach bubble, if
heart disease
not suspect heterotaxy syndrome
Atrial situs best determined by location of the liver

o Atrial morphology cannot be determined on Chamber organization can not be determined

radiographs but can also be inferred by bronchial from chest radiographs
branching pattern or by location of liver and Requires echocardiography or MRI
stomach
Situs inversus: Bronchus intermedius on the left CT Findings
(also left minor fissure) Useful to demonstrate visceral situs and anomalies of
Right and left bronchi of equal length (isomerism) aortic and venous vasculature
suggests situs ambiguous
MR Findings
Normal ratio of length of left/right main-stem
Excellent modality to determine situs, non-ionizing
bronchi> 1. 7, in isomerism ratio < 1.4
radiation especially useful for the young
o Situs ambiguous or heterotaxy syndrome
Atrial situs determined by morphology of the atria
Asplenia: Right-sided symmetry
o Right atrium
Polysplenia: Left-sided symmetry
Contains coronary sinus ostia
o Asplenia and polysplenia
Connects to suprahepatic portion of inferior vena
Discordant location stomach bubble and cardiac
cava (IVC)
apex
Location of superior vena cava (SVC) not as useful
Midline transverse liver
o Asplenia as the SVC is often duplicated
Atrial appendage: Wide base pyramidal shape
Bilateral minor fissures
Contains crista terminalis and pectinate muscles
Symmetrical main stem bronchi with narrow
o Left atrium
carinal angle
Contains ostia pulmonary veins
Lateral radiograph: Both pulmonary arteries
Atrial appendage: Narrow base and tubular shape
anterior to hilar bronchi (eparterial bronchi)
o Polysplenia Echocardiographic Findings
No minor fissure Best to characterize intracardiac anomalies
Symmetrical main stem bronchi with wide carinal
angle Imaging Recommendations
Prominent azygos vein Best imaging tool
Lateral radiograph: Both pulmonary arteries o Chest radiograph useful as preliminary survey
posterior to hilar bronchi (hyparterial bronchi) o Echocardiography and MRI to evaluate cardiac
o Dextroversion chamber anomalies
Transposition: Normal anterior to posterior
chamber relationships lost
No ventricular inversion I DIFFERENTIAL DIAGNOSIS
o Levoversion
Transposition Mislabeled Films
Ventricular inversion (reversed left and right Most common cause of misinterpretation, either
relationship) overlooked because films are thought to be mislabeled
o Bronchiectasis suggests Kartagener syndrome or overlooked because correctly labeled films are
o Once situs determined, next determine chamber flipped and viewed according to normal conventions
organization Mislabeling may result in operations on wrong side
Leads to malpractice awards
 HETEROTAXY SYNDROME
Normal: Left main-stem bronchus anterior and
Kartagener Syndrome inferior to the left pulmonary artery
Seen in 20% with situs inversus
Bronchiectasis, chronic sinusitis, and situs inversus
Due to structural abnormality of cilia I CLINICAL ISSUES
Situs inversus occurs in only 50% of patients
Presentation
Scimitar Syndrome Most common signs/symptoms
Hypoplasia right lung o Asymptomatic to severe cardiac symptomatology
Scimitar vertical vein coursing to the right o Midgut volvulus in heterotaxy syndrome
costovertebral angle Other signs/symptoms: Asplenia: Howel-]olly bodies
Heart displaced into right hemithorax, not on blood smear
dextrocardia
Demographics
Cardiac Volvulus
3 Follows right pneumonectomy
Age: Congenital but may not present until adults
Gender: Situs ambiguous more common in males
Heart herniates through surgical pericardial defect and
6 rotates with apex directed to the right costophrenic Natural History & Prognosis
angle Asplenia: Immunosuppressed for encapsulated bacteria
Cardiovascular collapse due to obstruction venous leading to sepsis
return Long term prognosis usually determined by cardiac
Surgical emergency requiring immediate reoperation defects
Incidence congenital heart disease
o Situs solitus: 0.75% incidence congenital heart
I PATHOLOGY disease
o Situs inversus: 3-5% incidence congenital heart
General Features disease
Genetics o Situs solitus + dextrocardia (dextroversion): 75%
o Most arise sporadically incidence congenital heart disease
o Evidence of both autosomal and X-linked o Situs inversus + levocardia (levoversion): 99%
inheritance, probably multifactorial incidence congenital heart disease
Epidemiology o Situs solitus + right aortic arch and situs inversus +
o Situs inversus present in 1 per 15,000 live births left aortic arch: Low incidence congenital heart
20% associated with Kartagener syndrome disease
o Situs ambiguous present in 1 per 10,000 live births o Levoversion: 99% incidence congenital heart disease
o 3-5% with congenital heart disease have o Dextroversion: 95% incidence congenital heart
dextrocardia disease
Situs inversus most common associated anomalies
o Transposition Treatment
o Atrioventricular discordance Surgical repairs of cardiac anomalies
Situs ambiguous most common associated anomalies Prophylactic Ladd procedure to prevent midgut
o Asplenia volvulus
Associated with cyanotic congenital heart disease Prophylactic antibiotics for asplenia
Single ventricle or atrium and conotruncal Pneumococcal vaccination for asplenia
anomalies
Total anomalous pulmonary venous return
Gastrointestinal malrotation I DIAGNOSTIC CHECKLIST
o Polysplenia
Left to right shunts (atrial septal defect, Consider
ventricular septal defect) Abdominal situs in any patient with dextrocardia
Left-sided obstructive lesions Image Interpretation Pearls
Double-outlet right ventricle
Discordance between cardiac apex and abdominal
Biliary atresia 10%
situs (stomach bubble and liver) suggests congenital
Partial anomalous pulmonary venous return
heart disease
Interruption IYC with azygous continuation 50%
Gastrointestinal malrotation
Gross Pathologic & Surgical Features I SELECTED REFERENCES
Pulmonary situs l. Garg N et al: Dextrocardia: an analysis of cardiac structures
o Eparterial bronchi in 125 patients. Int J Cardiol. 88(2-3):143-55; discussion
Normal: Right main stem bronchus posterior and 55-6,2003
superior to right pulmonary artery 2. Applegate KEet al: Situs revisited: imaging of the
o Hyparterial bronchi heterotaxy syndrome. Radiographies. 19(4):837-52;
discussion 53-4, 1999
 HETEROTAXY SYNDROME
[IMAGE GALLERY

(Left) Axial CECT shows

dextrocardia and left-sided
IVC (arrow) from the first
gallery patient. Polysplenia
heterotaxy. (Right) Frontal
radiograph shows situs
inversus. Right aortic arch
(white arrow), dextrocardia,
and right-sided stomach
(black arrow). One must
make certain that image has
been labeled correctly. 3
Heterotaxy should be
excluded. 7

Variant
(Left) Frontal radiograph
shows levocardia and left
aortic arch but stomach
bubble is on the right
(arrow). Discordant location
suggests underlying
heterotaxy syndrome. (Right)
Axial CECTshows midline
liver. Contrast filled stomach
on the right (arrow). No IVC
but enlarged azygous vein
(black curved arrow).
Multiple spleens in the right
upper quadrant (white
curved arrow). Po/ysp/enia
heterotaxy.

Typical
(Left) Axial CECT shows
bronchial isomerism (arrows)
in heterotaxy syndrome.
Enlarged azygos vein
(curved arrow). Bilateral
hyparterial bronchi
(/eft-sidedness). Polysplenia.
(Right) Axial CECT in
different patient shows
dextrocardia with apex into
the right hemithorax (white
arrows). Azygous vein is
enlarged (black arrow).
Heterotaxy syndrome with
interruption IVC and azygous
continuation.
 PERICARDIAL CYST

3
8

Coronal graphic shows the typical location of a Coronal MR T2WI shows a pericardial cyst with the
pericardial cyst at the right costophrenic (CP) angle typical findings of a smoothly marginated structure of
(curved arrows). The anomalous cyst forms as an uniformly high signal intensity, adjacent to the right CP
aU/pouching from the pericardial sac. angle (curved arrows).

I TERMINOlOGY Radiographic Findings

Radiography
Definitions o Double density at right CP angle
Pericardial cysts common benign disorder o Contour overlying the cardiac silhouette
Anomalous outpouching of parietal pericardium o Partly spherical with sharp smooth contours
o May rarely occur other places in mediastinum rather
than CP angle
IIMAGING FINDINGS In this case, difficult to distinguish from
General Features bronchogenic or thymic cyst
o May change shape with body positioning or
Best diagnostic clue
r.espiration
o Smoothly marginated
o Adjacent to heart at right anterior costophrenic CT Findings
angle NECT
o Fluid density by CT o Smoothly marginated
o Water signal intensity by MRI o Water attenuation (10 Hounsfield units), no
o Unilocular in 80%, 20% multiloculated septations
Location o Non-calcified
o Cardiophrenic (CP) angle o Wall imperceptible
Right 70% o Usually at CP angle, especially on right
Left 10-40% CECT
Size: 2-30 em in diameter o Homogeneous appearance
Morphology: Round, sharp margins o No internal enhancement
o No enhancing rim

DDx: Pericardial Cyst

Cardiac Mass Metastases

 PERICARDIAi CYST

Key Facts
Terminology Anechoic in appearance
Anomalous outpouching of parietal pericardium Top Differential Diagnoses
Imaging Findings Loculated Pleural Effusion
Smoothly marginated Bronchogenic Cyst
Adjacent to heart at right anterior costophrenic angle Hematoma
Fluid density by CT Esophageal Duplication Cyst
Water signal intensity by MRI Pericardial Metastases
Unilocular in 80%, 20% multiloculated Hydatid Cyst
Size: 2-30 em in diameter Pathology
May change shape with body positioning or Invariably connected to pericardium
respiration
Homogeneous appearance
Only a few show visible communication
pericardial sac
with 3
No internal enhancement
No enhancing rim Clinical Issues 9
Echocardiography primary tool to investigate Most common signs/symptoms: Usually
pericardium asymptomatic incidental finding

MR Findings Bronchogenic Cyst

TlWI Same imaging characteristics as pericardial cyst
o Uniform low or intermediate signal intensity (SI) Most commonly located in middle mediastinum
o Occasionally may contain highly proteinaceous around carina
fluid, which may have high SI on Tl weighted When infected or contain secretions, may appear as
images solid tumor or may have air-fluid level
T2WI
o Homogeneous Hematoma
o High signal intensity (follows that of water) M RI particularly useful
Tl C+ Acutely demonstrates homogeneous high signal
o No internal enhancement intensity on Tl WI and T2WI
o No rim-enhancement Subacutely shows heterogeneous signal intensity, areas
MR imaging findings are diagnostic, generally of high SI on Tl WI and T2W[
requiring no further intervention Chronically may show dark peripheral rim and low S[
areas that may represent calcification, fibrosis, or
Imaging Recommendations hemosiderin deposition on Tl WI
Best imaging tool: Echocardiography or MRI High SI areas on Tl WI or T2W[ may correspond to
Protocol advice hemorrhagic fluid
o Limited protocol needed No enhancement on Tl C+
Axial and coronal Tl WI and Tl C+
Axial and coronal T2WI Pericardial Fat Pad
Coronal imaging helpful to demonstrate Echo-free space may be seen byechocardiography;
relationship to heart and pericardium may be difficult to distinguish from pericardial fluid
Short axis and 4 chamber planes not necessary Fat density by CT distinguishing feature
Echocardiography primary tool to investigate Morgagni Hernia
pericardium
Bowel or mesenteric fat in anterior hernia sac
o Anechoic in appearance
o High sensitivity and ability to differentiate solid Enlarged Pericardial Lymph Nodes
from cystic masses Mantle radiation therapy: Cardiac blockers used to
o Defines relationships with cardiac chambers protect heart, area may be under treated
CT and MRI useful to o "Fat pad" sign: Enlarging recurrent nodes from
o Examine entire pericardium lymphoma in under treated pericardiallymph nodes
o Distinguish myocardial from pericardial disease o Appearance or enlargement of "fat pad" heralds the
o Further characterize pericardia I masses development of adenopathy
o Nodes may be irradiated since field was blocked
initially
I DIFFERENTIAL DIAGNOSIS May fill CP angle on frontal chest radiograph
On lateral view may be retrosternal or at level of
Loculated Pleural Effusion inferior vena cava or phrenic nerve
Fluid density at CT
Look for other loculations or free effusion Thymic Cysts or Thymolipoma
History pertinent; more common post-operatively Cysts have fluid density at CT or MRI
 PERICARDIAL CYST
Thymolipoma contains fat
Thymus usually separable from pericardium
ICLINICAL ISSUES
Esophageal Duplication Cyst Presentation
Most common signs/symptoms: Usually asymptomatic
Imaging characteristics identical to pericardial cyst
incidental finding
Adjacent to esophagus, majority are cervical
Other signs/symptoms
Bronchogenic Carcinoma o Occasionally may have chest pain
Separate from pericardium at CT o Pericardial tamponade may rarely occur
Bronchogenic carcinoma can directly extend into
Treatment
pericardium
Effusion and irregularly thickened pericardium or Generally incidental radiographic finding requiring no
pericardial mass treatment
Surgery if complicated by
Pericardial Metastases
3 Lung and breast cancer most common
o Chest pain
o Tamponade
Effusion and irregularly thickened pericardium or o Mistaken for malignancy
10
pericardial mass No literature to support percutaneous drainage
Enhancement common by CT or MRI
Most have low SI on T1 WI and high SI on T2WI
I SELECTED REFERENCES
Neurofibroma
1. Alpendurada F et al: Pericardial cyst--a clinical case. Rev
May cause CP angle mass Port Cardiol. 24(3):435-8, 2005
Generally solid, but may have cystic components 2. Nijveldt R et al: Pericardial cyst. Lancet. 365(9475):1960,
Enhancement internally with CT or MRI 2005
3. Guven A et al: A case of asymptomatic cardiopericardial
Hydatid Cyst hydatid cyst. Jpn Heart J. 45(3):541-5, 2004
Cystic mass with well-defined edges 4. Heirigs R et al: Images in cardiology: Pericardial cyst. Clin
Internal trabeculations correspond to daughter Cardiol. 27(9):507, 2004
membranes 5. Oyama N et al: Computed tomography and magnetic
May be pericardial or intra myocardial resonance imaging of the pericardium: anatomy and
May appear as solid mass if cyst replaced by necrotic pathology. Magn Reson Med 5ci. 3(3):145-52, 2004
6. Patel J et al: Pericardia I cyst: case reports and a literature
matter
review. Echocardiography. 21 (3):269-72,2004
o Contains membrane residues and granulomatous 7. Serwer BA et al: Images in clinical medicine. Pericardia I
foreign-body inflammatory reaction cyst. N EnglJ Med. 350(21):eI9, 2004
8. Uchiyama A et al: Infrasternal mediastinoscopic surgery for
Pancreatic Pseudocyst anterior mediastinal masses. Surg Endose. 18(5):843-6,
History pertinent 2004
Look for peri pancreatic inflammatory changes and 9. Walker MJ et al: Migrating pleural mesothelial cyst. Ann
fluid collections Thorac Surg. 77(2):701-2,2004
Usually extends through esophageal hiatus 10. Glockner JF: Imaging of pericardial disease. Magn Reson
Imaging Clin N Am. 11(1):149-62, vii, 2003
I I. Gossios K et al: Mediastinal and pericardial hydatid cysts:
an unusual cause of circulatory collapse. AJR Am J
I PATHOLOGY Roentgenol. 181(1):285-6,2003
12. Kim JH et al: Cystic tumors in the anterior mediastinum.
General Features Radiologic-pathological correlation. J Com put Assist
General path comments: Benign cyst of mediastinum Tomogr. 27(5):714-23, 2003
Etiology 13. Noyes BE et al: Pericardial cysts in children: surgical or
o Anomalous outpouching of parietal pericardium conservative approach? J Pediatr Surg. 38(8):1263-5, 2003
o Occurs by 4th week of gestation 14. Takeda S et al: Clinical spectrum of mediastinal cysts.
o Occurs as coalescing spaces form intraembryonic Chest. 124(1):125-32,2003
body cavity 15. Wang ZJ et al: CT and MR imaging of pericardial disease.
Radiographics. 23 Spec No:S167-80, 2003
Gross Pathologic & Surgical Features 16. Wildi SM et al: Diagnosis of benign cysts of the
mediastinum: the role and risks of EUS and FNA.
Invariably connected to pericardium
Gastrointest Endosc. 58(3):362-8, 2003
Only a few show visible communication with 17. Espinola-Zavaleta N et al: Three-dimensional
pericardial sac transesophageal echocardiography in tumors of the heart. J
Am Soc Echocardiogr. 15(9):972-9,2002
Microscopic Features 18. Breen JF: Imaging of the pericardium. J Thorac Imaging.
Fibrous tissue lined by single layer of bland 16:47-54,2001
mesothelium 19. Pezza no A et al: Value of two-dimensional
Differentiate from bronchogenic cysts and esophageal echocardiography in the diagnosis of pericardial cysts. Eur
duplication cyst by cell lining HeartJ. 4(4):238-46,1983
o Absence of bronchial or gastrointestinal epithelium 20. Feigin DS et al: Pericardial cysts: a radiologic-pathologic
respectively correlation and review. Radiology. 125: 15-20, 1977
 PERICARDIAL CYST
I IMAGE GALLERY

(Left) Frontal radiograph

shows a smoothly
marginated spherical opacity
overlying the cardiac
silhouette on the right
(arrows). Cross-sectional
imaging confirmed the
diagnosis of a pericardial
cyst. (Right) Lateral
radiograph shows the
anterior location of the
pericardia I cyst at the CP 3
angle (arrows). This is a
typical location by plain film, 11
but is nonspecific, prompting
further imaging.

(Left) Axial NECT shows a

bilobed pericardia I cyst at
the left cardiac apex, which
is atypical in appearance and
location (arrows). Note the
sma If pericardia I effusion or
thickening (open arrows).
(Right) Axial NECT shows
the typical water attenuation
10 Hounsfield units by
ROI analysis as shown),
location and unilocular
appearance of a pericardia I
cyst at the right CP angle
(arrows).

(left) Axial T1 WI MR shows

the lypical uniForm low
signal intensity of a
pericardial cyst (arrows).
T1WI with contrast (not
shown here) demonstrated
no enhancement. (Right)
Axial T2WI by MR shows the
pericardia I cyst to contain
uniform high signal intensity
(arrows) approximating that
of CSF fluid (open arrow).
 CORONARY ARTERY CALCIFICATION

3
12

Graphic shows calcification Ipictured in white) in the Axial CECT shows dense calcification in the left anterior
right and left coronary arteries. descending artery Iarrow) and the left circumflex artery
lopen arrow).

Location: Any of the coronary arteries, especially

ITERMINOlOGY proximal
Abbreviations and Synonyms Size: Coronary artery size is typically 5 mm or less
Morphology: Calcification manifests as high density
Coronary artery calcification (CAe)
within the course of the coronary arteries
Definitions
Radiographic Findings
Atherosclerotic heart disease the leading cause of
death in developed world General
o Coronary calcification has tram-track appearance
Coronary calcification a marker of atherosclerotic
o Quantity of calcification directly related to degree of
coronary artery disease (CAD)
stenosis
Direct relationship between coronary artery
o Myocardial calcification generally linear or arcuate
calcification and stenosis
o Valve calcification generally nodular or clumped
Direct relationship between coronary artery
calcification and myocardial infarction Coronary artery
o Posterior-anterior view: Cardiac triangle
Relationship is most marked in younger patients
Vertical border: Medial border spine
CT appears useful to screen for coronary artery
Superior diagonal border: Left heart border
calcification
Inferior border: Approximately 1/3rd the distance
from the left bronchus to diaphragm at the level
of the "shoulder of the left ventricle
IIMAGING FINDINGS Typically reflects left coronary artery calcification
General Features o Lateral view: Proximal calcification often evident
Best diagnostic clue: CT scan: Either Electron-Beam near aortic root
tomography (EBT) of multi detector CT (MDCT)

DDx: Cardiac Calcifications

Left Ventricular Pericardia I Mitral Annular

 CORONARY ARTERY CALCIFICATION

Key Facts
Terminology Frequent finding in otherwise healthy adults, signifies
Atherosclerotic heart disease the leading cause of atherosclerosis and CAD
death in developed world Quantity associated with likelihood of significant
Coronary calcification a marker of atherosclerotic stenosis (not necessarily related to the site of the
coronary artery disease (CAD) calcium)
Direct relationship between coronary artery Absence of calcification does not rule out unstable
calcification and stenosis plaque
Direct relationship between coronary artery Top Differential Diagnoses
calcification and myocardial infarction
Pericardial Calcification
Relationship is most marked in younger patients
Myocardial Calcification
CT appears useful to screen for coronary artery
Clinical Issues
calcification
Coronary artery calcification screening with CT may
3
Imaging Findings
be useful in atypical chest pain or with strong family 13
Coronary calcification has tram-track appearance
history of coronary artery disease
Quantity of calcification directly related to degree of
stenosis

Anterior calcification typically in left anterior
descending or right coronary artery
Posterior calcification typically in left circumflex
artery
a Visible calcification highly associated with
significant stenosis
CT Findings
CECT
a Coronary calcification visible but may be somewhat
obscured by contrast
a Soft plaque also visible
More sensitive than chest radiography to detect
calcium
Coronary artery calcification
a Frequent finding in otherwise healthy adults,
signifies atherosclerosis and CAD
a Quantity associated with likelihood of significant
stenosis (not necessarily related to the site of the
calcium)
a Absence of calcification does not rule out unstable
plaque
Consistent with a lowered risk for near term
cardiovascular event
Calcium score measured by EBT or MDCT (three
methods)
a Calcium score (Agatston)
Derived by computer calculating the area and
density of each coronary artery calcification
(above a certain threshold, typically 130 HU)
Conventional method described in 1990 - still
widely used
Most databases derived from this method
Limitations include lack of linearity with increases
in calcium and lack of accounting for volumetric
imaging
a Calcium score (volume)
Based on number of voxels exceeding threshold,
typically 130 HU
More reproducible than Agatston score
Prone to some partial volume effects
a Calcium score (mass)
Based on calibration with calcification with
known amount of hydroxyapatite
True physical measure
Appears reproducible among different scanners
a Calcium score compared to gender and age matched
populations
Databases generally use older Agatston scoring
method
a All methods based on acquisition of contiguous
2.5-3 mm sections without contrast
EBT advantages include somewhat better temporal
resolution and ionger experience with the
technique
MDCT advantages include better signal-to-noise
and wider availability
No clear overall advantage for either technique
a All methods require careful drawing of regions of
interest (ROl) around coronary calcifications for
accurate calcium scoring
Fluoroscopic Findings
Chest Fluoroscopy: Previously used to assess coronary
artery calcification
Imaging Recommendations
Best imaging tool: Noncontrast CT scan: EBT vs.
MDCT
Protocol advice
a Prospective ECG-gating
a 2.5-3 mm thick sections
I DIFFERENTIAL DIAGNOSIS
Pericardial Calcification
Usually right-sided (less cardiac motion)
Diffuse and extensive (focal)
Spares left atrium and apex (often involves
atrio-ventricular groove)
Lateral view: Over pulmonary outflow tract (under
pulmonary valve)
 CORONARY ARTERY CALCIFICATION

Myocardial Calcification Demographics

Usually left-sided Age: Males> 45, females> 55
Focal, apex typical location Gender: Male predominance in middle age
Spares AV groove Ethnicity: Caucasians and African-Americans have
Lateral view: Projects under pulmonic valve similar amounts of CAC
Natural History & Prognosis
I PATHOLOGY CAC is marker for CAD and its sequelae of angina and
myocardial infarction
General Features It often progresses in absence of intervention
General path comments: Calcification usually It may remain stable with aggressive therapy, typically
dystrophic due to abnormal tissue or flow does not regress
hemodynamics, may be degenerative
Treatment
3 Genetics: Multifactorial: Some genetic component,
especially hypercholesterolemia and diabetes Lifestyle modifications for CAD
More aggressive therapy with statins or other
14 Etiology
medications may be indicated
o Risk factors are same as for CAD
Percutaneous interventional or surgical bypass for
Hypertension
coronary artery disease if advanced
Diabetes
Smoking
Hypercholesterolemia
Obesity and sedentary lifestyle
I DIAGNOSTIC CHECKLIST
Family history Consider
Epidemiology: Most common in males> 45, females>
Obtain in patients at intermediate risk for CAD to
55 guide aggressiveness of treatment
Associated abnormalities
o Atherosclerosis is a systemic disease Image Interpretation Pearls
Myocardial infarction Careful drawing of ROI's necessary to avoid adding in
Stroke adjacent calcifications such as those in the mitral
Renal disease annulus or aortic valve or annulus
Peripheral vascular disease
Gross Pathologic & Surgical Features
I SElECTED REFERENCES
Onset occurs as early as puberty
o Primarily affects medium size muscular artery and 1. Bellasi A et al: Diagnostic and prognostic value of coronary
large elastic arteries artery calcium screening. Curr Opin Cardiol. 20(5):375-80,
2005
o Deposition of lipids, platelets, fibrin, cellular debris,
2. Kitamura A et al: Evaluation of coronary artery calcification
calcium by multi-detector row computed tomography for the
o Gross findings detection of coronary artery stenosis in Japanese patients.)
Fatty streak Epidemiol. 15(5):187-93,2005
Atheromatous plaques 3. Raggi P: Role of electron-beam computed tomography and
Complicated atheroma nuclear stress testing in cardiovascular risk assessment. Am
o Ultimately leads to luminal narrowing ) Cardiol. 96(8A):20-7, 2005
4. Taylor A) et al: Coronary calcium independently predicts
Microscopic Features incident premature coronary heart disease over measured
Calcification part of atheromatous plaque in cardiovascular risk factors: mean three-year outcomes in
the Prospective Army Coronary Calcium (PACC) project. )
atherosclerosis
Am Coll Cardiol. 46(5):807-14, 2005
5. Thompson BH et al: Update on using coronary calcium
screening by computed tomography to measure risk for
IClINICAllSSUES coronary heart disease. Int) Cardiovasc Imaging.
21(1):39-53,2005
Presentation 6. Greenland Pet al: Coronary artery calcium score combined
Most common signs/symptoms: Angina with Framingham score for risk prediction in
Other signs/symptoms asymptomatic individuals. lama. 291(2):210-5, 2004
o Shortness of breath 7. Nasir K et al: Electron beam CT versus helical CT scans for
o Many asymptomatic assessing coronary calcification: current utility and future
directions. Am Heart). 146(6):969-77, 2003
o Coronary artery calcification screening with CT may 8. Shaw L) et al: Prognostic value of cardiac risk factors and
be useful in atypical chest pain or with strong family coronary artery calcium screening for all-cause mortality.
history of coronary artery disease Radiology. 228(3):826-33, 2003
May modify risk obtained using traditional 9. Agatston AS et a1: Quantification of coronary artery
(Framingham) risk factors calcium using ultrafast computed tomography.) Am Coll
Best use probably in patients with intermediate Cardiol. IS(4):827-32, 1990
risk factors
 CORONARY ARTERY CALCIFICATION
[IMAGE GALLERY

(Left) Axial CECT shows

calci(ication in the left
anterior descending artery
(arrow). (Right) Chart shows
calcium score of 208.2 using
the Agatston method.

(Left) Lateral radiograph

shows anterior tram-track
calcification (arrow).
Calcification in this location
is in the left anterior
descending or right coronary
arteries. (Right) Axial CECT
shows dense calcification
(arrow) in the right coronary
artery.

(Left) Frontal radiograph

shows tram track
calcification (arrow)
projecting over the upper left
heart in the coronary
triangle. (Right) Axial CECT
shows that the calcification
in the previous chest
radiograph is located
primarily in the left anterior
descending coronary artery.
 LEFT ATRIAL CALCIFICATION

3
16

Coronal oblique graphic shows calcification in the LAteral radiograph shows concentric left atrial
lateral wall of the left atrium (arrow). calcifications in a patient with rheumatic fever (arrows).

o Left atrium
ITERMINOLOGY Lying in the center of the cardiac silhouette
Abbreviations and Synonyms beneath the carina and the main stem bronchi
Coconut atrium Size
o Variable
Definitions Usually 8-10 em in diameter on chest radiograph
Three general appearances according to extent of Morphology: Thin, smooth, and curvilinear
calcifications
o Extensive calcification of the wall Radiographic Findings
o Calcification of portions of the wall General
o Calcification confined to the area of the left atrial o Mural calcification
appendage Thin, curvilinear opacity partially or completely
MacCallum patch tracing the outline of the left atrium
o Jet lesion on posterior wall of the left atrium o Calcification can occasionally extend into
corresponding to the site of mitral regurgitant flow pulmonary veins
o Calcification usually more extensive than revealed
by radiographs
IIMAGING FINDINGS o Can be confused with mural thrombus calcifications
Thrombus calcification is usually laminated and
General Features non-linear (thicker)
Best diagnostic clue Intramural calcification is linear, non-laminated,
o Curvilinear density that traces outline of left atrium and marginal in distribution
Patients with history of rheumatic carditis Posteroanterior (PA) view
Location

DDx: Cardiac Calcifications

Calcific Aortic Stenosis Ventricular Cales Aortic Valve Cales

 LEFT ATRIAL CALCIFICATION

Key Facts
Terminology Valvular Calcification
Three general appearances according to extent of Aortic valve
calcifications Mitral valve
Extensive calcification of the wall Annular Calcifications
Calcification of portions of the wall Primary Cardiac Neoplasms
Calcification confined to the area of the left atrial Myxoma
appendage Osteogenic sarcoma
Coronary Artery Calcifications
Imaging Findings
Pathology
Completely calcified wall appears as a C-shaped
curvilinear opacity with opening of C lying anteriorly Calcification usually dystrophic due to abnormal
in the region of mitral annulus tissue or flow hemodynamics
NECT: More sensitive than chest radiography for Clinical Issues
3
detection of calcium History of rheumatic fever 17
Top Differential Diagnoses Middleaged patients
Myocardial Calcification Female predominance (3:1)

o Round or oval shell of calcium approximately 8-10 Mitral valve

cm in diameter o Common sequela of rheumatic mitral valve disease
Usually seen lying in the center of cardiac o Enlargement of the left atrium (LA), especially the
silhouette beneath the carina and main stem LA appendage
bronchi o Nodular or amorphous pattern on chest radiographs
Lateral view Pulmonary and tricuspid valves
o Completely calcified wall appears as a C-shaped o Calcifications: Rare
curvilinear opacity with opening of C lying
anteriorly in the region of mitral annulus
Annular Calcifications
Degenerative process
CT Findings o Most often seen in individuals older than age 40
NECT: More sensitive than chest radiography for (women> > men)
detection of calcium o Unless massive, calcification not clinically
important
Imaging Recommendations Mitral annulus
Best imaging tool o Echocardiography more sensitive than plain
o NECT radiographs for detection of calcifications
More sensitive than chest radiography o A,], U, or reverse C-shaped band-like calcification
Protocol advice: If thoracic CT is performed, gated Can appear O-shaped if the anterior leaflet also is
image sequences are helpful involved
o Calcification: Band-like and of uniform radiopacity
o Mitral valve calcification is nodular and more
I DIFFERENTIAL DIAGNOSIS irregular on chest radiographs
Aortic annulus
Myocardial Calcification o Associated with a calcified aortic valve
Usually in patients with sizable left ventricular infarcts May extend superiorly into ascending aorta or
Features inferiorly into interventricular septum
o Thin and curvilinear shaped
o Oriented toward the apex of the left ventricle Primary Cardiac Neoplasms
o Can rarely appear spherical or plate-like Myxoma
o Predilection for intra-atrial septum, especially fossa
Valvular Calcification ovalis
Aortic valve o Rarely calcify (16%)
o Bicuspid aortic valve o Echocardiography
Nodular, semilunar, or mushroom-shaped Characteristic narrow stalk
Thick, irregular, semilunar ring pattern with a Mobile and distensible
central bar or knob is typical o Heterogeneous low attenuation on CT scan
PA: Calcification is usually left paraspinous Osteogenic sarcoma
Lateral: Calcification is usually anterior o Most common location: Left atrium
o Atherosclerosis o Differentiated from myxoma on CT by
Calcification usually nodular Broad based attachment
Intimal calcification in wall of aorta Aggressive growth pattern
Diffuse aortic dilatation common Invasion of atrial septum
 LEFT ATRIAL CALCIFICATION
Infiltrative growth along the epicardium Long-standing mitral stenosis
Atrial fibrillation
Coronary Artery Calcifications
Mural thrombus
Strong association with atherosclerotic heart disease Systemic pulmonary emboli
Gated electron beam CT (EBCT) is gold standard in
detecting and quantifying coronary artery Demographics
calcifications Age
o Rapid image acquisition time virtually eliminates o Middle aged patients
motion artifact related to cardiac contraction Usually in fifth or sixth decade of life
Can also be detected via multi detector CT (MDCT), Gender
coronary angiography and intravascular ultrasound o Female predominance (3:1)
(IVUS) Higher female incidence of rheumatic
o EBCT and gated MDCT are extremely sensitive in involvement of the mitral valve
detecting vascular calcification Ethnicity: Native Hawaiian and Maori (both of
3 Polynesian descent) have a higher incidence of
rheumatic fever
18 I PATHOLOGY
Natural History & Prognosis
General Features Cardiovascular system symptoms usually chronic
General path comments o Symptoms usually present an average of 20 years
o Calcification usually dystrophic due to abnormal prior to recognition of left atrial calcification
tissue or flow hemodynamics Amount of calcification related to duration of
May be degenerative untreated disease
Genetics: No genetic predisposition Left atrial calcification may complicate valve
Etiology replacement due to
o Unknown o Impaired hemostasis
o Thought to be the end-result of repeated and o Potential embolization
extensive rheumatic auriculitis o Elevated left atrial pressure
Epidemiology
o Incidence of atrial calcification related to severity of
Treatment
original rheumatic attack and associated valvular Procedure of choice
damage o Total endoatriectomy of calcified left atrium
o Intra-atrial septum often free of calcifications o Reported hospital mortality rate of 12.5%
Associated abnormalities: Adherent mural thrombi o Mitral valvuloplasty does not provide cure
(common finding) Does not change intra-atrial pressures

Staging, Grading or Classification Criteria

Classified according to location of calcium and I DIAGNOSTIC CHECKLIST
dominant lesion
o Type A Consider
Calcification confined to the left atrial appendage Calcifications can be rarely identified in primary
only cardiac malignancies such as in
Dominant lesion is less-severe mitral stenosis o Leiomyosarcoma
Calcification almost always associated with o Paraganglioma
thrombus in the appendage
o Type B
Calcification of the left atrial appendage, the free I SELECTED REFERENCES
left atrial wall, and the mitral valve 1. Grebenc ML et al: Cardiac myxoma: imaging features in 83
Dominant lesion is mitral stenosis patients. Radiographies. 22(3):673-89, 2002
More severe than type A 2. Araoz et al: CT and MR Imaging of Primary Cardiac
o Type C Malignancies. Radiographies. 19:1421-1434 1999
Calcification of a MacCallum patch lesion in the 3. VallejoJL et al: Massive Calcification of the Left Atrium:
posterior left atrial wall Surgical Implications. Ann Thorac Surg. 60:1226-1229,
1995
Dominant lesion of mitral insufficiency 4. Duerinckx AJet al: Valvular Heart Disease. Radiol Clin
North Am. 32, 1994
5. Lee VSet al: Atypical and unusual calcifications of the
ICLINICAL ISSUES heart and great vessels: Imaging findings. AJR.
163:1349-55,1994
Presentation 6. Shaw DRet al: X-ray Appearance and Clinical Significance
Most common signs/symptoms of Left Atrial Wall Calcification. Invest Rad. 11:501-507,
o History of rheumatic fever 1976
Incidence in rheumatic population estimated at 7. Harthorne JW et al: Left Atrial Calcification. Review of
o.S%
Literature and Proposed Management. Circul 34:198-210,
1966
o Frequent association with
 LEFT ATRIAL CALCIFICATION

IIMAGE GALLERY

(Left) Frontal radiograph

shows an enlarged left
atrium as well as atrial
calcifications (open arrow).
Incidentally, there are mitral
annular calcifications as well
(curved arrow). (Right)
Lateral radiograph shows an
enlarged left atrium with
associated calcifications
(arrows). 3
19

(Left) Frontal radiograph

shows eccentric left atrial
calciFications in a
(arrows)
patient with left upper lobe
pneumonia. (Right) Frontal
radiograph shows
multichamber enlargement
of the cardiac silhouette in a
patient with left atrial
calcifications (arrows).
Patient had a history of
rheumatic fever.

(Left) Lateral radiograph

shows crescentic leFt atrial
calcifications (arrows) in a
patient with longstanding
mitral stenosis. (Right)
Frontal radiograph shows
large eccentric LA
calcifications (arrows) in a
patient who has undergone
multiple valvuloplasties for
rheumatic fever.
 VENTRICULAR CALCIFICATION

3
20

Graphic shows calcification in the wall of a true left Lateral radiograph shows thin, curvilinear calcification
ventricular aneurysm. Calcification generally seen with (arrows) in a patient with a large ventricular aneurysm.
large remote (more than 6 years) myocardial infarction. Wide mouth suggests a true ventricular aneurysm.

ITERMINOLOGY IIMAGING FINDINGS

Abbreviations and Synonyms General Features
Myocardial infarction (MI) Location
o Occurs most frequently in true left ventricular
Definitions aneurysms localized to the apical and anterolateral
Dystrophic calcification aspects of the left ventricular wall
o Calcification occurring in areas of myocardial o Rare in the right ventricle
necrosis, hemorrhage, or fibrosis o Isolated extensive papillary muscle calcification rare
o Can also be found without focal myocardial
Size
abnormality in older individuals o Normal left ventricular myocardium is 1 em thick at
o Not associated with elevated serum calcium or diastole
phosphorus levels o Right ventricular free wall is 2-3 mm thick
Metastatic calcification Morphology: Deposits are usually thin, curvilinear and
o Calcification occurring in otherwise normal tissue located within the periphery of the infarct or
o Associated with elevated levels of serum calcium aneurysm, in the distribution of the interventricular
o Common locations: Skin, cornea, lungs, stomach, septum and cardiac apex
and kidneys
Idiopathic cardiac calcification Radiographic Findings
o Calcification without any underlying etiology General
o Myocardial calcification most commonly
dystrophic, result of remote myocardial infarction
o Calcification underestimates size of underlying MI

DDx: Cardiac Calcification

 VENTRICULAR
Key Facts
Imaging Findings
Occurs most frequently in true left ventricular
aneurysms localized to the apical and anterolateral
aspects of the left ventricular wall
Rare in the right ventricle
Morphology: Deposits are usually thin, curvilinear
and located within the periphery of the infarct or
aneurysm, in the distribution of the interventricular
septum and cardiac apex
Myocardial calcification most commonly dystrophic,
result of remote myocardial infarction
Calcification underestimates size of underlying Ml
Top Differential Diagnoses
Pericardial Calcification
Valvular Calcification
Coronary Artery Calcification
o Overpenetrated examinations provide better
visualization of calcium
o Low kVp or dual energy substraction more sensitive
to detect calcification
Fluoroscopy
o More sensitive to the presence of calcium than
radiography, as pulsatile motion of calcium in the
beating heart improves visual acuity
o Rotating the patient may separate portions of
otherwise non-visualized calcifications from
overlapping spine and confirm anatomic location
(intracardiac vs. pericardial)
CT Findings
NECT: Myocardial calcification appears as dense,
irregular linear calcifications within left ventricle wall
CECT
o Distinct advantage over catheter ventriculography
its ability to define the external and internal borders
of the ventricular myocardium
o Ventricular myocardium and cavitary blood
enhance
o Mural thrombus does not enhance
o True aneurysm
Wide mouth
May contain mural thrombus
Primarily apical or antero-Iateral ventricular wall
Generally arise from left coronary artery
circulation
o False aneurysm
Narrow mouth
Primarily posterior, lateral, or diaphragmatic
ventricular wall
Generally arise from right coronary artery
circulation
Imaging Recommendations
Best imaging tool: Contrast-enhanced CT to detect
and characterize
CALCIFICATION
Left Atrial Calcifications
Cardiac Fibroma
Metastatic Cardiac Tumors
Calcified Hydatid Cysts
Pathology
General path comments: Takes approximately 6 years
to develop calcification in infarct
Associated abnormalities: Metastatic cardiac
calcification common in chronic renal disease and
may be a factor in cardiomyopathy
Clinical Issues
Calcified infarct at increased risk for sudden death
3
Myocardial calcification is found in 8% of cases of Ml 21
greater than 6 years old
I DIFFERENTIAL DIAGNOSIS
Pericardial Calcification
Occurs most often with previous acute inflammation
or blunt trauma; most common causes include
o Coxsackie or influenza A and B viral infection
o Granulomatous disease (TB, histoplasmosis)
o Hemopericardium following trauma
o Autoimmune disease (SLE, rheumatic heart disease)
Up to 50% of patients with constrictive pericarditis
have pericardial calcification
Pericardial tumors such as intrapericardial teratomas
and cysts occasionally calcify
Calcification most abundant along the right atrial and
ventricular borders, and may also be found in the AV
groove ..
in o Pericardium adjacent to the left ventncle IS usually
free of calcification, probably because of its vigorous
pulsations
o May present as a thin focal plaque, or a long
curvilinear layer following the cardiac contour
o Tends to be clunky and "ugly" in character, as
opposed to fine and curvilinear character of
myocardial calcification
o Rarely occurs along the left atrial border because of
absence of pericardium behind the left atrium
o Often obscured on frontal chest film owing to
underexposure of mediastinal structures
o Overpenetrated films or fluoroscopy helpful in
localization
Valvular Calcification
Mitral or aortic calcification
Valve calcification usually nodular
Mitral: Associated left atrial enlargement
Aortic: Associated enlargement ascending aorta and
left ventricular hypertrophy
Located within the heart, at the root of the aorta for
aortic valve and just posterior to major axis of the
heart on the lateral examination for the mitral valve
 VENTRICULAR
Coronary Artery Calcification
Coronary calcification has tram-track appearance
Frontal radiograph: Coronary triangle along the upper
left border of the heart
Forms in atherosclerotic plaque, not necessarily
stenotic
Left Atrial Calcifications
Coconut atrium
Patients with history of rheumatic carditis
Morphology: Thin, smooth, and curvilinear
Cardiac Fibroma
3 Second most common benign primary cardiac tumor
in children; but rare (- 100 cases reported since 1976)
22
o Often associated with arrhythmias
Increased prevalence of fibromas in Gorlin
syndrome (Nevoid basal cell carcinoma syndrome)
Dystrophic calcification common
Computed tomography
o Calcified homogeneous mass with soft tissue
attenuation that may be sharply marginated or
infiltrative
o CECT: Often demonstrate little or no contrast
material enhancement, because of their dense,
fibrous nature
Metastatic Cardiac Tumors
More common than primary cardiac malignancies
These tumors do not calcify sufficiently for plain film
diagnosis
Calcified Hydatid Cysts
Cardiac echinococcosis (hydatid cysts) account for
0.5-2% of all human cases of echinococcosis
Most often symptomless, but can present with chest
pain, valvular dysfunction, or sudden death
60% located in left ventricle, presumably due to richer
coronary circulation
o Calcification rare
I PATHOLOGY
General Features
General path comments: Takes approximately 6 years
to develop calcification in infarct
Etiology
o Excess calcium intake, chronic inflammation, and
malnutrition are associated with an increased risk
for development of cardiac calcifications
o Postulated mechanisms in myocardial soft tissue
calcification include
Carbon dioxide production in slowly metabolizing
tissue (infarcted myocardium)
Relative alkalinity
Calcium less soluble in alkaline environment
Epidemiology
o Acute myocardial infarction (AMI) is a leading cause
of morbidity and mortality in the United States
Approximately 1.3 million cases of nonfatal AMI
are reported each year, for an annual incidence of
approximately 600 per 100,000 people
CALCIFICATION
Associated abnormalities: Metastatic cardiac
calcification common in chronic renal disease and
may be a factor in cardiomyopathy
Gross Pathologic & Surgical Features
Aneurysms show paradoxical expansion with
ventricular contractions, as opposed to diverticula
which contract synchronously
Microscopic Features
Calcification part of the intimal plaque in
atherosclerosis
ICLINICAl ISSUES
Presentation
Most common signs/symptoms: Cardiac symptoms:
Chest pain/angina, shortness of breath
Other signs/symptoms: True aneurysm may serve as
arrhythmogenic source or result in congestive heart
failure (CHF)
Demographics
Age: AMI occurs most frequently in persons older than
45 years
Gender
o Male predilection for AMI exists in persons aged
40-70 years
Persons older than 70 years, no sex predilection
Ethnicity: Cardiovascular disease is the leading cause
of morbidity and mortality among African-American,
Hispanic, and Caucasian populations in USA
Natural History & Prognosis
Calcified infarct at increased risk for sudden death
Myocardial calcification is found in 8% of cases of MI
greater than 6 years old
False aneurysm is true perforation, may rupture
resulting in sudden death
Treatment
Dialysis for metastatic calcification
Medical and surgical therapy for coronary artery
disease
Left ventricular aneurysm resection
o Indications
Congestive heart failure
Arrythmia where arrythmogenic focus is
aneurysm
I SELECTED REFERENCES
1. Gowda RMet al: Calcifications of the heart. Radial Clin N
Am. 42:603-617, 2004
2. Boxt LMet al: Computed tomography for assessment of
cardiac chambers, valves, myocardium, and pericardium.
Cardiol Clin. 21:561-585, 2003
3. Braunwald: Heart Disease: A Textbook of Cardiovascular
Medicine, 6th ed.W.B. Saunders Company, 2001
4. Krasemann T et al: Calcification at the Left Cardiac Border.
Chest. 119:618-621,2001
5. Araoz et al: CT and MR Imaging of Benign Primary Cardiac
Neoplasms with Echocardiographic Correlation.
Radiographies. 20:1303-1319, 2000
 VENTRICULAR CALCIFICATION
IIMAGE GALLERY

(Left) Frontal radiograph

shows dystrophic ventricular
calcification (arrows) in
ventricular aneurysm from a
previous myocardial
infarction. Note the
abnormal left ventricular
contour. (Right! Lateral
radiograph shows calcified
left ventricular aneurysm
(arrows) in a patient that is 3
post placement of a thoracic
aortic endovascular stene.
23

(Left! Axial CECT shows

calcification in cardiac apex
(arrow) in a patient with a
left ventricular aneurysm.
Aneurysm contains a large
thrombus. (Right! Frontal
radiograph shows thin,
curvilinear calciFication
(arrows) in a left ventricular
aneurysm in patient with
remote history of MI.

(Left) Lateral radiograph

shows curvilinear
calcification (arrows) in a
patient with a large
wide-mouth left ventricular
aneurysm. (Right) Axial bone
CT shows moderate
calcification in the apex and
distal interventricular septum
(arrow) in a patient who has
undergone a prior median
sternotomy.
 VALVE AND ANNULAR CALCIFICATION

3
24

Radiograph shows artificial aortic (arrows), mitral Laleral radiograph shows aortic valve calcification (open
(curved arrows), and tricuspid valves (open arrows). arrow). Note the dense bar centrally (arrow) consistent
Recognition of valve calcification requires knowledge of with fused raphe in bicuspid valve.
the expected location of the valves.

ITERMINOlOGY o Valves overlap spine on the frontal view,

calcification best seen on the lateral view
Definitions Aortic valve
Calcium deposition along valves results in stiffening of o Often projected over the spine on frontal radiograph
the leaflets, stenosis, narrowing and resistance to o On lateral view, line drawn from carina to
blood flow through the valve sternodiaphragmatic junction passes through
Mitral annulus calcification a benign degenerative expected location of aortic valve
process o Found within middle third of cardiac silhouette on
lateral view
o Degenerative aortic valve calcification
I IMAGING FINDINGS Commisural fusion not a feature
Nodular calcific masses distort normal valve
General Features architecture
Best diagnostic clue: Calcification or increased opacity Signs of left heart failure usually found in patients
in the expected valve location with concomitant mitral regurgitation
o Bicuspid aortic valve calcification
Radiographic Findings Nearly circular calcification with an interior linear
Radiography bar is diagnostic
o Quantity of calcification directly related to degree of Linear calcification involving raphe and edges of
stenosis cusps
o Valve calcification generally nodular or clumped Vertical club or cobra head from calcification of
o Calcium first appears as fine speckled opacities and fused raphe
later coalesces into larger amorphous mass
o Calcified valve usually produces stenosis of the valve

DDx: Cardiac Calcifications

Left Ventricular Aneurysm Coronary

 VALVE AND ANNULAR CALCIFICATION

Key Facts
Terminology Valve calcification may be incidental without
Calcium deposition along valves results in stiffening hemodynamic stenosis
of the leaflets, stenosis, narrowing and resistance to Best imaging tool: Echocardiography: Procedure of
blood flow through the valve choice to determine valve morphology and function
Mitral annulus calcification a benign degenerative Top Differential Diagnoses
process Ventricular Calcification
Imaging Findings Atrial Calcification
Best diagnostic clue: Calcification or increased Pericardial Calcification
opacity in the expected valve location Coronary Artery Calcification
Quantity of calcification directly related to degree of Pathology
stenosis
Valve calcification generally nodular or clumped
Calcification usually dystrophic or degenerative
to abnormal tissue or flow hemodynamics
due 3
Calcified valve usually produces stenosis of the valve
Clinical Issues 25
Valve calcification usually central
Annulus calcification peripheral and rim-like Left atrial calcification may complicate valve
replacement due to risk of bleeding and embolization

Secondary manifestations: Aortic stenosis,
prominent ascending aorta, left ventricular
hypertrophy
Mitral valve
a On lateral view, mitral valve below and posterior to
line drawn from carina to sternodiaphragmatic
junction
a Secondary manifestations of mitral stenosis
Left atrium enlargement, double contour sign,
bulging of left atrial appendage
Pulmonary venous hypertension, pulmonary
vascular cephalization and interstitial pulmonary
edema
Long-standing: Hemosiderosis associated
interstitial lung disease
Mitral annulus
a Calcium first forms in or below mitral annulus at
junction between ventricular myocardium and
posterior mitral leaflet
a Uniform, band-like, C or horseshoe-shaped
calcification
a With involvement of anterior leaflet, can appear
a-shaped
a Measures 10 em in circumference
Aortic and mitral valve localization
a Posteroanterior (PA) view: Valves overlap adjacent to
spine, difficult to separate; clues
Aortic valve: In profile, horizontally positioned
Mitral valve: En face, vertically positioned
a Lateral view: Heart is football-shaped, "lace" the
football
Aortic valve is anterior, mitral valve is posterior to
laces
Pulmonic valve
a Superior to aortic valve on both frontal and lateral
radiographs
a Secondary manifestations of pulmonary valve
stenosis
Enlarged main and left pulmonary artery
Decreased pulmonary vascularity when severe
(usually cyanotic)
Upper lobe opacities from systemic collaterals
(pseudofibrosis)
Tricuspid valve
a Below and separated from pulmonic valve by
infundibulum of pulmonary outflow track
a Secondary manifestations of tricuspid stenosis
Right heart dilatation with right atrium behind
sternum, displacement of superior vena cava
medially, clockwise rotation of cardiac apex and
bowing of interventricular septum towards left
Dilation of superior vena cava and azygos vein
may also be seen
CT Findings
Valve calcification usually central
a Annulus calcification peripheral and rim-like
Valve calcification may be incidental without
hemodynamic stenosis
Echocardiographic Findings
Severity of the stenosis determined by the size of the
orifice
Imaging Recommendations
Best imaging tool: Echocardiography: Procedure of
choice to determine valve morphology and function
I DIFFERENTIAL DIAGNOSIS
Ventricular Calcification
Occurs most frequently in infarcts or aneurysms
localized to apical, anterolateral and septal walls of left
ventricle
Deposits are usually thin, curvilinear and located
within periphery of infarct or aneurysm, in
distribution of the interventricular septum and cardiac
apex
Takes approximately 6 years to develop calcification in
infarct
Atrial Calcification
Occurs most frequently in left atrium as sequela of
rheumatic endocarditis
 VALVE AND ANNULAR CALCIFICATION
Thin, smooth, curvilinear calcification of left atrial
appendage (most common)
"Egg shell" or "coconut" atrium
Pericardial Calcification
Clumpy amorphous calcification most abundant along
atrioventricular groove, right atrial and ventricular
borders
More commonly over right ventricle
50-70% have constrictive pericarditis
Often end stage sequelae of uremia, trauma, viral or
tuberculous myocarditis
Coronary Artery Calcification
3 Tubular morphology, may appear circular on end
Left anterior descending artery followed by left
circumflex and right coronary are most frequently
26
involved sites
I PATHOLOGY
General Features
General path comments
o Calcification usually dystrophic or degenerative due
to abnormal tissue or flow hemodynamics
o Calcific process begins in valvular fibrosa at points
of maximal cusp flexion (margins of attachment),
progression to heaped-up, nodular calcific masses
that prevent opening of cusps
o Distortion of cuspal architecture primarily at bases,
spares free cuspal edges
Etiology
o Aortic valve: Aging, congenital bicuspid aortic valve,
rheumatic aortic valve, syphilis, ankylosing
spondylitis
o Mitral valve: Rheumatic mitral valve disease (most
common)
o Mitral annulus calcification a degenerative process
o Pulmonic valve: Congenital, chronic pulmonary
hypertension, following tetralogy repair
o Tricuspid valve: Rheumatic tricuspid valve disease
(most common)
o Valve calcification uncommon in mitral valve
prolapse or in tricuspid or pulmonic valve pathology
Epidemiology
o Mitral annulus calcification more common in
elderly women, incidence increased in patients with
idiopathic hypertrophic subaortic stenosis (IHSS)
o Bicuspid aortic valve 2% of population
Gross Pathologic & Surgical Features
Bicuspid aortic valves: 90% calcified at surgery
I CLINICAL ISSUES
Presentation
Most common signs/symptoms
o Aortic stenosis
Exertional dyspnea alongside classic triad of
angina pectoris, syncope and heart failure
"Pulsus parvus et tardus" on physical exam
Crescendo-decrescendo systolic ejection murmur
with paradoxical S2 split on cardiac auscultation
o Mitral stenosis
Exertional dyspnea frequently accompanied by
cough and wheezing
Abrupt onset atrial fibrillation
Stress induced pulmonary edema (pregnancy)
Loud SI followed by S2 and "opening snap" with
low-pitched, rumbling diastolic murmur on
cardiac auscultation
o Pulmonic stenosis
Exertional dyspnea, fatigue
Systolic ejection click louder on expiration, with
ejection murmur audible at left upper sternal
border, transmitting to back on cardiac
auscultation
o Tricuspid stenosis
Fatigue due to limited cardiac output
Systemic venous congestion may result in
abdominal complaints of discomfort and swelling
Widely split SI with single S2 and diastolic
murmur along left sternal border on cardiac
auscultation
Demographics
Age
o Aortic calcification
Bicuspid valve below age 70
Calcific degenerative above age 70
More than 90% of patients with congenital
bicuspid valve have valve calcification by 40 years
o Mitral calcification: 3rd and 4th decades
o Mitral annulus calcification over age 60
Gender: Women seen more frequently
Natural History & Prognosis
Left atrial calcification may complicate valve
replacement due to risk of bleeding and embolization
Mitral annulus calcification
o Associated with doubled risk of stroke in elderly
Treatment
Surgical replacement abnormal valves
I SElECTED REFERENCES
1. Adler Yet al: Usefulness of helical computed tomography
in detection of mitral annular calcification as a marker of
coronary artery disease. lnt J Cardiol. 101(3):371-6,2005
2. Aksoy Yet al: Aortic valve calcification: association with
bone mineral density and cardiovascular risk factors.
Coron Artery Dis. 16(6):379-83,2005
3. Boxt LM:CT of valvular heart disease. Int J Cardiovasc
Imaging. 21(1):105-13, 2005
4. Koos R et al: Preliminary experience in the assessment of
aortic valve calcification by ECG-gated multislice spiral
computed tomography. Int J Cardiol. 102(2):195-200,2005
5. Molad Y et al: Heart valve calcification in young patients
with systemic lupus erythematosus: A window to
premature atherosclerotic vascular morbidity and a risk
factor for all-cause mortality. Atherosclerosis. 2005
6. Seo Y et al: Relationship between mitral annular
calcification and severity of carotid atherosclerosis in
patients with symptomatic ischemic cerebrovascular
disease. J Cardiol. 46(1): 17-24, 200S
 VALVE AND ANNULAR CALCIFICATION
I IMAGE GALLERY

(Left) Laleral radiograph

shows aortic valve
calcification (open arrows).
Valve calcification is Iypically
nodular or clumped (Right!
Laleral radiograph shows an
enlarged lefl alrium (arrows)
and faint calcification (open
arrow) of the mitral valve.

3
27

Typical
(Left) Fronlal radiograph
shows large
horseshoe-shaped
calcification of the mitral
annulus (open arrows). Left
atrium is not enlarged.
(Right! Laleral radiograph
shows C-shaped nodular
calcification of the mitral
annulus (arrows). Annulus
calcification is much larger
then the mitral valve.

(Left! Laleral radiograph of

calcification of the pulmonic
valve (arrow). Pulmonic
valve is more cephalad and
posterior than the aortic
valve. RighI pulmonary
arlery is also small (curved
arrow) due to severe
pulmonic slenosis. (Right!
Lateral radiograph shows
nodular curvilinear
calcification of the aortic
valve (arrow). Bicuspid valve
replaced for severe aortic
stenosis.
 AORTIC VALVE DYSFUNCTION

3
28

Graphic shows normal aortic valve and degenerative Axial CECT shows extensive calcification of the aortic
aortic valve with thickening along the base of the valve valve (arrow) and left ventricular hypertrophy consistent
resulting in aortic stenosis. With severe aortic stenosis with aortic stenosis. Calcification is central in aorta.
the left ventricle often hypertrophies.

Aortic stenosis leads to left ventricular hypertrophy

ITERMINOLOGY and angina, syncope, and heart failure
Abbreviations and Synonyms
Aortic regurgitation (AR)
Aortic insufficiency I IMAGING FINDINGS
Aortic stenosis (AS) General Features
Definitions Best diagnostic clue
Aortic dysfunction comprises aortic regurgitation and o For aortic stenosis: Aortic valve calcification
aortic stenosis o For aortic regurgitation: Dilated left ventricle and
AR may be acute or chronic aorta
Acute AR due to endocarditis, aortic dissection or Location: Aortic valve
trauma Size: In aortic stenosis, a valve size orifice size of < 1
Acute AR leads to poor tolerance of volume load by em-squared considered diagnostic
the normal left ventricle causing pulmonary edema
Radiographic Findings
Chronic AR due to multiple causes including
connective tissue disease, rheumatic heart disease, Radiography
o Aortic regurgitation
bicuspid valve, collagen vascular disease, and rarely
Large cardiac silhouette, especially left ventricle
syphilis
Dilated aorta
Chronic AR leads over time to left ventricle dilatation
Normal pulmonary vasculature until heart failure
and ultimately heart failure symptoms
supervenes
Aortic stenosis congenital or due to senile degenerative
o Aortic stenosis
changes or rheumatic fever
Normal cardiac silhouette size, cardiac apex may
be slightly up-turned

DDx: Aortic Dysfunction Mimics

5ubvalvular IH55 Coarctation Mitral Valve Disease

 AORTIC VALVE DYSFUNCTION
Key Facts
Terminology
Aortic dysfunction comprises aortic regurgitation and
aortic stenosis
AR may be acute or chronic
Acute AR due to endocarditis, aortic dissection or
trauma
Acute AR leads to poor tolerance of volume load by
the normal left ventricle causing pulmonary edema
Chronic AR due to multiple causes including
connective tissue disease, rheumatic heart disease,
bicuspid valve, collagen vascular disease, and rarely
syphilis
Aortic stenosis congenital or due to senile
degenerative changes or rheumatic fever
Aortic stenosis leads to left ventricular hypertrophy
and angina, syncope, and heart failure
Aortic valve calcification best identified on the
lateral view by tracing the ascending aorta toward
the aortic root
Dilated ascending aorta in some patients due to
posts ten otic dilatation; best observed along upper
right heart border on posteroanterior radiographs
CT Findings
CECT
a Aortic regurgitation
Left ventricular enlargement
Dilated ascending and often descending aorta
If transition point between sinus and tubular part
of aorta (sinotubular junction) effaced, suspect
connective tissue disease such as Marfan disease;
best observed on coronal reformats
Normal pulmonary vasculature in the absence of
heart failure
Septal lines, prominent vessels, airspace disease,
pleural effusions with cardiac decompensation
a Aortic stenosis
Central aortic valve calcification; distinguish from
aortic annulus calcification which is peripheral
and rim-like at the aortic root
Cine-CT images may show bicuspid valve
Left ventricular hypertrophy
Post-stenotic dilatation of aorta
Normal pulmonary vasculature in the absence of
heart failure
Septal lines, prominent vessels, airspace disease,
pleural effusions with cardiac decompensation
Angiographic Findings
Conventional
a Extent of AR assessed on a 1+ to 4+ (most severe)
basis
a In AS, peak pressure gradient can be assessed
MR Findings
TlWI
a Aortic regurgitation
Left ventricular enlargement
Dilated ascending and often descending aorta
Imaging Findings
For aortic stenosis: Aortic valve calcification
For aortic regurgitation: Dilated left ventricle and
aorta
Best imaging tool: Echocardiography
Top Differential Diagnoses
Subvalvular Aortic Stenosis
Supravalvular Aortic Stenosis
Mitral Valve Disease
Pathology
Associated abnormalities: Aortic stenosis associated 3
with bicuspid aortic valve and aortic coarctation
29
Clinical Issues
Aortic stenosis: Angina, syncope, dyspnea
If transition point between sinus and tubular part
of aorta (sinotubular junction) effaced, suspect
connective tissue disease such as Marfan disease;
best observed on coronal reformats
Pleural effusions may be present with
decompensation
a Aortic stenosis
Aortic valve calcification not visible
Left ventricular hypertrophy
Post-stenotic dilatation of aorta
Pleural effusions may be present with
decompensation
T2* GRE
a Aortic regurgitation
Left ventricular enlargement
Dilated ascending and often descending aorta
If transition point between sinus and tubular part
of aorta (sinotubular junction) effaced, suspect
connective tissue disease such as Marfan disease;
best observed on coronal reformats
Prominent vessels and pleural effusions may be
present with cardiac decompensation
Diastolic jet extending from valve plane into left
ventricle on flow sensitive images reflects
dephasing and may be qualitatively related to
severity of AR
Can calculate regurgitant fraction and systolic and
diastolic volumes
a Aortic stenosis
Aortic valve calcification not visible
Bicuspid valve may be visible on cine images
Left ventricular hypertrophy
Post-stenotic dilatation of aorta
Prominent vessels and pleural effusions may be
present with cardiac decompensation
Systolic jet extending from valve plane into aorta
on flow sensitive images reflects dephasing and
may be qualitatively related to severity of AS
Can calculate regurgitant fraction, systolic and
diastolic volumes
MRA
a Aortic regurgitation
 AORTIC VALVE DYSFUNCTION
Dilated ascending and descending aorta often
present
o Aortic stenosis
Post-stenotic dilatation of aorta evident
Fluoroscopic Findings
Chest Fluoroscopy: Rarely used; identifies valvular
calcification
Echocardiographic Findings
Echocardiogram
o Aortic regurgitation
Lack of coaptation of aortic valve leaflets
Evidence of left ventricular volume overload
3 Regurgitant fraction can be determined by
Doppler of regurgitant jet
30 o Aortic stenosis
Determine cause including bicuspid valve,
rheumatic, or senile
Assess gradient across valve and area of valve
orifice 1 cm squared considered critical)
SO mm Hg gradient considered significant
Imaging Recommendations
Best imaging tool: Echocardiography
I DIFFERENTIAL DIAGNOSIS
Subvalvular Aortic Stenosis
Idiopathic hypertrophic subaortic stenosis (IHSS):
Asymmetric thickening ventricular septum
Associated with ventricular septal defect
Supravalvular Aortic Stenosis
Hourglass narrowing above aortic sinuses
Associated with Marfan syndrome, William syndrome
Mitral Valve Disease
Left atrial enlargement
Valve more posterior on lateral view
I PATHOLOGY
General Features
Genetics: AR occurs in patients with Marfan syndrome
and other genetically-linked connective tissue diseases
Etiology
o Acute AR due to endocarditis, aortic dissection or
trauma
o Chronic AR causes include connective tissue disease,
rheumatic heart disease, bicuspid valve, collagen
vascular disease, and rarely syphilis
o Aortic stenosis may be congenital, due to senile
degenerative changes or to rheumatic fever
Associated abnormalities: Aortic stenosis associated
with bicuspid aortic valve and aortic coarctation
Gross Pathologic & Surgical Features
Aortic regurgitation causes valve fibrosis and
thickening if due to rheumatic heart disease
Aortic stenosis pathology shows calcification
beginning at the base of the cusps; rheumatic valves
show fibrosis and thickening along the commissural
edge
Microscopic Features
Aortic stenosis shows accumulation of lipid and
inflammatory cells
I CLINICAL ISSUES
Presentation
Most common signs/symptoms
o Aortic regurgitation: Chest pain, dyspnea
o Aortic stenosis: Angina, syncope, dyspnea
Demographics
Age
o In aortic regurgitation, age is variable except if
congenital
o In aortic stenosis, presentation in teens or twenties
due to unicuspid valve, fifth to seventh decade due
to bicuspid valve, and in later years due to senile
changes on a tricuspid valve
Gender
o Aortic regurgitation has a 3: 1 male predominance
o Aortic stenosis more common in males; bicuspid
aortic valve has a 4: 1 male predominance
Natural History & Prognosis
Aortic regurgitation
o In chronic AR, variable progression to left
ventricular failure; prognosis poor if valve not
replaced prior to left ventricular failure
Aortic stenosis
o In senile form, long asymptomatic period prior to
develop of angina, syncope or dyspnea
Treatment
Aortic regurgitation
o Medical management in mild to moderate AR
without significant cardiac enlargement
o Medical management in poor surgical candidates,
particular severe left ventricular compromise
o Medical management: Vasodilators and sometimes
inotropic agents
o Surgery reserved for symptomatic patients with
intact left ventricular function
Aortic stenosis
o Medical management: Endocarditis prophylaxis and
sometimes inotropic agents
o Aortic valve replacement indicated in severe AS with
symptoms, left ventricular dysfunction or if
undergoing bypass grafting or other value
replacement
o Alternatives: Ross procedure (pulmonary valve
moved to aortic position and pulmonary homograft
placed) and aortic balloon valvuloplasty
I SELECTED REFERENCES
1. Enriquez-Sarano M et al: Clinical practice. Aortic
regurgitation. N Engl J Med. 351 (IS): 1539-46,2004
 AORTIC VALVE DYSFUNCTION
I IMAGE GALLERY
Typical
(Left) Frontal radiograph
shows slight prominence of
the left ventricular apex in
this patient with aortic
stenosis and prior bypass
grafting. (Right) Lateral
radiograph shows
calcification in the aortic
valve (arrow) consistent with
aortic stenosis. Valve
calcification may be subtle.
Examine the base of the
3
aortic rool.
31

Typical
(Left) Sagillal oblique MR
cine shows loss of signal in
the aortic root (arrow)
during systole due to
turbulence caused by aortic
stenosis. (Right) Frontal
radiograph shows an
enlarged ascending aorta
(arrows) and dilated left
ventricle in a patient with
aortic regurgitation.

(Left) DSA shows contrast in

the left ventricle (arrow)
after aortic root injection
consistent with aortic
regurgitation. (Right)
Coronal MR cine shows loss
of signal in the left ventricle
(arrow) during diastole due
to turbulence caused by
aortic regurgitation.
 MITRAL VALVE DYSFUNCTION

32

Graphic shows location of the mitral valve in frontal Frontal radiograph shows left atrial enlargement with a
projection (arrow). Mitral dysfunction often leads to convex left atrial appendage (open arrow), double
enlargement of the left atrium (open arrows). density of the right heart (arrow), and uplifted left
bronchus (curved arrow).

ITERMINOLOGY I IMAGING FINDINGS

Abbreviations and Synonyms General Features
Mitral regurgitation (MR) Best diagnostic clue: Enlargement of left atrium
Mitral insufficiency Location: Left atrium
Mitral stenosis (MS)
Radiographic Findings
Definitions Radiography
Mitral regurgitation is categorized as acute or chronic o Frontal view
Acute MR often occurs in setting of ischemia and Convexity or straightening of the left atrial
causes acute pulmonary edema appendage along the left heart border below the
Chronic MR has multiple causes and leads to main pulmonary artery due to left atrial
progressive left heart failure enlargement
Mitral valve prolapse an important cause of MR Double density projecting over the right heart,
Mitral stenosis is typically due to rheumatic fever and reflecting superimposition of enlarged left atrium
leads to elevated left atrial and pulmonary venous over the right heart
pressures, and pulmonary congestion Elevation of the left main bronchus and splaying
Mitral stenosis may ultimately cause pulmonary of the carina by enlarged left atrium
hypertension Cephalization of flow due to pulmonary venous
hypertension
In acute MR, pulmonary edema sometimes
localized in right upper lobe; findings of left atrial
enlargement are often absent

DDx: Mitral Mimics

Aortic Regurgitation Cardiomyopathy Annular Calcification

 MITRAL VALVE DYSFUNCTION
Key Facts
Terminology
Mitral regurgitation is categorized as acute or chronic
Acute MR often occurs in setting of ischemia and
causes acute pulmonary edema
Chronic MR has multiple causes and leads to
progressive left heart failure
Mitral valve prolapse an important cause of MR
Mitral stenosis is typically due to rheumatic fever and
leads to elevated left atrial and pulmonary venous
pressures, and pulmonary congestion
Mitral stenosis may ultimately cause pulmonary
hypertension
Imaging Findings
Best diagnostic clue: Enlargement of left atrium
In chronic MR, left ventricular and left atrial
enlargement may be present
In MS, enlargement of central pulmonary arteries
from pulmonary hypertension
In MS, curvilinear calcification outlining left
atrium or the appendage due to stasis in atrial
fibrillation
In MS, pulmonary hemosiderosis or ossification
due to longstanding elevated venous pressures
a Lateral view
Posterior convexity of left atrial silhouette
Posterior displacement of left main bronchus
CT Findings
CECT
a Enlargement of left atrium
a Thickening and sometimes calcification of leaflets or
mitral valve apparatus on gated multidetector CT
a Thrombus, particularly in the left atrial appendage
Thrombus may calcify
a Pulmonary edema manifested as ground-glass
opacity, septal thickening, fissural thickening
a In chronic MR, left ventricular enlargement
Angiographic Findings
Conventional
a MR can be quantified on a 0 (none) - 4 (severe) scale
a Regurgitant volume calculated
a In MS, valve orifice can be calculated
MR Findings
TlWI
a Enlargement of left atrium
a In chronic MR, left ventricular enlargement
T2* GRE
a Enlargement of left atrium
a In chronic MR, left ventricular enlargement
a In MR, regurgitant jet (area of signal drop-out)
projects from mitral valve into left atrium during
systole
a In MS, stenotic jet projects from mitral valve into
left ventricle during diastole
Convexity or straightening of the left atrial
appendage along the left heart border below the main
pulmonary artery due to left atrial enlargement
Double density projecting over the right heart,
reflecting superimposition of enlarged left atrium
over the right heart
Elevation of the left main bronchus and splaying of
the carina by enlarged left atrium
Cephalization of flow due to pulmonary venous
hypertension
Top Differential Diagnoses
Aortic Valve Disease
Ischemic/Dilated Cardiomyopathy
3
Mitral Annular Calcification (Radiograph) 33
Ventricular Septal Defect (MR)
Left Atrial Myxoma (MS)
Echocardiographic Findings
Echocardiogram
a Left atrial and ventricular size readily evaluated
a In acute MR, ruptured papillary muscle visualized
a In MR, color Doppler shows jet extending from
mitral valve into left atrium during systole
a In MR, left ventricular size can be assessed as a
measure of disease progression
a In MS, color Doppler shows jet extending from
mitral valve into left ventricle during diastole
a Valve orifice diameter, gradient and estimated
pulmonary pressures can be calculated
a Normal orifice 4-6 sq em, critical MS is < 1 sq em
a Left atrial enlargement if AP diameter> 4.5 em
a Mixed MR/MS lesion can be assessed
a Mitral valve prolapse well demonstrated
Imaging Recommendations
Best imaging tool: Echocardiography primary
technique
Protocol advice: Gated multidetector CT or MRI are
useful in patients who undergo left atrial ablation for
atrial fibrillation
I DIFFERENTIAL DIAGNOSIS
Aortic Valve Disease
Valve calcification frequent, on lateral radiograph
more anteriorly located than mitral valve
Often enlarges ascending aorta with either valvular
stenosis or regurgitation
Ischemic/Dilated Cardiomyopathy
Generalized cardiac enlargement, left atrium may be
enlarged but is not out of proportion to ventricular
dilatation
Often signs of congestive heart failure due to left
ventricular dysfunction
Mitral Annular Calcification (Radiograph)
More common than valve, incidental finding in
elderly women
 MITRAL VALVE DYSFUNCTION
"C-shaped", larger than valve o In chronic MR, patients develop shortness of breath,
Left atrium normal orthopnea, and paroxysmal nocturnal dyspnea
o In MR, a holosystolic murmur may be auscultated
Ventricular Septal Defect (MR) o In MS, a diastolic murmur, accentuated first heart
Left atrium enlarged sound, and opening snap are audible
Right ventricle enlarged and main pulmonary artery Other signs/symptoms
may be enlarged due to shunt vascularity o Palpitations due to atrial fibrillation in either MR or
Small ascending aorta MS
left Atrial Myxoma (MS) o Atypical chest pain due to mitral valve prolapse
o Cough
50% have left atrial enlargement due to mass
o Hemoptysis late in MS
obstructing the mitral valve
May be calcified Demographics
Easily distinguished with echocardiography or
Age
3 cross-sectional imaging o In MR, patients with rheumatic fever tend to be
younger
34 o Congenital mitral stenosis presents in infancy
I PATHOLOGY o In MS, age of symptom onset is 20-50 years
Gender
General Features
o MR more common in women
Etiology o Mitral valve prolapse is present in about 6% of
o Mitral regurgitation women
In acute MR, papillary muscle dysfunction or oMS more common in women (2:1)
rupture
In chronic MR, rheumatic fever in young patients Natural History & Prognosis
Ischemic heart disease In acute ischemic MR, onset of pulmonary edema is
Mitral valve prolapse caused by myxomatous fulminant and the prognosis is guarded
degeneration [n chronic MR, volume overload may be
Endocarditis asymptomatic for years
Connective tissues disorders such as Marfan Ultimately, atrial fibrillation and finally heart failure
disease may occur
o Mitral stenosis 5 year survival for chronic MR is 80%
Rheumatic fever (accounts for majority) MS asymptomatic for up to 20 years; dyspnea develops
Congenital due to elevated venous pressure
Collagen vascular disease such as rheumatoid Atrial fibrillation (30-40%), thromboembolism (20%),
arthritis and systemic lupus erythematosus and pulmonary hypertension are complications
Endocarditis
Substantial mitral annular calcification Treatment
Epidemiology [n acute MR, aggressive treatment for pulmonary
o For MR, rheumatic fever most common in the edema
developing world and mitral valve prolapse accounts In chronic MR, medical treatment with diuretics and
for majority in developed countries after load-reducing agents during compensated phase
o MS occurs early in the developing world among Antibiotic prophylaxis in MR associated with mitral
patients with rheumatic fever valve prolapse
Associated abnormalities Surgery preferred with either mitral valve repair or
o In MS due to rheumatic fever, aortic and tricuspid replacement
valve involvement may also occur In MS, mitral valve replacement (balloon valvuloplasty
o MR and M5 occur together in 40% in young patients)

Gross Pathologic & Surgical Features

In MS, leaflets may be thickened with fused I DIAGNOSTIC CHECKLIST
commissures
Image Interpretation Pearls
Microscopic Features Mitral valve calcification uncommon on chest
In MR, myxomatous degeneration may be evident in radiography
mitral valve prolapse Mitral annular calcification common and manifests as
"J-shaped" density over the left heart

ICLINICAL ISSUES
Presentation I SELECTED REFERENCES
Most common signs/symptoms 1. Hayek E et al: Mitral valve prolapse. Lancet.
o In acute MR, sudden-onset pulmonary edema 365(9458):507-18,2005
2. Valocik G et al: Three-dimensional echocardiography in
mitral valve disease. EurJ Echocardiogr. 6(6):443-54, 200S
 MITRAL VALVE DYSFUNCTION
I IMAGE GALLERY
Typical
(Left) Frontal radiograph
shows lelt atrial enlargement
with a convex left atrial
appendage (open arrow),
double density 01 the right
heart (arrow), in mitral
stenosis. (Right) Lateral
radiograph shows lelt atrial
enlargement with posterior
convexity of the left atrium
and pulmonary
confluence
vein
(arrow), in mitral
3
stenosis.
35

(Left) Frontal radiograph

shows lelt atrial enlargement
with convex lelt atrial
appendage (open arrow),
double density 01 the right
heart (arrow), in mitral
stenosis/ regurgita lion.
(Right) Axial CECT shows
thickening and calcification
(arrow) of the mitral valve
chordae tendineae in mitral
stenosis.

Typical
(Left) Axial CECTshows
thrombus in the lelt atrial
appendage (arrow) in mitral
stenosis. (Right) Sagillal
oblique MR cine shows
signal void in the lelt
ventricle (arrow) due to
turbulence across the mitral
valve caused by mitral
stenosis.
 CONSTRICTIVE PERICARDITIS

3
36

Axial NEeT shows diffuse pericardial thickening in this Axial HASTE MR imaging again shows the thickened
patient with a history of constrictive pericarditis pericardium. The pericardium will appear as a
farrows). No calcification is seen. Normal pericardial hypointense structure on T1 WI and T2WI as seen here
thickness is 1-] mm. (arrows).

Pericardial calcifications highly suggestive

ITERMINOLOGY Associated signs of hepatic venous congestion,
Definitions enlargement of atria, dilated superior/inferior
Pericardial thickening with physiologic change vena cava and hepatic veins
o Causes include postsurgical, postradiation, Ascites, pleural effusions and pericardial effusion
postinfectious, posttraumatic, postmyocardial Location: Thickening may be isolated over right
infarction and idiopathic atrium, right ventricle or right atrioventricular groove
o Thickening does not necessarily indicate constrictive Morphology
disease o Reduced volume and narrow tubular configuration
o Calcification suggests likelihood of constrictive of right ventricle
physiology May see prominent leftward convexity or
o At times isolated to the right side of the heart sigmoid-shaped septum
Associated findings: Tubular ventricular configuration Radiographic Findings
and congestive heart failure Calcification
o Eggshell calcification predominantly inferior and
right-sided
I IMAGING FINDINGS o With constrictive pericarditis
General Features Widened superior mediastinum
Lack of pulmonary edema
Best diagnostic cl ue
Elevated diaphragms due to ascites
o Pericardial thickening in combination with heart
failure CT Findings
Thickness greater than 4-6 mm indicates NECT
pericardial thickening (normall-3 mm) o Constrictive pericarditis findings

DDx: Constrictive Pericarditis

Surgical Device Post-Op Finding Pericardia! Mass

 CONSTRICTIVE PERICARDITIS

Key Facts
Terminology
Causes include postsurgical, postradiation,
postinfectious, posttraumatic, postmyocardial
infarction and idiopathic
Thickening does not necessarily indicate constrictive
disease
Calcification suggests likelihood of constrictive
physiology
At times isolated to the right side of the heart
Associated findings: Tubular ventricular configuration
and congestive heart failure
May see prominent leftward convexity or
sigmoid-shaped septum
Small effusion difficult to distinguish from
thickening
Pericardial enhancement may indicate active
inflammatory process
MRI more sensitive in distinguishing pericardial
effusion from thickening
High sensitivity (approximately 95%) for
distinguishing constrictive pericarditis from
restrictive cardiomyopathy

Imaging Findings Top Differential

Diagnoses
Myocardial Calcification
3
Thickness greater than 4-6 mm indicates pericardial
thickening (normal 1-3 mm) Pericarditis without Constriction 37
Reduced volume and narrow tubular configuration of Restrictive Cardiomyopathy
right ventricle Neoplasm

Calcification highly suggestive Seen only as signal void

Pericardia I effusion/thickening (> 4-6 mm) High sensitivity (approximately 95%) for
May be limited to the right side of the heart distinguishing constrictive pericarditis from restrictive
o Small effusion difficult to distinguish from cardiomyopathy
thickening
Evaluate for attenuation characteristics of Echocardiographic Findings
pericardial effusion Primary diagnostic tool
Exudative effusion as seen with infection Effusion, when seen, well-delineated from thickening
hemorrhage, neoplasm has increased att~nuation Suboptimal in demonstration of pericardial thickening
Transudative effusion has water attenuation (> 10 o Transesophageal echo (TEE) allows better
Hounsfield units) visualization of pericardium
o Secondary signs of heart failure and tubular TEE limited by small field of view
configuration of ventricles Semi-invasive
CECT Imaging Recommendations
o Pericardial enhancement may indicate active
Best imaging tool
inflammatory process
o Echocardiography primary tool to investigate
Enhancement aids in distinguishing effusion from
pericardium
thickening
o CT and MRI useful to examine entire pericardium
Radiographic findings above not diagnostic of
o CT and MRI useful to distinguish myocardial from
constrictive pericarditis without clinical scenario of
pericardia I disease
physiologic constriction
Constrictive pericarditis vs. restrictive
MR Findings cardiomyopathy
TlWI o CT and MRI useful to further characterize pericardia I
o Low signal intensity band> 4-6 mm encompassed masses
by high signal intensity epicardial and pericardial fat
o Simple pericardial effusion low signal intensity
o Hemorrhagic effusion high signal intensity I DIFFERENTIAL DIAGNOSIS
T2Wl Myocardial Calcification
o Pericardium remains low signal intensity
o Occasionally in subacute forms, thickened Pericardial
pericardium may have moderate to high signal o Usually right-sided (less cardiac motion)
intensity on spin echo images o Diffuse and extensive
o Effusion very high signal intensity unless o Spare left atrium and apex
complicated by hemorrhage or proteinaceous o Atrioventricular (AV) groove
material o Lateral view: Over pulmonary outflow tract
Complicated effusion heterogeneous appearance Myocardial
with areas of lower signal intensity o Usually left-sided
Tl C+: Pericardial enhancement may be seen with o Focal
acute inflammatory process o Apex typical location
MRI more sensitive in distinguishing pericardial o Spares AV groove
effusion from thickening o Lateral view: Projects under pulmonary valve
o Calcification not seen
 CONSTRICTIVE PERICARDITIS
Pericarditis without Constriction Gross Pathologic & Surgical Features
Distinction is made based on physiologic changes > 50% with pericardial calcification will have
constrictive pericarditis
Restrictive Cardiomyopathy Thickening or calcification not diagnostic of
Similar physiologic changes by echocardiography and constrictive pericarditis unless patient also has
cardiac catheterization symptoms of physiologic constriction or restriction
Myocardial morphologic assessment helpful in
distinguishing the two
Look for associated myocardial thickening as seen I CLINICAL ISSUES
with sarcoidosis, amyloidosis, hypertrophic
obstructive cardiomyopathy (HOCM), lymphoma Presentation
MRI has reported accuracy of 93% for differentiation Most common signs/symptoms
between constrictive pericarditis and restrictive o Both constrictive pericarditis and restrictive
3 cardiomyopathy cardiomyopathy show
Restricted ventricular filling
Neoplasm Increased diastolic pressure in all chambers
38
Could cause restrictive physiology Equalization of atrial and ventricular pressures
Metastases usually cause effusion, not masses Other signs/symptoms
o Focal or multifocal areas of enhancing pericardium o Symptoms of heart failure
consider metastatic disease, especially in face of Dyspnea, shortness of breath, orthopnea
known primary Fatigability
o Lung and breast cancer most common, others Occasionally may present with hepatomegaly and
include lymphoma, thymoma, mesothelioma ascites
Primary tumors rare
o Sarcomas, large bulky tumors Treatment
Surgical stripping of pericardium, difficult to remove
entire pericardium
I PATHOLOGY May recur
General Features
General path comments I SELECTED REFERENCES
o Normal pericardial anatomy
Usually 2 mm thickness 1. Gilkeson RCet al: MR evaluation of cardiac and pericardial
malignancy. Magn Reson Imaging Clin N Am.
Lies between variable amounts of epicardial and 11(1):173-86, viii, 2003
pericardial fat 2. Glockner ]F: Imaging of pericardial disease. Magn Reson
Has fibrous and serous components Imaging Clin N Am. 11(1):149-62, vii, 2003
Fibrous elements attached to diaphragm, costal 3. Wang Z] et al: CT and MR imaging of pericardial disease.
cartilage and sternum Radiographies. 23 Spec No:SI67-80, 2003
Serous element thin mesothelial layer adjacent to 4. Breen]F: Imaging of the pericardium.] Thorac Imaging.
the heart 16(1):47-S4,2001
Intervening potential space usually contains IS-SO 5. Rozenshtein A et al: Plain-film diagnosis of pericardial
disease Semin Roentgenol. 34:195-204,1999
mm fluid 6. Watanabe A et al: A case of effusive-constructive
Etiology pericarditis: an efficacy of GO-OTPAenhanced magnetic
o Constrictive pericarditis caused by injury, iatrogenic, resonance imaging to detect a pericardialthickening. Magn
idiopathic processes Reson Imaging. 16(3):347-50, 1998
Most common etiologies today 7. White CS: MR evaluation of the pericardium and cardiac
Postsurgical malignancies. Magn Reson Imaging C1in N Am.
Radiation injury 4(2):237-51,1996
Dressler syndrome, status postmyocardial 8. White CS: MR evaluation of the pericardium. Top Magn
Reson Imaging. 7(4):258-66, 1995
infarction 9. Masui T et al: Constrictive pericarditis and restrictive
o Infectious cardiomyopathy: evaluation with MR imaging. Radiology.
Viral: Coxsackie B, influenza 182(2):369-73, 1992
Bacterial 10. Hoit 80: Imaging the pericardium. Cardio[ Clin.
Tuberculous 8(4):587-600, 1990
Fungal or parasitic; hydatid disease 11. Miller SW: Imaging pericardia[ disease. Radiol Clin North
o Metabolic Am. 27(6):1113-25, 1989
[2. Olson MC et al: Computed tomography and magnetic
Uremia resonance imaging of the pericardium. Radiographies.
o Inflammatory 9(4):633-49, 1989
Systemic lupus erythematosus 13. Sechtem U et al: MRIof the abnormal pericardium. A]RAm
Rheumatoid arthritis ] Roentgenol. 147(2):245-52, 1986
 CONSTRICTIVE PERICARDITIS

I IMAGE GAllERY

(Left) Axial TruFISP MR

imaging in the same patient
as shown in previous images
on first page shows the
pericardial thickening
(arrows) with elongation and
tubular configuration of the
right ventricle. (Right) Four
chamber TruF/SP MR
imaging shows elongation of
the ventricles. There is
bowing of the 3
intravenlricular septum
(arrows), with an 39
5-configuration to the
septum noted.

Typical
(Left) Short axis TruF/SP MR
imaging in the same patient
again shows flattening of the
intraventricular septum
farrows). (Right) Axial CECT
in a separate case shows
subtle bowing of the
intraventricular septum
towards the left ventricle
(arrow) with right atrial
enlargement. No pericardia I
thickening is seen.

(Left) Four chamber TruFISP

MR imaging shows mild
bowing of the
intraventricular septum
towards the left ventricle
(arrow) with right atrial
dilatation. No pericardial
thickening is seen. (Right)
Short axis TruFISP MR
imaging shows flattening of
the intraventricular septum,
which bows towards the left
ventricle in the same patient
(arrows).
 POSTCARDIAC INJURY SYNDROME

3
40

Anteroposterior radiograph after cardiac surgery shows Frontal radiograph of the same patient obtained two
a minimal enlargement of the heart silhouette. Midline weeks later shows a significant enlargement of the heart
vertically aligned sternotomy wires are seen. Normal silhouette. Bilateral pleural effusion is a/50 clearly visible.
fXJstoperalive appearance. Dressler syndrome.

ITERMINOLOGY I IMAGING FINDINGS

Abbreviations and Synonyms General Features
Postcardiac injury syndrome (PCIS): Best diagnostic clue: Cardiac enlargement (due to
Postpericardiotomy syndrome (PI'S) and pericardial effuSion) and often small to moderate sized
post-myocardial infarction syndrome (Dressler pleural effusion following cardiac injury
syndrome)
Radiographic Findings
Definitions Radiography
PCIS: Combinations of pericarditis, pleuritis, and o Chest radiograph usually abnormal (95%): Small
pneumonitis that develop after variety of injuries to pleural effusion and mild enlargement cardiac
the myocardium or pericardium silhouette
o Post-myocardial infarction syndrome (Dressler Conversely may be normal
syndrome): Autoimmune condition where the Pericardial and pleural effusion (30%)
pericardium becomes inflamed following a Pleural effusion alone (30%)
myocardial infarction or heart surgery Pericardial effusion alone (10%)
o Postpericardiotomy syndrome (PI'S): Autoimmune Consolidation, pleural and pericardial effusions
febrile illness associated with pericardial and (10%)
sometimes pleuropulmonary reaction that often o Pleural effusion (80%)
follows extensive pericardiotomy Unilateral (usually left-sided) or bilateral with
nearly equal frequency
Small to moderate in size
o Consolidation (50%)

DDx: Enlarged Cardiac Silhouette

Pleuropericarditis Pericardia I Cyst Thymolipoma

 POSTCARDIAC INJURY SYNDROME
Key Facts
Terminology
PClS: Combinations of pericarditis, pleuritis, and
pneumonitis that develop after variety of injuries to
the myocardium or pericardium
Imaging Findings
Best diagnostic clue: Cardiac enlargement (due to
pericardial effusion) and often small to moderate
sized pleural effusion following cardiac injury
Chest radiograph usually abnormal (95%): Small
pleural effusion and mild enlargement cardiac
silhouette
Widened pericardia I stripe> 2 mm in thickness; most
reliable sign of pericardial effusion
Approximately 120 ml of additional fluid can
accumulate in pericardium without an increase in
pressure
Usually unilateral (left-sided) patchy, basilar, and
mild in severity
Always occurs in association with pericardial or
pleural effusion, never seen in isolation
Pericardium
o Normal pericardium rarely identified as a thin line
along the left ventricular border on the frontal
radiograph
o Normal pericardium more frequently identified on
the lateral radiograph
Pericardial stripe: Curvilinear opacity 1-2 mm in
thickness just posterior to the lower sternal
segments
Normally seen in 70% normal chest radiographs
Pericardium sandwiched between mediastinal fat
and epicardial fat
Parallels the heart border
Occasionally wraps around the cardiac apex
o Pericardial effusion
Enlargement of cardiac silhouette not seen until MR Findings
accumulation of 250 ml of fluid
Cardiac shape: Triangular, globular, or
flask-shaped
Normal cardiac borders are effaced
Widened pericardial stripe> 2 mm in thickness;
most reliable sign of pericardial effusion
Widened subcarinal angle: Normal 40-70 degrees
Differential density sign: Increase in lucency of
the margin of the cardiac silhouette from
difference in tissue attenuation between
myocardium and fluid
o Pericardial tamponade
Approximately 120 ml of additional fluid can Imaging Recommendations
accumulate in pericardium without an increase in
pressure
In acute tamponade, heart size often normal
Pulmonary edema rare unless also have left
ventricular failure
Widened superior mediastinum due to dilatation
of superior vena cava
Rarely diagnosed or suggested on chest
radiographs
Top Differential Diagnoses
Other Causes of Pericardial Effusion
Cardiac Chamber Enlargement
Thymolipoma
Pericardial (Mesothelial) Cyst
Pathology
Autoimmune hypersensitivity reaction
Explains the latency of syndrome, response to
corticosteroid treatment and its tendency to relapse
Normal pericardium contains 25-50 ml of fluid
Clinical Issues 3
Usually anti-inflammatory agents, such as aspirin and
indomethacin 41
CT Findings
Evaluates entire pericardial space
Normal pericardial space < 2 mm thick anteriorly, up
to 4 mm thick near the diaphragm (where pericardial
fluid pools in supine position)
Fluid normally accumulates inferiorly and posteriorly
in dependent position posterior to left atrium and
ventricle
o Larger effusion accumulates anteriorly and encircles
the heart
Pericardial tamponade findings
o Pericardia I effusion
In acute tamponade effusion may not be large
o Atrial dilatation
o Superior and inferior vena cava or hepatic vein
dilatation
o Elongation ventricles
o Ascites
More sensitive than CT for detection fluid
Echocardiographic Findings
Unable to evaluate entire pericardium
Small effusions located posterior and inferior to the
left ventricle
Moderate effusions extend toward the heart apex
o Echo-free pericardial space (anterior plus posterior)
of 10-20 mm during diastole
Large effusion completely surround the heart
o Echo-free pericardial space more than 20 mm
Best imaging tool: Echocardiography primary modality
of choice to evaluate pericardium
I DIFFERENTIAL DIAGNOSIS
Other Causes of Pericardial Effusion
Hydrostatic
o Pulmonary arterial hypertension, congestive heart
failure, uremia, hypoalbuminemia
 POSTCARDIAC
Infections
o Viral, bacterial and fungal and mycobacterium
tuberculosis
Immunologic
o Systemic lupus erythematosus, rheumatoid arthritis,
periarteritis nodosa, rheumatic fever
Drugs
o Procainamide, hydralazine, Coumadin
Metastases
o Lung, breast, lymphoma
Trauma
o Blunt or penetrating chest trauma, radiation therapy
Idiopathic
o Hypothyroidism, usually massive pericardial
3 effusion
42 Cardiac Chamber Enlargement
Normal epicardial stripe
Normal pericardia I space on cross-sectional imaging
Thymolipoma
Enlarged soft tumor conforms to cardiac shape
Mixed attenuation (contains fat) anterior mediastinal
mass surrounding heart at cross-sectional imaging
Pericardial (Mesothelial) Cyst
Usually are smooth and round or oval
Located in the cardiophrenic angles
May simulate cardiac enlargement
I PATHOLOGY
General Features
Etiology
o Autoimmune hypersensitivity reaction
Myocardial or pericardial injury stimulates the
formation of antigens
Antigen-antibody complexes deposit in
pericardium, pleural and lung
Explains the latency of syndrome, response to
corticosteroid treatment and its tendency to
relapse
Epidemiology
o Any injury to the heart or pericardium
Post-myocardial infarction syndrome (Dressler
syndrome)
After coronary artery bypass graft (CABG) surgery
Complication of temporary and permanent pacing
Blunt chest trauma
o PPS: Incidence of 10-40%
o Dressler syndrome: Incidence of 5% has decreased
Due to reperfusion, multiple thrombolytic agents,
aggressive (I-adrenergic blockade,
angiotensin-converting enzyme inhibition, and
prompt coronary bypass
Onset 2-10 weeks after infarction (average 20
days)
Gross Pathologic & Surgical Features
Pericardium: Double-layered fibroserosal envelope that
surrounds the heart, great vessels, and veins
o Extends from the aorta and superior vena cava to
the central tendon of the diaphragm
INJURY SYNDROME
o Visceral pericardiallayer adherent to heart, parietal
fibrous pericardiallayer free
Normal pericardium contains 25-50 ml of fluid
o Slow accumulations may exceed 3 L without
tamponade
Normal pericardium limits the position and motion of
the heart, limits chamber dilatation, and provides a
barrier to spread of disease from the lungs
I CLINICAL ISSUES
Presentation
Most common signs/symptoms
o Clinical differential: Pulmonary embolism,
congestive heart failure, pneumonia, myocardial
infarction
o Course usually benign and self-limited: Malaise
(90%), fever (40%), pleuritic chest pain (90%),
pericardial friction rub
o Exudative pleural or pericardial fluid
(serosanguineous or bloody)
Other signs/symptoms: Elevated erythrocyte
sedimentation rate or leukocytosis
Complications: Tamponade
o Beck triad: Jugular venous distension, hypotension,
muffled heart sounds
o < 1% of patients with PPS
Demographics
Age: Parallels prevalence of coronary artery disease in
middle-aged and older adults
Natural History & Prognosis
Develops 2-3 weeks after cardiac surgery, uncommonly
it may develop up to 6 months later
Mild, self-limited inflammatory illness, however,
untreated may last for weeks or months
o May recur after stopping anti-inflammatory drugs
May lead to cardiac tamponade or constrictive
pericarditis in 1-2%
Treatment
Usually anti-inflammatory agents, such as aspirin and
indomethacin
Corticosteroids may be necessary in more severe cases
I DIAGNOSTIC CHECKLIST
Consider
PCIS in any patient who develops a pleural effusion
after injury to the heart
I SELECTED REFERENCES
1. Attili A et al: Postoperative cardiopulmonary thoracic
imaging. Radiol Clin N Am. 42:543- 564, 2004
2. Bendjelid Ket ai: Is Dressler syndrome dead? Chest.
126:1680-1682,2004
3. Breen JP: Imaging of the pericardium. J Thorac Imaging.
16:47-54,200]
4. Prince SEet al: Postpericardiotomy syndrome. Heart Lung.
26:]65-]68, ]997
 POSTCARDIAC INJURY SYNDROME

I IMAGE GALLERY
Typical
(Left) Anteroposterior
radiograph after cardiac
surgery. Normal appearance.
Midline vertically aligned
sternotomy wires are seen.
(Right) Anteroposterior
radiograph (portable) of the
same patient 10 days after
cardiac surgery. Cardiac
enlargement and bilateral
pleural effusion are seen
(loculated in the left)
3
(arrows). PC/5.
43

Typical
(Left) Frontal radiograph
demonstrates massive
cardiac enlargement and
mediastinal widening
(arrows). (Right) Axial CECT
of the same patient shows a
large pericardia I effusion in
the superior aortic recess
(arrows). PC/5.

(Left) Anteroposterior
radiograph shows pericardia I
effusion after cardiac surgery
in a 42 year old man 72
weeks after aortic valve
replacement (arrow). Acute
pulmonary edema (open
arrows). (Right) Axial NECT
(24 hours later) shows
bilateral pulmonary edema
characterized by eeo in
both upper lobes with a
predominant central
distribution. Also note small
bilateral pleural effusions.
 MYXEDEMA

3
44

Frontal radiograph shows massive cardiomegaly with a Axial NECT shows massive pericardia' effusion
water bottle shape probably from a pericardial effusion. extending to the left posterior chest wall from long
MediasUnum widened from substernal goiter (arrows). standing hypothyroidism. Fluid gradually resolved with
thyroid replacement therapy.

o Pleural effusions
I TERMINOLOGY Small to moderate in size, less than '4 of a
Abbreviations and Synonyms hemithorax
Hypothyroidism Unilateral or bilateral
No relationship between size and degree of
Definitions hypothyroidism
Chronic endocrine disease that slows multiple organ Resolves with replacement therapy
function due to lack of thyroid hormone o Pericardial effusions
Normal in 2S%
Marked cardiomegaly with "water bottle"
I IMAGING FINDINGS configuration
Double lucency sign on lateral chest radiograph
General Features (normally < 4 mm in width)
Best diagnostic clue: Massive cardiomegaly (pericardial Effusion may be massive, up to 4 liters
effusion) and thoracic inlet mass (goiter) Chronic and unchanging for years
Size: Pericardial effusions may be massive (over 4 Tamponade unusual
liters) Slowly resolves with replacement therapy
Radiographic Findings CT Findings
Radiography NECT: Pericardial effusion easily demonstrated
o Substernal goiter
Trachea deviated Nuclear Medicine Findings
Anterior superior mediastinal mass, may migrate Thyroid scanning
into posterior mediastinum o Useful to assess the thyroid anatomy and function

DDx: Hypothyroidism

Left Heart Failure Thymolipoma Lymphoma

 MYXEDEMA
Key Facts
Terminology Top Differential Diagnoses
Chronic endocrine disease that slows multiple organ Cardiomegaly
function due to lack of thyroid hormone Thymolipoma
Lymphoma
Imaging Findings
Best diagnostic clue: Massive cardiomegaly Clinical Issues
(pericardial effusion) and thoracic inlet mass (goiter) Effusions may be chronic and stable for years but will
Substernal goiter slowly resolve with thyroid hormone replacement
Tamponade unusual

Ultrasonographic Findings I CLINICAL ISSUES 3

Grayscale Ultrasound: Useful to detect nodules and
infiltrative disease in the thyroid
Presentation 45
Most common signs/symptoms: Weakness and loss of
energy (80%), decreased joint reflexes
I DIFFERENTIAL DIAGNOSIS Other signs/symptoms: Respiratory drive may be
depressed leading to alveolar hypoventilation and
Cardiomegaly sleep apnea
Massive cardiomegaly does not have water-bottle Demographics
shape
Age: Incidence increases with age
Thymolipoma Gender: Females exceed males (4: I)
Fatty mass at CT or MR Natural History & Prognosis
Lymphoma Effusions may be chronic and stable for years but will
Discrete nodes in all anatomic compartments of the slowly resolve with thyroid hormone replacement
mediastinum Treatment
Thyroid hormone replacement
I PATHOLOGY
General Features I DIAGNOSTIC CHECKLIST
Etiology: Effusions probably related to abnormal fluid Consider
and protein transport across the capillary wall
Always consider hypothyroidism in those with large or
Epidemiology: Incidence of thyroid abnormalities 5%,
massive pericardial effusion
Goiter estimated to affect 250 million worldwide
Gross Pathologic & Surgical Features
Pleural effusion analysis borderline between I SELECTED REFERENCES
transudates and exudates I. Smolar EN et al: Cardiac tamponade in primary myxedema
and review of the literature. Am J Med Sci. 272(3):345-52,
1976

I IMAGE GALLERY

(Left) Frontal radiograph shows marked cardiac enlargement. Left lower lobe ;5 partially atelectatic. Patient had unknown long-standing
hypothyroidism. (Center) Axial CECT shows a large pericardia I effusion in the same patient. Hearl actually normal in size. Left lower lobe
partially atelectatic (arrow). (Right) Lateral radiograph shows small pericardia! effusion widening the pericardial stripes. Inner stripe represents
epicardial fat (arrow), outer stripe pericardia I-mediastinal fat (curved arrow).
 LEFT ATRIAL MYXOMA

3
46

Atrial myxoma. Note classic septal attachment of atrial Sagittal oblique T1WI MR shows a sessile mass in the
myxoma (arrow). Atrial myxomas often cause anterior wall of the left atrium (arrow), the aortic root
secondary mitral valve obstruction. (curved arrow) and left pulmonary artery (open arrow).

Inferior vena cava

I TERMINOlOGY Left ventricle
Abbreviations and Synonyms Right ventricle
Myxoma Valve leaflets
Size: 1-15 cm diameter
Definitions Morphology
Most common benign tumor of the heart o Usually single, may be multiple in familial forms
o About 2/3 are smooth surfaced
o About 1/3 are villous
IIMAGING FINDINGS Villous tumors are more likely to develop embolic
complications
General Features
Best diagnostic clue Radiographic Findings
o Generally requires cross-sectional imaging to Radiography
visualize o Generally no abnormality of contour of heart on
o Chest radiograph generally normal plain films
Unless tumor obstructs valve (mitral or tricuspid), May be calcified, especially if origin right atrium
then findings consistent with valvular stenosis o If tumor obstructs mitral valve, findings may mimic
Location mitral stenosis
o Approximately 85% are located in the left atrium Enlarged left atrium
attached to atrial septum, usually at the fossa ovalis Pulmonary vascular congestion
o Most of the rest are in the right atrium May prolapse across mitral or tricuspid valve
o Occasionally biatrial CT Findings
o Rare sites
NECT

DDx: Cardiac Masses

Cardiac Angiosarcoma Cardiac Metastasis Cardiac Lymphoma

 LEFT ATRIAL MYXOMA

Key Facts
Terminology Top Differential Diagnoses
Most common benign tumor of the heart Intracardiac Thrombosis
Cardiac Metastasis
Imaging Findings Cardiac Lipoma
Approximately 85% are located in the left atrium Primary Cardiac Malignancies
attached to atrial septum, usually at the fossa ovalis Cardiac Lymphoma
Size: 1-15 em diameter
Villous tumors are more likely to develop embolic Clinical Issues
complications Symptoms of valvular obstruction (40%)
If tumor obstructs mitral valve, findings may mimic Constitutional (30%): Fatigue, weight loss, fever
mitral stenosis Peripheral embolization (50% to brain causing stroke
Calcification is seen in about half of tumors in the or mycotic aneurysms)
right atrium Gender: Approximately 60% in females
Cardiac ultrasound can be useful to assess mobility of May become infected, producing fever, weight loss
tumor and septic emboli
Best imaging tool: Echocardiography May recur after removal in 5%

a Tumor may not be visible without intravenous Cardiac ultrasound can be useful to assess mobility of
contrast tumor
a Calcification is seen in about half of tumors in the a Can assess hemodynamic degree of obstruction,
right atrium myxoma often prolapse through mitral valve
a Calcification is rare in left atrial tumors a Stalk often well visualized
CECT
a Filling defect in cardiac chamber Imaging Recommendations
Ovoid lesion with lobular (75%) or smooth (25;(,) Best imaging tool: Echocardiography
contour
About 80% are low attenuation on CT
Generally no contrast-enhancement I DIFFERENTIAL DIAGNOSIS
Occasionally cystic Intracardiac Thrombosis
May have stalk
Common, associated with atrial fibrillation and mitral
May change in position during cardiac cycle
valve disease
May prolapse through mitral or tricuspid valve
a Generally no associated adenopathy Thrombus in the cardiac chambers can have very
a Generally no pericardial effusion similar appearance to myxoma
Usually located adjacent to posterior and lateral atrial
MR Findings wall and appendage
Similar to CT
Cardiac Metastasis
Majority are inhomogeneous on MR
a Hypointense on T1 WI May be larger than myxoma
a Hyperintense on T2WI More often multiple
a Positive enhancement with gadolinium More often enhancing
Calcification not as well identified as on CT Often associated with pericardial effusion
May be well visualized without gadolinium using Look for other adjacent metastatic sites: Lung,
bright blood imaging mediastinum
Additional imaging planes may be helpful: Four Most common primary sites: Lung, breast, melanoma
chamber view, long axis views Cardiac lipoma
Nuclear Medicine Findings Often in interatrial septum
Show fat density on CT and fat signal on MR
Positron emission tomography (PET) can show the
tumor Primary Cardiac Malignancies
Most have low mitotic rate, not very metabolically Most often angiosarcoma, less common fibrosarcoma
active and liposarcoma
a If very active uptake is seen, consider other Also may have associated pericardial effusion,
diagnoses: Angiosarcoma, lymphoma adenopathy, lung metastases
May be detected with gated cardiac blood pool
scanning Cardiac lymphoma
Generally have increased uptake on PET scanning
Echocardiographic Findings Look for other areas of involvement: Lung,
Generally the initial imaging modality mediastinum
Hyperechogenic tumor
 LEFT ATRIAL MYXOMA
Pericardial Metastases
Often associated with pericardial effusion
Often extend beyond the left atrial wall
Most common primary sites: Lung, breast, melanoma
Pericardial Primary Tumors
Solitary fibrous tumor
Cysts
Cardiac Sarcoidosis
Can rarely produce cardiac masses
Usually have lung or mediastinal disease typical of
sarcoidosis
Cardiac Wegener Granulomatosis
Very rarely can produce cardiac masses
Cavitary lung masses
Papillary Fibroelastoma
Most common tumor of the valvular epithelium
Solitary (rarely multiple) arising from aortic or mitral
valve
Most have a stalk
May embolize and cause stroke
Pathogenesis: Mechanical damage, organized
thrombus, latent infectious complication of
cytomegalovirus
[PATHOLOGY
General Features
General path comments: Soft gelatinous or friable
frond-like tumor, may be firm
Genetics
o Carney triad: Gastric leiomyosarcoma, pulmonary
chondromas, extra-adrenal paragangliomas
Recurrent cardiac myxomas
Skin lesions: Pigmented nevi
o Familial multiple myxomas: Less than 10% of all
myxomas
Etiology: Unknown cell of origin, probably primitive
mesenchymal cell
Epidemiology: 90% sporadic
Associated abnormalities
o Tumor emboli can lead to intracranial aneurysms
o Peripheral emboli can lead to ischemic changes
o If obstructing mitral valve, left atrial enlargement
and pulmonary venous congestion
o If obstructing tricuspid valve, ascites, anasarca
Gross Pathologic & Surgical Features
Hemorrhage, thrombus and hemosiderin present in
80%
Calcification common (50%)
Microscopic Features
Most commonly rings and syncticial chains of
myxoma cells embedded in myxomatous matrix
May contain various other elements
o Hematopoietic, glandular, mesenchymal, and atopic
endocrine elements; rarely thymic tissue
I CLINICAL ISSUES
Presentation
Most common signs/symptoms
o Symptoms of valvular obstruction (40%)
Left atrium: Orthopnea, dyspnea
Right atrium: Peripheral edema, hepatic
congestion, ascites
o Constitutional (30%): Fatigue, weight loss, fever
o Arrhythmias
o Peripheral embolization (SOOAJ to brain causing
stroke or mycotic aneurysms)
Myocardial infarction, from embolization to
coronaries
In presence of valvular mitral stenosis, can protect
from large tumor emboli
Other signs/symptoms
o About 70% express interleukin-6 (IL-6)
Can lead to symptoms similar to connective tissue
disease
o Elevated erythrocyte sedimentation rate (ESR)
o May have auscultation findings
Resembling mitral valve disease
Tumor plop in about 15%
o May have electrocardiographic abnormalities
Mainly signs of left atrial hypertrophy
Arrhythmias rarely
Transient heart blocks
Demographics
Age
o Mean age at presentation is SO
o Range from 1 month to 81 years
Gender: Approximately 60% in females
Natural History & Prognosis
Very slow growing
Some may be managed conservatively in poor
operative candidates
May become infected, producing fever, weight loss
and septic emboli
3 year survival over 95%
Treatment
Surgical resection
o Traditional approach is via median sternotomy
o May require valve replacement
May recur after removal in 5%
May be a role for ablation in selected patients
Newer minimally invasive techniques promising
I SELECTED REFERENCES
1. Niuarchos C et al: Ascites and other extracardiac
manifestations associated with right atrial myxoma
Angiology. 56(3):357-60, 2005
2. Grebenc ML et al: Cardiac myxoma: imaging features in 83
patients Radiographies. 22(3):673-89, 2002
3. 5puentrup E et al: Visualization of cardiac myxoma
mobility with real-time spiral magnetic resonance imaging
Circulation. 104(19):E101-1, 2001
4. Araoz PA et al: CT and MR imaging of benign primary
cardiac neoplasms with echocardiographic correlation
Radiographies. 20(5):1303-19, 2000
 LEFT ATRIAL MYXOMA
I IMAGE GALLERY
Typical
(Leh) Frontal radiograph
shows mild cardiomegaly but
otherwise normal cardiac
contours in a patient with left
atrial myxoma. (Right) Axial
NECT shows mild pericardiaI
thickening (open arrow) but
the left atrial myxoma
(arrows) is only minimally
lower in attenuation than the
surrounding blood.

(Left) Axial T1WI MR shows

a sessile mass of the anterior
wall of the left atrium
(arrow) adjacent to the
aortic root (curved arrow).
The right atrium is shown by
the open arrow. (Right) Axial
STIR MR shows the sessile
mass (arrows) in the anterior
wall of the left atrium clearly
visible without gadolinium.
Differential would include
thrombus. Left atrium is not
enlarged.

Variant
(Left) Lateralradiograph
shows densely calcified left
atrial myxoma (arrows).
Patient had multiple transient
ischemic attacks, possibly
related to the myxoma.
(Right) Axial CECT shows
myxoma involving the
interatrial septum and
extending into the right
atrium (arrow), with tumor
embolism in a right lower
lobe pulmonary artery
branch (curved arrow).
Tumor is of decreased
density compared to
contrasted heart chambers
but was not fat density.
 METASTASES PERICARDIUM

Frontal radiograph shows widened mediastinal contour Axial NECT shows diffuse pericardial thickening
and large left pleural effusion (arrow) in a patient with (arrows) without significant fluid attenuation in patient
Hodgkin disease. with Hodgkin disease involving pleural (curved arrows)
& pericardial spaces.

o Pericardial fluid with high attenuation

ITERMINOlOGY o Pericardial masses usually with fluid
Abbreviations and Synonyms Location
Malignant pericardial effusion o Pericardium may extend variable distance up aorta
Primary intrathoracic malignant effusions and pulmonary artery
o May be diffuse or loculated
Definitions Size
Involvement of pericardium with malignant disease o Tumor and fluid may be minimal or large
o Lymphatic spread o Larger fluid component may produce tamponade
o Hematogenous spread o Relatively small tumor component may produce
o Direct extension tamponade
o Intraluminal tumor spread, then invasion Morphology
Pericardial involvement seen in about 5-10% of late o Fluid may be heterogeneous in attenuation
stage cancers o Fluid may be loculated in irregular collections
About 15% of cytologically examined pericardial
effusions are malignant
Radiographic Findings
In about 40% of cases, pericardial effusion presenting Radiography
site of disease o Chest film may be normal
o May show typical findings of pericardial fluid
May simulate cardiomegaly (if quantity of fluid
I IMAGING FINDINGS exceed 250 ml)
Water-bottle heart, if large
General Features Oreo cookie sign on lateral view
Best diagnostic clue

DDx: Other Pericardial Masses

Primary Angiosarcoma Partial Pericardia! Absence Pericardia! Cyst

 METASTASES PERICARDIUM
Key Facts
Terminology Enlarged Pericardial Lymph Nodes
Involvement of pericardium with malignant disease Pathology
Pericardial involvement seen in about 5-10% of late Most common primaries: Breast, lung, ovary
stage cancers
In about 40% of cases, pericardial effusion presenting Clinical Issues
site of disease Asymptomatic (50%)
Tamponade (50%)
Imaging Findings
Nonmalignant causes of pericardial effusion occurs in
Chest film may be normal 50% of cancer patients
May show typical findings of pericardial fluid About 1/3 die within a month of detection, usually
Pleural effusions in 50% from tamponade
Top Differential Diagnoses Pericardiocentesis: Primary treatment choice with
catheter drainage
3
Primary Cardiac Tumors
Epicardial Fat Pad Recurrent effusions in 50%, sclerosing agents help to 51
Pericardial Cyst prevent but rate still 5-20%
Radiation or drug induced pericarditis

Separation of pericardial and epicardial fat by fluid Signs of tamponade: Right ventricular or atrial
attenuation diastolic collapse
a May show unusual cardiac contour
a Pleural effusions in 50% Imaging Recommendations
Best imaging tool
CT Findings a Echocardiography initially
NECT Limited evaluation of right ventricle
a Water attenuation of effusion visible on noncontrast May not show entire extent of pericardium
imaging Poor demonstration of associated findings,
a Fluid may be circumferential or loculated, lentiform adenopathy
a Effusion may show heterogeneous densities a Cardiac gated MR for further evaluation
a Calcifications rare except in certain tumors
Osteosarcoma
Angiosarcoma I DIFFERENTIAL DIAGNOSIS
Some tumors with psammomatous calcium
a May have lobular soft tissue along inner or outer Primary Cardiac Tumors
margin of fluid Very rare
a Look for associated signs of malignancy Include sarcomas (myxo-, lipo-, angio-, fibro-, osteo-,
Lung, bone metastases leiomyo-, rhabdo-)
Adenopathy Also undifferentiated sarcoma, synovial sarcoma,
Pleural effusions neurofibrosarcoma
CECT Malignant fibrous histiocytoma
a May better demonstrate solid and cystic Mesothelioma
components Malignant teratoma, pheochromocytoma
a Can show associated myocardial involvement
a Better demonstration of associated adenopathy
Epicardial Fat Pad
Fat density at CT
MR Findings Often history of steroid use
TlWl Pericardia I Cyst
a Better demonstration of extent of disease than CT
Particularly good for myocardiai invasion Congenital
Better characterization of tissue types, fat Homogeneously water attenuation, smoothly
a No advantage for mediastinal adenopathy marginated
a Does not show calcifications No soft tissue components

Echocardiographic Findings Effects of Treatment

Procedure of choice for detection, quantity and quality
of suspected effusions
a Moderate effusions: Echo-free space 10-20 mm
during diastole
a Severe effusions: Echo-free space> 20 mm
Can determine right and left ventricular function
Chemotherapy or radiation induced myocardial
damage with cardiomegaly
Nephrotic syndrome with pericardial effusion
Radiation or drug induced pericarditis
a Radiation dose usually exceeds 3,000 cGy
a Common drugs: Doxorubicin and
cyclophosphamide
 METASTASES PERICARDIUM
Absence of Pericardium Microscopic Features
May produce abnormal cardiac contour or position Immune markers can help discriminate among
If total, heart is rotated and shifted into right chest different cell types
If partial, focal bulge along left heart border near main Psammoma bodies
pulmonary artery o Lung cancer
o Ovarian cancer
Morgagni Hernia
Fat from presence of mesenteric or omental fat
Gas from bowel loops ICLINICAl ISSUES
Enlarged Pericardial Lymph Nodes Presentation
Cardiac blockers often used with mantle therapy to
Most common signs/symptoms
prevent premature coronary arteriosclerosis o Asymptomatic (50%)
3 Recurrence may be manifested by rounded
cardiophrenic masses (fat pad sign)
o Related to valve obstruction
Shortness of breath, chest pain, cough, peripheral
52 Thymic Cysts edema
Fluid density at CT or MRI o Arrhythmia: Palpitations and dizziness
Most often seen after treatment of anterior mediastinal o Tamponade (50%)
lymphoma Shortness of breath out of proportion to chest film
findings
Loculated Pleural Effusion Dyspnea (95%)
Fluid density at CT Kussmaul sign: Increased distention of jugular
Usually separate from pericardium veins with inspiration
Friedreich sign: Rapid diastolic descent of the
venous pulse
I PATHOLOGY Pulsus paradoxus: Decrease of more than 10 mm
Hg in diastolic pressure on inspiration
General Features o Tumor emboli
General path comments Symptoms vary depending on site of emboli
o Malignant effusions are overwhelmingly metastases Other signs/symptoms
Primary cardiac tumors are much more rare o Pericardial fluid cytology accuracy 80-90%
Most common primaries: Breast, lung, ovary Nonmalignant causes of pericardial effusion
Etiology occurs in 50% of cancer patients
o Tamponade results from accumulation fluid in
pericardial sac Demographics
Hemodynamic compromise: Decreased cardiac Age: Age determined by incidence of malignancy
output, progressive decrease in cardiac diastolic Gender: Equal sex distribution
filling
Natural History & Prognosis
Hemodynamic compromise occurs when norma!
Very poor prognosis
quantity of pericardial fluid 50 ml) increases to
200-1,800 ml Over 80% die within 5 years of detection
o About 1/3 die within a month of detection, usually
Less fluid required to produce tamponade when
accumulation rapid from tamponade
o Only primary with significant survival: Lymphoma
Epidemiology
o Other common primaries o Only approximately 10% 1 year survival
Esophageal, can lead to esophageal-pericardial Treatment
fistula Goal: Relief of tamponade
Papillary thyroid carcinoma o Options
Malignant thymoma Pericardiocentesis: Primary treatment choice with
Renal cell carcinoma catheter drainage
Melanoma Pericardia! sclerosis
o Rare primaries Pericardia! window (surgical)
Mesothelioma Pericardiectomy
Endometrial o Recurrent effusions in 50%, sclerosing agents help to
Myxoid liposarcoma prevent but rate still 5-20%
Colon cancer
Head and neck carcinoma
Carcinoid I SELECTED REFERENCES
Gross Pathologic & Surgical Features 1. Gonzalez Valverde FM et al: Pericardial tamponade as
Over 90% are of epithelial origin initial presentation of papillary thyroid carcinoma. EurJ
o Lung is most common primary in males 5urg Oneol. 31:205-7, 200S
o Breast is most common primary in females
 METASTASES PERICARDIUM
I IMAGE GALLERY
Typical
(Left) Axial CECT shows
loculated pericardia I fluid
with irregular contour
(arrows), due to metastatic
breast cancer. Bilateral
pleural effusions are also
present, left larger than right.
(RighI) Axial CECT shows
minimal pericardia!
thickening and fluid (arrows)
as well as tumor infiltration
of the left atrium (open
arrow) in a patient with
non-Hodgkin lymphoma.

Typical
(Left) Axial NECT shows
relatively high attenuation
fluid in the pericardial space
(arrows) in a patient who
has had an esophageal
pull-up from esophageal
carcinoma (open arrow).
(RighI) Axial NECT shows
mild pericardia I thickening
with a small effusion
(arrows) in a patient with
adenocarcinoma of the lung.

Typical
(Lefl) Axial NECT shows
moderate pericardial effusion
(arrows) with attenuation
similar to water in a patient
with metastatic invasive
thymoma. A small right
pleural effusion is also
present. (Right) Axial NECT
shows mixed attenuation
complex fluid and solid
pericardial masses in a
patient with Hodgkin
disease. A pericardia Idrain
(arrow) is in place and a left
pleural effusion.
 Congenital
Arteriovenous Malformation, Pulmonary 11-4-2
Partial Anomalous Venous Return 11-4-6
Scimitar Syndrome 11-4-8
Idiopathic Pulmonary Artery Dilatation 11-4-12
Congenital Interruption Pulmonary Artery 11-4-16
Anomalous Origin Left Pulmonary Artery 11-4-20

Infectious
Septic Emboli, Pulmonary 11-4-22

Inflammatory - Degenerative
Vasculitis, Pulmonary 11-4-26
Wegener Granulomatosis, Pulmonary 11-4-28
Veno-Occlusive Disease, Pulmonary 11-4-32

Toxic - Metabolic
Takosis, Pulmonary 11-4-34
Ulidt Drug Abuse, Pulmonary 11-4-38
Smoke Inhalation 11-4-42
Silo-Filler's Disease 11-4-46

Vascular
Pulmonary Emboli 11-4-50
Pulmonary Artery Hypertension 11-4-54
Pulmonary Artery Aneurysm 11-4-58
High Altitude Pulmonary Edema 11-4-62
Neurogenic Pulmonary Edema 11-4-66

Neoplastic
Pulmonary Artery Sarcoma 11-4-70
Tumor Emboli, Pulmonary 11-4-74
Capillary Hemangiomatosis, Pulmonary 11-4-78
 ARTERIOVENOUS MALFORMATION, PULMONARY

Clinical photograph shows several characteristic Corresponding NECT shows a tubular opacity in the
telangiectases on the tongue and lower lip of an right upper lobe with demonstration of a feeding artery
asymptomatic 67 year old woman with HHT and a and draining vein (arrows). Intervening lung normal.
4 solitary PAVM. Thesefindings are characteristics of an PAVM.

2
Hepatopulmonary syndrome: Association of
ITERMINOlOGY hepatic dysfunction with hypoxemia in the
Abbreviations and Synonyms absence of intrinsic cardiopulmonary disease,
Pulmonary arteriovenous fistulae, pulmonary intrapulmonary vascular dilatations and PAVMs
arteriovenous aneurysms, and pulmonary
arteriovenous malformations (PAVMs)
Hereditary hemorrhagic telangiectasis (H HT) or IIMAGING FINDINGS
Osler-Weber-Rendu (OWR) syndrome General Features
Hemangiomas of the lung, cavernous angiomas of the
Best diagnostic clue: Nodule(s) with feeding artery(s)
lung, pulmonary telangiectases
and draining vein
Definitions Location: Periphery of the lower lobes
Spectrum of abnormal direct communications Size: Variable
between pulmonary arteries and pulmonary veins Morphology: Single or multiple well-circumscribed
Congenital or acquired nodules with feeding artery(s) and draining veins
o Congenital: Most of congenital PAVMs associated Radiographic Findings
with hereditary hemorrhagic telangiectasia
Radiography
o Acquired: Liver disorders (hepatopulmonary
o Round or oval nodule of uniform density
syndrome), systemic diseases, venous anomalies,
Distinguishing characteristic: Connected to
and after palliation of complex cyanotic congenital
feeding artery(s) and draining vein
heart disease
Lobulated but sharply-defined
May be multiple (33%), solitary (66%)
Size: 1-5 cm in diameter; occasionally
undetectable on chest radiographs

DDx: Arteriovenous Malformation

Carcinoid Septic Emboli

 ARTERIOVENOUS MALFORMATION, PULMONARY

Key Facts
Terminology Top Differential Diagnoses
Spectrum of abnormal direct communications Carcinoid
between pulmonary arteries and pulmonary veins Metastases
Congenital: Most of congenital PAVMs associated Septic Emboli
with hereditary hemorrhagic telangiectasia Solitary Pulmonary Nodule
Imaging Findings Pathology
Best diagnostic clue: Nodule(s) with feeding artery(s) Multiple AVMs highly associated with HHT (90%)
and draining vein
Morphology: Single or multiple well-circumscribed Clinical Issues
nodules with feeding artery(s) and draining veins Epistaxis presenting feature in HHT due to associated
Round or oval nodule of uniform density nasal telangiectasia (80%)
Lobulated but sharply-defined Hemorrhagic complications increased in pregnancy
May be multiple (33%), solitary (66%) CNS complications (40%)
Located in lower lobes (50-70%) in medial third of Recurrence possible but rare
the lung Treat all AVMs with feeding artery> 3 mm in
diameter

Located in lower lobes (50-70%) in medial third of

Echocardiographic Findings
4
the lung
Calcification rare (occasional phlebolith) Contrast echocardiography: Excellent tool for 3
Surrounding lung usually normal evaluation of cardiac and intrapulmonary shunts
With hemorrhage, arteriovenous malformations o Identify small right-to-left shunts even when they
(AVM) may be obscured are not suggested by gas exchange data
o Pulsations and changes in size with Valsalva and Imaging Recommendations
Mueller maneuvers
Best imaging tool
o Infarcts may develop after embolotherapy
o CECT for detection and characterization
More common with peripheral AVMs
o Pulmonary angiography still important for
Often heralded by pleurisy and pleural effusion
therapeutic planning
CT Findings Angiography supplies detailed information on
Pre-treatment morphology, complexity, and size of PAVMs
o Procedure of choice to screen for AVMs
o More sensitive than pulmonary angiography
o Nodule with feeding artery(s) and draining vein I DIFFERENTIAL DIAGNOSIS
Circumscribed, noncalcified, homogeneous
density
Carcinoid
o Useful to plan embolotherapy May enhance at CT
Simple (70%): Single feeding artery No connecting artery or vein
Complex (10%): Multiple feeding arteries Metastases
After embolotherapy < 1 month May also have feeding vessels at CT
o 2/3 disappear or shrink o "Cherry stem" appearance
o 1/3 same size Not identified on chest radiographs
Because either thrombosed or persistent perfusion Feeding arteries much smaller than feeding arteries of
After embolotherapy > 1 month AVMs
o If same size suspect embolization failure due to No large draining veins
persistent perfusion
Septic Emboli
MR Findings
May have visible feeding vessels, usually do not
MR angiography similar to CT for detection enhance after contrast administration
Significant limitation when screening for small lesions Cavitation is frequent
Nuclear Medicine Findings Solitary Pulmonary Nodule
Tc-99m labeled macroaggregates may be used to Most commonly: Bronchogenic carcinoma,
estimate size of right to left shunt by measuring granuloma, hamartoma, carcinoid, metastasis
activity accumulation in kidney (normally Only AVM has feeding artery and vein
macroaggregates will not pass through pulmonary
capillary bed)
 ARTERIOVENOUS MALFORMATION, PULMONARY
I PATHOLOGY Hemorrhagic complications increased in
pregnancy
General Features o Paradoxic embolism to central nervous system (CNS)
General path comments ConSiderable morbidity and mortality
o Pathophysiology o CNS complications (40%)
Right to left shunt with high flow and low Transient ischemic attacks (TIAs) and stroke in
resistance 20-40%
Hypoxemia uncorrected with 100% 02 Migraine in 43%
Genetics Cerebral abscess in 9%
o Multiple AVMs highly associated with HHT (90%) Seizures in 8%
Autosomal dominant disorder with mutations Other signs/symptoms
localized to chromosome locus 9q 33-34 or o Hypoxemia exaggerated in upright position
OWR-1 (orthodeoxia) due to increased shunting in lower
Prevalence of approximately one in 40,000 to one lobe AVMs
in 2,000 o May lead to high output congestive heart failure
Penetrance is age-related and is nearly complete Demographics
by age 40 Age: 10% of cases are identified in infancy or
Etiology: Unknown childhood
Epidemiology
Gender
o 15-35% of patients with HHT have PAVM o M:F = 1:2
4 o 50% of patients with PAVM have mucocutaneous Male predominance in newborns
telangiectases
4 Associated abnormalities Natural History & Prognosis
o 5-13% of patients with HHT have intracerebral Growth typically slow but maybe rapid
AVMs Recurrence possible but rare
02-17% have hepatic AVMs o Periodic screening CT every 5 years
Family members should be screened for HHT
Gross Pathologic & Surgical Features
Pathologic AV communications may occur in simple Treatment
or complex forms Treatment of choice: Selective embolization
o Tangled web of vessels (Medusa head or can of o Intravascular coils or balloons: Polyvinyl alcohol
worms) (Ivalon), wool coils, and stainless steel coils
o Draining veins usually larger than feeding arteries o Treat all AVMs with feeding artery> 3 mm in
o Classified as simple or complex diameter
Simple: Solitary feeding artery Surgery rarely used
Complex: More than one feeding artery o Large isolated malformations
Several malformations (often termed "multiple" Complications
type) o Minor (15%)
Innumerable telangiectatic anastomoses widely Chest pain and pleurisy
spread in both lungs or in at least one lobe of the o Major (5%)
lung (diffuse or telangiectatic type) Paradoxical embolization of coils or balloons
(0.5%)
Microscopic Features More common with simple than complex AVM
Intervening lung normal Infarction: More common with distal than central
Vessels have thin-walls AVMs
o At risk for rupture and hemorrhage

IClINICAllSSUES
I SELECTED REFERENCES
1. Bittles MAet al: Multidetector CT angiography of pediatric
Presentation vascular malformations and hemangiomas: utility of 3-D
Most common signs/symptoms reformatting in differential diagnosis. Pediatr Radiol. 2005
2. Jeong WK et al: Telangiectatic Pulmonary Arteriovenous
o Symptomatic 40-60 years of age Malformation. J Thorac Imag. ]8:113-] ]5,2003
Usually, a single PAVM < 2 cm in diameter does 3. Khurshid I et al: Pulmonary arteriovenous malformation.
not cause symptoms Postgrad MedJ. 78:]9]-]97,2002
o Hemoptysis, cyanosis and clubbing in the presence 4. Lawler LPet al: Multi-detector row CT of thoracic disease
of right to left shunt with emphasis on 3D volume rendering and CT
o Hemorrhage angiography. Radiographies. 21:]257-]273, 200]
Epistaxis presenting feature in HHT due to 5. Faughnan MEet al. Diffuse pulmonary arteriovenous
associated nasal telangiectasia (80%) malformations; characteristics and prognosis. Chest.
117:31-38,2000
Hemoptysis also common 6. Remy-Jardin M et al: Spiral CT angiography of the
Hemothorax serious and may be fatal pulmonary circulation. Radiology. 2]2:6] 5-636, ]999
 ARTERIOVENOUS MALFORMATION, PULMONARY

I IMAGE GALLERY

(Leh) Frontal radiograph

shows a small solitary
pulmonary nodule (SPN) in
the right upper lobe (arrow).
A branching opacity is also
visible in the inferior part of
the nodule (open arrow)
suggestive of an AVM.
(Right) Angiography of the
right main pulmonary artery
demonstrates the opacity
was an arteriovenous
malformation with a dilated
feeding artery and a dilated
draining vein (arrows).

4
Typical 5
(Left) Frontal radiograph of
the left lower lung shows a
single, bilobed PAVM with
smooth lobulated borders
(arrows). (Right)
Angiography of the same
patient confirms the vascular
nature of the nodule and
shows the typical findings of
a large arteriovenous
malformation in the left
lower lobe (arrow).

Typical
(Leh) Axial NEeT shows a
slightly lobulated opacity in
the right upper lobe with
demonstration of a feeding
artery and draining vein
(arrows). These findings
confirm the vascular nature
of the lesion. (Right) 3D
gadolinium-enhanced MRA
shows a solitary AVM in the
right upper lobe. A feeding
artery originating from the
right superior pulmonary
artery and a draining vein
are clearly demonstrated
(arrow).
 PARTIAL ANOMALOUS VENOUS RETURN

Axial CECT MIP shows left superior pulmonary vein Coronal CECT shows a scimitar vein draining into the
(arrows) draining into the left brachiocephalic vein supradiaphragmatic IVC. No hypoplasia of lung. Right
(curved arrow). Partial anomalous venous return. pulmonary artery normal. PAPVR in scimitar syndrome.
(Courtesy C. Abbott, MO).

o Left-to-right shunt: Commonly associated with atrial

!TERMINOLOGY septal defect (ASD)
Abbreviations and Synonyms Dilatation of intra parenchymal vessels
Partial anomalous pulmonary venous return (PAPVR) Dilatation right ventricular cavity and right
atrium and normal sized left atrium
Definitions o Recognition abnormal vein into systemic vein
Congenital anomaly where pulmonary vein(s) drain
into systemic veins (total or partial)
MR Findings
Anomalous connections: Supracardiac, cardiac, Better depicts septal defects
infradiaphragmatic, mixed
I DIFFERENTIAL DIAGNOSIS
I IMAGING FINDINGS Left Superior Vena Cava (SVC)
Radiographic Findings 2 vessels ventral to the left upper lobe bronchus: Left
Radiography superior vena cava and left superior pulmonary vein
o Usually normal, rare to identify abnormal vein o Vessels absent with PAPVR
o En face vein a solitary pulmonary nodule mimic o Drains into coronary sinus, right sided SVC may be
o Left-to-right shunt: When shunt> 2:1 absent
Cardiomegaly, pulmonary vascular plethora, Pulmonary Varix
pulmonary artery hypertension (late)
Acquired or development dilatation pulmonary vein at
CT Findings its entrance to left atrium
CECT
Lobulated mass or nodule posterior to the heart

DDx: Venous Anomalies

Left Superior Vena Cava Left Superior Intercostal Vein

 PARTIAL ANOMALOUS VENOUS RETURN

Key Facts
Terminology Clinical Issues
Congenital anomaly where pulmonary vein(s) drain Contralatera'l pneumonectomy: PAPVR shunt may
into systemic veins (total or partial) now account for majority of cardiac output

Top Differential Diagnoses Diagnostic Checklist

Left Superior Vena Cava (SVC) Discovery of PAPVR, look carefully for ASD: Cause of
Pulmonary Varix paradoxical emboli and stroke
Left Superior Intercostal Vein

o None, usually incidental radiographic finding

Left Superior Intercostal Vein o Swan-Ganz catheterization
2-4 intercostal veins and accessory hemiazygos form Equal oxygen saturation all chambers
the left superior intercostal vein which drains into the
left brachiocephalic vein, enlarges with SVC or left Natural History & Prognosis
brachiocephalic vein occlusion Shunt less than 2:1, normal life span
Aortic "nipple" on chest radiograph
Treatment
Options, risks, complications
4
o Advise surgeon, inadvertent clipping will result in
I PATHOLOGY persistent lobar edema
7

General Features o Contralateral pneumonectomy: PAPVR shunt may

Etiology: Congenital anomaly where normal systemic now account for majority of cardiac output
venous connection to the lung bud fails to involute May require reimplantation of aberrant vein into
Epidemiology: Incidence 0,5% left atrial appendage
Associated abnormalities Consider surgical or percutaneous closure of ASD
o Scimitar syndrome: Vertical vein along with variable
degrees of right lung hypoplasia
o ASD: Up to 90% with anomalous vein into SVC I DIAGNOSTIC CHECKLIST
Sinus venosus type high in septum near SVC
Image Interpretation Pearls
Gross Pathologic & Surgical Features Discovery of PAPVR, look carefully for ASD: Cause of
PAPVR right lung more frequent than left paradoxical emboli and stroke
Anomalous vein usually drains into nearest systemic
vein, commonly SVC on the right and left
brachiocephalic vein on the left ISElECTED REFERENCES
1. Demos TC et al: Venous anomalies of the thorax. AJR Am J
Roentgenol. 182(5):1139-50,2004
IClINICALISSUES 2. RemY-Jardin M et al: Spiral CT angiography of the
pulmonary circulation. Radiology. 212(3):615-36, 1999
Presentation
Most common signs/symptoms

I IMAGE GALLERY

(Left) Axial CECT shows left PAPVR (arrow) connecting to vertical vein (curved arrow) at the level of the aorticopulmonary window. Vein will
drain cephalad to the left brachiocephalic vein, (Center) Axial CECT shows no vessels veneral to the left main bronchus (arrow) in subject with
partial anomalous venous return to the left brachiocephaJic vein. (Right) Axial CECT in comparison shows two vessels ventral to the left main
bronchus, the left superior pulmonary vein (arrow) and a left superior vena cava (curved arrow).
 SCIMITAR SYNDROME

Frontal radiograph shows vertical vein coursing towards Coronal eTA shows scimitar vein draining into the
the right costovertebral angle (arrow). Mild supradiaphragmatic IVC The right pulmonary artery is
cardiomegaly. Normal pulmonary vascularity. Normal normal. Scimitar syndrome. (Courtesy G. Abbott, MD).
lung volumes. (Courtesy G. Abbott, MD).

8
Location: Right lung almost exclusively (left lung
ITERMINOlOGY extremely rare)
Abbreviations and Synonyms Size: Hypoplastic lung may be mild or marked
Hypogenetic lung syndrome, congenital pulmonary Morphology: Anomalous vein usually drains into
venolobar syndrome subdiaphragmatic inferior vena cava

Definitions Radiographic Findings

Congenital hypoplasia of the right lung and Radiography
anomalous pulmonary venous drainage to the inferior o Anomalous vein (75%)
vena cava (IVC) Gently curved tubular shadow descending from
Partial anomalous pulmonary venous drainage: One or the right mid-lung towards the right
more pulmonary veins connect to a systemic vein costovertebral angle
Vein gets larger as it descends towards the
diaphragm
IIMAGING FINDINGS Shaped like a Turkish sword (scimitar syndrome)
o Small right hemithorax with mediastinal and
General Features cardiac shift into the right hemithorax
Best diagnostic clue Due to hypoplasia lung
o Vertical vein: Gently curved vein in the right Hypoplasia may be mild or marked
mid-lung towards the right costovertebral angle, Cardiac shift may be mistaken for dextrocardia
vein enlarges as it descends towards the diaphragm (actually dextroposition)
o Anomalous vein shaped like a Turkish sword o Left lung
(scimitar sign)

DDx: Scimitar Syndrome

Congenital Interruption PA Sequestration Chronic Thromboembolism

 SCIMITAR SYNDROME

Key Facts
Terminology Top Differential Diagnoses
Hypogenetic lung syndrome, congenital pulmonary Congenital Interruption Pulmonary Artery
venolobar syndrome Pulmonary Sequestration
Congenital hypoplasia of the right lung and Wandering Vein
anomalous pulmonary venous drainage to the Fibrosing Mediastinitis
inferior vena cava (IVe) Chronic Thromboembolic Occlusion
Swyer-James Syndrome
Imaging Findings
Vertical vein: Gently curved vein in the right Pathology
mid-lung towards the right costovertebral angle, vein Oxygenated blood drains into right atrium producing
enlarges as it descends towards the diaphragm a left-to-right shunt
Anomalous vein shaped like a Turkish sword (scimitar Associated cardiac anomalies (25%)
sign)
Morphology: Anomalous vein usually drains into
Clinical Issues
subdiaphragmatic inferior vena cava 50% asymptomatic
Recurrent infections common
Without cardiac anomalies, usually normal life span

Hyperinflation with large left pulmonary artery 4

(depends on degree of hypoplasia right pulmonary
MR Findings
Similar to CT 9
artery)
Increased vascularity from left-to-right shunt Advantages
o Small right hilum o Nonionizing
o Associated anomalies o Better for cardiac shunts
Cardiomegaly: Secondary to pulmonary artery Disadvantages
hypertension, left-to-right shunt, or cardiac o Less useful for lung or bronchial anatomy
anomalies Echocardiographic Findings
Vertebral anomalies Useful to evaluate for shunts and estimate pulmonary
Bronchogenic cyst sometimes associated artery pressure
Extremely rare accessory diaphragm
Diaphragmatic hernia Imaging Recommendations
Best imaging tool
CT Findings
o CT to determine hypoplasia of lung, size of
CECT pulmonary arteries, and course of draining vein
o Bronchial anomalies, associated o Useful to determine extent of associated anomalies
Absent minor fissure, mirror imaging branching, Protocol advice: CTA with multiplanar reconstructions
bilobed right lung
Horseshoe lung: Bridge of lung fusing the two
lungs across the posterior mediastinum I DIFFERENTIAL DIAGNOSIS
Horseshoe lung: Ominous finding often associated
with lethal cardiac anomalies Congenital Interruption Pulmonary Artery
Bronchial diverticula No vertical vein
o Cardiovascular anomalies, associated Aortic arch on opposite side of interrupted artery
Dilated right ventricle in left-to-right shunts and Normal bronchial branching
pulmonary hypertension Volume reduction affected lung marked similar to
Enlarged main pulmonary artery marked hypoplasia in scimitar syndrome
o Anomalous vein
Precisely define drainage into systemic vein Pulmonary Sequestration
o Occasional systemic arterialization of the lung from No vertical vein
the descending thoracic aorta or upper abdominal Systemic supply to sequestered lung, usually from
aorta descending or abdominal aorta
HRCT More common in the left costovertebral angle
o Hypoplastic lung: Mosaic perfusion with Lung usually dense but may be hyperlucent,
hypoattenuation of the hypoplastic lung simulating hypogenetic lung
o Secondary bronchiectasis from recurrent infections Normal venous drainage in intralobar sequestration
o Infections may be secondary to seeding of lung from
bronchial diverticula Wandering Vein
Anatomic variant
Angiographic Findings Pulmonary vein may normally wander in lung with
Not required for diagnosis, CT and MRI have replaced normal drainage into left atrium
Normal lung size
 SCIMITAR SYNDROME
o Symptoms of left-to-right shunt
Fibrosing Mediastinitis Dyspnea on exertion
No vertical vein o Symptoms of pulmonary artery hypertension
Pulmonary veins may be obstructed Nonspecific: Dyspnea, easy fatigue, chest pain,
Endemic geographic area for Histoplasmosis Raynaud phenomenon
Focal hilar or mediastinal mass narrowing nearby Paradoxical embolization with septal defects
airways or vessels Other signs/symptoms
Calcification of mass common (60-90%) o Recurrent infections common
Chronic Thromboembolic Occlusion o Systolic murmur
< 1% of patients following acute thromboembolism Demographics
No vertical vein Age: Any age
Small artery due to organized thrombus
Normal pulmonary venous anatomy, size may be Natural History & Prognosis
small from diminished blood flow Without cardiac anomalies, usually normal life span
Swyer-James Syndrome Treatment
No vertical vein Cardiac surgery or intervention for associated cardiac
Volume loss affected lung anomalies
Normal pulmonary venous anatomy Lung resection sometimes required for removal of
bronchiectatic segments
4 !PATHOLOGY
None required specifically for scimitar syndrome

10
General Features I DIAGNOSTIC CHECKLIST
Genetics: Sporadic
Epidemiology: 2 per 100,000 births Image Interpretation Pearls
Scimitar vein may be subtle, should be examined for
Gross Pathologic & Surgical Features carefully when the right hemithorax is small and
Anomalous vein drains all or a portion of the right hyperlucent
lung
o Oxygenated blood drains into right atrium
producing a left-to-right shunt I SELECTED REFERENCES
Hypogenetic lung
1. Konen E et al: Congenital pulmonary venolobar syndrome:
o Lobar agenesis to focal hypoplasia
spectrum of helical CT findings with emphasis on
Anomalous drainage to computerized reformatting. Radiographies. 23(5):1175-84,
o Subdiaphragmatic lVC (most common) 2003
o Hepatic veins 2. Kamijoh M et al: Horseshoe lung with bilateral vascular
o Portal vein anomalies: a rare variant of hypogenetic lung syndrome
o Azygos vein (scimitar syndrome). Pediatr Int. 44(4):443-5, 2002
o Coronary sinus 3. Zylak CJ et al: Developmental lung anomalies in the adult:
o Right atrium radiologic-pathologic correlation. Radiographies. 22 5pec
No(525-43, 2002
Arterial supply 4. Vrachliotis TG et al: Hypogenetic lung syndrome:
o Hypoplastic or normal right pulmonary artery functional and anatomic evaluation with magnetic
o Systemic supply from abdominal aorta common resonance imaging and magnetic resonance angiography. )
Associated cardiac anomalies (25%) Magn Reson Imaging. 6(5):798-800, 1996
o Atrial septal defect (most common) 5. Cairns RA et al: Pediatric case of the day. Hypogenetic lung
o Ventricular septal defect syndrome (Scimitar syndrome) with right-sided congenital
o Tetralogy of Fallot diaphragmatic hernia. Radiographies. 15(2):496-9, 1995
o Patent ductus arteriosus 6. Woodring)H et al: Congenital pulmonary venolobar
syndrome revisited. Radiographies. 14(2):349-69, 1994
o Coarctation of the aorta
7. Pomerantz 5M et al: False pulmonary artery catheter
o Persistence of the left superior vena cava measurements due to the scimitar (hypogenetic lung)
o Pulmonic stenosis syndrome. Potential for iatrogenic pulmonary edema.
Bronchial anomalies Chest. 103(6):1895-7, 1993
o Mirror image branching (left branching pattern) 8. Heron CW et al: Anomalous systemic venous drainage
o Bronchial diverticula occurring in association with the hypogenetic lung
o Horseshoe lung syndrome. Clin Radiol. 39(4):446-9, 1988
9. Gilman M) et al: Hypoplastic right pulmonary artery in the
hypogenetic lung syndrome.) Com put Assist Tomogr.
6(5):1015-8,1982
IClINICAL ISSUES
Presentation
Most common signs/symptoms
o 50% asymptomatic
 SCIMITAR SYNDROME
I IMAGE GALLERY

(Left) Frontal radiograph

shows small right
hemithorax. Dextroposition.
Left pulmonary artery
(arrow) and vascularity to
left lung increased.
Anomalous vein not
identified. (Right) Coronal
CTA oblique MIP shows the
anomalous vein coursing
below the diaphragm to join
the IVC.

4
11
(Left) Axial CECT same
patient as above shows the
enlarged anomalous veins in
the right lower lobe. IVC
(arrows) is distended from
the left-to-right shunt.
Hypoplastic lung (curved
arrows) is lucent and small in
volume. (Right) Axial CECT
same patient shows
dextroposition of the heart
into the right hemithorax.
Right pulmonary artery is
small (arrow).

(Left) Axial CECT sequential

images shows scimitar vein
(arrows) coursing directly
into the right atrium. (Right)
Coronal CTA oblique MIP
better demonstrate the
course of the anomalous
vein into the right atrium
than the axial images.
 IDIOPATHIC PULMONARY ARTERY DILATATION

Frontal radiograph shows an enlarged main pulmonary Axial CECT shows enlarged main pulmonary artery.
artery (arrow) in a young female. Heart size is normal. Main pulmonary artery is larger than ascending aorta.
Pulmonary vascularity is otherwise normal. Both the right and left pulmonary artery are normal.
4
12 o Focal convexity caudal to aortic arch and cephalad
ITERMINOlOGY to left main bronchus due to enlarged main
Definitions pulmonary artery (2n.d mogul)
Normal resting mean pulmonary artery pressure < 20 a Remainder of pulmonary arteries usually normal
mm Hg Left or right pulmonary arteries may also be
enlarged in continuation with enlarged main
Enlargement of the main pulmonary artery with or
without dilatation of the right and left pulmonary pulmonary artery
arteries a Normal heart size
a Idiopathic pulmonary artery dilatation a diagnosis a Normal pulmonary vascularity
of excl usion a No pleural or pericardial effusions
CT Findings
CECT
IIMAGING FINDINGS a Normal pulmonary artery
Transverse diameter of main pulmonary artery <
General Features
28.6 mm measured at bifurcation perpendicular to
Best diagnostic clue: Focal convexity between the
long axis of pulmonary artery
aortic arch and left main bronchus (2nd mogul)
Main pulmonary artery smaller diameter than
Location: Main pulmonary artery with possible
adjacent ascending aorta
extension into the right or left pulmonary artery
Transverse diameter of right interlobar pulmonary
Morphology: Smooth enlargement main pulmonary
artery: < 16 mm men and < 14 mm women
artery a Enlarged main pulmonary artery
Radiographic Findings a Right and left pulmonary arteries may also be
Radiography enlarged

DDx: Enlarged Pulmonary Artery

Pulmonary Artery Hypertension Pulmonary Valve Stenosis Pulmonary Artery Sarcoma

 IDIOPATHIC PULMONARY ARTERY DILATATION
Key Facts
Terminology Adenopathy
Enlargement of the main pulmonary artery with or Pathology
without dilatation of the right and left pulmonary Epidemiology: 0.6% all congenital cardiac anomalies
arteries
Idiopathic pulmonary artery dilatation a diagnosis of Clinical Issues
exclusion Asymptomatic
Discovered fortuitously on chest radiographs
Imaging Findings Age: Any age, typically recognized 20-30's
Hemodynamics now estimated using Gender: Females more common
echocardiographic techniques Long term observation useful to exclude underlying
Top Differential Diagnoses pathology
Pulmonary Vavle Stenosis None required
Pulmonary Artery Hypertension Diagnostic Checklist
Pulmonary Embolus Left upper lobe pulmonary arteries will be enlarged in
Pulmonary Artery Sarcoma those with pulmonary artery stenosis due to jet effect
Mediastinal Germ Cell Tumor of blood flow across the pulmonic valve

o Peripheral arteries normal o Can evaluate for left-to-right shunts 4

Left upper lobe arteries same size as right upper o Limited in evaluating pulmonary artery size
13
lobe arteries useful to help exclude pulmonary
Imaging Recommendations
artery stenosis
o Normal right ventricle Best imaging tool: MRA or CECT to evaluate
Width right ventricular cavity smaller then left pulmonary arteries and exclude mediastinal mass or
ventricular cavity other cause of enlarged pulmonary artery on chest
Interventricular septum not convex into the left radiographs
ventricular cavity
No hypertrophy right ventricular wall
o Normal size atria I DIFFERENTIAL DIAGNOSIS
Left-to-right shunts usually enlarge one of the Pulmonary Vavle Stenosis
atria
Murmur
HRCT Enlarged main and left pulmonary artery, right
o No mosaic perfusion
pulmonary artery usually normal
o No septal thickening
Diameter left upper lobe vessels larger than mirror
Angiographic Findings image vessels right upper lobe due to jet effect of
Not necessary for diagnosis blood across the pulmonic valve
Hemodynamics now estimated using Dilated right ventricle or right ventricular wall
echocardiographic techniques hypertrophy
Right heart catheterization may be required to exclude Valve calcification rare
elevated pulmonary artery pressures Pulmonary Artery Hypertension
MR Findings Enlarged main and central pulmonary arteries with
No ionizing radiation, especially useful for follow-up rapid pruning peripheral arteries
of young patients Primary (typically young women) or secondary to
Helpful to evaluate for cardiac valve dysfunction underlying cardiopulmonary disease
Equal to CT to evaluate size of pulmonary arteries Right ventricular hypertrophy and dilatation of right
Equivalent to CT for accuracy ventricle
May have pericardia I effusion
Echocardiographic Findings Secondary lung abnormalities
Echocardiogram o Emphysema or fibrosis in secondary pulmonary
o Useful to exclude other causes of enlarged hypertension
pulmonary artery o Mosaic perfusion, septal thickening, and small
o Useful to evaluate hemodynamic parameters pleural effusion in pulmonary veno-occlusive
Doppler echocardiography used to estimate disease
pulmonary artery pressures
Size of tricuspid regurgitant jet used to estimate Pulmonary Embolus
pressure according to modified Bernoulli equation Enlarged pulmonary artery due to lodgment of the clot
o Useful to evaluate for valvular heart disease in the enlarged pulmonary artery (Fleischner sign)
Doppler provides information on tricuspid and May have focal opacities from pulmonary infarcts
pulmonary valvular regurgitation Predisposing conditions for thromboembolic disease
 IDIOPATHIC PULMONARY ARTERY DILATATION
Chronic thromboembolism may enlarge central
pulmonary arteries due to development of secondary Demographics
pulmonary hypertension Age: Any age, typically recognized 20-30's
Gender: Females more common
Pulmonary Artery Sarcoma
Rare cause of enlarged pulmonary artery Natural History & Prognosis
Usually symptomatic with dyspnea and chest pain Long term observation useful to exclude underlying
Lobulated pulmonary artery enlargement, most pathology
commonly located main pulmonary artery True idiopathic dilatation: Normal life span
May have hematogenous metastases Treatment
o Rare cause of tree-in-bud opacities
Options, risks, complications
Mediastinal Germ Cell Tumor o Rarely compress tracheobronchial tree
Anterior mediastinal mass centered on the main o Case reports of arterial dissection, probably due to
pulmonary artery underlying connective tissue disorder
Teratoma: 25% contain calcification None required
May have murmur of pulmonary valve stenosis Recognition that it is a benign entity
Normal or compressed pulmonary artery at CT
Adenopathy I DIAGNOSTIC CHECKLIST
Sarcoid common disease that causes bilateral
Consider
4 symmetric hilar enlargement
Hilum more lobulated, may have abnormal Idiopathic dilatation as a cause of enlarged main
14 mediastinal contours from adenopathy pulmonary artery, diagnosis of exclusion
Normal pulmonary arteries at CT Exclude other causes of enlarged pulmonary artery:
Commonly pulmonary hypertension, pulmonic valve
stenosis, left-to-right shunts
I PATHOLOGY Image Interpretation Pearls
General Features Left upper lobe pulmonary arteries will be enlarged in
Etiology those with pulmonary artery stenosis due to jet effect
o Unknown of blood flow across the pulmonic valve
o Congenital: Possible unequal division of truncus
arteriosus
Epidemiology: 0.6% all congenital cardiac anomalies I SElECTED REFERENCES
Associated abnormalities: Ascending aortic hypoplasia 1. Ring NJ et al: Idiopathic dilatation of the pulmonary
or dilatation artery. BrJ Radiol. 75(894):532-5, 2002
2. Hoeftel JC: Idiopathic dilatation of the pulmonary artery:
Gross Pathologic & Surgical Features report of four cases. Magn Reson Imaging. 19(5):761,2001
Normal but thinned arterial wall 3. Corbi Pet al: [Idiopathic aneurysm of the pulmonary
artery trunk. Report of a case]. Ann Med Interne (Paris).
Microscopic Features 150(1):67-9, 1999
No specific abnormality 4. Ugolini Pet al: Idiopathic dilatation of the pulmonary
artery: report of four cases. Magn Reson Imaging.
17(6):933-7, 1999
5. Chang RYet al: Idiopathic dilatation of the pulmonary
I CLINICAL ISSUES artery. A case presentation. Angiology. 47(1):87-92,1996
6. Andrews R et al: Pulmonary artery dissection in a patient
Presentation with idiopathic dilatation of the pulmonary artery: a rare
Most common signs/symptoms cause of sudden cardiac death. Br Heart J. 69(3):268-9,1993
o Asymptomatic 7. Asayama J et al: Echocardiographic findings of idiopathic
o Discovered fortuitously on chest radiographs dilatation of the pulmonary artery. Chest. 71(5):671-3,
o Diagnostic criteria 1977
Simple dilatation of pulmonary trunk with or 8. Wilhelm WC et al: Acquired pulmonic stenosis due to
without involvement of the rest of the arterial tree cardiac compression by a benign teratoma. Ann Thorac
Surg. 7(1):38-41, 1969
Absence of intracardiac or extracardiac shunts 9. Befeler B et al: Idiopathic dilatation of the pulmonary
Absence of chronic cardiopulmonary disease artery. Am J Med Sci. 254(5):667-74, 1967
Absence of arterial disease such as vasculitis
o Specifically exclude
Pulmonary artery hypertension: Pre and
post-capillary
Pulmonary artery stenosis
Left-to-right shunts
Other signs/symptoms: Occasional slight cardiac
murmur along the left sternal border
 IDIOPATHIC PULMONARY ARTERY DILATATION

I IMAGE GALLERY
Typical
(Left) Frontal radiograph
shows enlarged tubular
shaped main pulmonary
artery (arrow) in a young
female. She was
asymptomatic with no
murmur at auscultation.
Heart size normal.
Pulmonary vascularity
normal. (Right) Axial GeT
shows an enlarged main and
left pulmonary artery. She
was asymptomatic.
Idiopathic dilatation main
pulmonary artery.

4
Typical 15
(Left) Frontal radiograph
shows enlarged main and
lobar pulmonary arteries.
Hearl size is normal.
(Courtesy P. Agarwal, MO).
(Right) Axial radiograph
shows enlarged main and left
pulmonary arteries. Main
pulmonary artery is larger
than ascending aorta.
(Courtesy P. Agarwal, MO).

Typical
(Left) Axial radiograph shows
enlarged main and right
pulmonary artery. Left lower
lobe pulmonary artery is
normal. After excluding other
causes, diagnosis idiopathic
dUatation main pulmonary
artery. (Courtesy P. Agarwal,
MO). (Right) Axial CECT in
patient with idiopathic
dilatation of main pulmonary
artery. Left upper lobe and
right upper lobe arteries are
equal in size.
 CONGENITAL INTERRUPTION PULMONARY ARTERY

Frontal radiograph shows small left hemithorax and Lateral radiograph shows right pulmonary artery (arrow)
marked hyperinflation of the right lung with shift of the and absence of the left pulmonary artery (curved
mediastinal to the left. R.;ghtinterlobar artery is enlarged arrow). Congenital interrupUon left pulmonary artery
4 (arrow). Right aortic arch (curved arrow).

16
Substantial loss of volume: Elevation of
!TERMINOlOGY hemidiaphragm, shift of mediastinum and heart
Abbreviations and Synonyms Small or indistinct hilum
Pulmonary artery agenesis, unilateral absence of a Fine peripheral interstitial thickening (secondary
pulmonary artery to collateral vessels feeding the lung)
Rib notching (intercostal collateral vessels)
Definitions a Unaffected hemithorax
Congenital interruption of pulmonary artery Marked compensatory hyperinflation herniating
development, more common on the left into affected hemithorax
Hilum larger than normal (artery must carry
entirety of cardiac output)
IIMAGING FINDINGS a Cardiomegaly from associated cardiac anomalies, or
heart failure associated with secondary pulmonary
General Features artery hypertension
Best diagnostic clue: Small hemithorax with small
hilum, aortic arch on the opposite side CT Findings
Location: Left more common than right CECT
o Absent pulmonary artery
Radiographic Findings o Normal bronchial branching pattern
Radiography o Signs of collateral circulation affected side
a Aortic arch on opposite side of the absent Enlarged bronchial arteries (70%), internal
pulmonary artery mammary arteries (60%), lateral thoracic artery
a Affected hemithorax (15%)

DDx: Small Hilum

Fibrosing Mediastinitis Chronic Pulmonary Embolism Scimitar

 CONGENITAL INTERRUPTION PULMONARY ARTERY
Key Facts
Terminology Swyer-James Syndrome
Congenital interruption of pulmonary artery Scimitar Syndrome
development, more common on the left Pathology
Imaging Findings Right interruption associated congenital anomalies
less frequent
Best diagnostic clue: Small hemithorax with small
Left interruption higher incidence of congenital
hilum, aortic arch on the opposit~ side
cardiovascular anomalies
Aortic arch on opposite side of the absent pulmonary
artery Clinical Issues
Fine peripheral interstitial thickening (secondary to Recurrent pulmonary infections in 33%
collateral vessels feeding the lung) Hemoptysis 20%
No air trapping at expiration Asymptomatic 15%
Top Differential Diagnoses Pulmonary hypertension 20%
May be discovered when develop high altitude
Fibrosing Mediastinitis
pulmonary edema o'r pulmonary hypertension during
Chronic Thromboembolic Occlusion
pregnancy
Takayasu Arteritis

Hypertrophy extra pleural fat with tortuous

I DIFFERENTIAL DIAGNOSIS
4
intercostal arteries (40%)
17
Serrated pleural thickening 75% Fibrosing Mediastinitis
Subpleural parenchymal bands 66% Endemic geographic area for histoplasmosis
Mosaic attention 33% Commonly also obstructs nearby veins and airways
Diameters of peripheral pulmonary blood vessels Focal hilar or mediastinal mass narrowing nearby
in affected lung smaller airways or vessels
HRCT Calcification of mass common (60-90%)
o Bronchiectasis complication of recurrent infections
(25%) Chronic Thromboembolic Occlusion
Embryologically, airway development separate < 1% of patients following acute thromboembolism
from arteries, branching pattern in affected lung Chronic thrombus
normal o Eccentric location
o Mosaic attenuation o Arterial lumen decreased more than 50%
In affected lung (35%) probably due to hypoxic o Arterial stenosis or webs
vasoconstriction o Complete occlusion affected artery
In unaffected lung (50%) probably due to Bronchiectasis common
overperfusion of unaffected lung Volume loss in affected lung much less than
No air trapping at expiration congenital interruption pulmonary artery
o Lung attenuation equal or even slightly more
radiopaque compared with the unaffected lung due Takayasu Arteritis
to hyperemia Nonspecific (probably autoimmune) granulomatous
aortitis
Angiographic Findings Pulmonary artery involvement (50%)
Usually not required for diagnosis, CT and MRI have o Most common abnormalities: Stenosis or occlusion
replaced of segmental or subsegmental upper lobe branches
Embolization of systemic collaterals for recurrent or Usually affects multiple arteries especially aorta
life-threatening hemoptysis (abnormal contour)
Volume loss in affected lung much less than
MR Findings congenital interruption pulmonary artery
Similar to CT CT or MRI demonstrated wall thickening with or
Nuclear Medicine Findings without enhancement of affected vessels
V/QScan Swyer-James Syndrome
o No perfusion to affected side Air-trapping on expiratory radiographs
o Ventilation affected side normal Volume loss in affected lung much less than
Imaging Recommendations congenital interruption pulmonary artery
Lung attenuation decreased compared with the
Best imaging tool: CT and or MRI to evaluate central
unaffected lung
pulmonary arteries
Perfusion decreased but present on perfusion scanning
 CONGENITAL INTERRUPTION PULMONARY ARTERY
Scimitar Syndrome Natural History & Prognosis
Scimitar sign: Curved anomalous venous trunk, Determined by associated cardiac anomalies or
resembling a Turkish sword, in right medial pulmonary artery hypertension
costophrenic sulcus near right heart border, that
increases in caliber in a caudad direction Treatment
Right lung hypoplasia Options, risks, complications
o Risk for development of high altitude pulmonary
edema
I PATHOLOGY o Risk of developing pulmonary hypertension during
pregnancy
General Features May require termination of pregnancy
Etiology Revascularization of absent artery in infancy
o Normal pulmonary artery develops from the Hemoptysis
proximal aspect 6th aortic arch during 1st 16 weeks o Angiographic embolization of systemic collaterals
intrauterine development o May require pneumonectomy
Proximal arch becomes the proximal segment of Pulmonary hypertension
the right and left pulmonary artery o Same as for primary pulmonary hypertension
Right: Distal part loses connection with the arch
Left: Distal part maintains connection as the
ductus arteriosus I DIAGNOSTIC CHECKLIST
4 Intrapulmonary vessels remain intact
Consider
Epidemiology
18 o 1 per 200,000 population Recognition important: Counseling for pregnancy and
o Right pulmonary interruption: 33% travel to high altitude
o Left pulmonary interruption: 66% Image Interpretation Pearls
Associated abnormalities
Unilateral expiratory air trapping suggests Swyer-James
o Right interruption associated congenital anomalies
rather than congenital interruption pulmonary artery
less frequent
Group 1: Associated left-to-right shunt (typically
patent ductus arteriosus)
I SElECTED REFERENCES
Group 2: Associated with pulmonary hypertension
Group 3: Isolated anomaly 1. Welch Ket al: Isolated unilateral absence of right proximal
o Left interruption higher incidence of congenital pulmonary artery: surgical repair and follow-up. Ann
Thorac Surg. 79(4):1399-402, 2005
cardiovascular anomalies
2. Dogan R et al: About unilateral absence of intrapericardial
Tetralogy of Fallot pulmonary artery. J Cardiovasc Surg (Torino). 45(1):81-4,
Septal defects 2004
Coarctation of the aorta 3. Ten Harkel AD et al: Isolated unilateral absence of a
Transposition of the great arteries pulmonary artery: a case report and review of the
literature. Chest. 122(4):1471-7,2002
Gross Pathologic & Surgical Features 4. Chow B et al: Unilateral absence of pulmonary perfusion
Systemic collaterals mimicking pulmonary embolism. AJRAm J Roentgenol.
o Bronchial, intercostal, internal mammary, long 176(3):712,2001
thoracic, and subdiaphragmatic arteries 5. Maeda S et al: Isolated unilateral absence of a pulmonary
artery: influence of systemic circulation on alveolar
capillary vessels. Pathollnt. 51(8):649-53, 2001
6. Vergauwen S et al: Unilateral absence of a pulmonary
I CLINICAL ISSUES artery. J Beige Radiol. 81(5):254, 1998
7. Baran R et al: Congenital isolated unilateral absence of
Presentation right pulmonary artery. Thorac Cardiovasc Surg.
Most common signs/symptoms 41(6):374-6,1993
o Recurrent pulmonary infections in 33% 8. Rios B et al: High-altitude pulmonary edema with absent
o Dyspnea 40% right pulmonary artery. Pediatrics. 75(2):314-7,1985
o Hemoptysis 20%
o Asymptomatic 15%
Other signs/symptoms
o Pulmonary hypertension 20%
o May be discovered when develop high altitude
pulmonary edema or pulmonary hypertension
during pregnancy
Demographics
Age: 15 years at diagnosis, adults may have time delay
of more than 30 years
 CONGENITAL INTERRUPTION PULMONARY ARTERY

I IMAGE GALLERY

Typical
(Leh) Frontal angiography
shows enlarged right
pulmonary artery (rom the
initial case. Left pulmonary
artery is absent. (Right) Axial
CECT shows interruption of
the left pulmonary artery.
Right pulmonary artery is
slightly enlarged. Collateral
supply through bronchial
arteries (arrow) and enlarged
left internal mammary artery
(curved arrow).

4
19
(Leh) Frontal radiograph
shows small right hemithorax
and hyperinflation of the left
hemithorax. Right hilum is
small. Left aortic arch. Fine
reticular peripheral interstitia!
thickening right lung.
Congenital interruption right
pulmonary artery. (Right)
Frontal angiography shows
enlarged subdiaphragmatic
vessels supplying the right
lung.

(Leh) Frontal radiograph

shows small right
hemithorax, hyperinflation of
the left hemithorax with
herniation into the right
hemithorax. Small right
hilum. Left aortic arch.
(Right) Axial MR cine shows
normal main and left
pulmonary artery. Congenital
interruption right pulmonary
artery.
 ANOMALOUS ORIGIN LEFT PULMONARY ARTERY

Axial TI WI MR shows origin of the left PA from the Axial TI WI MR shows the anomalous pulmonary vessel
distal main right pulmonary artery (open arrow) and the coursing between the trachea (arrow) and esophagus
vessel coursing obliquely (arrow) to the left lung. (open arrow). (Courtesy L. Haramati, MO).
4 (Courtesy L. Haramati, MO).

20
I TERMINOlOGY Radiographic Findings
Radiography
Abbreviations and Synonyms o Leftward deviation and right-sided compression of
Pulmonary artery sling lower trachea
Anomalous pulmonary artery (PA) o Hyperinflation of right lung or right middle and
Aberrant left pulmonary artery lower lobe
Definitions o On lateral, density due to anomalous left pulmonary
Anomalous origin of left pulmonary artery from artery between trachea and esophagus
posterior aspect of right pulmonary artery CT Findings
Causes compression of trachea and right main
CECT
bronchus o Vessel arising from distal main right PA in right
Associated with complete tracheal rings, mediastinum
tracheomalacia, tracheal hypoplasia o Courses leftward and passes between trachea and
esophagus
o Extends into left lung with normal lobar branching
[IMAGING FINDINGS o No normal connection between main pulmonary
General Features artery and left lung
o Hyperinflation of right lung or right middle and
Best diagnostic clue: Vessel coursing between trachea
lower lobe
and esophagus
o Complete tracheal rings and tracheomalacia
Location: Middle mediastinum
Size: Left pulmonary artery size may be slightly MR Findings
decreased TlWl

DDx: Pulmonary Sling Mimics

Double Arch Interrupted Inferior Vena Cava Absent Left Pulmonary Artery
 ANOMALOUS ORIGIN LEFT PULMONARY ARTERY

Key Facts
Terminology Imaging Findings
Anomalous origin of left pulmonary artery from Best diagnostic clue: Vessel coursing between trachea
posterior aspect of right pulmonary artery and esophagus
Causes compression of trachea and right main
Top Differential Diagnoses
bronchus
Associated with complete tracheal rings, Vascular Ring
tracheomalacia, tracheal hypoplasia Mediastinal Mass
Absent Left Pulmonary Artery

o Vessel arising from distal main right PA IClINICAllSSUES

o Courses leftward passing between trachea and
esophagus Presentation
Fluoroscopic Findings Most common signs/symptoms
o Majority have respiratory symptoms
Upper Gl Stridor most common, occasional apnea
o Anterior impression of midesophagus
o Rightward deviation of esophagus Wheezing
Recurrent pneumonias
4
Other signs/symptoms: Dysphagia, failure to thrive 21
I DIFFERENTIAL DIAGNOSIS Demographics
Age: Two-thirds present on first day of life
Vascular Ring
Gender: No gender predilection
Location posterior to esophagus, not between
esophagus and trachea Treatment
Surgical ligation of anomalous vessel with
Mediastinal Mass
reanastomosis in normal location
Rarely localized to tracheo-esophageal groove
Tracheoplasty may be required
Absent Left Pulmonary Artery
Hemithorax small with small hilum
I SElECTED REFERENCES
1. Fiore AC et al: Surgical treatment of pulmonary artery sling
[PATHOLOGY and tracheal stenosis. Ann Thorac Surg. 79(1):38-46;
discussion 38-46, 2005
General Features 2. Berdon WE: Rings, slings, and other things: vascular
Etiology: Embryonic regression of normal left compression of the infant trachea updated from the
pulmonary artery with development from a right midcentury to the millennium--the legacy of Robert E.
Gross, MD, and Edward B. D. Neuhauser, MD. Radiology.
pulmonary artery collateral vessel 216(3):624-32,2000
Associated abnormalities: Assorted congenital heart 3. ROSENBERGBFet al: Anomalous course of left pulmonary
lesions in 15% artery with respiratory obstruction. Radiology.
67(3):339-45,1956

I IMAGE GAllERY

(Left) Axial CECT shows the left PA arising (arrow) from the right PA and coursing between trachea and esophagus. Patient has metastatic giant
cell tumor (open arrow). (Center) Axial CECT shows a more inferior section with the pulmonary sling (open arrow). (Right) Axial CECT shows
the anomalous pulmonary artery (arrow) coursing toward the left lung.
 SEPTIC EMBOLI, PULMONARY

Frontal radiograph shows multiple cavitary lesions and Axial CECT in the same patient shows the focal area of
wedge-shaped opaciUes throughout the lungs. A more cavitalion as well as another smaller cavitary lesion in
well-defined area of cavitaUon is seen in the left upper this patient with septic emboli. (Courtesy J. Speckman,
4 lobe (arrows). MO).

22
o 1-3 cm peripheral poorly marginated nodular or
I TERMI NOLOGY wedge-shaped opacities
Definitions May change in number or appearance (size or
Septic emboli degree of cavitation) from day to day
o Source: Tricuspid endocarditis, indwelling catheters, o Usually basilar (due to gravity and blood flow)
pacemaker wires o Evolve rapidly, cavitation common (50%)
o Associated with IV drug abusers, alcoholism, skin Cavity wall often thick
infection, peripheral septic thrombophlebitis Lack air-fluid level
o Peripheral wedge-shaped opacities from infarcts o Often complicated by empyema
o Rapidly evolve and cavitate CT Findings
CECT
o Multiple discrete nodules with varying degrees of
I IMAGING FINDINGS cavitation
General Features Air bronchograms (25%)
Subpleural, wedge-shaped areas of increased
Best diagnostic clue: Multiple patchy areas of
attenuation with rim-like peripheral enhancement
consolidation rapidly evolving into cavitary nodules
o Vessel may be seen leading directly to the nodules
Location: Primarily lung bases
Feeding vessel sign, found in 60-70% of patients
Size: Usually small 3 cm diameter)
o No intravascular clots
Morphology: Ill-defined nodules with rapid cavitation
o Pleural effusion, may be loculated in empyema
Radiographic Findings
Radiography

DDx: Cavitary lesions

Pneumonia Lung Cancer Pulmonary Infarct

 SEPTIC EMBOLI, PULMONARY

Key Facts
Terminology Often complicated by empyema
Subpleural, wedge-shaped areas of increased
Source: Tricuspid endocarditis, indwelling catheters,
attenuation with rim-like peripheral enhancement
pacemaker wires
Vessel may be seen leading directly to the nodules
Associated with IV drug abusers, alcoholism, skin
infection, peripheral septic thrombophlebitis Top Differential Diagnoses
Peripheral wedge-shaped opacities from infarcts Thromboemboli
Imaging Findings Pneumonia
Best diagnostic clue: Multiple patchy areas of Metastases
consolidation rapidly evolving into cavitary nodules Foreign Body Embolus
1-3 cm peripheral poorly marginated nodular or Pathology
wedge-shaped opacities Staphylococcus aureus most common organism
May change in number or appearance (size or degree
of cavitation) from day to day Clinical Issues
Usually basilar (due to gravity and blood flow) Radiographic abnormalities may precede positive
Cavity wall often thick blood cultures
Lack air-fluid level

4
Imaging Recommendations Foreign Body Embolus
Best imaging tool: Chest radiographs suggestive, CT Catheter fragment may be seen 23
useful in characterizing septic emboli and extent of CT helpful for visualization of foreign body
disease
Protocol advice: CT: More sensitive than chest
Iodinated Oil Embolism
radiographs for both lung and pleural disease Can occur after transcatheter oil chemoembolization
for hepatocellular carcinoma
o Diffuse bilateral foci of consolidation appear within
I DIFFERENTIAL DIAGNOSIS 2-5 days of chemoembolization
Seen previously with lymphangiography
Thromboemboli o Subtle fine nodular areas of increased opacity
Focal areas of increased opacity, linear atelectasis, Hydatid Embolism
pleural effusion
Rare complication of cardiac or hepatic echinococcosis
Decreased vascularity in peripheral lung, Westermark
Occlusion of pulmonary arteries and branches by
sign
cystic lesions (daughter cysts)
Pleural-based areas of increased opacity, Hampton sign
Enlargement of central pulmonary artery, Fleischner Fat Embolism
sign Com plication of long bone fracture
CTA shows filling defect in pulmonary artery Free fatty acids may initiate toxic reaction in
Pulmonary infarction may cavitate endothelium vs. obstruction of the pulmonary
o Central necrosis with hemorrhage vasculature by fat globules, red blood cells and
Rare for venous emboli to cavitate platelets
Pneumonia Findings of acute respiratory distress syndrome rather
than cavitary nodules
Bacterial
o Staphylococcus, Streptococcus, gram-negatives Tumor Embolism
o Area of consolidation may be solitary or multiple Seen with hepatocellular carcinoma, breast, renal cell
o Associated empyema may also develop carcinoma, gastric, prostate and choriocarcinoma
Fungal Radiologic findings are often minimal or nonspecific
o Aspergillosis, candida common Focal or diffuse heterogeneous areas of increased
o lmmunocompromised host opacity
Actinomycosis May have "tree-in-bud" appearance
o Often associated with chest wall invasion Large emboli more rare
Metastases Other Embolic Disease
May also cavitate Venous air embolism, amniotic fluid embolism
o Squamous cell carcinoma or sarcomas o Radiographically may have edema pattern
Do not rapidly evolve Talc embolism
Wegener Granulomatosis Cement (Polymethylmethacrylate) embolism
Usually radiographically distinct; small vessels
Nodules with varying degrees of cavitation
affected, small nodules
Do not rapidly evolve
o Nodules rarely cavitate
May have subglottic stenosis
 SEPTIC EMBOLI, PULMONARY
I PATHOLOGY
General Features
General path comments
o Septic emboli occur from infected embolic material
o Infective endocarditis
Occurs as result of non bacterial thrombotic
endocarditis, with injury to endothelial surface of
the heart
Transient bacteremia leads to seeding of lesions
with adherent bacteria
Subsequent infective endocarditis develops
Etiology
o In IV drug abusers: Staphylococcus aureus is most
common 50% of cases)
o Other organisms include Streptococci, fungi,
gram-negative rods (Pseudomonas, Serratia)
o Tricuspid valve affected most commonly, aortic
valve may also be involved
o Other types of endocarditis
4 Prosthetic valve endocarditis
Native valve endocarditis: Rheumatic valvular
24 disease, congenital heart disease, mitral valve
prolapse with an associated murmur, degenerative
heart disease
Fungal endocarditis: leu patients on
broad-spectrum antibiotics as well as IV drug users
Epidemiology
o Source septic emboli
Indwelling venous catheters
Tricuspid valve endocarditis in IV dpug abusers
Also associated with alcoholism, skin infection,
peripheral septic thrombophlebitis
Rarely pacemaker wires
Immunologic deficiencies, particulariy lymphoma,
organ transplants
o Staphylococcus aureus most common organism
Associated abnormalities
o Also complicating endocarditis: Myocardial
infarction, pericarditis, cardiac arrhythmia, cardiac
valvular insufficiency, congestive heart failure, Sinus
of Valsalva aneurysm, aortic root or myocardial
abscesses
o Mycotic aneurysms, arthritis, myositis,
glomerulonephritis, acute renal failure, stroke,
mesenteric or splenic abscess or infarct
Gross Pathologic & Surgical Features
Septic emboli: Necrotic infected lung
Microscopic Features
No specific features; inflammatory response, necrosis,
may see infective organism
[CLINICAL ISSUES
Presentation
Most common signs/symptoms: Fever, cough, and
hemoptysis
Other signs/symptoms
o With endocarditis: Petechiae, splinter hemorrhages
(dark red linear lesions in the nailbeds)
Osler nodes (tender subcutaneous nodules usually
found on distal pads of digits)
Janeway lesions (nontender maculae on palms
and soles)
Roth spots (retinal hemorrhages with small, clear
centers)
o Lemierre syndrome
Postanginal sepsis or necrobacillosis, uncommon
but potentially life-threatening complication of
acute pharyngotonsillitis from spread of infection
to parapharyngeal space
Infection of the para pharyngeal space
Upper respiratory infection, immunocompetent
host
Anaerobic infection
Septic phlebitis jugular vein
Septic emboli
Adult respiratory distress syndrome (ARDS)
Natural History & Prognosis
Radiographic abnormalities may precede positive
blood cultures
Often rupture into pleural space and result in
empyema
Treatment
Therapy with broad spectrum antibiotics, long term
with endocarditis
Percutaneous drainage for associated empyema
Surgery
o To remove infected source
o To replace heart valves
I SElECTED REFERENCES
1. Aslam AFet al: Staphylococcus aureus infective
endocarditis and septic pulmonary embolism after septic
abortion. IntJ Cardiol. 105(2):233-5,2005
2. Cook RJet al: Septic pulmonary embolism: presenting
features and clinical course of 14 patients. Chest.
128(1):162-6,2005
3. Goldenberg NA et ai: Lemierre's and Lemierre's-like
syndromes in children: survival and thromboembolic
outcomes. Pediatrics. 116(4):e543-8, 2005
4. Parambil JG et al: Causes and presenting features of
pulmonary infarctions in 43 cases identified by surgical
lung biopsy. Chest. 127(4):1178-83,2005
5. Gormus N et al: Lemierre's syndrome associated with septic
pulmonary embolism: a case report. Ann VaseSurg.
18(2):243-5,2004
6. Wittram C et al: CT angiography of pulmonary embolism:
diagnostic criteria and causes of misdiagnosis.
Radiographies. 24(5):1219-38, 2004
7. Han D et al: Thrombotic and nonthrombotic pulmonary
arterial embolism: spectrum of imaging findings.
Radiographies. 23(6):152]-39,2003
8. Wong KSet al: Clinical and radiographic spectrum of
septic pulmonary embolism. Arch Dis Child. 87(4):312-5,
2002
9. Lioulias A et al: Acute pulmonary embolism due to
multiple hydatid cysts. EurJ Cardiothorac Surg. 20: 197-9,
2001
10. RossiSEet al: Nonthrombotic pulmonary emboli. AJR.
174:1499-508,2000
11. Screaton NJ et al: Lemierre Syndrome: Forgotten but Not
Extinct-Report of Four Cases. Radiology, 213(2): 369 - 74,
1999
 SEPTIC EMBOLI, PULMONARY
I IMAGE GALLERY

(Left) Axial NECT shows

multiple small ground-glass
opacities, some with central
cavitation (arrow). The
diffuse septic embolic
disease in this patient is
secondary to a tricuspid
valve vegetation. (Right)
Axial NECT at a lower level
shows multiple small
airspace opacities with a
ground-glass appearance
(arrows) from septic emboli
as well as a peripheral
wedge-shaped opacity
(curved arrow).

4
25
(Left) Axial NECT shows
ill-defined peripheral
wedge-shaped opacities with
surrounding ground-glass
changes. This patient had a
history of tricuspid valve
vegetation with emboli to the
lungs. (Right) Axial NECT at
a lower level shows a very
typical area of peripheral
wedge-shaped opacity
consistent with embolic
disease, as can be seen with
bland or septic emboli.

(Left) Axial CECT shows a

less typical appearance of
septic embolus with findings
of a well-defined
non-cavitating nodular
opacity. Imaging at other
levels did show additional
small cavitary lesions. (Right)
Ax~/CECTinthesame
patient shows a small cavity
in the superior segment or
the right lower lobe
(arrows), as well as a cavity
adjacent to the right upper
bronchus (curved arrow).
 VASCULITIS, PULMONARY

Axial HRCT shows peripheral airspace opacity in the left Axial HRCT shows peripheral airspace disease and
upper lobe and both lower lobes. Patient diagnosed consolidation and hazy density in the anterobasal
with Churg-Strausssyndrome. segment of the right lower lobe. Patient diagnosed with
Churg-Strausssyndrome.

26 Large vessel vasculitis of aorta and occasionally

ITERMINOLOGY pulmonary arteries
Abbreviations and Synonyms Granulomatous inflammation
Small vessel vasculitis
Definitions IIMAGING FINDINGS
Pulmonary vasculitis often part of a systemic small or
large vessel vasculitis General Features
Affects pulmonary arteries, capillaries, or veins Best diagnostic clue: Ground-glass opacity on
Spectrum includes Wegener granulomatosis, high-resolution CT
Goodpasture disease and Churg-Strauss syndrome, Radiographic Findings
microscopic polyangiitis, Behcet disease, and Takayasu
Radiography
disease
o Imaging features nonspecific
o Churg-Strauss syndrome (allergic granulomatosis)
o Hazy opacity is typical due to pulmonary
Asthmatics with eosinophilia
hemorrhage
Medium and small vessel vasculitis
o Large vessel vasculitis in Behcet disease or Takayasu
o Microscopic polyangiitis (MPA)
disease associated with pulmonary artery aneurysms
Affects kidneys, peripheral nerves, skin, and lungs
10-15% of patients develop alveolar hemorrhage CT Findings
o Behcet disease CECT
Widespread involvement of large and small vessels o Behcet disease may show pulmonary artery
Pulmonary aneurysms that may hemorrhage aneurysms
o Takayasu disease o Takayasu disease may demonstrate irregularity and
beading of large pulmonary arteries

DDx: Vasculitis Mimics

Pneumocystis Cryptogenic Organizing Pneumonia Pulmonary Edema

 VASCULITIS, PULMONARY
Key Facts
Terminology Imaging Findings
Pulmonary vasculitis often part of a systemic small or Best diagnostic clue: Ground-glass opacity on
large vessel vasculitis high-resolution CT
Affects pulmonary arteries, capillaries, or veins
Spectrum includes Wegener granulomatosis,
Top Differential Diagnoses
Goodpasture disease and Churg-Strauss syndrome, Diffuse Pulmonary Infection
microscopic polyangiitis, Behcet disease, and Cryptogenic Organizing Pneumonia
Takayasu disease

HRCT Gross Pathologic & Surgical Features

o Ground-glass opacity or consolidation without lobar
predilection due to hemorrhage Churg-Strauss: Granulomatous lesions
o III-defined centrilobular nodules due to Microscopic Features
hemosiderin-laden macrophages Inflammatory cells in walls of small arteries with
Imaging Recommendations variable thrombosis
Best imaging tool: HRCT to characterize lung Churg-Strauss: Eosinophilia 4
27
I DIFFERENTIAL DIAGNOSIS I CLINICAL ISSUES
Diffuse Pulmonary Infection Presentation
Often considered in differential diagnosis of focal or Most common signs/symptoms
diffuse ground-glass opacities o Nonspecific shortness of breath or hemoptysis
Requires culture to exclude o Sedimentation rate often elevated

Cryptogenic Organizing Pneumonia Demographics

Identical radiograph findings Age: Any age
Opacities may be migratory Gender: Takayasu disease much more common in
women
Treatment
I PATHOLOGY Steroids and cytotoxic agents
General Features
Etiology: Unknown: May be autoimmune or response
to infection or an environmental insult
I SELECTED REFERENCES
Epidemiology 1. Ravenel JG et al: Pulmonary vasculitis: CT features. Semin
o Behcet disease common in Turkey and Asia Respir Crit Care Med. 24(4):427-36, 2003
2. Hansell OM: Small-vessel diseases of the lung:
o Takayasu disease common in Asia
CT-pathologic correlates. Radiology. 225(3):639-53, 2002
3. Burns A: Pulmonary vasculitis. Thorax. 53(3):220-7,1998

I IMAGE GALLERY

(Left) Frontal radiograph shows diffuse fairly symmetric airspace disease. Patient diagnosed with lupus vasculitis. (Center) Axial HRCT shows two
areas of airspace disease and other scattered poorly-defined nodules. Patient diagnosed with microscopic polyangiitis. (Right) Axial HRCT shows
two predominant areas of patchy airspace disease. Patient diagnosed with microscopic polyangiitis.
 WEGENER GRANULOMATOSIS, PULMONARY

Frontal radiograph shows multiple bilateral lung Axial CECT demonstrates marked subglottic mucosal
nodules, including some cavitary nodules (arrows), in a irregularity & soft tissue thickening (arrows) in a woman
man with Wegener granulomatosis. with Wegener granulomatosis. Airway involvement is
4 more common in women.

28 Morphology: Characteristic lesion: Organized

ITERMINOlOGY palisading granuloma
Abbreviations and Synonyms Radiographic Findings
Circulating anti neutrophil cytoplasmic antibodies Single or multiple nodules
(c-ANCA) o Sharp or ill-defined from surrounding hemorrhage
Wegener granulomatosis (WG) o 50% of lung nodules & masses cavitate
Definitions o Cavities frequently thick-walled, but can be
Necrotizing granulomatous vasculitis of thin-walled
small-to-medium vessels without associated infection o Rapid enlargement suggests hemorrhage or
superinfection
o Variable size, can coalesce into large masses
IIMAGING FINDINGS o Can be unilateral: 15%, solitary: 25%
o When multiple usually < 10 in number
General Features o Can regress spontaneously
Best diagnostic clue: Multiple cavitary lung nodules & Focal or multi focal consolidation can also cavitate
large airway narrowing Diffuse consolidation from hemorrhage
Location Patterns: Cavitary nodules, focal consolidation &
o Focal airway involvement most common in diffuse airspace disease from hemorrhage
subglottic area: 20% Peripheral airway stenosis can result in segmental or
o Lung nodules tend to be bronchocentric or lobar atelectasis
subpleural & peripheral Subglottic stenosis often visible, but overlooked on
Size: Nodules range in size up to 10 cm chest radiography
Pleural effusion: 20%, pneumothorax rare

DDx: Cavitating Lung Masses

Bronchi%a/veo/ar Carcinoma Candida Lung Abscess Haemophi/us Lung Abscess

 WEGENER GRANULOMATOSIS, PULMONARY

Key Facts
Imaging Findings Pathology
Best diagnostic clue: Multiple cavitary lung nodules & Etiology: Unknown, suspect inhaled antigen
large airway narrowing Necrotizing vasculitis & granulomatosis in lung
50% of lung nodules & masses cavitate nodules, associated hemorrhage common
Focal or multifocal consolidation can also cavitate Subglottic stenosis, tracheobronchial stenosis &
Patterns: Cavitary nodules, focal consolidation & ulcerating tracheobronchitis
diffuse airspace disease from hemorrhage Focal necrotizing glomerulonephritis
Subglottic stenosis often visible, but overlooked on
Clinical Issues
chest radiography
Pleural effusion: 20%, pneumothorax rare Most common signs/symptoms: Sinusitis, rhinitis &
otitis media
Top Differential Diagnoses Classic Wegener triad: Sinus, lung & renal disease
Bacterial, Fungal & Tuberculosis Infection Renal failure: Most common cause of death
Metastatic Disease With systemic glucocorticoids & cyclophosphamide
Septic Emboli Survival at 24 months: 80%
Rheumatoid Necrobiotic Nodules Disease relapse 50%, drug side effects 42%
Pulmonary-Renal Syndromes

Hilar or mediastinal adenopathy uncommon 4

Metastatic Disease
Post-therapy
o Parenchymal findings should start to clear within 1 Squamous cell carcinoma or sarcoma metastases often 29
week cavitate
o If no improvement, suspect superinfection Septic Emboli
o Complete normalization averages 1 month (2-6 Nodules evolve rapidly, blood culture positive
weeks)
Relapse Rheumatoid Necrobiotic Nodules
o Appearance of pulmonary relapse different in 23% History of joint disease
o Relapse involves airways more often
Pulmonary-Renal Syndromes
CT Findings Microscopic polyangiitis
Like metastases, nodules can have feeding vessels o No necrotizing granulomatous inflammation
Peripheral wedge-shaped consolidation from infarct o Antineutrophil cytoplasmic antibody vasculitis
Some nodules have CT halo sign from surrounding Churg-Strauss syndrome
hemorrhage o Peripheral blood eosinophilia> 100A,& asthma
Trachea & bronchi concentrically thickened, either o Antineutrophil cytoplasmic antibody vasculitis
focal or long segments Goodpasture syndrome
o Anti-glomerular basement membrane antibodies
Echocardiographic Findings o Pulmonary hemorrhage & glomerulonephritis
Echocardiographic abnormalities related to WG: 36% Polyarteritis nodosa
o Pericardia I effusion: 19% o Renal infarcts, renal arterial disease, lung
Imaging Recommendations involvement much less common than WG
Best imaging tool: CT is more sensitive, particularly for Lymphomatoid granulomatosis (B-celllymphoma)
evaluating possible airway involvement o Central or peripheral nerve involvement, skin
disease
Protocol advice
o Conventional CT protocols are usually sufficient o Multiple cavitary lung nodules indistinguishable
o Including glottis is helpful, because of frequent from WG
subglottic involvement Systemic lupus erythematosus
o Multiplanar reconstructions are particularly useful o Autoimmune disorder characterized by antinuclear
for evaluating airways antibodies
o Pulmonary hemorrhage & serositis

I DIFFERENTIAL DIAGNOSIS
I PATHOLOGY
Bacterial, Fungal & Tuberculosis Infection
General Features
Radiographic findings can be indistinguishable
Differentiate by culture or special stains Genetics: Complex, multifactorial genetic basis
Etiology: Unknown, suspect inhaled antigen
Primary Bronchogenic Carcinoma Epidemiology: Prevalence 3:100,000 in US
Squamous cell carcinoma most likely to cavitate
 WEGENER GRANULOMATOSIS,
Gross Pathologic & Surgical Features
Necrotizing vasculitis & granulomatosis in lung
nodules, associated hemorrhage common
Subglottic stenosis, tracheobronchial stenosis &
ulcerating tracheobronchitis
Focal necrotizing glomerulonephritis
Microscopic Features
Necrotizing vasculitis of small-to-medium vessels,
necrotizing granulomatosis & hemorrhage
Staging, Grading or Classification Criteria
ELK classification
o Ear, nose, throat
o Lung
o Kidney
Diagnosis of WG by
o Involvement of ~ 1 of ELK classification locations
o Confirmatory pathological findings
American College of Rheumatology criteria
4 o Abnormal urinary sediment (red cell casts or > 5 red
blood cells per high power field)
30 o Nodules, cavities or fixed infiltrates on chest
radiography
o Oral ulcers or nasal discharge
o Granulomatous inflammation on biopsy
If patients have ~ 2 of these criteria
o Diagnosis of WG confirmed with sensitivity of 88%
& specificity of 92%
IClINICAL ISSUES
Presentation
Most common signs/symptoms: Sinusitis, rhinitis &
otitis media
Other signs/symptoms
o Saddle-node deformity & perforation of nasal
septum
o Voice changes & stridor
o Hearing loss, vertigo & chondritis
o Cough, fever, night sweats, dyspnea, wheezing,
hemoptysis & chest pain
o Proteinuria, microscopic hematuria & t serum
creatinine
o Myalgias, joint pain & swelling
o Conjunctivitis, scleritis, episcleritis & orbital
pseudotumor
o Palpable purpura, Raynaud phenomenon
Classic Wegener triad: Sinus, lung & renal disease
However, WG can affect any part of body
Frequency of systemic involvement during disease
course
o Upper airways, 92%; lower airways, 85%; renal, 80%;
joints, 67%; eye, 52%; skin, 46%; & nerve, 20%
o Prevalence of organ involvement progresses after
diagnosis, particularly for glomerulonephritis
Limited WG: Lung only, usually evolves into systemic
disease
Circulating antineutrophil cytoplasmic antibodies
(c-ANCA)
o In WG patients c-ANCA is usually directed against
proteinase-3, an antigen found in neutrophils
PULMONARY
o 70-90% of patients with active WG have positive
c-ANCA
o Positive c-ANCA supports diagnosis, but is neither
necessary nor sufficient
o Therapy for WG is very toxic, so c-ANCA levels
should not replace histologic confirmation
Nasal, paranasal sinus, lung or renal biopsy for
diagnosis
Demographics
Age: Any age, mean age at diagnosis 40-55 years
Gender
oM = F
o Airway involvement much more common in
women
Ethnicity: 80-97% Caucasian, 2-8% African-American
Natural History & Prognosis
Renal failure: Most common cause of death
Subglottic stenosis occurs later in course of disease
Median survival without treatment: 5 months
With systemic glucocorticoids & cyclophosphamide
o Survival at 24 months: 80%
o Disease relapse 50%, drug side effects 42%
Treatment
Systemic involvement
o Systemic glucocorticoids & cyclophosphamide
o Treatment with cyclophosphamide
t Risk of transitional cell carcinoma
t Risk of myeloproliferative disorder
Less extensive involvement or cyclophosphamide
toxicity
o Systemic glucocorticoids & cytotoxic agents
Trimethoprim-sulfamethoxazole prophylaxis for t
risk of Pneumocystis pneumonia
Tracheobronchial disease can require stent placement
I SELECTED REFERENCES
1. Jagiello P et al: Complex genetics of Wegener
granulomatosis. Autoimmun Rev.4(1):42-7, 2005
2. Oliveira GH et al: Echocardiographic findings in patients
with Wegener granulomatosis. Mayo Clin Proc.
80(11):]435-40,2005
3. Langford CA et al: Rare diseases.3: Wegener's
granulomatosis. Thorax. 54(7):629-37, ]999
4. Frazier AAet al: Pulmonary angiitis and granulomatosis:
radiologic-pathologic correlation. Radiographics.
18(3):687-710; quiz 727,1998
5. Screaton NJ et al: Tracheal involvement in Wegener's
granulomatosis: evaluation using spiral CT. Clin Radiol.
53(11):809-] 5, ]998
6. Daum TE et al: Tracheobronchial involvement in
Wegener's granulomatosis. Am J Respir Crit Care Med.
]51(2 Pt 1):522-6, 1995
7. DeRemee RA:Wegener's granulomatosis. Curr Opin Pulm
Med. 1(5):363-7, 1995
8. Hoffman GS et al: Wegener granulomatosis: an analysis of
]58 patients. Ann Intern Med. 116(6):488-98, 1992
9. Aberle DR et al: Thoracic manifestations of Wegener
granulomatosis: diagnosis and course. Radiology. 174(3 Pt
1):703-9, 1990
10. Leavitt RYet al: The American College of Rheumatology
1990 criteria for the classification of Wegener's
granulomatosis. Arthritis Rheum. 33(8):1101-7,1990
 WEGENER GRANULOMATOSIS, PULMONARY

I IMAGE GALLERY

(Left) Frontal radiograph

shows bilateral thin-walled
lung cavities (arrows). A
fluid level is visible in a right
lower lobe cavity (curved
arrow). (Right) Axial CECT
shows a solid nodule (arrow)
in superior segment of left
lower lobe & a cavitating left
upper lobe mass (curved
arrow) in a patient with
Wegener granulomatosis.

4
Typical 31
(Left) Axial CECT shows
cavitating nodule in right
middle lobe, right lower lobe
consolidation &
wedge-shaped consolidation
in left lung. Halo sign
(arrows) is seen around
lower lobe consolidation.
(Right) Axial CECT shows
predominantly peripheral
ground-glass opacities,
consistent with acute
hemorrhage, in a patient
with Wegener
granulomatosis.

Variant
(Left) Coronal CECT shows
thickening of trachea &
bronchus intermedius
(arrows). Right upper lobe
bronchus (curved arrow) is
narrow & irregular. A stent
(open arrows) is seen in left
main bronchus. (Right) Axial
CECT demonstrates a small
pericardia I effusion (arrow)
& a moderate left pleural
effusion (open arrow) in a
patient with Wegener
granulomatosis.
 VENO-OCCLUSIVE DISEASE, PULMONARY

Frontal radiograph shows enlarged cen/Jal pulmonary Frontal radiograph shows subtle Kerley B lines (arrows).
arteries (arrows) (rom pulmonary arterial hypertension. Seplallines are unusual in pulmonary hypertension and
(Courtesy HP McAdams, MO). suggest pulmonary veno-occlusive disease or
4 pulmonary capillary hemangiomatosis.

32
ITERMINOlOGY CT Findings
Abbreviations and Synonyms HRCT
o Centrilobular ground-glass nodular opacities (85%)
Pulmonary veno-occlusive disease (PVOD), pulmonary o Thickened interlobular septa (60%), smooth or
vaso-occlusive disease, isolated pulmonary venous nodular
sclerosis, obstructive disease of the pulmonary veins, o Pericardial effusion (75%)
venous form of primary pulmonary hypertension o Mild lymphadenopathy, average 12 mm (70%)
Definitions Imaging Recommendations
Rare cause of pulmonary hypertension due primarily Best imaging tool: HRCT important to separate PVOD
to occlusion of post-capillary venous radicles from primary pulmonary hypertension (PPH)

I IMAGING FINDINGS I DIFFERENTIALDIAGNOSIS

General Features Pulmonary Capillary Hemangiomatosis
Best diagnostic clue: Pulmonary arterial hypertension
+ Kerley B lines (PCH)
Identical radiographic findings; some investigators
Radiographic Findings view PCH as sequela of PVOD
Enlarged pulmonary arteries (100%)
Nonspecific basilar interstitial thickening Mediastinal Fibrosis
Cardiomegaly (90%) Major pulmonary veins obstructed by partially
Pleural effusions, small (75%) calcified mass

DDx: PVOD Mimics

Capillary Hemangiomatosis Pulmonary Hypertension Mediastinal Fibrosis

 VENO-OCCLUSIVE DISEASE, PULMONARY

Key Facts
Terminology Top Differential Diagnoses
Rare cause of pulmonary hypertension due primarily Pulmonary Capillary Hemangiomatosis (PC H)
to occlusion of post-capillary venous radicles Mediastinal Fibrosis
Pulmonary Hypertension
Imaging Findings
Best diagnostic clue: Pulmonary arterial hypertension Pathology
+ Kerley B lines Etiologies: Viral infection, chemotherapeutic drugs,
Centrilobular ground-glass nodular opacities (85%) autoimmune diseases, pregnancy, bone marrow
transplantation

Proliferation of thin-walled capillaries ~ capillary

Pulmonary Hypertension hemangiomatosis
Pre-capillary: PPH (young women); Secondary:
Chronic thromboembolism
Findings portending poor response to epoprostenol ICLINICAl ISSUES
(prostacyclin) therapy (more suggestive of PVOD or
PC H) Presentation
o Centrilobular ground-glass opacities, septal lines, Most common signs/symptoms 4
pleural effusions, lymphadenopathy, pericardial o Progressive dyspnea, lethargy and non productive
effusion cough, occasionally preceded by flu-like illness 33
o Normal pulmonary capillary wedge pressure

I PATHOLOGY Demographics
Age: Any age most < 50 years
General Features Gender: M:F = 2:1 in adults but equal in children
Etiology
o Insult that causes thrombosis of pulmonary veins
Natural History & Prognosis
Etiologies: Viral infection, chemotherapeutic Prognosis very poor: Most patients die within 2 years
drugs, autoimmune diseases, pregnancy, bone of diagnosis
marrow transplantation Treatment
Epidemiology: Unknown incidence: PVOD may Single or double-lung transplantation the only therapy
account for up to 10% of cases of PPH capable of significantly prolonging life in PVOD
Gross Pathologic & Surgical Features Therapies such as vasodilators, immunosuppressive
Pathologic hallmark: Extensive occlusion of agents and anticoagulants ineffective
pulmonary veins by fibrous tissue
Microscopic Features I SELECTED REFERENCES
Eccentric intimal thickening of small veins within 1. Resten A et al: Pulmonary hypertension: CT of the chest in
lobular septa; recanalization of occluded veins over pulmonary venoocclusive disease. AJR Am J Roentgenol.
time 183(1):65-70, 2004

I IMAGE GALLERY
 TALCOSIS, PULMONARY

Frontal radiograph shows inferior perihilar masses from Axial HRCT shows that these areas of progressive
talcosis (arrows). Masses not definitely calcified. massive fibrosis are of calcific attenuation (arrows)
Background tiny miliary nodules like sandpaper. Long highly suggesUve of talcosis. Compare to last gallery
4 history of IV drug abuse. image.

34 Size: Nodules pinpoint in size

ITERMINOlOGY Morphology: Pinpoint nodularity
Abbreviations and Synonyms Radiographic Findings
Illicit drug use, simple pneumoconiosis, complicated Radiography
pneumoconiosis, progressive massive fibrosis o Inhalational
Definitions Multiple small or tiny miliary nodules
Four forms: 3 inhalational, 1 intravenous Predominantly upper lung zones
May evolve into progressive massive fibrosis (PMF)
o Inhalation pure talc (talcosis)
o Inhalation talc and silica (talco-silicosis) Lower zone reticular opacities and pleural changes
in those with asbestos contamination
o Inhalation talc and asbestos (talco-asbestosis)
o Intravenous illicit drug use Enlarged hilar lymph nodes with egg-shell
calcification (especially in silico-talcosis)
o Intravenous
Miliary "pinpoint" nodules
IIMAGING FINDINGS Occasionally lymphadenopathy
General Features PMF in perihilar regions
Best diagnostic clue Calcification in PMF usually not recognized on
o Diffuse fine-granular nodularity with high radiographs
attenuation perihilar conglomeration Emphysema, either centriacinar (upper lung
o Basilar pan lobular emphysema in intravenous zones) or panacinar (lower lung zones)
Ritalin abusers Pulmonary artery hypertension in severe disease
Location: Nodularity diffuse, progressive massive o Talc pleurodesis
fibrosis perihilar, emphysema lower lobes

DDx: Takosis

Silicosis Sarcoidosis Metastatic Calcification

 TALCOSIS, PULMONARY

Key Facts
Terminology Amiodarone Toxicity
Four forms: 3 inhalation ai, 1 intravenous Pathology
Imaging Findings Inhalational: Nodules in lymphatic distribution
(centrilobular and subpleural)
Miliary "pinpoint" nodules
Intravenous: Nodules perivascular distribution
Emphysema, either centriacinar (upper lung zones) or
(centrilobular) with occasional tree-in-bud opacity
panacinar (lower lung zones)
Crystals birefringent under polarized light
PMF in perihilar distribution and of high attenuation
(highly suggestive of talcosis) Clinical Issues
Top Differential Diagnoses Natural progression from simple nodularity to
progressive massive fibrosis similar to silicosis
Sarcoidosis No specific treatment for pneumoconiosis available
Metastatic Pulmonary Calcification
Silicosis Diagnostic Checklist
Cellulose Granulomatosis Intravenous talcosis for PMF of calcific density
Amyloidosis Diffuse nodularity, perihilar PMF, and basilar
Neurofibromatosis em h sema hi hi su estive of Ritalin abuse

Often used for pleurodesis due to the intense

4
inflammation induced by talc
I DIFFERENTIAL DIAGNOSIS
35
Pleural thickened with irregular deposits of Sarcoidosis
calcification in the most dependent lung (dorsal) No occupational exposure, PMF less likely
due to settling of talc in supine position Nodules usually larger and tend to cluster (galaxy sign)
CT Findings Peribronchovascular distribution of nodules
NECT Metastatic Pulmonary Calcification
o Progressive massive fibrosis may be of high No PMF
attenuation Emphysema if present admixed with ground-glass
Highly suggestive of talcosis opacities or consolidation
HRCT Centrilobular nodules larger and mulberry shape and
o Inhalational tend to cluster
Centrilobular and subpleural nodules, may calcify Predominantly disease of upper lung zones
Aggregation of nodules into PMF (identical to
silicosis) Silicosis
Architectural distortion adjacent to PMF Occupational history
Pleural and diaphragmatic plaques identical to Nodules tend to be larger than talc
those froin asbestos PMF usually more cephalad in upper lung zones and
Pleural thickening can sometimes be dramatic not high in attenuation
o Intravenous Talc and silica may be admixed together
Emphysema may be upper lung zone or
predominantly lower lung zone (even in the Cellulose Granulomatosis
absence of smoking) Cellulose filler in oral medications
Methylphenidate (Ritalin) especial proclivity for Cellulose particles trapped in arterioles leading to
severe lower lobe panacinar emphysema granulomatous reaction
Ritalin may result in severe lower lung zone HRCT: Centrilobular nodules and tree-in-bud pattern
panacinar emphysema alone without nodularity No PMF
Centrilobular (fine granular appearance) nodules Amyloidosis
and tree-In-bud opacities
Also may be related to IV drug abuse
Nodules spare emphysematous lung, but Nodular form: Multiple small scattered pulmonary
otherwise uniform throughout the lung
nodules
Nonspecific ground-glass opacities May calcify but calcification in small nodules rare
PMF in perihilar distribution and of high
attenuation (highly suggestive of talcosis) Neurofibromatosis
PMF superimposed on fine-nodular pattern Upper lobe bullae
Lower lobe reticular interstitial fibrosis
Imaging Recommendations
Not nodular
Best imaging tool: HRCT for characterization of Cutaneous and skeletal stigmata of neurofibromatosis
interstitial lung disease and detection of high
attenuation conglomerate masses Amiodarone Toxicity
Used to treat tachyarrhythmia
Accumulates in lung and liver
 TALCOSIS, PULMONARY
Focal areas of consolidation randomly distributed
Focal lung abnormalities and liver of high attenuation
due to drug which contains 3 iodine molecules
o Pulmonary function tests
Mixed obstruction (from emphysema) and
restriction (from interstitial lung disease)
Severe reduction in diffusion capacity

I PATHOLOGY Demographics
Age
General Features o Middle age and older for inhalational forms
General path comments o Younger men for intravenous form
o Talc: Magnesium silicate Gender: Men both for occupational inhalational forms
o Talc used in paper, plastics, cosmetic, construction, and intravenous drug abuse
rubber, and drug industries
Natural History & Prognosis
o Less fibrogenic than silica and asbestos
Not a known carcinogen
Etiology
o Inhalational Natural progression from simple nodularity to
progressive massive fibrosis similar to silicosis
Occupational exposure in mining, milling,
Slow progression even without further inhalational or
packaging of talc
intravenous exposure
Cosmetic use (talcum powder) common but
disease from inhalation extremely rare Treatment
o Intravenous No specific treatment for pneumoconiosis available
Talc (and cellulose) common filler in oral
4 medication; common drugs: Amphetamines,
Prevention: Respirators in dusty environments, dust
control to reduce ambient dust concentrations
methylphenidate (Ritalin), hydromorphone Removal from work environment or transfer to less
36 (Dilaudid), pentazocine (Talwin), propoxyphene dusty environment
(Darvon) Smoking cessation
Not meant to be ground up and injected Occupational dust control and regulation for
intravenously inhalational forms
Particles trapped in small arterioles leading to Drug treatment for intravenous abusers
infarction, ischemia, granulomatous
inflammation
Reduction of the capillary bed may result in I DIAGNOSTIC CHECKLIST
panacinar emphysema
Epidemiology: Latency period 20 years for inhalational Consider
form Intravenous talcosis for PMF of calcific density
Gross Pathologic & Surgical Features Image Interpretation Pearls
Inhalational: Nodules in lymphatic distribution Diffuse nodularity, perihilar PMF, and basilar
(centrilobular and subpleural) emphysema highly suggestive of Ritalin abuse
Intravenous: Nodules perivascular distribution
(centrilobular) with occasional tree-In-bud opacity
o Subpleural lung tends to be spared I SElECTED REFERENCES
Microscopic Features L Marchiori E et al: Inhalational pulmonary talcosis:
Inhaled and intravenous high-resolution CT findings in 3 patients. J Thorac
Imaging. 19(1):41-4,2004
o Granulomatous interstitial inflammation
2. Han D et al: Thrombotic and non thrombotic pulmonary
o Needle-shaped crystals both free and in arterial embolism: spectrum of imaging findings.
macrophages Radiographies. 23(6):1521-39, 2003
o Crystals birefringent under polarized light 3. Ward S et al: Talcosis associated with IV abuse of oral
Inhaled medications: CT findings. AJR Am J Roentgenol.
o Interstitial fibrosis or poorly-defined fibrotic nodules 174(3):789-93,2000
o Difficult to separate talc from contaminants of silica 4. Stern EJ et al: Panlobular pulmonary emphysema caused by
and asbestos i.v. injection of methylphenidate (Ritalin): findings on
chest radiographs and CT scans. AJR Am J Roentgenol.
162(3):555-60, 1994
5. Padley SP et al: Pulmonary talcosis: CT findings in three
IClINICAllSSUES cases. Radiology. 186(1):125-7, 1993
6. Gibbs AE et al: Talc pneumoconiosis: a pathologic and
Presentation mineralogic study. Hum Pathol. 23(12):1344-54,1992
Most common signs/symptoms 7. Pare JP et al: Long-term follow-up of drug abusers with
o Inhalational intravenous talcosis. Am Rev Respir Dis. 139(1):233-41,
Dry cough and chronic dyspnea progressing to cor 1989
pulmonale in end stage disease
o Intravenous
Progressive dyspnea and COPD
Other signs/symptoms
 TALCOSIS, PULMONARY

IIMAGE GALLERY

(Left) Frontal radiograph

shows basilar emphysema,
mild diffuse nodularity in the
upper lung zones and focal
wedge-shaped perihilar mass
on the left (arrows). Central
arteries are mildly enlarged.
IV drug abuser. (Right) Axial
HRCT shows diffuse
panlobular emphysema
(arrows) with lucent lung
devoid of small blood
vessels. Right middle lobe
relatively spared Highly
consistent with IV Ritalin
abuse.

4
37
(Left) Frontal radiograph
shows marked hilar
distortion with perihilar
masses and volume loss in
the upper lobes (arrows) in
this IV drug abuser. (Right)
Axial CECT shows
homogeneous irregularly
shaped perihilar masses
(arrows) from progressive
massive fibrosis in the same
patient. Few small nodules
adjacent to the right perihilar
mass.

(Left) Axial HRCT shows at

the level of aortic arch.
Diffuse ground-glass
opacities with pinpoint
nodularity throughout the
upper lobes. From the
original patient. (Right) Axial
HRCT shows at the level of
inferior pulmonary veins.
Bilateral PMF (arrows). PMF
surrounded by ground-glass
opacities and numerous
pinpoint nodules.
 IlliCIT DRUG AB...,SE, PULMONARY

Frontal radiograph shows diffuse ground-glass opaciUes Frontal radiograph shows mulUple flame shaped foci
uniformly distributed through out the lung. Intubated. (curved arrows) one of which is cavitated (arrow).
Crack lung. OpaciUes resolved over 48 hours. Septic emboli.
4
38 o Pneumonia and infection
ITERMINOlOGY One of the most common sequelae for pulmonary
Abbreviations and Synonyms disease in illicit drug abuse
Intravenous (IV) General immunosuppression from nutritional
debility
Definitions High rate of AIDS transmission from infected
Typical drugs: Heroin, cocaine, methamphetamine, needles
codeine, methadone, methylphenidate Higher risk for tuberculosis
o Aspiration
Obtundation and stupor places at risk, especially
IIMAGING FINDINGS from opiates
Focal opacities unilateral or bilateral in dependent
General Features lung (especially superior segments lower lobes)
Best diagnostic clue: Young adult in emergency room May lead to lung abscess
with unexplained diffuse or focal lung disease Repeated episodes may eventually result in
bronchiectasis
Radiographic Findings o Pulmonary hemorrhage
Radiography: Multiple radiographic abnormalities Severe diffuse alveolar damage (DAD), most
none of which are specific for illicit drug abuse common with cocaine and crack
Lung Bilateral diffuse nonspecific consolidation, often
o Atelectasis worse in lower lung zones
Variable from subsegmental to lobar o Cardiogenic pulmonary edema
Often associated with drug induced respiratory Myocardial depression, ischemia, and infarction
depression and stupor from vasoconstriction, especially cocaine

DDx: lung Abnormalities Young Adults

Pneumonia Smoke Inhalation Hypersensitivity Pneumonitis

 ILLICIT DRUG ABUSE, PULMONARY

Key Facts
Imaging Findings Pneumothorax and pneumomediastinum
Tracheal stenosis
Best diagnostic clue: Young adult in emergency room
with unexplained diffuse or focal lung disease Top Differential Diagnoses
Atelectasis Pneumonia
Pneumonia and infection Atypical Edema
Aspiration Aspiration
Pulmonary hemorrhage Contusion Blunt Chest Trauma
Cardiogenic pulmonary edema Hypersensitivity Pneumonitis
Noncardiac pulmonary edema ("crack lung", heroin
lung) Pathology
Talcosis Most common complications of IV drug use: Septic
Emphysema emboli, community-acquired pneumonia,
Cryptogenic organizing pneumonia tuberculosis
Septic emboli No specific features for drug induced damage
Pulmonary hypertension
Aortic dissection
Clinical Issues
Mortalitv rate 3-4% per year

Small pleural effusions (pleural effusion Often < 10 in number and < 1 cm in size 4
uncommon in other etiologies) Etiology: Infected needle, endocarditis, tricuspid
39
o Noncardiac pulmonary edema ("crack lung", heroin valve vegetations
lung) o Pulmonary hypertension
Nonspecific consolidation, usually peripheral and Often due to talc granulomatosis
bilateral Some vasoconstrictive drugs may result in acute
Occasionally focal or predominately upper lung hypertension
zones o Aortic dissection
Normal heart size Acute hypertensive crisis, induced most
No pleural effusions commonly from cocaine
Etiology: Direct endothelial damage or neurogenic Pleural
pulmonary edema if intracerebral insult that o Pneumothorax and pneumomediastinum
results in increased intracranial pressure Direct needle laceration when attempting to
Develops within a few hours of intoxication, access neck veins
resolves over 24-48 hours Prolonged valsalva maneuver leading to
Methadone may have prolonged resolution over barotrauma when inhaling crack cocaine
several days (due to prolonged absorption from Airway
the GI tract) o Tracheal stenosis
o Talcosis Long term sequelae of thermal injury from
Develops slowly over time with chronic IV drug smoked cocaine abuse
abuse Skeletal
Fine-micronodular pattern, ground-glass opacities, o Involvement from septicemia and occasionally
panacinar emphysema direct injury
Progressive massive fibrosis (PMF) often lower o Diskitis and vertebral osteomyelitis
lung and of high density Vertebral body collapse and paraspinal mass
o Emphysema o Costochondritis and necrotizing fascitis
Associated with IV drug abuse in younger men o Septic arthritis: Sternoclavicular and
(30-40's) acromioclavicular joints
Often paraseptal type predominantly in upper
Imaging Recommendations
lung zones from IV cocaine or heroin
IV methadone and methylphenidate results in Best imaging tool
predominantly lower lobe panacinar emphysema o Chest radiographs usually suffice for detection and
Frequency up to 2% of chronic IV drug abuse follow-up
o CT increased sensitivity for pleuro-pulmonary
o Cryptogenic organizing pneumonia
Associated with cocaine inhalation abnormalities
Focal lower lobe chronic air space opacities
o Amyloidosis
Secondary to long term drug abuse
I DIFFERENTIAL DIAGNOSIS
Multiple pulmonary nodules, may be calcified Pneumonia
Vascular
Focal or diffuse due to community-acquired
o Septic emboli
pneumonias
Multiple pulmonary nodules with rapid cavitation
 IlliCIT DRUG ABUSE, PULMONARY
Most common cause of focal or diffuse opacities in
young adult
Atypical Edema
Neurogenic pulmonary edema
o Any cause of increased intracranial hypertension
o Edema slightly more prominent in upper lung zones
o Subglottic edema
Negative pressure pulmonary edema
o Neck burns or bruises from suicidal or homicidal
insult
o Edema more prominent in upper lung zones
Smoke inhalation
o Skin burns, carbonaceous sputum
o Edema more prominent in upper lung zones
Aspiration
Generally occurs in those obtunded and unable to
protect their upper airway
May have underlying esophageal motility disorder
4 Contusion Blunt Chest Trauma
Focal nonsegmental opacity immediately following
40 trauma
May have associated rib fractures
Hypersensitivity Pneumonitis
Miliary nodular pattern in mid and lower lung zones
Air-trapping at expiratory CT in addition to
centrilobular nodules
Detailed exposure history important
I PATHOLOGY
General Features
Etiology
o Drug effects
Cocaine: Sympathomimetic agents
Narcotics: Respiratory depression
o Staphylococcus most common organism in septic
embolism
o Damage and obliteration of capillary bed may result
in bullous emphysema
o Capillary damage may due to direct drug toxicity or
due to intermediate immunologic reaction
Epidemiology
o Most common complications of IV drug use: Septic
emboli, community-acquired pneumonia,
tuberculosis
o More than 1.5 million IV drug users in North
America
o 25% of HIV/ AIDS seen in IV drug users
o Edema common developing in up to 50% of
narcotic overdose
Associated abnormalities: Hepatitis Band C in more
than 50% long time IV drug abusers
Gross Pathologic & Surgical Features
No specific features for drug induced damage
Microscopic Features
Pulmonary edema may have features of both capillary
leak and hydrostatic edema
Granulomas from injected talc or methylcellulose
Birefringent crystals from talc particles
Angiothrombosis: Intravascular foreign material mixed
with thrombi in various stages of organization
IClINICALISSUES
Presentation
Most common signs/symptoms
o Hemoptysis common, especially with cocaine abuse
o Needle tracks: Sclerosed veins and scars at common
in jection sites
o Chest pain in cocaine abuse highly associated with
myocardial ischemia
Other signs/symptoms: Toxicologic analysis of blood
and urine
Demographics
Age: Any age but primarily young adults 18-25 years of
age
Gender: Males twice more common than females
Natural History & Prognosis
Mortality rate 3-4% per year
Treatment
Addiction treatment
I DIAGNOSTIC CHECKLIST
Consider
IV drug abuse in young adults with unexplained
radiographic abnormalities in the ER
Consider in young adult presenting with myocardial
infarction
I SELECTED REFERENCES
1. Wolff AJet al: Pulmonary effects of illicit drug use. Clin
Chest Med. 25(1):203-16, 2004
2. Gotway MBet al: Thoracic complications of illicit drug use:
an organ system approach. Radiographies. 22 5pec No
5119-35, 2002
3. Tashkin DP: Airway effects of marijuana, cocaine, and
other inhaled illicit agents. CUff Opin Pulm Med.
7(2):43-61,2001
4. O'Donnell AE:HIV in illicit drug users. Clin Chest Med.
17(4):797-807, 1996
5. O'Donnell AEet al: Pulmonary complications associated
with illicit drug use. An update. Chest. 108(2):460-3, 1995
6. 5tern EJet al: Panlobular pulmonary emphysema caused by
Lv. injection of methylphenidate (Ritalin): findings on
chest radiographs and CT scans. AJRAm J Roentgenol.
162(3):555-60, 1994
7. McCarroll KAet al: Lung disorders due to drug abuse. J
Thorac Imaging. 6(1):30-5, 1991
8. Heffner JE et al: Pulmonary reactions from illicit substance
abuse. Clin Chest Med. 11(1):151-62, 1990
9. Seaman ME: Barotrauma related to inhalational drug abuse.
J Emerg Med. 8(2):141-9,1990
10. Gurney JW et al: Pulmonary cystic disease: comparison of
Pneumocystis carinii pneumatoceles and bullous
emphysema due to intravenous drug abuse. Radiology.
173(1):27-3], ]989
11. Goldstein DSet al: Bullous pulmonary damage in users of
intravenous drugs. Chest. 89(2):266-9, ]986
 IlliCIT DRUG ABUSE, PULMONARY

IIMAGE GALLERY

(Left) Axial NEeT shows

small peripheral cavitated
nodules (arrows) from septic
emboli. IV drug abuse.
Nodules typically are small,
peripheral and cavitate
quickly after development.
(Right) Axial CECT shows
diffuse ground-glass
opacification and
intralobular reticular
thickening (crazy paving
pattern). Subpleural lung
spared. No pleural effusions.
Crack lung resolved over 48
hours.

4
41
(Left) Frontal radiograph
shows diffuse patchy
consolidation throughout the
righllung from pneumonia
or aspiration in IV drug user.
Large abscess cavity in right
upper lobe (arrows). (Right)
Axial CECT shows large
abscess cavity in right upper
lobe with air-fluid level
(arrow). Focal consolidation
in apical posterior segment
left upper lobe.

Typical
(Left) Frontal radiograph
shows fine nodular diffuse
interstitial pattern and
bilateral focal infrahilar
opacities. Ta/cosis and
progressive massive fibrosis
(arrows). (Right) Frontal
radiograph shows severe
bullous emphysema
predominately in the upper
lobes (arrows) in a young IV
drug abuser.
 SMOKE INHALATION

Frontal radiograph at admission. Carbonaceous sputum. Anteroposterior radiograph within 24 hours of smoke
Hypoxic. Mild central bronchial wall thickening (arrow) inhalation vicUm developed diffuse noncardiogenic
and tram-tracking in left upper lobe (open arrow), chest edema most severe in the upper lung zones.
4 was otherwise normal.

42
o Acute: Up to 48 hours
I TERMI NOLOGY
Initial radiograph often normal
Definitions Diffuse peribronchial wall thickening (85%),
Inhalation injury to upper and lower respiratory tract subjective
from smoke Conical narrowing of the subglottic trachea from
edema (35%), never isolated: Individuals will also
have peribronchial wall thickening
I IMAGING FINDINGS Subsegmental atelectasis: Airways narrowed from
mucosal edema and airway casts
General Features Uncomplicated course resolves over 3-5 days
Best diagnostic clue: Acutely, bronchial wall Pleural effusions may develop without
thickening and subglottic edema following smoke parenchymal abnormality, probably related to
inhalation (uncommon) hypoproteinemia from skin burns
Location o Subacute: 3 days to end of hospitalization
o Parenchymal abnormalities predominately in the Noncardiogenic pulmonary edema
perihilar and upper lung zones Superimposed hydrostatic pulmonary edema,
o Typically normal acutely pneumonia, or pulmonary emboli common
o Delayed: Weeks to months after hospital discharge
Radiographic Findings Hyperinflation and small nodules in previously
Radiography affected lung due to bronchiolitis obliterans (BO)
o Severity of injury dependent on concentration and o Complications
length of exposure, airways predominant Barotrauma due to positive pressure ventilation
manifestation 1st 24 hours followed by lung common
parenchyma over next 24-48 hours

DDx: Smoke Inhalation

NPE From Epidural Aspiration Mitral Regurgitation

 SMOKE INHALATION
Key Facts
Terminology
Inhalation injury to upper and lower respiratory tract
from smoke
Imaging Findings
Parenchymal abnormalities predominately in the
perihilar and upper lung zones
Diffuse peribronchial wall thickening (85%),
subjective
Conical narrowing of the subglottic trachea from
edema (35%), never isolated: Individuals will also
have peribronchial wall thickening
Subsegmental atelectasis: Airways narrowed from
mucosal edema and airway casts
Superimposed pneumonia common especially in
those with cutaneous burns, suspect with
worsening of parenchymal abnormalities after the
first 48 hours, develops in up to 40%
Cardiogenic pulmonary edema commonly
superimposed due to large volumes of
administered fluid, especially in those with
cutaneous burns
Adult respiratory distress syndrome (ARDS) is a
severe complication with high mortality rate
Pulmonary emboli after first 3 days
CT Findings
CECT
o Acute
Rarely used to evaluate initial presentation but
would be expected to be more sensitive
Ground-glass opacities due to edema or mosaic
perfusion from small airway occlusion
o Subacute
Problem solving tool for suspected complications
such as pulmonary embolus
Maybe helpful to investigate parenchymal
abnormalities, especially in patients with a
complicated clinical course
HRCT
o Delayed
Bronchiectasis
Mosaic pattern of attenuation (air-trapping) from
BO
Other Modality Findings
Xenon-l33 Ventilation scanning
o Air-trapping and delayed washout acutely
o Maybe abnormal when chest radiograph normal,
rarely used today
Imaging Recommendations
Best imaging tool
o Chest radiography surveillance to detect and
monitor course of disease
o CT as problem solving tool or to evaluate
unexplained radiographic abnormalities
Superimposed pneumonia common especially in
those with cutaneous burns, suspect with worsening
of parenchymal abnormalities after the first 48 hours,
develops in up to 40%
Top Differential Diagnoses
Hydrostatic Pulmonary Edema
Pneumonia
Aspiration
Neurogenic Pulmonary Edema (NPE)
Mitral Regurgitant Pulmonary Edema (MRPE)
Negative Pressure Pulmonary Edema (NPPE)
Pathology
Severity of chemical pneumonitis dependent on
composition and concentration of smoke and length
of exposure
o HRCT to detect late airways complications of 4
bronchiectasis or BO
43
I DIFFERENTIAL DIAGNOSIS
Hydrostatic Pulmonary Edema
Smoke inhalation has more proclivity for the upper
lung zones
Fluid overload common in smoke inhalation due to
massive fluid administration for burns
Pneumonia
Clinical history different from entrapment in fire
Superimposed pneumonia common in smoke
inhalation, develops> 48 hours after admission
Aspiration
Identical radiographic findings
Hypoxic neurologically impaired victim of smoke
inhalation at high risk for aspiration
Neurogenic Pulmonary Edema (NPE)
Requires a central nervous system insult that will raise
intracranial pressure
Hypoxic neurologically impaired victim often requires
head CT to exclude intracranial pathology
Mitral Regurgitant Pulmonary Edema (MRPE)
Pulmonary edema due to heart failure in patient with
incompetent mitral valve
Edema diffuse but more severe in the right upper lobe
due to directional back flow through the right superior
pulmonary vein
Enlarged heart, usually normal in smoke inhalation
Responds quickly to diuretic and inotropic support
Negative Pressure Pulmonary Edema (NPPE)
Develops acutely following relief of upper airway
obstruction, most commonly laryngospasm
Victims of smoke inhalation at risk for NPPE due to
subglottic edema
 SMOKE INHALATION
I PATHOLOGY
General Features
General path comments
o Smoke consists of gases and fine particulate material
Carbonaceous particles (soot) absorb noxious
substances in the gas acting as a delivery vehicle
to the respiratory mucosa
o Severity of chemical pneumonitis dependent on
composition and concentration of smoke and length
of exposure
Injury may occur from upper airways to
pulmonary capillary bed
o Airway wall
Spectrum beginning with edema and
inflammatory cells, proceeding to hemorrhage,
necrosis, ulceration and charring
o Airway casts common causing widespread plugging
Casts composed of neutrophils, shed bronchial
epithelium, mucin, and fibrin
Mean reduction in cross-sectional area: Bronchi
(30%), bronchioles (10%) 48 hours after injury
44 o Pathophysiology: General
Gas concentrations in the lung determined by
ventilation perfusion ratio (V/0]
Normal upright lung, V/Q ratio highest in upper
lung zone, therefore concentration of inhaled gas
concentrated in the nondependent lung
o Pathophysiology: Thermal injury
Rare, inhaled gases rapidly cooled by the upper
respiratory tract
If occurs, limited to the upper respiratory tract
and larynx
Seen with superheated steam and explosions
o Pathophysiology: Asphyxiation due to carbon
monoxide and carbon dioxide
Carbon monoxide displaces oxygen producing
profound hypoxemia
Carbon dioxide reduces ambient oxygen
concentration
o Pathophysiology: Pyrolysis
Cyanide gas from natural and synthetic fabric and
plastics (especially polyvinyl chloride-I'Ve)
Hydrogen chloride from combustion pVC
combines with water to produce hydrochloric acid
Etiology
o Nitric oxide (NO)
Smoke induced release from epithelial cells and
alveolar macrophages
NO causes loss of hypoxic vasoconstriction and
increased vascular permeability
o Bronchial blood flow markedly increased (8x)
May contribute to pulmonary edema
In animal models, bronchial artery occlusion
lessens severity of smoke injury
Gross Pathologic & Surgical Features
Diffuse friable airway mucosa with ulceration and
charring, consolidated lung
Microscopic Features
Acute: Diffuse alveolar damage with hyaline
membrane formation
Delayed: Bronchiolitis obliterans
ICLINICAL ISSUES
Presentation
Most common signs/symptoms: Dyspnea, wheezing,
burns, singed nasal hairs, carbonaceous sputum
Other signs/symptoms
o Elevated carboxyhemoglobin (from carbon
monoxide inhalation)
o Wheezing common due to airway injury
Bronchoscopic findings in smoke inhalation, often
used acutely for diagnosis
o Laryngeal edema, airway ulceration and charring
depending on severity
Delayed symptoms months later: Dyspnea,
nonproductive cough
Pulmonary function tests
o Decreased maximum expiratory flow volume and
forced vital capacity
Demographics
Age: Any age, favors those who are physically unable
to escape a fire
Firefighters
o Long term risk of obstructive lung disease
Natural History & Prognosis
Variable, depends on extent of initial injury
Abnormal chest radiograph within 48 hours of
exposure poor prognostic sign
Treatment
Supportive, intubation and ventilation with
supplemental oxygen to counter hypoxia
Serial cultures for infectious surveillance
Steroids may be detrimental
Promising: NO inhibitors and aerosolized
acetylcysteine and heparin to reduce airway casts
I DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Peribronchial thickening acutely and subglottic edema
Pulmonary edema more common in the upper lung
zones
I SELECTED REFERENCES
1. Wittram C et al: The admission chest radiograph after
acute inl1alation injury and burns. BrJ Radiol.
67(800):751-4,1994
2. Peitzman ABet al: Smoke inhalation injury: evaluation of
radiographic manifestations and pulmonary dysfunction. J
Trauma. 29(9):1232-8; discussion 38-9,1989
3. Lee MJ et al: The plain chest radiograph after acute smoke
inhalation. Clin Radiol. 39(1):33-7, 1988
4. Teixidor HSet al: Smoke inhalation: radiologic
manifestations. Radiology. 149(2):383-7, 1983
 SMOKE INHALATION

I IMAGE GALLERY

(Left) Anteroposterior
radiograph initial study
following smoke inhalation.
Subtle central bronchial wall
thickening (arrow) extending
into upper lobes. Rapidly
progressed to pulmonary
edema. (Right) Frontal
radiograph shows mild
consolidation throughout the
upper lung zones.
Differential includes
neurogenic pulmonary
edema, negative pressure
edema, or aspiration.

4
Typical 45
(Left) Frontal radiograph
shows mifd consolidation
throughout the right lung
and left upper lobe. Note
thickened body wall from
burn edema (open arrow)
and thickened bronchial
walls (arrows). (Right)
Frontal radiograph shows
subg/ollic narrowing
(arrows) on admission. In
house fire, sustained 70%
skin burns and had
carbonaceous sputum.
Patient electively intubated
to protect the airway.

(Left) Axial H RCT shows

mosaic attenuation from
bronchiolitis obliterans
(Right) Axial HRCT at the
level of the carina shows
extensive mosaic attenuation
due to bronchiolitis
obliterans which developed
several years following
smoke inhalation.
 SILO-FILLER'S DISEASE

Graphic shows consequences of acute silo filler's Frontal radiograph shows diffuse vague airspace
disease. Diffuse alveolar damage with proteinaceous opacities and ill-define nodules. Chemical pneumonitis
hemorrhagic fluid-filling alveoli. The small airways may due to toxic inhalation of N02 ISFD).
4 be damaged leading to bronchiolitis obliterans.

46 Morphology: Airspace disease; miliary to small nodules

I TERMINOLOGY
Radiographic Findings
Abbreviations and Synonyms
Radiography
Silo filler's disease (SFD), nitrogen dioxide (N02), nitric o Initial radiograph may be normal
oxide (NO), carbon dioxide (C02) o Onset: Immediate to first 48 hours
Definitions o Hemorrhagic pulmonary edema
Occupational disease that results from pulmonary 6-12 hours after exposure, usual
exposure to oxides of nitrogen Ill-defined, alveolar opacities, pulmonary edema
Inhalation of toxic gases from freshly stored silage o Resolves over 3-5 days
o Also formed during welding, glassblowing, ice o Superimposed pneumonia common complication
resurfacing with Zamboni, manufacture of lacquers o Adult respiratory distress syndrome (ARDS)
and dyes, rocket propellants, and fertilizers, metal o Normal heart size
cleaning; rayon and food bleaching, nitrocellulose o Bronchiolitis obliterans, rare
2-4 weeks after exposure, usual (range, weeks to
incineration
months)
Diffuse ill-defined small or miliary nodules
I IMAGING FINDINGS Diffuse reticulonodular opacities
Hyperinflation
General Features CT Findings
Best diagnostic clue: Diffuse pulmonary edema within
hours of toxic fume inhalation HRCT
o Nonspecific appearance
Location: Diffuse lung involvement o Bilateral airspace and ground-glass opacities, patchy
Size: Variable or diffuse

DDx: Toxic Inhalation

Smoke Inhalation Pneumonia Paraquat Inhalation

 SILO-FILLER'S DISEASE
Key Facts
Terminology
Occupational disease that results from pulmonary
exposure to oxides of nitrogen
Inhalation of toxic gases from freshly stored silage
Imaging Findings
Best diagnostic clue: Diffuse pulmonary edema
within hours of toxic fume inhalation
Initial radiograph may be normal Pathology
Onset: Immediate to first 48 hours
Superimposed pneumonia common complication
Adult respiratory distress syndrome (ARDS)
Cryptogenic organizing pneumonia
Bronchiolitis obliterans, late Clinical Issues
Best imaging tool: Chest radiographs suffice for
extent of injury and monitoring course
o Pleural effusions
o Cryptogenic organizing pneumonia
Peripheral wedge-shaped opacities with air
bronchograms
Subpleural nodules
Ground-glass opacities
Small pleural effusions
Bronchovascular bundle thickening
o Bronchiolitis obliterans, late
Mosaic attenuation
Geographic regions of ground-glass opacification
and hyperlucency
Hyperlucent lung: Decreased vascularity and
air-trapping
Ground-glass opacification with increased
vascularity
Centrilobular nodules
Tree-in-bud appearance
Nuclear Medicine Findings
V/QScan
o Xenon-133 ventilation scanning
Air-trapping and delayed washout
Maybe abnormal when chest radiograph normal
Rarely used, replaced by CT
Imaging Recommendations
Best imaging tool: Chest radiographs suffice for extent
of injury and monitoring course
Protocol advice: Serial radiographs, for initial 4 days
and at 1 week, 1 month, and 3 months
I DIFFERENTIAL DIAGNOSIS
Other Agricultural Lung Diseases
Other toxic gases: Hydrogen sulfide (H2S), ammonia,
carbon dioxide, methane
o Toxic swine and dairy manure exposure (dung lung)
o Can cause anesthesia, asphyxiation, aspiration,
pneumonia
o Can cause ARDS and death, rare
o Anhydrous ammonia inhalation
Protocol advice: Serial radiographs, for initial 4 days
and at 1 week, 1 month, and 3 months
Top Differential Diagnoses
Other Agricultural Lung Diseases
Smoke Inhalation
Fluid Overload
Pneumonia
Dependent on composition and concentration of gas
and length of exposure
During the harvest months, September to October
Preventive: Avoid freshly-filled silo for 14 days (gases
dissipate a few weeks after ensilage)
o From leaks in fertilizer tanks and hoses, and toxic 4
levels in animal buildings
o Can cause chemical bronchitis, pneumonitis, 47
asthma like symptoms, bronchiolitis obliterans
Organic dust toxicity syndrome
o Inflammation due to bacterial cell wall endotoxin
inhalation, primarily gram-negative bacteria
o Usually seen in the spring from moldy dust silage
(silo-filler's disease in fall)
o Radiographs usually normal, HRCT may show
ill-defined centrilobular nodules
Pesticide exposure
o Paraquat lung: Usually absorbed through skin
o Rapid pulmonary fibrosis, end stage lung within 30
days, often fatal
Smoke Inhalation
Acutely, bronchial wall thickening and subglottic
edema
Perihilar and upper lung zone pulmonary edema
Subsegmental atelectasis
Skin burns
Fluid Overload
Identical radiographic findings
Fluid overload, common
Pneumonia
Identical radiographic findings
Superimposed pneumonia common, develops 48
hours after admission
Any worsening of consolidation after 48 hours should
be considered superinfection
Aspiration
Identical radiographic findings and similar course
I PATHOLOGY
General Features
General path comments
o Severity of chemical pneumonitis
 SILO-FILLER'S DISEASE
Dependent on composition and concentration of
gas and length of exposure
Greatest injury to lower respiratory tract
a Pathophysiology
Toxic gas concentrations dependent on V/Q ratio
Normal upright lung, V/Q ratio highest in upper
lung zone
Etiology
a Anaerobic bacteria ferment green forage crops in
silos, corn, oats
a Provides slightly acidic, well preserved good
feedstock for livestock during the winter
a Nitrogen in the plant undergoes 2 oxidation steps to
form NO and then N02
a Fumes forms rapidly in farm silos filled with fresh
organic material
a Hours after stored, toxic and lethal levels of N02
develop
a Gas continues to form during the first 10 days after
filling the silo
a Grain grown under drought conditions with heavy
fertilization, extremely high levels
a N02, heavier than air, settles on top of silage,
48
yellowish-orange in appearance with bleach-like
odor
a SFD: Inhalation of N02
a High levels of C02 in the silo stimulate deeper
inspiration causing higher delivered dose
a With high concentrations, farmer overcome within
2-3 minutes
a N02 combines with water in lung to produce nitrous
and nitric acid
a Free radical generation, protein oxidation, lipid
peroxidation, cell membrane damage
a Altered immune function
Epidemiology
a S cases per 100,000 silo-associated farm workers per
year
a Under-reported
a During the harvest months, September to October
a Severity dependent on concentration and length of
exposure
Gross Pathologic & Surgical Features
Profound chemical pneumonitis and pulmonary
edema
a Noncardiac hemorrhagic pulmonary edema
a Proteinaceous fluid exudates
a Hyaline membrane formation
Microscopic Features
Damaged type I pneumocytes and ciliated airway cells
Extensive damage of the respiratory epithelium in the
small bronchi and bronchioles
Infiltration of the alveolar walls with lymphocytes
Filling of alveolar spaces with macrophages
Hemorrhage
Diffuse alveolar damage with hyaline membrane
formation
Patches of pneumonia
Subacute findings
o Small palpable nodules and hemorrhagic areas
a Cryptogenic organizing pneumonia
Chronic findings: Small airways damage, bronchiolitis
obliterans
I CLINICAL ISSUES
Presentation
Most common signs/symptoms
o Signs and symptoms depend on duration of
exposure and concentration of gas
a May be asymptomatic, 1/2 to 42 hours after
exposure
a Most symptomatic exposures are mild and
self-limiting
a Cough, light headedness, dyspnea, fatigue, and
upper airway and ocular irritation
a Laryngeal spasm, wheezing, bronchiolar spasm
a Cyanosis, vomiting, vertigo, and a loss of
consciousness
a Pulmonary edema may take up to 48 hours to
develop
a Sudden death from
Respiratory failure/arrest; pulmonary
edema/ ARDS, asphyxiation
Late: Bronchiolitis obliterans
Methemoglobinemia, further impairs oxygen delivery
o N02 binds to hemoglobin forming nitrosyl
hemoglobin
Relapse of symptoms of dyspnea, nonproductive
cough months later
a Heralds onset of bronchiolitis obliterans
Demographics
Age: Usually adults, but can affect children
Gender: Male farm workers, most common
Natural History & Prognosis
Variable, depends on extent of initial injury
1/3 with severe exposure die from pulmonary edema
and bronchiolitis obliterans
Treatment
Monitor for 48 hours, possibility of sudden pulmonary
edema
Oxygen
Supportive, mechanical ventilation: Positive
end-expiratory pressure (PEEP)
Serial cultures for infectious surveillance
Antibiotics for superimposed infections
Steroids, to prevent or treat cryptogenic organizing
pneumonia and bronchiolitis obliterans
Preventive: Avoid freshly-filled silo for 14 days (gases
dissipate a few weeks after ensilage)
I SELECTED REFERENCES
1. Pipavath 5), Lynch DA, Cool C, Brown KK,Newell )D.
Radiologic and pathologic features of bronchiolitis. A)RAm
J Roentgenol. 185(2):354-63, 2005
2. Kirkhom 5Ret al: Agricultural lung diseases. Environ
Health Perspect. 108 5uppI4:705-12, 2000
3. Gumey)W et al: Agricultural disorders of the lung.
Radiographies. 11: 625-34, 199]
 SILO-FILLER'S DISEASE

I IMAGE GALLERY
Typical
(Left) Frontal radiograph
normal. Farmer with
symptoms of light
headedness and dyspnea
presented after he was
acutely exposed to NO, in a
freshly filled silo. (Right)
Anteroposterior radiograph
in same patient shows new
diffuse opacities in both
lungs (arrows) that represent
non-cardiac permeability
edema.

4
49
(Left) Frontal radiograph
shows bilateral diffuse small
nodular opacities (arrows).
(Right! Frontal radiograph
close-up in same patient
shows well and ill-defined
nodules and reticular
opacities (arrows). Silo
filler's disease.

(Left) Frontal radiograph 6

weeks after NO, inhalation
shows bibasilar ill-defined
airspace opacities (arrows).
Initial findings of pulmonary
edema had completely
cleared. Radiographic
relapse due to delayed onset
of cryptogenic organizing
pneumonia. (Right) Axial
HRCT shows mosaic pattern
of perfusion. Open arrows
show hyperlucent oligemic
lung, regions of bronchiolitis
obliterans. Ground-glass
opacities (curved arrows)
represent redistribution of
flow to normal lung.
 PULMONARY EMBOLI

Drawing shows an intravascular filling defect in the left Axial eTA in a patient with severe dyspnea shows
interlobar pulmonary artery (curved arrow). cast-like filling defects involving right and left main
pulmonary arteries (open arrows). Diagnosis: Saddle
emboli.

50
I TERMI NOlOGY Radiographic Findings
Radiography
Abbreviations and Synonyms o Chest radiograph nonspecific, 10% normal
Pulmonary embolism (PE), ventilation perfusion scan o Vascular alteration: Oligemia (Westermark sign) due
(V/Q scan), deep venous thrombosis (DVT), inferior to vascular obstruction
vena cava (IVC) Focal enlargement central pulmonary artery
Definitions (knuckle sign) due to physical presence of clot
Embolization of thrombi to the pulmonary o Subsegmental atelectasis (Fleischner lines), airspace
vasculature, usually from lower extremities, opacities, pleural effusion, elevated
abdominal/pelvic veins hemidiaphragm(s)
o Pulmonary infarcts, uncommon; < 10% embolic
episodes
I IMAGING FINDINGS Hampton hump: Pleural-based wedge-shaped
opacity with rounded apex pointing toward the
General Features hilum
Best diagnostic clue: CTA showing emboli as Usually in lower lung zones
intravascular filling defects May develop immediately or delayed 2-3 days
Location: Main, lobar, segmental, subsegmental following embolus
pulmonary arteries Melting sign: Initially ill-defined, over time
Size: Variable, occlusion of large arteries to peripheral become sharply defined and shrink
subsegmental vessels 50% clear completely usually within 3 weeks;
Morphology: CTA: Usually tubular casts of veins others leave linear scars (F1eischner lines)

DDx: Intravascular Filling Defects

Lymph Node Flow Artifact Vein

 PULMONARY EMBOLI

Key Facts
Terminology Total cutoff of vascular enhancement; arterial
Embolization of thrombi to the pulmonary occlusion may enlarge vessels caliber
vasculature, usually from lower extremities, Detection of disease other than PE in 70% of patients
abdominal/pelvic veins V/Q Scanning: More likely to provide diagnosis when
lungs are normal
Imaging Findings
Chest radiograph nonspecific, 10% normal
Top Differential Diagnoses
Vascular alteration: Oligemia (Westermark sign) due Hilar Lymph Nodes or Lymphadenopathy
to vascular obstruction Veins with Flow-Related Artifacts
Subsegmental atelectasis (Fleischner lines), airspace Mucus Plugging
opacities, pleural effusion, elevated In Situ Thrombosis
hemidiaphragm(s) Pulmonary Artery Sarcoma
Hampton hump: Pleural-based wedge-shaped opacity Tumor Emboli
with rounded apex pointing toward the hilum Clinical Issues
CTA: Directly visualizes intraluminal clot as a filling Outcome: Good outcome with appropriate therapy
defect

More common in those with underlying
cardiopulmonary disease
CT Findings
NECT: Rarely can see intravascular hyperattenuation
that represents PE
Standard of care for suspected PE; rapid, noninvasive,
and readily available
High sensitivity and specificity (> 90%); high
inter-observer agreement
CTA: Directly visualizes intraluminal clot as a filling
defect
Acute PE: Partial intraluminal filling defects, sharp
interface surrounded by contrast
o Eccentric or peripheral intraluminal filling defects,
form acute angles with vessel wall
o Total cutoff of vascular enhancement; arterial
occlusion may enlarge vessels caliber
o Right ventricular failure (RVF): Dilation of right
ventricle, leftward bowing of interventricular
septum
o RVF: Dilated IVC with reflux of contrast into hepatic
veins
o Subsegmental atelectasis
o Infarcts: Pleural-based wedge-shaped opacities with
no contrast-enhancement; may cavitate
Chronic PE: Mural-based crescent-shaped intraluminal
defect
o Defects form obtuse angles with vessel wall
o Intimal irregularities, recanalization, webs, bands,
flaps or occlusion
o Stenotic vessels, smaller in caliber than uninvolved
same order vessels
o Extensive bronchial or other systemic collateral
vessels
o Mosaic perfusion pattern
o Pulmonary arterial hypertension, main pulmonary
artery diameter> 30 mm
o Pericardia! effusion
o Chronic PE, thrombus measures approximately 87
HU; acute PE measures approximately 33 HU
Advantages of CTA
o Detection of disease other than PE in 70% of 4
patients
Pneumonia, lung cancer, metastases, 51
pneumothorax
Pericarditis, acute myocardial infarction, aortic
dissection
o Can be combined with scanning abdomen/pelvis
and lower extremities for DVT
Sensitivity and specificity> 90%
Complete, partial or juxtamural filling defect;
venous wall enhancement
Causes for indeterminate or false negative scan
o Poor bolus, image noise, beam hardening, stair step
or respiratory artifacts
o Flow-related, admixing of unenhanced IVC blood
during inspiration
o Partial volume effect; prefer to see filling defect on at
least 2 seq uen tial images
o Potential to miss subsegmental emboli
Angiographic Findings
Never gained widespread acceptance, not universally
available
Vascular filling defect, abrupt occlusion or pruning of
vessels, oligemia
Potential to miss central and subsegmental emboli
MR Findings
Less available than CT; less resolution; limited role for
PE imaging
o Gradient-recalled echo imaging with gadolinium
enhancement
o Shows main, lobar and segmental fulling defects
o Sensitivity approximately 90%, specificity 80-95%
Nuclear Medicine Findings
V/Q Scanning: More likely to provide diagnosis when
lungs are normal
o Normal perfusion scan excludes embolus; high
probability scan diagnostic of embolus
o High percentage of nondiagnostic intermediate
probability scans (> 60%)
o High sensitivity but very poor specificity
 PULMONARY EMBOLI
o Hughes Stovins syndrome: Multiple pulmonary
Ultrasonographic Findings artery aneurysms and peripheral venous thromboses
Lower limb ultrasonography, low sensitivity, high Epidemiology
specificity o Common, considered 3rd most common cause of
When positive, imaging of lungs optional death
50% of patients with PE have no DVT o Found in 15% of autopsies; cause of death in 9% of
Imaging Recommendations autopsies
o 1.5% of CECT scans performed for other reasons
Best imaging tool: CTA of pulmonary arteries and
show PE
abdominal/pelvic and lower extremity veins
Protocol advice Gross Pathologic & Surgical Features
o " 20 gauge catheter in an antecubital vein; 100-150 Deep venous clot fragments in right heart, an average
cc contrast injection at 3-4 cc/sec of 8 pulmonary vessels emboli zed
o 15-17 sec delay; 1.25-3 mm thick slices through Hemodynamic consequences: > 50% reduction
chest with single breathold vascular bed, pulmonary hypertension and right heart
o 3-4 minute delay to image abdomen/pelvis, lower failure
extremities; '" 5 mm thick slices at 3 cm intervals
from knees to abdomen Microscopic Features
Intraluminal thrombus, branching lines of
fibrin-platelet aggregates, surrounded by red and white
IDIFFERENTIAl DIAGNOSIS blood cells
4 Hilar lymph Nodes or lymphadenopathy
52 Reformats to show extraluminallocation IClINICAllSSUES
Veins with Flow-Related Artifacts Presentation
Extend toward left atrium; do not track with bronchi Most common signs/symptoms: Dyspnea, tachypnea,
Mucus Plugging pleuritic chest pain, syncope or asymptomatic
Track with pulmonary arteries; originate from central Other signs/symptoms
bronchi o No telltale signs, symptoms, or laboratory studies
that strongly suggest PE
In Situ Thrombosis D-dimer assay, high sensitivity, poor specificity
Behcet disease: Pulmonary artery aneurysms,
Demographics
hemorrhage, arthritis, orogenital ulcers
Age: Variable: Infants to elderly
Pulmonary Artery Sarcoma Gender: M = F
Lobulated mass that demonstrates enhancement
Natural History & Prognosis
Tumor Emboli Most pulmonary emboli resolve without sequelae
Invasion of IVC or hepatic veins from prostate, breast, Outcome: Good outcome with appropriate therapy
renal cell carcinoma, hepatoma o Good following negative pulmonary angiograms or
Vascular beading, tree-in-bud appearance CT 1% embolic rate)
o Chronic PE: Surgery may result in good outcome
Pneumonia and/or Atelectasis Mortality untreated disease, up to 30%
Common in critically ill, nonspecific opacities must o Poor survival with pulmonary artery hypertension,
consider embolus cor pulmonale
CTA shows enhancement of vessels in opacified lung
Pneumonia and edema generally "fade" away; infarcts Treatment
"melt" away Anticoagulation and fibrinolysis; hemorrhage
complications in 2-15%
Foreign Bodies: Vertebroplasty Glue IVC filter if contra indications to drug therapy or for
Smaller thinner or different density than thrombi recurrent emboli
Endarterectomy for chronic organizing pulmonary
emboli
I PATHOLOGY
General Features I SElECTED REFERENCES
Etiology
o Immobile patients: Hospitalized, intensive care unit, 1. Wittram C et al: CT angiography of pulmonary embolism:
diagnostic criteria and causes of misdiagnosis.
post-operative, post-trauma
Radiographies. 24(5): 1219-38, 2004
o Venous stasis or inflammation: Phlebitis, pregnancy, 2. Oaehee Han et al: Thrombotic and Nonthrombotic
obesity Pulmonary Arterial Embolism: Spectrum of Imaging
o Hypercoagulable states: Acquired, inherited, Findings RadioGraphies. 23: 1521, 2003
malignancy, pregnancy
 PULMONARY EMBOLI
I IMAGE GAllERY

(Left) Axial CTA shows filling

defect in a right lower lobe
segmental pulmonary artery
(open arrow). Peripheral
wedge-shaped opacity
represents infarction, a/so
termed Hampton hump
(curved arrows). (Right)
Axial CTA shows enlarged
main pulmonary artery
indicating pulmonary arterial
hypertension (arrow). The
etiology is likely due to the
left main pulmonary artery
embolus (curved arrow).

4
53
(Left) Axial CTA in a patient
with aortic dissection
(arrow) shows hyperdense
thrombus expanding the
right main pulmonary artery
(curved arrow). Right
inter/obar artery shows no
enhancement (open arrow).
(Right) Axial CTA in same
patient shows decreased
vascularity or oligemia of
right lung (arrows),
Westermark sign.

(Left) Axial NECT shows a

tubular structure or increased
attenuation (arrow) that
represents a thrombus in the
left main pulmonary artery.
Diagnosis: PE. (Right) Axial
CTA shows a filling defect
(open arrow) in a right lower
lobe segmental pulmonary
artery. The thrombus is
mural-based with obtuse
angles and most likely
represents subacute or
chronic PE.
 PULMONARY ARTERY HYPERTENSION

Frontal radiograph shows massive enlargement of the Lateral radiograph shows marked enlargement of the
main (arrows) and central pulmonary arteries with rapid right (black arrow) and left (curved arrow) pulmonary
arterial tapering from severe pulmonary artery arteries. Right ventricular dilatation (white arrows).
4 hypertension due to primary pulmonary hypertension. Long-standing primary pulmonary hypertension.

54 a Associated findings with secondary disease:

ITERMINOlOGY
Definitions
Elevated mean pulmonary artery pressure>
at rest (> 30 mm Hg during exercise)
Classified as pre- or post-capillary
I IMAGING FINDINGS
General Features
Best diagnostic clue: Dilatation central pulmonary
arteries with rapid tapering, right ventricular (RV)
hypertrophy
Radiographic Findings
Radiography
a Pre-capillary hypertension
Dilatation central pulmonary arteries with rapid
pruning of peripheral pulmonary arteries
Normal sized heart early or cardiomegaly with
right ventricular hypertrophy
Intimal calcification with severe long-standing
hypertension
Emphysema, bronchiectasis, honeycombing
a Post-capillary hypertension: Pulmonary
25 mm Hg veno-occlusive disease (PVOD)
Normal size left atrium
Edema, septal thickening and small pleural
effusions
a Post-capillary hypertension: Left atrial obstruction
Increased size left atrium
Hemosiderosis: Nodular interstitial thickening
with calcified nodules
a Normal transverse diameter of right interlobar
pulmonary artery
Abnormal: > 16 mm men, > 14 mm women
Sensitivity for mild hypertension = 50%, severe
hypertension = 75%
a Post-treatment thromboendarterectomy
Reduction in size pulmonary arteries
Immediate reperfusion edema, self-limited, clears
in 3-4 days
CT Findings
CTA

DDx: Enlarged Pulmonary Arteries

~ ""

,/ A. ~
\_4~,f)
Pulmonary Valve Stenosis Idiopathic Dilatation Adenopathy
 PULMONARY ARTERY HYPERTENSION
Key Facts
Terminology Idiopathic Dilatation .
Elevated mean pulmonary artery pressure> 2S mm Pulmonary Valve Stenosis
Hg at rest (> 30 mm Hg during exercise) Pathology
Classified as pre- or post-capillary
Prevalence in men: 10% above age 3S and 2S% above
Imaging Findings age 6S
Best diagnostic clue: Dilatation central pulmonary 1% with acute pulmonary emboli will develop
arteries with rapid tapering, right ventricular (RV) chronic thromboemboli
hypertrophy Clinical Issues
Intimal calcification with severe long-standing No cure, 30% respond to medical therapy
hypertension
Normal main pulmonary artery smaller diameter Diagnostic Checklist
than adjacent ascending aorta Septal thickening may represent post-capillary
Pericardial thickening or effusion common hypertension, important to recognize before
prostaglandin therapy
Top Differential Diagnoses
Adenopathy

a Normal transverse diameter of main pulmonary 4

artery < 28.6 mm Angiographic Findings
Measured at bifurcation, perpendicular to long Contrast injection has increased risk of complications, 55
axis primarily used for pressure measurements
a Normal main pulmonary artery smaller diameter MR Findings
than adjacent ascending aorta
Less sensitive and specific than CT
a Normal cross-section artery-to-bronchus ratio", 1
More difficult in dyspneic patients
a Good correlation between size of central pulmonary
arteries and mean pulmonary artery pressure Nuclear Medicine Findings
a Acute right ventricular dysfunction Ventilation-perfusion
Usually secondary to acute pulmonary embolus a Usually low probability scans except in patients with
Right ventricular dilatation (cavity wider than left chronic thromboemboli which has a high
ventricular cavity) probability pattern
Convex deviation of interventricular septum a Need to reduce the number of particles to avoid risk
towards the left ventricular cavity of acute right heart failure from occlusion of
a Peripheral lobular or wedge-shaped opacities in capillary bed
those who develop pulmonary infarcts
Chronic thromboembolism and tumor emboli Imaging Recommendations
a Pericardial thickening or effusion common Best imaging tool: CT useful to exclude chronic
a Cirrhosis and portal hypertension in Schistosomiasis pulmonary embolism as a cause of hypertension and
HRCT to look for PVOD prior to institution of prostaglandin
a Mosaic perfusion pattern common in pulmonary therapy
hypertension
Geographic ground-glass attenuation represents
normal or hyperperfused lung I DIFFERENTIAL DIAGNOSIS
Vessels in hypoattenuated lung have decreased
caliber due to either vascular obstruction or Adenopathy
hypoxic vasoconstriction Hilum more lobulated may have abnormal mediastinal
Geographic ground-glass opacities less contours from adenopathy
well-defined than that seen with small airways Typical examples: Sarcoidosis, lymphoma
disease Idiopathic Dilatation
a Post-capillary hypertension: Additional findings
Young women, unilateral enlargement main and left
beyond pre-capillary hypertension
pulmonary artery, no pressure gradient
Central and gravity dependent ground-glass
opacities, septal thickening Pulmonary Valve Stenosis
Centrilobular nodules Unilateral enlargement main and left pulmonary
Pleural and pericardial effusions artery, left upper lobe vessels larger than mirror image
Mediastinal adenopathy right upper lobe vessels (due to jet effect of flow across
a Centrilobular nodules may also represent cholesterol pulmonic valve)
granulomas which are seen in up to 2S% of patients
with pulmonary hypertension
 PULMONARY ARTERY HYPERTENSION
o Nonspecific symptoms: Dyspnea, easy fatigue, chest
I PATHOLOGY pain
General Features o Raynaud phenomenon
General path comments: Hemodynamic vascular Other signs/symptoms: Pulmonary veno-occlusive
changes due to elevated pressure from either pre- or disease often preceded by flu-like illness
post-capillary obstruction Swan-Ganz catheterization: Normal resting mean
pulmonary artery pressure < 20 mm Hg
Etiology
o Pre-capillary o Pre-capillary
Congenital left to right shunt, (e.g., atrial septal Elevated mean pulmonary artery pressure and
defect) increased resistance
Chronic thromboemboli Normal pulmonary capillary wedge pressure
Tumor embolism: Hepatoma, gastric carcinoma, (PCWP)
renal cell, right atrial sarcoma, breast, o Post-capillary
intravascular lymphomatosis Elevated mean pulmonary artery pressure and
Infection: Schistosomiasis, AIDS increased resistance
IV drug abuse: Talcosis Elevated PCWP (may be normal, PVOD patchy in
Portal hypertension (2%) distribution)
Primary pulmonary hypertension (PPH) Demographics
Lung disease: End-stage interstitial lung disease or Gender: Primary hypertension (women 3:1)
chronic emphysema Ethnicity: Schistosomiasis endemic in Middle East,
4 o Post-capillary Africa, and Caribbean
PVOD
56 Mediastinal fibrosis (affecting pulmonary veins) Natural History & Prognosis
Left atrial obstruction: Mitral stenosis; LV Poor, 5 year survival for untreated primary pulmonary
dysfunction, myxoma hypertension
Epidemiology 5 year survival for chronic thromboembolic
o Prevalence in men: 10% above age 35 and 25% hypertension 30%
above age 65 End-stage complications: Right heart failure,
o PPH: Women 3rd decade pulmonary artery dissection, pulmonary artery
o 1% with acute pulmonary emboli will develop thrombosis
chronic thromboemboli
o Pulmonary veno-occlusive disease, 1/3rd children Treatment
o Schistosomiasis most common cause worldwide No cure, 30% respond to medical therapy
Oxygen of little benefit
Gross Pathologic & Surgical Features Anticoagulation for thromboemboli: Consider inferior
Chronic emboli may have webs and bands and cava filter and thromboendarterectomy
recanalized clots Calcium channel blockers (potential lethal rebound if
discontinued)
Microscopic Features Prostaglandin 12 (epoprostenol) for primary
Necrotizing arteritis and capillary plexiform lesion in hypertension
PPH and left-to-right shunts o Vasodilator given by continuous IV infusion
o Plexiform lesion: Focal disruption of internal elastic o Side effects: Jaw pain, erythema, diarrhea, arthralgias
lamina of muscular pulmonary artery by a o May cause acute pulmonary edema and right heart
glomeruloid plexus of endothelial channels failure in those with post-capillary hypertension
Capillary hemangiomatosis in PVOD Lung with or without heart transplant
Pulmonary vein intimal fibrosis, recanalized thrombi
and webs in PVOD
Centrilobular cholesterol granulomas in 25% of those I DIAGNOSTIC CHECKLIST
with pulmonary hypertension
Staging, Grading or Classification Criteria Image Interpretation Pearls
Septal thickening may represent post-capillary
Heath-Edwards microscopic grading
hypertension, important to recognize before
o Grade I: Muscularization of pulmonary arteries
prostaglandin therapy
o Grade II: Intimal proliferation
o Grade III: Subendothelial fibrosis
o Grade IV: Plexiform lesions
I SELECTED REFERENCES
o Grade V: Rupture dilated vessels
o Grade VI: Necrotizing arteritis 1. Frazier AAet al: From the archives of the AFIP:Pulmonary
vasculature: Hypertension and infarction. Radiographics.
20:491-524; quiz 530-491,532,2000
2. 5herrick AD et al: Mosaic pattern of lung attenuation on
I CLINICAL ISSUES CT scans: Frequency among patients with pulmonary
artery hypertension of different causes. A)R.169:79-82,
Presentation 1997
Most common signs/symptoms
 PULMONARY ARTERY HYPERTENSION
IIMAGE GALLERY

Typical
(Left) Axial CECT shows
enlarged main pulmonary
artery (black arrow) from
pulmonary artery
hypertension. Moderate
pericardia I effusion (white
arrows) common in
pulmonary artery
hypertension. (Right) Axial
CECT shows dilatation of the
right ventricle (arrows)
which is larger in width than
the left ventricular cavity
(curved arrow) in patient
with pulmonary
hypertension and cor
pulmonale.

(Left) Frontal radiograph

shows moderate
cardiomegaly and enlarged
main and central pulmonary
arteries due to chronic
thromboembolic
hypertension. (Right) Frontal
radiograph following
thromboendarterectomy.
Heart has decreased in size
and reduclion in size main
and central pulmonary
arteries.

Typical
(Left) Axial CECT shows
obstructed inferior
pulmonary vein due to
mediastinal fibrosis (arrow)
resulting in post-capillary
pulmonary artery
hypertension. (Right) Axial
HRCT shows enlarged
central bronchovascular
structures (arrows), smooth
septal thickening (curved
arrow), and mosaic
perfusion from post-capillary
pulmonary artery
hypertension.
 PULMONARY ARTERY ANEURYSM

Frontalgraphic shows Swan-Canz catheter is too far Coronal angiography shows focal aneurysm (arrow) at
peripheral in subsegmental branch of the left lower segmental branch right lower lobe from Swan-Canz
lobe. Balloon overdistension has ruptured the artery and induced false aneurysm. False aneurysms are at risk (or
4 will lead to a falseaneurysm. rupture.

58
ITERMINOlOGY Radiographic Findings
Radiography
Abbreviations and Synonyms
a Central pulmonary artery aneurysms (main and
Pulmonary artery dissection lobar pulmonary arteries)
Definitions Enlarged lobulated central pulmonary arteries
True aneurysm: Wall composed of all layers of the (may be multiple)
artery wall Asymmetric enlargement central pulmonary
False aneurysm: Perforation of wall, aneurysm wall artery
composed of surrounding adventitia Displaced intimal calcification suggests dissecting
Dissecting aneurysm: Intimal disruption with aneurysm (double wall sign)
extension into media of arterial wall a Peripheral pulmonary artery aneurysms (segmental
arteries to periphery)
Nonspecific solitary pulmonary nodule
I IMAGING FINDINGS Nodule adjacent to cavitary disease in mycotic
aneurysms (Rasmussen)
General Features Swan-Ganz induced false aneurysm
Best diagnostic clue o Pre-aneurysm
a Focal dilatation pulmonary artery Persistent inflation of Swan-Ganz balloon or
a Elliptical shaped solitary pulmonary nodule (SPN) persistent location of Swan-Ganz tip 3-4 em past
adjacent to right lower lobe segmental artery the lobar pulmonary arteries
Morphology: Elliptical shape sharply marginated o Most common location right lower lobe (mirrors the
nodule oriented along axis of pulmonary artery most frequent placement of Swan-Ganz catheter),
rare in upper lobes

DDx: Pulmonary Artery Aneurysm

Carcinoid Pulmonary Valve Stenosis Idiopathic Dilatation

 PULMONARY ARTERY ANEURYSM
Key Facts
Imaging Findings
Elliptical shaped solitary pulmonary nodule (SPN)
adjacent to right lower lobe segmental artery
Aneurysm will enhance with intravenous contrast,
identical to enhancement of adjacent pulmonary
arteries
May be easily overlooked on mediastinal windows,
mistaken as normal artery
Top Differential Diagnoses
Carcinoid Tumors
Idiopathic Dilatation Pulmonary Artery
Pulmonary Valve Stenosis
Pulmonary Artery Hypertension
o Sharply defined elliptical shape
o Main axis parallels normal course of pulmonary
arteries
o Perihilar adjacent to segmental pulmonary artery
o Size usually < 3 cm
o Often pre-existing surgical changes of coronary
bypass grafting
o Rupture
Focal consolidation in area of Swan-Ganz catheter,
size dependent on quantity of hemorrhage
Hemothorax if rupture into pleural space
Hemorrhage will obscure aneurysm
CT Findings
NECT: Often misinterpreted as hilar adenopathy
CECT
o Swan-Ganz induced false aneurysm
Aneurysm will enhance with intravenous contrast,
identical to enhancement of adjacent pulmonary
arteries
May be easily overlooked on mediastinal
windows, mistaken as normal artery
o Dissecting aneurysm
Enlarged central pulmonary arteries from
pre-existing hypertension
Intimal flap: False lumen may by thrombosed
Pericardial effusion suggest rupture of false lumen
o Behcet disease
In situ thromboses
PA aneurysms (often multiple)
Thromboembolic disease
Intracardiac thromboses
Angiographic Findings
Usually not required for diagnosis
Primarily used for interventional therapy with coils or
detachable balloons
MR Findings
Multi planar capability useful for small aneurysm
White blood sequences more useful for
characterization
Pathology
Direct rupture of vessel by inflated balloon or by
catheter tip
Systemic vasculitis (Hughes-Stovin disease, Behcet
disease)
Rasmussen aneurysm
Clinical Issues
Swan-Ganz false aneurysm: Mortality rate 45-65%
Diagnostic Checklist
Pulmonary artery aneurysm for any pulmonary
nodule adjacent to segmental artery, especially in
those who have pre-existing cardiac disease
(cardiomegaly, coronary artery bypass surgery)
Carcinoid tumors may also intensely enhance
o Black blood sequences less useful due to lack of
signal from surrounding lung (poor conspicuity)
Imaging Recommendations
Best imaging tool: CTA with reformations for
characterization of pulmonary vascular lesions
I DIFFERENTIAL DIAGNOSIS
Carcinoid Tumors
Segmental location similar to Swan-Ganz induced false
aneurysm
May enhance, but not to the same degree as false
aneurysm
May calcify (central nidus common)
Idiopathic Dilatation Pulmonary Artery
Young asymptomatic women
Focal dilatation main pulmonary artery, pulmonary
artery pressures not elevated
Peripheral arteries normal
Pulmonary Valve Stenosis
Enlarged main and left pulmonary artery from jet
effect across stenotic valve
Left upper lobe arteries larger than mirror image
arteries in the right upper lobe (due to increased flow)
Calcified pulmonary valve (rare)
Right apical opacities (cyanotic pseudofibrosis) from
collateral vessels and scarring from infarcts
Pulmonary Artery Hypertension
Enlarged central pulmonary arteries with pruning of
distal arteries
No intimal flap or thickening of wall
I PATHOLOGY
General Features
Genetics
o Behcet disease
 PULMONARY ARTERY ANEURYSM
Associated with human leukocyte antigen B5 Dissection pulmonary artery
(HLA-B5) a Rare and usually lethal complication chronic
Etiology pulmonary hypertension, rupture into pericardium
a 5wan-Ganz false aneurysm tamponade and sudden death
Direct rupture of vessel by inflated balloon or by Behcet disease
catheter tip a Recurrent oral ulceration (99%) and recurrent
Pressure in inflated balloon: 300 mm Hg - genital ulcers (60%)
inflating pressure up to 900 mm Hg a Other: Ocular lesions (70%), skin hypersensitivity
Swan-Ganz catheter may migrate distally after (80%)
correct placement dl)e to stretching of the catheter a Thrombophlebitis 15%
at body temperature Hugh-Stovin syndrome
Normal tip of Swan-Ganz catheter should be just a Dural sinus thrombosis and peripheral
beyond the hilum at the level of the interlobar thrombophlebitis
pulmonary artery
a Central pulmonary artery aneurysm Demographics
Pulmonary artery hypertension: Primary or Age
secondary a Swan-Ganz false aneurysm
Congenital anomalies: Left to right shunts, Older adult mirroring prevalence of those
especially patent ductus arteriosus requiring hemodynamic monitoring
Mycotic aneurysms (especially tuberculosis), a Dissecting aneurysm
Follows distribution of primary and secondary
4 bacterial endocarditis, syphilis, septic emboli
Systemic vasculitis (Hughes-Stovin disease, Behcet pulmonary hypertension
disease) o Behcet disease and Hugh-Stovin syndrome
60 30 years of age
a Peripheral pulmonary artery aneurysm
Blunt or lacerating trauma Gender
Mycotic aneurysms (especially tuberculosis) o Swan-Ganz false aneurysm more common in
a Rasmussen aneurysm females
Aneurysm of small to medium pulmonary artery o Behcet disease and Hugh-Stovin syndrome more
branches adjacent to tuberculous cavity common in males (2.3:1)
Erosion of cavity into artery producing a false Ethnicity: Behcet disease: Asians and North Africans
aneurysm Natural History & Prognosis
a Behcet and Hugh-Stovin syndrome have similar
Swan-Ganz false aneurysm: Mortality rate 45-65%
pathology and may be related
Epidemiology Treatment
a 0.1 11, of cases with a Swan-Ganz catheter Swan-Ganz false aneurysm or Rasmussen aneurysm
a Swan-Ganz false aneurysm risk factors: Pulmonary o Interventional coil or balloon embolization
hypertension, anticoagulation, females> 60 years of o Surgical segmentectomy
age, repeat manipulation during cardiopulmonary Surgical repair main pulmonary artery aneurysm: High
bypass surgery morbidity and mortality
Associated abnormalities: Rasmussen aneurysm Corticosteroids and cytotoxic agents for vasculitis
associated with tuberculosis, either primary or
secondary
Gross Pathologic & Surgical Features I DIAGNOSTIC CHECKLIST
Pulmonary artery dissecting aneurysm Consider
a Main pulmonary artery site in 80%, lobar arteries in Pulmonary artery aneurysm for any pulmonary nodule
20%
adjacent to segmental artery, especially in those who
a In contrast to arterial dissection, false lumen tends have pre-existing cardiac disease (cardiomegaly,
to rupture into the pericardium coronary artery bypass surgery)
Microscopic Features Image Interpretation Pearls
Behcet and Hugh-Stovin syndrome: Necrotizing Carcinoid tumors may also intensely enhance
lymphocytic vasculitis
Beware history of catheterization before biopsying SPN

I CLINICAL ISSUES I SELECTED REFERENCES

Presentation 1. Khattar RSet al: Pulmonary artery dissection: an emerging
Most common signs/symptoms cardiovascular complication in surviving patients with
a Swan-Ganz false aneurysm chronic pulmonary hypertension. Heart. 91(2):142-5, 2005
2. Abreu ARet al: Pulmonary artery rupture induced by a
May be asymptomatic, incidental SPN to pulmonary artery catheter: a case report and review of the
life-threatening hemorrhage literature. J Intensive Care Med. 19(5):291-6,2004
a Rasmussen aneurysm: Hemoptysis may be 3. Erkan D et al: Is Hughes-Stovin syndrome Behcet's disease?
life-threatening Clin Exp Rheumatol. 22(4 Suppl 34):564-8, 2004
 PULMONARY ARTERY ANEURYSM
I IMAGE GALLERY
Typical
(Left) Frontal radiograph
shows solitary pulmonary
nodule approximately 2 em
peripheral to the right
interlobar pulmonary artery
(arrow). Angiogram shown
in opening gallery.
Swan-Canz false aneurysm.
(Right! Axial CECT shows
contrast-enhancement in the
aneurysm (arrow).
Aneurysm is larger than the
adjacent pulmonary arteries.
Aneurysm could be easily
overlooked.

4
Typical 61
(Left) Frontal radiograph
shows sharply marginated
elliptical nodule (arrows) in
the left lower lobe adjacent
to a segmental artery (curved
arrow). Long axis oriented
parallel to the pulmonary
artery. (Right! Axial CECT
shows pulmonary artery
aneurysm (arrow) with a
small eccentric thrombus
(curved arrow). Patient had
history of hemodynamic
monitoring with a
Swan-Ganz catheter.

Typical
(Left! Coronal CECT
mu/tip/anar shows mycotic
aneurysm (arrow) in
subsegmental artery right
lower lobe. Dense lobar
consolidation right lower
lobe and focal consolidation
left lung. (Right! Axial CECT
shows thrombosed
dissection (arrows) of the
right pulmonary artery in this
young woman with long
standing pulmonary artery
hypertension. Displaced
flattened right superior
pulmonary vein (curved
arrow).
 HIGH ALTITUDE PULMONARY EDEMA

Frontal radiograph shows widespread perihilar Frontal radiograph shows similar findings of basilar
consolidation. Heart size is normal. No pleural effusion. perihilar consolidation in trekking partner who a/50
Trekker became dyspneic at high altitude. Made a became ill at high alWude. HAPE oftell has variable
4 complete rapid recovery after descent. edema pattern. (Courtesy P. Stark, MOJ.

62
Radiographic appearance changes between
ITERMINOLOGY episodes
Abbreviations and Synonyms Size: Extent parallels clinical severity
Noncardiogenic pulmonary edema Radiographic Findings
Definitions Radiography
Noncardiogenic pulmonary edema that usually occurs o Normally 10-15% increase in size main and right
at altitudes above 3,000 meters (10,000 feet) interlobar pulmonary artery at high altitude
Capillary stress failure due to hypoxic pulmonary o Normal initially
artery vasoconstriction and permeability edema Develops in 18-48 hours after arriving at altitude
oEdema
Nonconfluent and asymmetric
IIMAGING FINDINGS Alveolar more common than interstitial patterns
Mild disease more often peripheral (50%) and
General Features upper lung zone predominant (40%)
Best diagnostic clue: Diffuse lung disease at high When unilateral (uncommon) right> left (4:1)
altitude Peripheral in 50%, uniform or central in 50%
Location May fluctuate several times between upper zonal
o Variable, may predominate in upper lung zones or and diffuse over first 4 days
unilateral o Normal heart size
Upper lung zones early and rarely spared o Pleural effusions absent
Lung adjacent to diaphragm usually spared o Resolves over 5 to 10 days
When unilateral usually right lung o Each episode of high altitude pulmonary edema
(pathophysiology unknown) (HAPE) tends to differ in the same individual

DDx: High Altitude Pulmonary Edema

Aspiration Neurogenic Pulmonary Edema Mitral Regurgitation

 HIGH ALTITUDE PULMONARY EDEMA
Key Facts
Terminology Pulmonary Embolism
Noncardiogenic pulmonary edema that usually Negative Pressure Pulmonary Edema
occurs at altitudes above 3,000 meters (10,000 feet) Pathology
Capillary stress failure due to hypoxic pulmonary Irregular hypoxic basal vasoconstriction and
artery vasoconstriction and permeability edema overperfusion of other vascular zones
Imaging Findings 0.5-15% previously healthy individuals after rapid
Normally 10-15% increase in size main and right ascent to elevations above 2,500-3,000 m above sea
interlobar pulmonary artery at high altitude level
Develops in 18-48 hours after arriving at altitude Associated abnormalities: Acute mountain sickness in
May fluctuate several times between upper zonal and 50%
diffuse over first 4 days Clinical Issues
Top Differential Diagnoses Rarely develops after 4 days at altitude
Neurogenic Pulmonary Edema (NPE) Descent to lower altitude critical
Aspiration
Pneumonia

Imaging Recommendations Smoke Inhalation

4
Best imaging tool: Chest radiographs usually sufficient Similar radiographic pattern 63
for detection and monitoring '. May have carbonaceous particles in sputum and skin
burns
Develops within few hours of smoke inhalation
I DIFFERENTIAL DIAGNOSIS
Neurogenic Pulmonary Edema (NPE) I PATHOLOGY
Often upper lung zone predominant
Requires central nervous system (CNS) insult that General Features
acutely raises intracranial pressure General path comments: Pulmonary edema with
Develops acutely or subacutely following CNS insult features of both hydrostatic and noncardiogenic
edema
Aspiration Genetics: Hypoxic vasoconstriction response variable
May be predominately upper lobes in supine comatose and maybe genetically determined
patient (gravitationally directed to posterior segments Etiology
upper lobes) o Known features important in pathogenesis
Aspiration extremely common with obtunded patients Rapid rise in pulmonary artery pressure
Associated wi th fever Hypoxia
Pneumonia o Edema has features of both hydrostatic (pulmonary
overperfusion) and noncardiogenic (capillary) leak
Identical radiographic findings
Overperfusion with increased pulmonary arterial
Associated with fever
pressures but normal capillary wedge pressures
Resolves more slowly
Capillary stress failure with leakage of protein rich
Pulmonary Embolism fluid
Predisposed due to venous stasis and hyperviscosity at o Stress failure
altitude Pulmonary capillaries extremely thin
Infarcts unusual in otherwise healthy young adults When subjected to high intravascular pressures,
Clot may enlarge central pulmonary arteries capillaries may fail (pressures> 40 mm Hg)
Initially hydrostatic edema develops according to
Negative Pressure Pulmonary Edema Starling law
Develops acutely following relief of upper airway Disruption of capillary endothelium leads to
obstruction noncardiogenic pulmonary edema
Victims often confused due to hypoxia o Physiology
Most common following laryngospasm Alveolar oxygen concentration highest in the
non dependent lung (due to ventilation/perfusion
Mitral Regurgitant Pulmonary Edema ratio)
Mitral regurgitant pulmonary edema predominant in At 3000 m, alveolar oxygen 70% of that at sea
right upper lobe level
Cardiomegaly Irregular hypoxic basal vasoconstriction and
overperfusion of other vascular zones
 HIGH ALTITUDE PULMONARY EDEMA
Redistribution and overperfusion often to the o Dyspnea and dry cough, fever, tachycardia,
upper lung zones where hypoxic vasoconstriction tachypnea, and hypotension
least o Rales at auscultation
o Stress failure differential diagnosis o Acute mountain sickness (50%)
Neurogenic pulmonary edema Headache, fatigue, insomnia, anorexia, nausea,
High altitude pulmonary edema and vomiting
Exercise-induced pulmonary hemorrhage in Symptoms begin 2-3 hours after ascent (in
thoroughbred horses contrast HAPE > 18 hours)
o Risk factors Usually self-limited, disappears spontaneously
Previous history of HAPE (up to 60% redevelop after 2-3 days
HAPE) May not occur in those with HAPE
Rapid ascent Other signs/symptoms: Respiratory alkalosis due to
Physical exercise at altitude hyperventilation
Unilateral absence of a pulmonary artery
Epidemiology Demographics
00.5-]5% previously healthy individuals after rapid Age: Any age but more common in young adults (due
ascent to elevations above 2,500-3,000 m above sea to treks at high altitudes)
level Gender: Women may be less susceptible than men
o Often goes unrecognized in otherwise healthy adults Natural History & Prognosis
at lower altitudes
Clinically evident on 2nd day at altitude
4 Associated abnormalities: Acute mountain sickness in
50%
Rarely develops after 4 days at altitude
Outcome depends on descent to lower altitudes and
64 Gross Pathologic & Surgical Features access to supportive care
Increased lung weight from pulmonary edema Mortality up to 10%
Airways filled with fluid, often blood tinged Treatment
Dilated right atrium and right ventricle
Prevent with acclimatization allows time for vascular
Normal left ventricle
adapt ion
Microscopic Features Descent to lower altitude critical
Diffuse alveolar damage pattern Supplemental oxygen
o Neutrophilic infiltrate, rare lymphocytes and Portable hyperbaric chamber (Gamow bag)
monocytes temporizing until descent possible
o Focal hemorrhage common Vasodilators (such as nifedipine) may reduce incidence
o Occasional hyaline membranes in those with previous HAPE
Inhaled nitric oxide may alleviate hypoxic
Staging, Grading or Classification Criteria vasoconstriction
Hultgren severity classification
Mild
o Dyspnea with exertion, rales may be present, heart I DIAGNOSTIC CHECKLIST
rate < 110
o Edema less than 25% of one lung involved Consider
Moderate In those with acute mountain sickness and cerebral
o Dyspnea with ordinary effort, rales usually present, edema may also develop neurogenic pulmonary
heart rate 110-120 edema
o Edema less than 50% of one lung
Image Interpretation Pearls
Serious
o Dyspnea at rest, cough, rales present, heart rate Upper lung edema also seen with smoke inhalation,
120-140 neurogenic pulmonary edema, mitral regurgitant
o Bilateral edema of at least half of each lung pulmonary edema and negative pressure pulmonary
edema - many of which include CNS symptoms
Severe
o Severe cyanosis, prominent rales, productive cough,
mentally impaired, heart rate> 140
o Bilateral edema of more than 50% of each lung I SELECTED REFERENCES
1. West JIl: Invited review: pulmonary capiilary stress failure.
J Appl Physio!. 89(6):2483-9;discussion 97, 2000
IClINICALISSUES 2. Hultgren HN et al: Lung pathoiogy in high-altitude
pulmonary edema. Wilderness Environ Med. 8(4):218-20,
Presentation 1997
3. Vock Pet ai: Variable radiomorphologic data of high
Most common signs/symptoms
aititude pulmonary edema. Features from 60 patients.
o Underlying pulmonary or cardiac disease, alcohol, Chest. 100(5):1306-11, 1991
respiratory depressants, and respiratory infections 4. Vock Pet al: High-altitude pulmonary edema: findings at
may enhance vulnerability to altitude illness high-altitude chest radiography and physical examination.
Radiology. 170(3 Pt 1):661-6, 1989
 HIGH ALTITUDE PULMONARY EDEMA
I IMAGE GALLERY
Typical
(Left) Frontal radiograph
shows enlarged heart (not
typica/) and severe perihilar
consolidation. Hemoptysis
and dyspnea at high altitude.
Also had symptoms of acute
mountain sickness. (Right!
Anteroposterior radiograph
shows homogeneous
consolidation in the right
upper lobe. Patient
complained of shortness of
breath following a mountain
climb. Cleared within 24
hours.

4
65
(Left! Anteroposterior
radiograph shows diffuse
nonspecific central
pulmonary edema. Edema
resolved quickly after
descent to lower altitude.
(Right! Anteroposterior
radiograph shows mild
central consolidation and
heart size upper limits of
normal in trekker. Patient
had mild shortness of breath
and hemoptysis.

Typical
(Left! Anteroposterior
radiograph shows diffuse
consolidation predominantly
left-sided and predominantly
involving the upper lung
zones. High altitude
pulmonary edema. (Right!
Anteroposterior radiograph
shows moderately dense
consolidation centrally and
in the upper lung zones with
sparing of the costophrenic
angles. Intubated. High
altitude pulmonary edema.
 NEUROGENIC PULMONARY EDEMA

Anteroposterior radiograph shows homogeneous Anteroposterior radiograph shows severe consolidaUon

consolidaUon throughout the upper lung zones (arrows) throughout the upper lung zones following trauma.
in patient following subarachnoid hemorrhage. DifferenUal included contusion or aspiraUon. PaUent
4 sustained epidural hematoma.

66 When unilateral usually right lung

ITERMINOLOGY (pathophysiology unknown)
Abbreviations and Synonyms Size: Variable extent dependent on extent of CNS
Neurogenic pulmonary edema (NPE) injury

Definitions Radiographic Findings

Any central nervous system (CNS) injury (including Radiography
seizures) that causes a rapid increase in intracranial o Onset acute (minutes) or subacute (12 hours)
pressure (ICP) following CNS insult
Capillary stress failure, edema based on both o Edema pattern often asymmetric
hydrostatic mechanisms and noncardiogenic Upper lung predominant common finding
(capillary) leak Usually bilateral but rare unilateral involvement
(typically right lung)
Radiographically, edema pattern asymmetric, often
upper lung zone predominant o Septal (Kerley) lines usually absent
o Normal heart size
o Pleural effusions absent
I IMAGING FINDINGS o Resolves over 24-48 hours
Resolution usually rapid < 24 hours (33%)
General Features Resolution between 24-72 hours (20%)
Best diagnostic clue: Pulmonary edema pattern after Resolution between 3-7 days (20%)
CNS insult Rarely persists more than 7 days (5%)
Location Imaging Recommendations
o Variable often upper lung zone or unilateral
Best imaging tool: Chest radiographs usually sufficient
for detection and monitoring

DDx: Neurogenic Pulmonary Edema

Aspiration Mitral Regurgitation

 NEUROGENIC PULMONARY EDEMA
Key Facts
Terminology Cardiogenic Pulmonary Edema
Any central nervous system (CNS) injury (including Pneumonia
seizures) that causes a rapid increase in intracranial Contusion
pressure (ICP) High Altitude Pulmonary Edema
Capillary stress failure, edema based on both Smoke Inhalation
hydrostatic mechanisms and noncardiogenic Pathology
(capillary) leak May result from any CNS injury that leads to rapid
Radiographically, edema pattern asymmetric, often increase in intracranial pressure (ICP)
upper lung zone predominant Underdiagnosed and unrecognized, NPE actually
Imaging Findings quite common
Onset acute (minutes) or subacute (12 hours) 70% incidence in fatal cases of subarachnoid
following CNS insult hemorrhage
Resolves over 24-48 hours Clinical Issues
Top Differential Diagnoses Characteristic rapid development of respiratory
Aspiration failure 4 hours)

Protocol advice: Cranial CT or MRI useful to evaluate 4

CNS etiology Mitral Regurgitant Pulmonary Edema
Mitral regurgitant pulmonary edema predominant in 67
right upper lobe
I DIFFERENTIAL DIAGNOSIS Cardiomegaly

Aspiration Negative Pressure Pulmonary Edema

Develops acutely following relief of upper airway
May be predominately upper lobes in supine comatose
patient (gravitationally directed to posterior segments obstruction
upper lobes) Victims often confused due to hypoxia
Aspiration extremely common with CNS insults Most common following laryngospasm
Resolves more slowly than (NPE)
Both associated with fever
I PATHOLOGY
Cardiogenic Pulmonary Edema
Usually not predominately upper lobe General Features
Cardiomegaly and pleural effusions less likely with General path comments: Pulmonary edema with
NPE features of both hydrostatic edema and
Kerley B lines more common, rarely seen with NPE noncardiogenic (capillary) leak edema
Etiology
Pneumonia o Known features important in pathogenesis
Identical radiographic findings ICP
Both associated with fever Requires intact cervical spinal cord (NPE blocked
Resolves more slowly than NPE with C7 cord transection)
Release catecholamines producing "sympathetic
Contusion storm"
Immediately following trauma Marked elevation of epinephrine and
Motor vehicle accidents could give rise to both norepinephrine in blood
contusions and NPE NPE blocked by alpha-adrenergic blocking agents
Resolution similar such as phentolamine
Traumatic pneumatoceles not seen with NPE o Pulmonary venoconstriction from catecholamines
High Altitude Pulmonary Edema Markedly increases pulmonary capillary wedge
pressure and may cause capillary stress failure or
Similar radiographic pattern
"blast injury"
Seen with ascent> 3,000 meters (10,000 feet)
o Edema has features of both hydrostatic (pulmonary
CNS insults from acute mountain sickness may result
venoconstriction) and noncardiogenic capillary leak
in high altitude pulmonary edema
Initially edema due to hydrostatic mechanisms
Smoke Inhalation and later due to capillary leak
Similar radiographic pattern o Stress failure
May have carbonaceous particles in sputum and skin Pulmonary capillaries extremely thin
burns When subjected to high intravascular pressures,
Develops within few hours of smoke inhalation capillaries may fail (pressures> 40 mm Kg)
 NEUROGENIC PULMONARY
Initially hydrostatic edema develops according to
Starling's law
Disruption of capillary endothelium leads to
noncardiogenic pulmonary edema
o Stress failure differential diagnosis
Neurogenic pulmonary edema
High altitude pulmonary edema
Exercise-induced pulmonary hemorrhage in
thoroughbred horses
o CNS site responsible for NPE not fully established
Animal studies suggest hypothalamus or medulla
Epidemiology
o May result from any CNS injury that leads to rapid
increase in intracranial pressure (lCP)
Most commonly trauma but can be seen with
grand mal seizures and electroshock therapy
o Underdiagnosed and unrecognized, NPE actually
quite common
70% incidence in fatal cases of subarachnoid
hemorrhage
4 50% incidence in head trauma
33% incidence in status epilepticus
68 Associated abnormalities
o CNS injury usually obvious from subarachnoid
hemorrhage, mass effect with herniation, subdural
fluid collection
CT or MRI may be normal with seizure induced
NPE
Gross Pathologic & Surgical Features
Increased lung weight from pulmonary edema
Microscopic Features
Protein rich pulmonary edema with hyaline
membrane formation
Hemorrhage more common in severe cases
ICLINICAllSSUES
Presentation
Most common signs/symptoms
o Dyspnea
o Nonspecific signs: Tachycardia, tachypnea
o Rales and rhonchi
o Mild leukocytosis common (leading to erroneous
diagnosis of pneumonia)
o Fever common (usually secondary to CNS insult)
o Mild hemoptysis or pink frothy sputum
o Hypoxia may require ventilatory support
o Protein rich sputum (due to capillary leak)
Other signs/symptoms
o Swan Ganz catheterization
Usually pulmonary artery pressures and cardiac
output normal
If pressure monitored at the time of CNS insult
(subarachnoid hemorrhage in the ICU) there is a
transient rise in pulmonary capillary wedge
pressure
By the time patients reach hospital, pulmonary
artery pressures have returned to normal
EDEMA
Demographics
Age: Any age but more common in young adults (due
to trauma related injuries and ruptured intracranial
aneurysms)
Gender: None
Natural History & Prognosis
Develops within minutes to hours of CNS insult
o Characteristic rapid development of respiratory
failure 4 hours)
Outcome depends on successful treatment of CNS
cause
Treatment
Supportive
o Supplemental oxygen
o Often hyperventilated (to decrease CO,) which
decreases intracranial pressure
o Mechanical ventilation including positive
end-expiratory pressure (PEEP) may be necessary to
maintain oxygenation
PEEP may increase intracranial pressure and
should be used cautiously
Alpha-adrenergic blocking agents early, benefit
unproven but works in experimental animals
Dilantin or other anticonvulsants for seizures
I DIAGNOSTIC CHECKLIST
Consider
Underdiagnosed, usually mistaken for aspiration,
pneumonia, or cardiogenic edema
Image Interpretation Pearls
Review neuroimaging studies
o NPE a diagnosis of exclusion once contusion,
aspiration, pneumonia excluded
Upper lung zone predominance also seen with smoke
inhalation, high altitude pulmonary edema, mitral
regurgitant pulmonary edema, and negative pressure
pulmonary edema: Many of which include CNS
symptoms
I SElECTED REFERENCES
1. Fontes RBet al: Acute neurogenic pulmonary edema: case
reports and literature review.J Neurosurg Anesthesiol.
15(2):144-50, 2003
2. WestJB: Invited review: pulmonary capillary stress failure.
J Appl Physiol. 89(6):2483-9;discussion 97, 2000
3. Rogers FBet al: Neurogenic pulmonary edema in fatal and
nonfatal head injuries. J Trauma. 39(5):860-6; discussion
66-8, 1995
4. EllSR:Neurogenic pulmonary edema. A review of the
literature and a perspective. Invest Radiol. 26:499-506,
1991
5. West JB et al: Stress failure in pulmonary capillaries. J Appi
Physiol. 70:1731-42, 1991
6. Colice GL: Neurogenic pulmonary edema. Clin Chest Med.
6(3):473-89,1985
7. Malik AB:Mechanisms of neurogenic pulmonary edema.
Circ Res. 57(1):1-18, ]985
8. Felman AH:Neurogenic pulmonary edema. Observations
in 6 patients. AJR.112: 393-6, 1971
 NEUROGENIC PULMONARY EDEMA
I IMAGE GALLERY
Typical
(Left) Frontal radiograph
shows severe consolidation
predominantly in the upper
lobes following intracranial
bleed while in the intensive
care unit. (Right) Frontal
radiograph shows mild
homogeneous consolidation
centrally with sparing of the
lung bases. Edema more
marked on the left.

4
Typical 69
(Left) Frontal radiograph
shows homogeneous
consolidation throughout the
upper lung zones (arrows).
Patchy atelectasis in the
bases was chronic. NPE due
to subarachnoid
hemorrhage. (Right) Frontal
radiograph shows
homogeneous consolidation
throughout the upper lung
zones (arrows) from
subarachnoid hemorrhage.

Variant
(Left) Frontal radiograph
shows homogeneous
consolidation throughout the
right lung from ruptured
intracranial aneurysm. NPE
may be unilateral, often the
right lung. (Right) Frontal
radiograph shows marked
interstitial edema with Kerley
B lines. Patient suffered
subarachnoid bleed from
ruptured aneurysm.
 PULMONARY ARTERY SARCOMA

Frontal graphic shows characteristic features of Axial CfCT shows a large mass filling the right
pulmonary artery sarcoma. Lobulated tumor involving pulmonary artery (arrows) and expanding its diameter.
the main pulmonary artery. There is direct extension of bulky tumor into the right
4 lower lobe.

70
ITERMINOlOGY I IMAGING FINDINGS
Abbreviations and Synonyms General Features
Intimal sarcoma Best diagnostic clue
o Pulmonary emboli unresponsive to standard
Definitions treatment
Malignancy of the wall of the pulmonary artery, or o Symptoms often nonspecific
other great vessel Location
Very rare o Central or peripheral pulmonary arteries
o Only 400 total reported in 2,000 involving o Other great vessels
pulmonary artery, aorta or inferior vena cava o Can also be in smaller vessels, capillaries
Diagnosis often delayed Size: Wide range, from 1-20 cm
o Misdiagnosis as pulmonary embolism common Morphology
o Leads to higher stage at diagnosis o Filling defect in pulmonary artery or other great
Tumors may arise in other great vessels of the vessel
mediastinum o Often dilates the involved vessel
o Inferior vena cava (IVC), most common site overall
o Superior vena cava (SVC) Radiographic Findings
o Azygos vein Radiography
o Aorta o Chest radiography often normal
o Pulmonary veins o May show peripheral pulmonary infarcts
o May show enlargement or lobularity of central
pulmonary artery
o Nodules, variable-sized

DDx: Pulmonary Artery Filling Defects

/~;'C::>.'
..
l ;~~,
- '"-: \
..
o " _ .. ,~ - 0
,. ... ,.~ .? . I

Emboli Metastasis (Hepatoma)

"'~''',~'
;/
Fibrosis
 PULMONARY ARTERY SARCOMA

Key Facts
Terminology Pathology
Malignancy of the wall of the pulmonary artery, or May arise from smooth muscle: Leiomyosarcoma
other great vessel May arise from mesenchymal cells of intima: Intimal
Very rare sarcoma
Can be radiation-induced
Imaging Findings
Pulmonary emboli unresponsive to standard Clinical Issues
treatment Tumors in pulmonary artery mimic pulmonary
Chest radiography often normal embolism
May show enlargement or lobularity of central Tumors in superior vena cava produce SVC syndrome
pulmonary artery Very poor prognosis
Often expands the diameter of the involved vessel If misdiagnosed as pulmonary embolism,
thrombolytic therapy may lead to life-threatening
Top Differential Diagnoses hemorrhage
Pulmonary Embolism
Hilar Adenopathy Diagnostic Checklist
Intravascular Metastases Look for enhancement in "clot"

CT Findings
Often accompanying symptoms of deep venous 4
thrombosis
CECT 71
o Filling defect in central or peripheral pulmonary Hilar Adenopathy
vessels May compress central pulmonary vessels
May show enhancement Should be visibly external to lumen of vessels
Generally unilateral May be calcified, if granulomatous
May extend beyond lumen
Often expands the diameter of the involved vessel Intravascular Metastases
Filling defect may be lobulated Renal cell carcinoma: Generally limited to right atrium
o If capillaries involved, may show mosaic perfusion and inferior vena cava
o Look for metastases, lung or bone most common Hepatoma
Melanoma
MR Findings Breast cancer
Multiplanar imaging can be important in delineating History important
extent of disease
Cine imaging may be useful in determination of Hilar Fibrosis
involvement of vital structures May be mass-like
May show enhancement with gadolinium within the May calcify
tumor Should decrease vessel diameter rather than expand
o Distinguishes this from bland thrombus or artery
embolism Look for other signs of granulomatous disease,
calcifications in other sites
Nuclear Medicine Findings
PET: Uptake within tumor demonstrating metabolic
activity I PATHOLOGY
Imaging Recommendations General Features
Best imaging tool Genetics: PS3 may play role in pathogenesis
o CTA Etiology
o Chest MR o May arise from smooth muscle: Leiomyosarcoma
Protocol advice Most often seen in tumors of superior and inferior
o Use of IV contrast essential vena cava, pulmonary veins
o May show enhancement within the tumor o May arise from mesenchymal cells of intima: Intimal
sarcoma
Undifferentiated
I DIFFERENTIAL DIAGNOSIS More common in elastic arteries, pulmonary
artery or aorta
Pulmonary Embolism Poorer prognosis than leiomyosarcomas
Do not typically fill entire lumen of central vessel o Can be radiation-induced
Generally bilateral After mantle radiation to mediastinum
Do not enhance Long latent period, up to 2S years
Rarely expand diameter of involved vessel Epidemiology
Generally have associated risk factors for emboli
 PULMONARY ARTERY SARCOMA
o Wide age range
o Median age approximately SO Natural History & Prognosis
Associated abnormalities Very poor prognosis
o Metastatic disease If misdiagnosed as pulmonary embolism, thrombolytic
therapy may lead to life-threatening hemorrhage
Lung
Brain Treatment
Bone Resection tumor
Pleura o Reconstruction of vessel wall or placement of
Gross Pathologic & Surgical Features conduit
Bulky tumors adherent to and filling lumen of vessel o May require pneumonectomy for central lesions
Stent for symptomatic relief
Microscopic Features Heart lung transplant for rare highly selected patients
Various subtypes of sarcoma
o Leiomyosarcomas make up about 20% of pulmonary
artery sarcomas I DIAGNOSTIC CHECKLIST
o Osteosarcoma
Most often seen in tumors arising in pulmonary Image Interpretation Pearls
artery Large filling defect in pulmonary artery
May calcify with osseous matrix May expand lumen
o Malignant fibrous histiocytoma Single unilateral lesion
o Spindle cell sarcoma Look for direct spread to adjacent structures,
o Myxoid sarcoma metastases
72 o Pleomorphic sarcoma Look for enhancement in "clot"
o Undifferentiated sarcoma
o Angiosarcoma: Rarest type in great vessels
More common in heart I SELECTED REFERENCES
Pulmonary capillary hemangiomatosis 1. Kerr KM:Pulmonary artery sarcoma masquerading as
o Low grade tumor of capillaries chronic thromboembolic pulmonary hypertension. Nat
o Leads to pulmonary arterial hypertension C1in Pract Cardiovasc Med. 1:108-12,2005
Tumors arising from pulmonary veins may show 2. Rizzo Eet al: Intimal sarcoma of pulmonary artery:
positive stains for hormone receptors multi-slice ECG-gated computed tomography findings with
3D reconstruction. EurJ Cardiothorac Surg. 27:919, 2005
3. Totaro M et al: Cardiac angiosarcoma arising from
pulmonary artery: endovascular treatment. Ann Thorac
I CLINICAL ISSUES Surg. 78:1568-70, 2004 .
4. Tsunezuka Yet al: Primary chondromatous osteosarcoma
Presentation of the pulmonary artery. Ann Thorac Surg. 77:331-4,2004
Most common signs/symptoms 5. Yi CA et al: Computed tomography in pulmonary artery
o Symptoms vary with site of origin sarcoma: distinguishing features from pulmonary embolic
o Tumors in pulmonary artery mimic pulmonary disease. J Comput AssistTomogr. 28:34-9, 2004
embolism 6. Yi ES:Tumors of the pulmonary vasculature. Cardiol C1in.
Dyspnea 22:431-40,2004
7. Croitoru AG et al: Primary pulmonary artery
Shortness of breath leiomyosarcoma. Cardiovasc Pathol. 12:166-9,2003
Chest pain 8. KimJH et al: Primary leiomyosarcoma of the pulmonary
Hemoptysis artery: a diagnostic dilemma. Clin Imaging. 27:206-11,
Fever 2003
o Tumors in superior vena cava produce SVC 9. Tschirch Fr et al: Angiosarcoma of the pulmonary trunk
syndrome mimicking pulmonary thromboembolic disease. A case
Dilated superficial veins report. Acta Radial. 44:504-7, 2003
10. Dennie CJ et al: Intimal sarcoma of the pulmonary arteries
Edema seen as a mosaic pattern of lung attenuation on
Headache high-resolution CT. AJR.178:1208-10,2002
Neck swelling 11. Mattoo A et al: Pulmonary artery sarcoma: a case report of
o Tumors in IVC can extend into liver surgical cure and 5-year follow-up. Chest. 122:945-7,2002
May produce Budd-Chiari syndrome 12. Talbot 5M et al: Combined heart and lung transplantation
o Tumors in pulmonary veins may mimic left heart for unresectable primary cardiac sarcoma. J Thorac
failure Cardiovasc Surg. 124:1145-8,2002
13. Thurer RLet al: FOG imaging of a pulmonary artery
Demographics sarcoma. Ann Thorac Surg. 70:1414-5,2000
Age: Median age SO 14. Oliai BRet a1:Leiomyosarcoma of the pulmonary veins.
Gender Am J Surg Pathol. 23:1082-8, 1999
o No sex difference for most sites 15. Mader MT et al: Malignant tumors of the heart and great
vessels: MR imaging appearance. Radiographies. 17:145-53,
o Tumors of pulmonary veins have female 1994
predominance
 PULMONARY ARTERY SARCOMA
I IMAGE GAllERY
Variant
(Left) Coronal CECT shows
tumor arising in the SVC,
filling and expanding the
lumen (arrows). There are
large collateral vessels in the
supraclavicular regions
(curved arrows). (Right)
Axial CECT shows same
patient as previous image,
with tumor filling and
expanding the SVC (white
arrow). There are enlarged
collateral vessels in internal
mammary (white curved
arrow), azygos (black
curved arrow) and
paravertebral areas (black
arrow).
4
73
(Left) Axial CECT shows
tumor invasion of the azygos
vein (arrow) and a large
mass posteriorly in the
pleural space. (Right) Frontal
radiograph shows
enlargement of the left main
and interlobar pulmonary
arteries (arrows) with
volume 1055 in the left lung.

Typical
(Left) Axial NECT in same
patient shows tumor within
the left main pulmonary
artery (arrows) with slight
expansion of the diameter of
the vessel. (Right) Axial
CECT shows an image at a
slightly lower pas Won than in
previous image, with tumor
filling the majority of the
lumen of the left main
pulmonary artery (arrows).
Inhomogeneity in tumor may
represent slight
contrast~enhancement.
 TUMOR EMBOLI, PULMONARY

Axial graphic shows varied findings for tumor emboli Coronal CECT shows hepatoma metastasis to right
from centrilobular nodules (arrow) to tree-in-bud atrium (arrow) and tumor embolus to left interlobar
opaciUes (open arrows). artery (open arrow).
4
74
I TERMI NOlOGY Radiographic Findings
Nonspecific appearance
Abbreviations and Synonyms o Normal radiograph, common
Non-thrombotic pulmonary embolism o Focal or diffuse heterogeneous opacities
Definitions Miliary disease
Occlusion of the pulmonary vasculature by tumor cells o May resemble lymphangitic carcinomatosis
Reticular interstitial thickening
o Cardiomegaly
I IMAGING FINDINGS o Pulmonary artery hypertension
Enlarged main and central pulmonary arteries
General Features with peripheral pruning
Best diagnostic clue: Normal chest radiograph with CT Findings
progressive hypoxia in patient with malignancy
CTA
Location o Large filling defects in main, lobar, segmental
o Variable: Large central to centrilobular pulmonary
pulmonary arteries
arteries
o Peripheral wedge-shaped areas of attenuation
Medium or small sized arteries, usual
Represent infarction
Size: CTA: Variable sized filling defects or vascular
o Filling defects or mass in right atrium or ventricle
beading
o Mosaic perfusion
Morphology: Intra-arterial metastases, no proliferation
Small vessel occlusion
within extravascular tissue
No air-trapping
CECT
o Normal CT, not uncommon

DDx: Pulmonary Artery Filling Defects

Sarcoma Pulmonary Embolism In-Situ Thrombosis

 TUMOR EMBOLI, PULMONARY
Key Facts
Terminology Top Differential Diagnoses
Occlusion of the pulmonary vasculature by tumor Pulmonary Embolism
cells Pulmonary Artery Sarcoma
Behcet Disease
Imaging Findings Infection
Best diagnostic clue: Normal chest radiograph with
progressive hypoxia in patient with malignancy Pathology
Dilated and beaded peripheral subsegmental Hepatocellular carcinoma that invades hepatic veins
pulmonary arteries Renal cell carcinoma that invades renal vein and
Tree-in-bud appearance inferior vena cava
Centrilobular nodules Angiosarcomas that involve systemic veins or right
Miliary pattern heart
Nodular attenuation surrounded by a halo of
ground-glass opacification Clinical Issues
Large filling defects in main, lobar, segmental Rarely identified prior to death
pulmonary arteries Prognosis: Grave
No imaging is pathognomonic

o Right heart enlargement

Imaging Recommendations
4
o Main pulmonary artery, > 3 cm
o Variant: Lung mass with invasion of pulmonary Best imaging tool 75
veins or left atrium o No imaging is pathognomonic
Systemic embolization to brain, extremities, o HRCT or ventilation perfusion imaging may suggest
kidneys diagnosis
HRCT o CTA for large vessel emboli
o Focal, multi focal, unilateral, bilateral Protocol advice
o Dilated and beaded peripheral subsegmental o Ventilation perfusion imaging
pulmonary arteries Macroaggregated albumin may further occlude
o Tree-in-bud appearance small vessels
Represents filling of centrilobular arteries with Potential for cardiac arrest due to right heart
tumor cells failure
o Centrilobular nodules Reduce number of particles to 100,000 to 200,000
Represent arteriolar involvement in patients with pulmonary hypertension
o Miliary pattern
Represents diffuse hematogenous intravascular
dissemination I DIFFERENTIAL DIAGNOSIS
o Nodular attenuation surrounded by a halo of
Pulmonary Embolism
ground-glass opacification
Increased frequency in patients with cancer
Ill-defined margins
Represents peritumoral hemorrhage Difficult to distinguish acutely
Most cases respond to treatment and do not progress
Angiographic Findings
Pulmonary Artery Sarcoma
Poor sensitivity and specificity; rarely performed
Pruning and tortuosity of 3rd to 5th order arteries Solid intraarterial lobulated filling defect/mass
Delayed filling of the segmental pulmonary arteries May enhance with intravenous contrast
Subsegmental pulmonary artery filling defects May expand artery
Large filling defects in main, lobar, segmental Behcet Disease
pulmonary arteries Pulmonary artery aneurysms with in-situ thromboses
Cytology of blood aspirated from wedged Swan Ganz
catheter Infection
Variant: Bronchogenic carcinoma with systemic Tree-in-bud appearance usually due to small airways
arterial tumor emboli bronchiolitis
o Filling defect or occlusion of medium sized or small Mosaic attenuation with air trapping on expiration
arteries No pulmonary artery hypertension
Nuclear Medicine Findings CT Halo Sign
V/Q Scan Invasive aspergillosis
o Ventilation perfusion mismatched defects o Usually hematopoietic malignancy, not solid organ
Multiple small peripheral subsegmental perfusion o Neutropenic, usually febrile patient
defects o Fulminant, rapid progression
Visualization of the fissures Candidiasis
 TUMOR EMBOLI, PULMONARY
o Intensive care patients, sepsis
o Usually on broad spectrum antibiotics with central Microscopic Features
lines Involves multiple levels of the microscopic pulmonary
Wegener granulomatosis vasculature
o Renal failure, midline lethal granulomatosis o From the elastic arteries down to the alveolar septal
o May have multiple cavitary nodules capillaries
Tuberculoma and hemoptysis Thrombotic microangiopathy of pulmonary tumors
o Upper lobe location o Extensive fibrocellular intimal hyperplasia of small
Bronchioloalveolar cell carcinoma pulmonary arteries
o Airways involvement, not intravascular o Initiated by tumor microemboli
o No extra pulmonary source for emboli Progressive and irreversible obstruction of the vascular
bed
Metastases with Extravascular Involvement Peri tumoral halos: Hemorrhage due to rupture of
HRCf or CTA to show pulmonary, lymphatic or fragile vessels
airway spread
Treatment-Related Pulmonary Disease ICLlNICAL ISSUES
Hypersensitivity lung disease
o May be secondary to chemotherapeutic drugs, Presentation
especially methotrexate Most common signs/symptoms
o Centrilobular fuzzy nodules o Rarely identified prior to death
4 o Diffuse ground-glass opacities o Tumor thrombi can produce a subacute and
o Responds to steroids progressive clinical picture
76 Cryptogenic organizing pneumonia Other signs/symptoms
o Airways abnormality o Progressive dyspnea, cough
o Small centrilobular nodules o Chest and abdominal pain
o Peripheral wedge-shaped opacities that may o Hypoxia
resemble infarcts o Acute right heart failure
o Responds to steroids o Progressive cor pulmonale over weeks or months
o Ascites

I PATHOLOGY Demographics
Age: Childhood to elderly
General Features Gender: M:F
Etiology
o Occlusion of the pulmonary microvasculature by
Natural History & Prognosis
tumor cells Prognosis: Grave
o Primary tumors include o Dependent on response to chemotherapy and
Hepatoma anticoagulation
Renal cell carcinoma Treatment
Angiosarcoma Diagnosis: Cytology of blood aspirated from wedged
Breast Swan Ganz catheter
Gastric carcinoma
Bronchogenic carcinoma
Prostatic carcinoma I SElECTED REFERENCES
Pancreatic carcinoma
Osteosarcoma, chondrosarcoma 1. Han D et al: Thrombotic and non thrombotic pulmonary
Choriocarcinoma arterial embolism: spectrum of imaging findings.
Radiographies. 23(6):1521-39, 2003
Thyroid carcinoma 2. Roberts KEet al: Pulmonary tumor embolism: a review of
o Occurs with tumors that tend to invade systemic the literature. Am) Med. 15;115(3):228-32,2003
veins 3. Seo)B et al: Atypical pulmonary metastases: spectrum of
Hepatocellular carcinoma that invades hepatic radiologic findings. Radiographies. 21(2):403-17, 2001
veins 4. Kim AEet al: Pulmonary tumor embolism presenting as
Renal cell carcinoma that invades renal vein and infarct on computed tomography.) Thorac Imaging.
inferior vena cava 14:135-137,1999
Angiosarcomas that involve systemic veins or 5. Moores LKet al: Diffuse tumor microembolism: a rare
cause of a high-probability perfusion scan. Chest.
right heart 111:1122-1125, 1997
Epidemiology: 2-26% of autopsies 6. Shepard)A et al: Pulmonary intravascular tumor emboli:
dilated and beaded peripheral pulmonary arteries at CT.
Gross Pathologic & Surgical Features Radiology. 187:797-801, 1993
Tumor thromboemboli, from large central to small 7. Chan CK et al: Pulmonary tumor embolism: a critical
peripheral arteries review of clinical, imaging, and hemodynamic features.)
Thorac Imaging. 2:4-14,1987
 TUMOR EMBOLI, PULMONARY

I IMAGE GAllERY
Typical
(Lefl) Axial CECT in a patient
with renal cell carcinoma
with renal vein involvement
shows small peripheral
tumor embolus, left lower
lobe (arrow). (RighI) Axial
CECT in same patient as
previous image shows
tree-in-bud tumor emboli
(arrow).

4
Typical 77
(Left) Axial CECT in a patient
with right hilar lung cancer
shows centrilobular nodules
(arrows) that represent
tumor emboli. (RighI) Axial
H RCT in a patient with right
atrial rhabdomyosarcoma
shows innumerable small
tumor emboli. Open arrow
shows tree-in-bud
appearance. Ground-glass
halos (arrows) represent
hemorrhage due to fragile
neovascular tissue. Halos are
a/50 seen with
choriocarcinoma and
osteosarcoma.

Variant
(Left) Anteroposterior DSA
shows thrombosis of the
right brachial artery (arrow).
During archery the patient
hit his antecubital f055a with
the string and presented with
a cold arm. Embolectomy
showed tumor cells in the
thrombus. (RighI) Frontal
radiograph in same patient
as previous image shows
right upper lobe
bronchogenic carcinoma
(arrow). Diagnosis: Systemic
tumor embolus.
 CAPILLARY HEMANGIOMATOSIS, PULMONARY

~;r~~-.
-'

Axial HRCT prone image shows septal thickening Coronal HRCT shows septal thickening (arrow) and
(arrow) and ground-glass opacity consistent with ground-glass opacity consistent with edema. Main and
edema. rightpulmonary arteriesare enlarged (open arrows).

78
o Cardiomegaly (90%)
ITERMINOlOGY o Pleural effusion, small (75%)
Abbreviations and Synonyms o Nonspecific basilar interstitial abnormalities
Pulmonary capillary hemangiomatosis (PCH) CT Findings
Definitions HRCT
Described in ] 978 by Wagenvoort o In addition to radiographic abnormalities
Rare idiopathic disorder that causes pulmonary o Ground-glass opacities (85%)
hypertension due to lung invasion by proliferation of Poorly defined centrilobular ground-glass nodular
thin-walled alveolar capillaries opacities (70%)
o Thickened interlobular septa (60%), may be smooth
or nodular
IIMAGING FINDINGS o Pericardia! effusion (75%)
o Mild lymphadenopathy, average ]2 mm (70%)
General Features
Imaging Recommendations
Best diagnostic clue: CT findings of pulmonary artery
Best imaging tool: HRCT important to separate PCH
hypertension and a diffuse centrilobular nodular
from primary pulmonary hypertension
pattern
Location: Diffuse no predominant pulmonary
parenchymal location
I DIFFERENTIAL DIAGNOSIS
Radiographic Findings
Pulmonary Veno-Occlusive Disease (PVOD)
Radiography
a Enlarged pulmonary arteries (100%) Identical radiographic findings; some investigators
view PCH as sequela of PVOD

DDx: PCH Mimics

Pulmonary Hypertension PVOD (Courtesy HP. McAdams)

 CAPILLARY HEMANGIOMATOSIS, PULMONARY
Key Facts
Terminology Mild lymphadenopathy, average 12 mm (70%)
Rare idiopathic disorder that causes pulmonary Top Differential Diagnoses
hypertension due to lung invasion by proliferation of Pulmonary Veno-Occlusive Disease (PVOD)
thin-walled alveolar capillaries
Primary Pulmonary Hypertension (PPH)
Imaging Findings Chronic Pulmonary Thromboemboli
Best diagnostic clue: CT findings of pulmonary artery Clinical Issues
hypertension and a diffuse centrilobular nodular
Median survival three years from diagnosis
pattern
Pericardial effusion (75%)

Lacks capillary invasion

ICLINICAl ISSUES
Primary Pulmonary Hypertension (PPH)
Presentation
Primarily young women
Finding portending poor response to epoprostenol Most common signs/symptoms: Dyspnea, hemoptysis;
(prostacyclin) therapy (more suggest of PVOD or PCH) occasionally asymptomatic
o Centrilobular ground-glass opacities, septal lines, Other signs/symptoms: Stigmata of pulmonary
pleural effusions, lymphadenopathy,
effusion
pericardial hypertension
4
Demographics
Age: Range 6-7] years, most commonly 20-40 years 79
Chronic Pulmonary Thromboemboli
Chronic emboli can be identified at CTA Gender: No gender predominance
Centrilobular nodules, septal lines and pleural Natural History & Prognosis
effusions all iess common Typically develop progressive pulmonary hypertension
Succumb to respiratory failure or hemoptysis
Median survival three years from diagnosis
I PATHOLOGY
Treatment
General Features Steroids, cytotoxic agents ineffective
Genetics: Unknown but one report of a familial cluster Alpha-interferon may be useful in some cases
Etiology: Unknown but may be associated with use of Lung transplantation efficacious
oral contraceptives o Single lung transplantation or pneumonectomy can
Associated abnormalities: Secondary hemothorax be performed if the disease is limited to one lung
Microscopic Features
Proliferation of thin-walled capillaries with iung
I SELECTED REFERENCES
invasion
Secondary obstruction of pulmonary venules causing 1. Lawler LP et al: Pulmonary capillary hemangiomatosis:
pulmonary hypertension multidetector row CT findings and clinico-pathologic
correlation. J Thocac Imaging. 20(1):61-3, 200S

I IMAGE GALLERY

(Left) Axial HRCT shows a small to moderate right and minimal left pleural effusion (arrows). Enlarged main pulmonary artery (open arrow).
(Center) Axial HRCT shows septal thickening (open arrow) and some groundglass opacity consistent with edema. Small left pleural effusion
(arrow). (Courtesy P. Stark, MO). fRight) Axial HRCT shows multiple centrilobular nodules (arrows) in a 26 year woman with pulmonary
capillary hemangiomatosis. (Courtesy E. Pallisa, MO).
 APICAL LUNG HERNIA
1
2

Coronal oblique graphic shows a defect in Sibson fascia, Frontal radiograph shows an apical lung hernia
aI/owing herniation of right lung apex into neck. extending into right neck (arrow).

ITERMINOlOGY Imaging Recommendations

Abbreviations and Synonyms Best imaging tool
o Fluoroscopy
Cervical lung hernia, cervical pneumatocele
Reducibility of hernia can be assessed dynamically
Definitions Hernia worsens with Valsalva maneuver, cough,
Protrusion of lung apex into neck through defect in maximum inspiration or forced expiration
Sibson fascia, which covers lung cupola Protocol advice
o Conventional CT protocols demonstrate apical lung
hernia well
I IMAGING FINDINGS o Coronal & sagittal CT reformations show anatomy
of apical hernia better
General Features
Best diagnostic clue: Gas-filled mass that changes with
inspiration & expiration, bulging into neck I DIFFERENTIALDIAGNOSIS
Location
o Typically unilateral, but can be bilateral Paratracheal Air Cyst
o Six times more common on right Mucosal herniation through tracheal wall
Size: Can enlarge to 10 cm Apical Bulla
Morphology: Lung protruding into neck, usually with
Sharply defined air space ~ 1 cm with thin wall
constriction at hernia aperture
Can cause tracheal deviation or subclavian venous Zenker Diverticulum
obstruction Posterior herniation of mucosa & submucosa through
defect in inferior constrictor pharyngeal muscle

DDx: Apical Lucencies

Paratracheal Air Cyst Zenker Diverticulum

 APICAL LUNG HERNIA

Key Facts
1
Imaging Findings Killian-Jamieson Diverticulum 3
Best diagnostic clue: Gas-filled mass that changes Pathology
with inspiration & expiration, bulging into neck Defect allows lung to protrude between scalenus
Top Differential Diagnoses anticus & sternocleidomastoid muscles
Para tracheal Air Cyst Clinical Issues
Apical Bulla Most common signs/symptoms: Asymptomatic
Zenker Diverticulum t Risk of pneumothorax with placement of central
Lateral Pharyngeal Diverticulum venous catheter or tracheostomy
Laryngocele

Lateral Pharyngeal Diverticulum
Mucosal herniation through thyrohyoid
Laryngocele
Dilated appendix of laryngeal ventricle
Killian-Jamieson Diverticulum
Anterolateral outpouching of cervical esophagus just
below cricopharyngeus muscle
I PATHOLOGY
General Features
General path comments: Apical hernia: 1/3 of lung
hernias, most lung hernias intercostal
Genetics: Can be familial
Etiology: Usually congenital in children & acquired in
adults, some cases are post-operative
Epidemiology: Very rare condition
Gross Pathologic & Surgical Features
Sibson fascia (suprapleural membrane)
o Endothoracic fascia covering lung cupola
Defect allows lung to protrude between scalenus
anticus & sternocleidomastoid muscles
I CLINICAL ISSUES
Presentation
Most common signs/symptoms: Asymptomatic
Other signs/symptoms
o Cough, dyspnea, dysphagia & hoarseness
membrane o Soft, bulging mass in supraclavicular region
Clinical Profile
o Asthma, emphysema or chronic bronchitis
o Weight lifter, wind instrument player
Demographics
Age: Can present in childhood or adulthood
Gender: M:F; 2:1
Natural History & Prognosis
Many apical hernias resolve without treatment
Treatment
Antitussives as necessary, surgery usually unnecessary
Surgery may be appropriate for incarcerated or
symptomatic hernias or for cosmesis
t Risk of pneumothorax with placement of central
venous catheter or tracheostomy
I SELECTED REFERENCES
1. Gupta M et al: Lung hernia after en bloc cervicothoracic
resection. J Thorac Cardiovasc Surg. 130(2):607-8, 2005
2. McAdams HP et al: Apical lung hernia: radiologic findings
in six cases. AJR Am J Roentgenol. 167(4):927-30, 1996
3. Moncada R et al: Congenital and acquired lung hernias. J
Thorac Imaging. 11(1):75-82, 1996
4. Bhalla M et al: Lung hernia: radiographic features. AJR Am
J Roentgenol. 154(1):51-3, 1990
5. Grunebaum Met al: Protrusion of the lung apex through
Sibson's fascia in infancy. Thorax. 33(3):290-4, 1978

I IMAGE GALLERY

(Left) Frontal radiograph magnification view shows an apical hernia extending into right neck. (Center) Frontal radiograph magnification view
during Valsalva maneuver in same patient demonstrates ballooning of apical lung hernia. (Right) Axial CECT shows small right apical lung hernia
(arrow). Other images showed continuity of hernia with remainder of right/ung.
 YELLOW-NAIL SYNDROME
1
4

Frontal radiograph shows large right pleural effusion Axial NEeT shows loculated moderate-sized left pleural
(arrows) in patient with yellow nail syndrome. Denver effusion (arrows) and subtle bronchiectasis (open
shunt had been placed to chronically drain effusion. arrow) in yellow nail syndrome.
Subsegmental atelectasis left lower lobe (open arrow).

I TERMINOlOGY Other Modality Findings

Paranasal sinuses: Chronic sinusitis
Definitions
Triad of slow-growing dystrophic yellow nails,
lymphedema, and pleural effusions
o Often associated with pericardial effusion,
I DIFFERENTIALDIAGNOSIS
rhinosinusitis, and bronchiectasis Dermatologic Manifestations of Pulmonary
Disease
[IMAGING FINDINGS Clubbing: Congenital heart disease and chronic
pleuropulmonary disease
Radiographic Findings Hypertrophic pulmonary osteoarthropathy: Lung
Radiography cancer or lung abscess
o Chronic pleural effusions (60%) Sarcoidosis: Lupus pernio (violaceous indurated
Unilateral or bilateral, small to moderate in size plaques on nose and face)
Often recur after initial drainage Pulmonary arteriovenous malformations (hereditary
o Lungs usually normal, may have recurrent hemorrhagic telangiectasias): Telangiectasias
pulmonary infections and bronchiectasis widespread but characteristically oral and nasal
Fat embolism: Nondependent petechia
CT Findings Churg-Strauss vasculitis: Palpable purpura
HRCT Relapsing polychondritis: Saddle nose deformity,
o Bronchiectasis (40%): Often associated with sinusitis deformity cartilaginous ear
o Rarely pulmonary cysts Alpha-l antitrypsin deficiency: Ulcerative panniculitis

DDx: Chronic Pleural Effusions

Rheumatoid Arthritis Asbestos Effusion Lymphangiomyomatosis

 YELLOW-NAIL SYNDROME

Key Facts 1
Terminology Bronchiectasis (40%): Often associated with sinusitis 5
Triad of slow-growing dystrophic yellow nails, Paranasal sinuses: Chronic sinusitis
lymphedema, and pleural effusions Pathology
Often associated with pericardia I effusion, Etiology: Impaired lymphatic drainage
rhinosinusitis, and bronchiectasis .
Clinical Issues
Imaging Findings
Nails: Excessively curved from side-to-side, thick and
Chronic pleural effusions (60%)
dystrophic, pale yellow to green in color, growth rate
Unilateral or bilateral, small to moderate in size 50% of normal
Often recur after initial drainage

Symptoms predate diagnosis by 10 years

Chronic Pleural Effusions o Nails: Excessively curved from side-to-side, thick and
Rheumatoid arthritis dystrophic, pale yellow to green in coJor, growth
o More common in men rate 50% of normal
o Typically low in glucose o Yellow nail triad (2 of 3 required for diagnosis)
o May also have bronchiectasis Yellow nails 90%
Asbestos effusion Lymphedema 80% (usually the first
o Earliest manifestation of asbestos exposure manifestation)
o Effusion typically resolves after 6 months Pleuropulmonary symptoms 60%
o May be blood tinged o Lymphedema: Symmetrical, non pitting and mild -
Lymphangiomyomatosis primarily of the lower extremities
o Young women child-bearing age Other signs/symptoms: Pleural effusion exudative:
o Diffuse cystic lung disease, not bronchiectasis May be chylous, typically high percentage of
o Chylous effusions lymphocytes
Demographics
I PATHOLOGY Age: Median age at presentation 40-50 years of age
(range birth to geriatrics)
General Features Gender: Females slightly more common (1.5:1)
Etiology: Impaired lymphatic drainage
Natural History & Prognosis
Microscopic Features May be reversible
Pleura: Lymphocytic pleuritis associated with
moderate fibrosis and dilated lymphatics Treatment
No specific treatment: Recurrent pleural taps,
pleurodesis, pleuroperitoneal shunts and rarely
ICLINICAL ISSUES pleurectomy

Presentation
Most common signs/symptoms I SELECTED REFERENCES
o Pleuropulmonary involvement leads to shortness of 1. Alkadhi H et al: Yellow nail syndrome. Respiration.
breath and productive cough 72(2):197,200S

IIMAGE GALLERY

(Left) Axial NECT shows loculated left pleural effusion (arrows) and bilateral pleural thickening (curved arrows) in yellow nail syndrome.
Hypertrophy extrapleural fat (open arrows) common with chronic pleural thickening. (Center) Axial NECT shows left pleural effusion and pleural
thickening right hemithorax (arrows). Subsegmental bronchi are thickened and slightly dilated (open arrows). (Right) Axial NECT shows bilateral
pleural effusions, moderate in size on the left and small on the right (arrows). Pericardium is normal (open arrow).
 EXUDATIVE PLEURAL EFFUSION
1
6

Frontal radiograph shows sharply marginated large left Axial CECT in same pauent shows loculated pleural fluid
pleural based opacity (open arrow) with obtuse angle with split pleura sign (arrow). Consolidated lung
with chest waif (curved arrow). (curved arrow). Pleural fluid showed Actinomycosis.

I TERMINOlOGY CT Findings
Pleural fluid: Water to high attenuation, homogeneous
Definitions
or heterogeneous attenuation
Pleural inflammation or pleurisy and/or lymphatic o CECT: May not differentiate between transudative,
obstruction exudative, and chylous effusions
Increased permeability of the pleural surface with o Fluid -fluid level: Dependent layering of high
accumulation of proteinaceous pleural fluid attenuation contents suggests acute hemorrhage
o Loculated effusion: Lenticular configuration,
smooth margins, homogeneous attenuation,
IIMAGING FINDINGS displaces adjacent lung
General Features Pleura: Surfaces may be smooth thin, uniform
o Malignant pleural disease: CT may not differentiate
Best diagnostic clue: Loculated pleural thickening
benign from malignant effusions
Morphology: CT: High attenuation fluid, thick pleural
Pleural thickening: Circumferential, nodular,
rind, nodularity, loculation, septations
mediastinal pleural involvement
Radiographic Findings Parietal pleural thickening> 1 cm
Radiography o Empyema: Elliptical with smooth inner surface
o Radiography: Appearance identical to transudative Parietal and visceral pleural enhancement (split
effusion pleura sign)
o Decubitus views useful to show free flow or Sharply defined border between empyema and
loculation lung
o Air-fluid level: Consider bronchopleural fistula, Bowing of vessels and bronchi away from
Boerhaave syndrome, trauma, empyema empyema

DDx: Exudative Effusion

Pancoast Tumor Transudates

 EXUDATIVE PLEURAL EFFUSION

Key Facts
1
Terminology Malignant pleural disease: CT may not differentiate 7
Increased permeability of the pleural surface with benign from malignant effusions
accumulation of proteinaceous pleural fluid Empyema: Elliptical with smooth inner surface
Parietal and visceral pleural enhancement (split
Imaging Findings pleura sign)
Radiography: Appearance identical to transudative
effusion
Top Differential Diagnoses
Air-fluid level: Consider bronchopleural fistula, Transudative Effusion
Boerhaave syndrome, trauma, empyema Fibrothorax
CECT: May not differentiate between transudative, Primary or Metastatic Pleural Disease without
exudative, and chylous effusions Effusion
Fluid -fluid level: Dependent layering of high Diagnostic Checklist
attenuation contents suggests acute hemorrhage Empyema requires early chest tube drainage; lung
Loculated effusion: Lenticular configuration, smooth abscess treated with antibiotics
margins, homogeneous attenuation, displaces
adjacent lung

Lung: Assess for abscess, tumor, bronchopleural fistula,

pulmonary embolism
I DIFFERENTIAL DIAGNOSIS
o Abscess: Round, relatively thick wall Transudative Effusion
Lack discrete boundary between lesion and lung CT: No pleural thickening or nodularity; diagnosis by
No displacement of bronchi or vessels away from pleural fluid analysis
lesion
o Encased small lung with mesothelioma Fibrothorax
Extrapleural fat: Empyema: Thickening of extra pleural No fluid with either CT or ultrasound
subcostal tissues, including fat
o Soft tissue stranding or infiltration of extra pleural Primary or Metastatic Pleural Disease
fat, seen with infection or tumor without Effusion
Mediastinum: Assess for lymphadenopathy, CT: Solid enhancing pleural lesions
mediastinitis, esophageal perforation
o Cardiac surgery or myocardial infarction
o Mediastinal infiltration suggests tumor or infection I PATHOLOGY
Chest wall involvement: Assess for extension of tumor
or infection (empyema necessitans) General Features
Subdiaphragmatic: Assess for associated subphrenic or General path comments
liver abscess, tumor, pancreatitis o Exudative effusion
Ratio of pleural fluid and serum protein levels>
Fluoroscopic Findings 0.5
Esophagram: To assess for perforation Ratio of pleural fluid and serum lactate
Nuclear Medicine Findings dehydrogenase (LDH) levels> 0.6, and
PET: In patients with known extra pleural malignancy, Pleural fluid LDH level> 2/3 of the upper limit for
uptake in fluid or pleura suggests neoplasm serum LDH levels
Etiology
Ultrasonographic Findings o Neoplastic: Metastases, (lung, breast, lymphoma,
Grayscale Ultrasound ovarian, gastric) mesothelioma, lymphoma
o May not distinguish transudate from exudate when o Infectious: Para pneumonic effusion
fluid is anechoic Bacterial: Anaerobic, aerobic, mixed pathogens,
Exudate: Echogenic, loculated fluid, with actinomycosis
septations and pleural thickening Viral, mycoplasma
o Assess for subdiaphragmatic cause, subphrenic or Tuberculosis, fungal: Coccidioidomycosis,
liver abscess, pancreatitis aspergillus, mucor, blastomycosis
o To guide thoracentesis puncture site Parasites: Amebiasis, echinococcus
o Collagen vascular disease: Rheumatoid arthritis,
Imaging Recommendations systemic lupus erythematosus (SLE)
Best imaging tool: CECT: To assess pleura, lung, o Drug induced SLE (hydralazine, procainamide,
mediastinum isoniazid, phenytoin, and chlorpromazine)
Protocol advice: Administer contrast to show split o Drug induced (nitrofurantoin, dantrolene,
pleura sign, tumor enhancement bromocriptine and other dopamine agonists,
amiodarone, interleukin-2)
 EXUDATIVE PLEURAL EFFUSION
1 o Pulmonary embolism
o Benign asbestos effusion
8 o Meigs syndrome: Ovarian fibroma, ascites, pleural
effusion
o Injury to thoracic duct, traumatic or neoplastic
(lymphoma)
o Post-cardiac surgery or myocardial infarction
(Dressler syndrome)
o Trauma with hemothorax
o Uremic pleuritis
o Yellow nail syndrome: Rhinosinusitis, pleural
effusions, bronchiectasis, lymphedema, yellow nails
o GI diseases: Perforated esophagus, pancreatic
disease, subdiaphragmatic abscess
Epidemiology: Pulmonary embolism: 3/4 have
exudative effusions; suggests pulmonary infarction
Gross Pathologic & Surgical Features
Fluids in pleural space: Blood, pus, chyle, exudate,
neoplastic
Microscopic Features
Immunohistochemistry and electron microscopy to
differentiate mesothelioma from adenocarcinoma
Para pneumonic effusion, neutrophils and bacteria;
mycotic pleurisy, granulomas
Chylothorax: High lipid content (neutral fat, fatty
acids); low cholesterol
o Sudanophilic fat droplets; triglyceride level> 110
mg/dL
Cholesterol effusion: Cholesterol crystals, up to 1
gm/dL; low neutral fat and fatty acids
IClINICAllSSUES
Presentation
Most common signs/symptoms: Fever, dyspnea on
exertion, chest pain, asymptomatic
Other signs/symptoms
o Post cardiac injury syndrome: (Dressler syndrome)
After myocardial infarction, cardiac surgery, blunt
chest trauma, pacemaker implantation, or
angioplasty
Fever, pleuropericarditis, unilateral or bilateral
small to moderate effusions, lung opacities
Manifests weeks after event
o High antinuclear antibody, rheumatoid factor titer,
cholesterol crystals
o Low glucose, pH, complement
Low glucose < 60 m/dL: Tuberculosis (TB),
malignancy, rheumatoid, empyema, hemothorax, SLE
Pleural fluid features
o Bloody: Trauma, anticoagulation, iatrogenic,
metastases, uremia
o Blood-tinged: Metastases, mesothelioma, benign
asbestos effusion, pulmonary embolism,
tuberculosis, pancreatitis
o Milky: Chylous
o Brown: Amebic abscess
o Black: Aspergillus
o Yellow-green: Rheumatoid; golden, iridescent:
Chronic chylothorax, tuberculosis, rheumatoid
o Opaque: Mesothelioma, chronic empyema; putrid
odor: Anaerobic infection
Thoracentesis: Always indicated if suspect exudative
effusion
o Fluid for chemical, bacteriologic, and cytologic
examination
o Large effusions: Relieves dyspnea; remove < 1,000
mLat a time
CT or ultrasound guided pleural biopsy
o Core sample required for mesothelioma; fine needle
aspiration for metastatic lesions
Open biopsy or video-assisted thoracic surgery (VATS)
often needed to establish the diagnosis
Treatment dependent on underlying etiology
o Antibiotics, thoracentesis, chest tube drainage,
chemotherapy, surgery, steroids
Chest tube placement
o For empyema, hemothorax, large malignant
effusions, for pleurodesis or fibrinolysis
Para pneumonic effusion: pH < 7.0 glucose < 40
mg/dL represents empyema
Placement of chest tubes for empyema indicated
early in course of infection
Without drainage fibrothorax may be seen as early
as 7 days from onset of empyema
May require decortication of fibrothorax
o Drainage not indicated for tuberculous effusion;
indicated for tuberculous empyema
o Pleural blood does not clot, can usually be
withdrawn easily

Demographics
Age: Usually in adults I DIAGNOSTIC CHECKLIST
Gender: Male predominance: Rheumatoid arthritis,
pancreatitis Image Interpretation Pearls
Important to distinguish empyema from lung abscess
Natural History & Prognosis o Empyema requires early chest tube drainage; lung
Malignant pleural effusion, life expectancy 3-6 months abscess treated with antibiotics
Benign asbestos effusion manifests 5 to > 30 years after
exposure
Treatment
I SELECTED REFERENCES
Exudative effusions may show 1. Kuhlman JE et al: Complex disease of the pleural space:
radiographic and CT evaluation. Radiographies.
o High amylase, red blood cell (RBC), LDH level,
17(1):63-79, ]997
lymphocytes, neutrophil, eosinophil, or plasma cell 2. Muller NL: Imaging of the pleura. Radiology 186:297-309,
counts 1993
 EXUDATIVE PLEURAL EFFUSION

I IMAGE GALLERY 1
9
Typical
(Left) Frontal radiograph
shows right pleural base
opacity. Note obtuse angle
with chest wall and blunted
coslophrenic sulcus
(arrows). (Right) Axial CECT
in same patient shows right
pleural effusion, with smooth
thin pleural line (arrows).
Appearance resembles
transudate. Thoracentesis
showed empyema.

Typical
(Left) Frontal radiograph
shows opacification at the
left lower hemothorax and
multiple air-fluid levels
(arrows). (Right)
Esophagram in same patient
shows extravasation of
barium into left pleural space
(arrow). Boerhaave
syndrome.

Typical
(Left) Axial CECT shows
large left effusion with
dependent high attenuation
(arrow). Mediastinal pleura
thickened (curved arrow)
indicating malignant
effusion. Fluid cytology,
adenocarcinoma. (Right)
Axial CECT in a patient on
anticoagulants shows
heterogeneous attenuation of
large right pleural fluid
collection (arrow). Chronic
hemothorax.
 APICAL PLEURAL CAP
1
10

Apical caps (arrows) are common and a normal Frontal radiograph magnified view right apex shows
process of aging. Apical caps must be differentiated apical cap a few mm thick (arrows). Margins are sharp
from other more significant pathology. and slightly undulating. No rib destruction.

ITERMINOLOGY I DIFFERENTIALDIAGNOSIS
Definitions Pancoast Tumor (Superior Sulcus Tumor)
Radiographic term used to describe pleural thickening From bronchogenic carcinoma
at the lung apex May have rib destruction (causing pain) or Horner
syndrome (involvement of sympathetic ganglia)
Inferior margin may be smooth or indistinct
IIMAGING FINDINGS Tuberculosis
Radiographic Findings Cicatricial scarring, often containing small calcified
Radiography nodules with hilar retraction due to atelectasis
o Sharp, smooth or undulating margin usually < 5 mm Hypertrophy of extra pleural fat common at CT
thick
Radiation Fibrosis
o Bilateral (12%) more common than unilateral (10%)
Right (22%) more common than the left (17%) Lung apex included in field for head and neck cancers,
Hodgkin lymphoma, breast carcinoma (supraclavicular
CT Findings therapy)
NECT Pleural Effusion (Supine)
o Nonspecific findings: Irregular soft tissue opacities
and pleural thickening at lung apex Lung apex most dependent portion of the pleural
o Extrapleural fat often hypertrophied space in supine position
o May have paracicatricial emphysema adjacent to Aortic Transection
abnormal lung Blood from transection dissects along subclavian
artery producing a cap

DDx: Apical Cap

Pancoast Tumor Supine Pleural Effusion Aspergillomas

 APICAL PLEURAL CAP

Key Facts
1
Terminology Tuberculosis 11
Radiographic term used to describe pleural thickening Radiation Fibrosis
at the lung apex Pleural Effusion (Supine)

Imaging Findings Pathology

Sharp, smooth or undulating margin usually < 5 mm Epidemiology: Incidence increases with age, age 40:
thick 5% age 70: 50%

Top Differential Diagnoses Clinical Issues

Pancoast Tumor (Superior Sulcus Tumor) Normal process of aging

Unusual as only radiographic finding of transection Epidemiology: Incidence increases with age, age 40:
5% age 70: 50%
Aspergillus Superinfection
New pleural thickening adjacent to pre-existing Gross Pathologic & Surgical Features
cavitary disease Lung apex normally covered by thicker fascia (Sibson
Aspergilloma may not be radiographically evident fascia)
(seen at CT) Overall apical cap similar histologically to pulmonary
infarct
Lipoma or Extrapleural Fat
Hypertrophy or excess deposition of extra pleural fat Microscopic Features
Usually bilateral, enlarged body habitus Hyaline fibrosis of visceral pleura (identical to pleural
Fat density at CT plaque) 50%
Lung collapsed (elastic skeleton contracted like an
Peripheral Upper Lobe Collapse accordion) and filled with collagen
More common in neonates Cicatricial emphysema may be seen adjacent to the
Represents collapse of the apical - posterior segments scar
with sparing of the anterior segment
Pleural Metastases or Mesothelioma
Uncommon location as only site, malignant thymoma
I CLINICAL ISSUES
may spread extrapleurally around lung apex Presentation
Most common signs/symptoms: Asymptomatic
radiographic abnormality
I PATHOLOGY
Natural History & Prognosis
General Features Normal process of aging
Etiology
o Chronic ischemia
Normally pulmonary artery pressure just sufficient I SElECTED REFERENCES
to get blood to lung apex
With aging, apex becomes ischemic leading to 1. Yousem 5A: Pulmonary apical cap: a distinctive but poorly
recognized lesion in pulmonary surgical pathology. Am J
pleural-parenchymal fibrosis 5urg Pathol. 25(5):679-83, 2001

I IMAGE GALLERY

(Left) Frontal radiograph shows bilateral apical caps (arrows) slightly thicker on the right. Chest radiograph was otherwise normal. (Center) Axial
CECT shows inhomogeneous 50ft tissue opacity at the left apex (arrow). Discrete reticular opacities at the edge of the opacity (open arrow).
Apical cap. (Right) Axial CECT shows that apical cap is primarily composed of extrapleural fat (arrow).
 SYSTEMIC LUPUS ERYTHEMATOSUS, PULMONARY
1
12

Frontal radiograph shows diffuse relicular and nodular Axial HRCT shows pleural thickening/small effusions of
interstitial lung disease, more prominent at the bases, SLE,right> left, with some calcium deposition farrows).
typical of systemic lupus erythematosus fSLE). There is also a small amount of subpleural fal deposilion
(open arrow).

ITERMINOLOGY Radiographic Findings

Radiography
Abbreviations and Synonyms
o Pleural effusion or pleural thickening 50%
Systemic lupus erythematosus (SLE), lupus, lupus Usually small, either unilateral or bilateral
erythematosus (LE) o Consolidation
Definitions Pneumonia (conventional or opportunistic)
Chronic collagen vascular disease Alveolar hemorrhage
o May manifest as cough, dyspnea and pleuritic chest Acute lupus pneumonitis (1-4%)
pain Infarcts from thromboembolism
o Thoracic manifestations in 70% Cryptogenic organizing pneumonia
o Other manifestations include: Arthritis, serositis, o Elevated diaphragm/atelectasis 20%
photosensitivity, renal, hematologic and central Related to respiratory muscle/diaphragmatic
nervous system involvement dysfunction
"Shrinking lung syndrome"
o Cardiac enlargement
IIMAGING FINDINGS Pericardial effusion
Renal failure
General Features o Only 1-6% have evidence of interstitial lung disease
Best diagnostic clue (lLD) on chest X-ray (CXR) or clinically
o Pleural thickening or effusion most common CT Findings
Unexplained, small, bilateral, pleural effusions or
More sensitive than chest radiography
pleural thickening in young women
o Signs of ILD seen in 60% of symptomatic patients
o 38% of asymptomatic patients with normal CXR

DDx: Intersitial Lung Disease

Amiodarone Toxicity Desquamative Interstitial Pneumonia

 SYSTEMIC LUPUS ERYTHEMATOSUS, PULMONARY

Key Facts 1
Terminology Bronchiectasis or bronchial wall thickening 33% 13
Chronic collagen vascular disease Thromboembolic disease due to antiphospholipid
May manifest as cough, dyspnea and pleuritic chest antibodies
pain Ground-glass opacity (GGO) from associated
Thoracic manifestations in 70% Pneumonia
Other manifestations include: Arthritis, serositis, Acute lupus pneumonitis
photosensitivity, renal, hematologic and central Alveolar hemorrhage
nervous system involvement Top Differential Diagnoses
Imaging Findings Cardiogenic Pulmonary Edema
Pleural effusion or pleural thickening 50% Pneumonia
Consolidation Usual Interstitial Pneumonia (UIP)
Elevated diaphragm/atelectasis 20% Nonspecific Interstitial Pneumonitis (NSlP)
HRCT may resemble UIP Many drugs produce SLE pattern
Bibasilar subpleural reticular opacities Clinical Issues
Honeycombing Antiphospholipid antibodies in 40%
Centrilobular nodules ("tree-in-bud") 20%

HRCT
o More sensitive than CXR or pulmonary function Usual Interstitial Pneumonia (UIP)
tests (PFTs) Interstitial lung disease with honeycombing (rare with
o HRCT may resemble VIP SLE)
Inhomogeneous Nonspecific Interstitial Pneumonitis (NSIP)
Bibasilar subpleural reticular opacities
Cellular NSIP identical, fibrotic NSIP: Honeycombing
Honeycombing
o Centrilobular nodules ("tree-in-bud") 20% Drug Toxicity
o Bronchiectasis or bronchial wall thickening 33% Many drugs produce SLE pattern
o Possible findings of chronic interstitial pneumonitis
(3-13%) Rheumatoid Arthritis
Extensive ground-glass opacities Interstitial thickening less common with SLE
Coarse linear bands
Viral Pleuropericarditis
Honeycomb cysts
Other possible CT findings Identical appearance but limited course
o Mild adenopathy < 2 cm 20%
o Pulmonary embolism
Thromboembolic disease due to antiphospholipid I PATHOLOGY
antibodies General Features
o Ground-glass opacity (GGO) from associated
General path comments
Pneumonia
o Collagen vascular disease involving
Acute lupus pneumonitis
Blood vessels (vasculitis and PHT)
Alveolar hemorrhage
Serosa surfaces and joints
o Pulmonary artery (PA) enlarged from pulmonary
Kidneys, central nervous system, skin
hypertension (PHT) (5-14%)
Etiology
Usually primary
o Immune system
May be secondary due to chronic pulmonary
SLE effects complement system, T suppressor cells
emboli (PE)
and cytokine production
o Cavitating pulmonary nodules
Results in generation of autoantibodies
May be secondary to infarction
o Unknown: Majority of cases
o Drug-induced lupus - 90% due to
Procainamide
I DIFFERENTIAL DIAGNOSIS Hydralazine
Cardiogenic Pulmonary Edema Isoniazid
Interstitial thickening less common with SLE Phenytoin
History aids in diagnosis Thyroid blockers
Anti-arrhythmic drugs
Pneumonia Anticonvulsants
Identical radiographic findings, often seen with SLE Antibiotics
o Renal and central nervous system disease usually
Goodpasture Syndrome absent
Parenchymal changes more severe than SLE Anti-DNA antibodies absent
 SYSTEMIC LUPUS ERYTHEMATOSUS, PULMONARY
1 o Thromboembolic disease
Related to anticardiolipin antibody
Acute lupus pneumonitis
mortality
and hemorrhage high

14 May require life long anticoagulation Most common cause of death sepsis or renal disease
Epidemiology
o Primarily occurs in women (10:1)
Treatment
o 50 cases per 100,000 Steroids or immunosuppressants
NSAIDs may be effective for mildly symptomatic
Gross Pathologic & Surgical Features pleurisy
Pulmonary pathology nonspecific
o Vasculitis, hemorrhage, or BOOP

Microscopic Features
I SELECTED REFERENCES
I. Kocheril SV et al: Comparison of disease progression and
Hematoxylin bodies pathognomonic
mortality of connective tissue disease-related interstitial
o But rare in lung 1%)
lung disease and idiopathic interstitial pneumonia.
Alveolar hemorrhage reflects diffuse endothelial injury Arthritis Rheum. 53(4):549-57, 2005
Pleural findings are nonspecific 2. Filipek MS et al: Lymphocytic interstitial pneumonitis in a
o Lymphocytic and plasma cell infiltration, fibrosis, patient with systemic lupus erythematosus: radiographic
and fibrinous pleuritis and high-resolution CT findings.] Thorac Imaging.
19(3):200-3, 2004
3. Lalani et al. Imaging Findings in Systemic Lupus
Erythematosus. RadioGraphies. 24: 1069-1 086, 2004
ICLINICAL ISSUES 4. Najjar M et al: Cavitary lung masses in SLE patients: an
Presentation unusual manifestation of CMV infection. Eur Respir ).
24(1):182-4,2004
Most common signs/symptoms: Pleuritic pain present 5. Nomura A et al: Unusual lung consolidation in SLE.
in 45-60% of patients and may occur with or without Thorax. 58(4):367, 2003
a pleural effusion 6. Saito Y et al: Pulmonary involvement in mixed connective
Eleven diagnostic criteria tissue disease: comparison with other collagen vascular
o Any four present constitute diagnosis of SLE diseases using high resolution CT.] Com put Assist Tomogr.
o Skin 80%: Malar rash; photosensitivity; discoid 26(3):349-57, 2002
7. Rockall AG et al. Imaging of the pulmonary manifestations
lesions
of systemic disease. Postgrad Med]. 77:621-638,2001
o Oral ulceration 15%
8. Keane MP et al. Pleuropulmonary manifestations of
o Arthropathy 85% (nonerosive) systemic lupus erythematosus. Thorax 55: 159-166, 2000
o Serositis (pericardial or pleural) 50% 9. Mayberry ]P et al. Thoracic Manifestations of Systemic
o Renal proteinuria or casts 50% Autoimmune Diseases: Radiographic and High-Resolution
o Neurologic epilepsy or psychosis 40% CT Findings. Radiographies. 20: 1623-1635, 2000
o Hematologic anemia or pancytopenia 10. Murin S et al. Pulmonary manifestations of systemic lupus
o Immunologic abnormalities erythematosus. C1in Chest Med. 19:641-665, 1998
o Positive antinuclear antibody test 11. Munoz-Rodriguez F] et al. Shrinking lungs syndrome in
systemic lupus erythematosus: improvement with inhaled
Pleural disease usually painful
beta-agonist therapy. Lupus. 6:412-414,1997
o Antinuclear antibody (ANA), anti-DNA antibodies, 12. Ooi GC et al: Systemic lupus erythematosus patients with
and LE cells found in pleural fluid respiratory symptoms: the value of HRCT. Clin Radiol.
o Exudative effusion with higher glucose and lower 52(10):775-81,1997
lactate than rheumatoid arthritis 13. Sant SM et al: Pleuropulmonary abnormalities in patients
Pulmonary hemorrhage may not result in hemoptysis with systemic lupus erythematosus: assessment with high
o Mortality 50-90% resolution computed tomography, chest radiography and
o Often associated with glomerulonephritis pulmonary function tests. C1in Exp RheumatoJ.
15(5):507 -13, 1997
Antiphospholipid antibodies in 40%
14. Fenlon HM et al: High-resolution chest CT in systemic
Pulmonary function: Restrictive with normal diffusion lupus erythematosus. A]R. 166:301-7, 1996
capacity reflects diaphragm dysfunction 15. Bankier AA et al: Discrete lung involvement in systemic
Acute lupus pneumonitis lupus erythematosus: CT assessment. Radiology.
o Rare, life-threatening, immune complex disease 196(3):835-40, 1995
o Fever, cough, hypoxia requiring mechanical 16. McDonagh] et al: High resolution computed tomography
ventilation of the lungs in patients with rheumatoid arthritis and
interstitial lung disease. Br] RheumatoJ. 33(2): 118-22, 1994
Obliterative bronchiolitis rarely reported with SLE
17. Wiedemann HP et al: Pulmonary manifestations of
o With or without organizing pneumonia
systemic lupus erythematosus.] Thorac Imaging. 7:1-18,
Respiratory muscle dysfunction seen in up to 25% of 1992
SLE patients 18. Cush]] et aJ. Drug-induced lupus: clinical spectrum and
pathogenesis. Am] Med Sci. 290:36-45, 1985
Natural History & Prognosis 19. Haupt HM et al.l The lung in systemic lupus
Chronic disease (> 10 years) except in acute lupus erythematosus. Analysis of the pathologic changes in 120
pneumonitis patients. Am] Med. 71:791-798,1981
At risk for thromboembolic disease, opportunistic
infections
Chronic disease
 SYSTEMIC LUPUS ERYTHEMATOSUS, PULMONARY
I IMAGE GAllERY 1
15

(Left) Frontal radiograph

shows a diffuse fine nodular
pattern with a basilar
predominance in this patient
with SLE-related hemorrhage.
The lungs are well inflated
and there is no adenopathy.
(Right) Axial HRCT shows
lower lobe eeo with
bronchiectasis and septal
thickening.

(Left) Axial HRCT shows

centrilobu/ar nodules that
can be seen with SLE
(arrows). There is some
associated intralobular septal
thickening. (Right) Axial
NECT shows intralobular
septal thickening (black
arrows), bronchiectasis,
centrilobular nodules (white
arrows) and mild pleural
reactive change.
Lymphadenopathy and
centrilobular emphysema are
also seen.

Typical
(Left) Axial HRCT also shows
septal thickening, severe
bronchiectasis, centri/obular
nodules (arrows) and mild
pleural reactive changes, as
well as subpleural cystic
changes. (Right) Axial CECT
shows thromboembolic
disease with thrombus
(arrows) within the right
middle lobar PA branches, as
well as irregular wall
thickening of the lower lobe
PA, suggesting chronic PE.
 EMPYEMA
Key Facts
1
Terminology Presence of septae are important for treatment 17
Most commonly para pneumonic planning
Empyema necessitatis Top Differential Diagnoses
Tension empyema Pleural Metastases
Imaging Findings Mesothelioma
Pleural effusion with evidence of loculation in patient Iatrogenic Pleural Loculation
with febrile illness Pathology
No motion on decubitus views Overall incidence about 1% after lung resection for
Lentiform collections on lateral or frontal view cancer
One-sided lesion About 75% have associated bronchopleural fistula
Collections in fissures produce pseudotumor
Most findings are not specific for empyema Clinical Issues
Split pleura sign, not specific for empyema Early thoracentesis essential to diagnosis
Thickening of extrapleural fat Median hospital stay about 20 days
CT essential for treatment planning, to define Antibiotics and drainage are first lines of therapy
location of loculations

Typical of any pleural lesion, not specific for o Presence of septae are important for treatment
empyema planning
o Collections in fissures produce pseudotumor Increased likelihood that surgical intervention will
Appearance of rounded mass on one view, be needed
flattened in orthogonal view
May simulate lung mass
Imaging Recommendations
Best imaging tool: Chest radiography best initial study,
CT Findings CT often needed to plan intervention
CECT Protocol advice: Intravenous (IV) contrast can be
o Most findings are not specific for empyema useful to demonstrate pleural enhancement, but not
May also be seen in other causes of loculated essential
pleural fluid
Primary or metastatic tumor, sterile reactive
collections, fluid from abdominal sources I DIFFERENTIAL DIAGNOSIS
o Split pleura sign, not specific for empyema
Enhancement of the irritated visceral and parietal Pleural Metastases
pleura split by fluid Most common tumors: Breast, ovary, lung, malignant
o Pleural thickening thymoma
May also be seen in noninfected pleural fluid Pleural thickening or nodularity often absent
collections, thicker is more suggestive of empyema
Mesothelioma
o Thickening of extrapleural fat
Almost always unilateral thick rind, often with little
Also seen in noninfected fluid collections, most
fluid
commonly present in tuberculous or fungal
May have calcified plaques from asbestos
disease
o CT essential for treatment planning, to define Iatrogenic Pleural Loculation
location of loculations Pleurodesis, often for malignant effusions
o Tuberculous pleuritis produces thick calcification,
rib thickening, adjacent trapped lung Abdominal Causes
Endometriosis, cyclic symptoms, complex cystic
MR Findings collections
MR offers no advantages over CT in imaging of Pancreatic pseudocyst, may erode through diaphragm
empyema to reach pleural space
Ultrasonographic Findings
Grayscale Ultrasound
o Complex fluid collections, with internal echos and I PATHOLOGY
septations General Features
o Shadowing from gas pockets
Etiology
o May also appear as simple, nonseptated fluid
o Most often due to spread from adjacent pneumonia
collections
Effusions in setting of pneumonia may be bland
o Presence of echogenic material or septae may also be
or infected
seen in bland fluid collections
In tuberculosis, bland effusions are due to delayed
hypersensitivity reaction to tuberculous antigens
 EMPYEMA
1 In tuberculosis, reactive effusions are much more
common than actual empyema
18 Both bland and infected para pneumonic effusions
may be similar in imaging appearance
Overall effusions related to pneumonia more
common in patients with diabetes mellitus
a Can be related to procedures, iatrogenic
Overall incidence about] % after lung resection
for cancer
May occur early or late
Late cases may be due to hematogenous seeding of
bland fluid collections
About 75% have associated bronchopleural fistula
May occur due to breakdown of bronchial stump
Staphylococcus most common organism, also
streptococcus, anaerobes, gram-negative rods
o May occur from fistulous connections to Gl tract or
skin
Chest wall infections, fasciitis
Tumor erosion from skin or esophagus
Epidemiology: Most series have more men than
women
Associated abnormalities
o Bronchopleural fistula
May be related to necrotic infection
May be related to tumor invasion of airways
Gross Pathologic & Surgical Features
Thickened pleural rind
Often tightly adherent to underlying lung
Purulent fluid
If underlying lung noncompliant, re-expansion after
drainage may not be possible ("trapped lung")
Microscopic Features
Fibrinous exudate
Microbial organisms
May be associated hemorrhage
Staging, Grading or Classification Criteria
Empyema evolves through 3 stages, none of which are
distinct radiographically
o Evolution may take weeks but may evolve in days
Exudative stage
o Sterile fluid with normal glucose and normal pH
Fibrinopurulent stage
o Accumulation of neutrophils, bacteria, and fibrin
o Glucose and pH decreases
Chronic organizing stage
o Pleural peel develops and encases the lung
o Exudate is thick and frankly purulent
ICLINICAl ISSUES
Presentation
Most common signs/symptoms: Chest pain, fever,
rigors
Other signs/symptoms: Tuberculous empyemas may
have fewer symptoms
Demographics
Age
a Median age in most series around 50
o Can occur in children, mostly related to
pneumonias
o In elderly, outcome is often determined by other
associated disease
Gender: M > F
Natural History & Prognosis
Early diagnosis depends on high index of suspicion
o Early thoracentesis essential to diagnosis
o Imaging features and symptoms nonspecific
In post-operative empyema, panendoscopy essential
o To exclude fistulous connections to airways or
esophagus
Median hospital stay about 20 days
Mortality overall about 10%
o Poorer outcome if infection is fungal or if patient is
afebrile, indicating inadequate host response
Treatment
Antibiotics and drainage are first lines of therapy
Tube thoracostomy
o All loculated pockets must be drained
o Imaging guidance often needed to access separate
areas of involvement
o Fibrinolytic agents can be infused into pleural space
Break up loculations, facilitate complete drainage
Use has led to less need for surgical intervention,
shorter hospital stays
Video-assisted thoracoscopy
o Essential in complex cases, direct visualization,
physical disruption of loculations
Open drainage
o Required for complex or unresponsive cases, can
assess underlying lung
Drainage will not be successful if lung cannot
expand to fill the space
May require thoracoplasty to eliminate potential
space
Particularly a problem in tuberculous empyema
o After pneumonectomy, may require long-term open
drainage
Clagget window
I SELECTED REFERENCES
1. Wurnig PN et al: Video-assisted thoracic surgery for pleural
empyema. Ann Thorac Surg. 81:309-13, 2006
2. Bramley D et al: Tension empyema as a reversible cause for
cardiac arrest. Emerg Med J. 22:919-20, 2005
3. Cheng G et al: A retrospective analysis of the management
of parapneumonic empyemas in a country teaching facility
from 1992 to 2004. Chest. 128:3284-90,2005
4. Falguera M et al: Etiology and outcome of
community-acquired pneumonia in patients with diabetes
mellitus. Chest. 128:3233-9, 2005
5. Misthos Pet al: Early use of intrapleural fibrinolytics in the
management of postpneumonic empyema. A prospective
study. Eur J Cardiothorac Surg. 28:599-603, 2005
6. Tsai TH et al: Community-acquired thoracic empyema in
older people. JAm Geriatr Soc. 53:1203-9, 2005
7. Evans AL et al: Radiology in pleural disease: state of the art.
Respirology. 9:300-12, 2004
 EMPYEMA
IIMAGE GALLERY 1
19
Typical
(Left) Axial CECT shows a
chest wall infection at the
site of prior anterior
mediastinoscopy (arrow)
that eroded inward to the left
anterior pleural space.
(Right) Frontal radiograph
shows a psudowmor on the
right (arrows) from a large
area of loculated pleural fluid
related to a pneumonia.

Typical
(Left) Frontal radiograph
shows complex right sided
pleural disease with several
air fluid levels (arrows)
suggesting a connection to
either the airways or
esophagus. (Right) Frontal
radiograph shows typical
features of pleural
involvement with
tuberculosis. This may
represent either a reactive
process or empyema, with
dense calcification in the
pleural space.

Typical
(Left) Axial NECT shows a
farge air pocket posteriorly
on the right with a small air
fluid level (arrow) in a
patient with sporotrichosis.
(Right) Axial CECT shows a
thoracic window to allow
long term drainage of a
postoperative empyema after
right pneumonectomy. There
is still a small amount of
residual fluid present
(arrow).
 BRONCHO-PLEURAL FISTULA
1
20

Axial graphic shows abnormal fistulous connection Axial NEeT shows abnormal connection between the
between a right upper lobe airway and the rightpleural right mainstem bronchus and a rounded mass in the
space, with purulent pleural effusion. acljacent left lung or pleural space, which was due to
aspergillus.

I TERMI NOLOGY IIMAGING FINDINGS

Abbreviations and Synonyms General Features
Broncho-pleural fistula (BPF) Best diagnostic clue
Parenchymal-pleural fistula (PPF) o Drop in fluid level after pneumonectomy
o Development of hydropneumothorax without
Definitions intervening pleural intervention or procedure
Abnormal connection between the pleura and an Most hydropneumothoraces are related to pleural
airway tubes or thoracentesis
o Rare Location
o High mortality rate o Bronchial stump
o May develop due to necrotizing infection o Peripheral airway
o May also develop due to tumor Size: Variable-sized air-fluid level in pleural space
o Most often related to surgery Morphology: Loculated fluid collections containing air
May also rarely be iatrogenic due to trauma from
support lines Radiographic Findings
True BPF involves abnormal connection between a Radiography
central airway and pleural space o Loculated pleural collections
PPF is connection between distal airway and pleural May extend into fissures, produce pseudotumor
space One or multiple air fluid levels
o Less common Length of air-fluid levels unequal between the
o May occur due to necrotizing peripheral infection or frontal and lateral chest radiographs
tumor o Pneumomediastinum, in some cases
o After pneumonectomy, air-fluid levels are expected

DDx: Hydropneumothorax

Spontaneous Esophageal Fistula Pancreatic Pseudocyst

 BRONCHO-PLEURAL FISTULA

Key Facts 1
Terminology Pathology 21
Abnormal connection between the pleura and an Occur in about 6% of pneumonectomy cases, 4%
airway overall after lung surgery
True BPF involves abnormal connection between a
central airway and pleural space Clinical Issues
Expectoration of purulent material
Imaging Findings Mortality rate about 25% overall
Drop in fluid level after pneumonectomy Up to one third of BPFs may close spontaneously
Actual connection to bronchus may not be visible Chronic empyema (> 5 years) may lead to
Decubitus radiographs contraindicated: Pleural fluid malignancy
may spill into lung Adequate drainage of pleural fluid collections and
antibiotic therapy are essential
Top Differential Diagnoses
Gas Forming Infection in Pleural Space Diagnostic Checklist
Hydropneumothorax Unequal lengths of air-fluid level on frontal and
Esophagopleural Fistula lateral radiographs consistent with pleural collections
Empyema Necessitatis

Over time, fluid should rise and air decrease

(typically 1/2-2/3 fill in 1 week)
I DIFFERENTIAL DIAGNOSIS
Air is often entirely absorbed over time Gas Forming Infection in Pleural Space
(completely fills in 2-4 months time) Rare (Clostridium perfringens and Bacteroides fragilis)
Any decrease in fluid (without intervening pleural Most gas in pleural space from instrumentation or
instrumentation) indicates development of BPF fistulas
CT Findings Hydropneumothorax
NECT Spontaneous, due to pneumothorax with bleeding
o Loculated pleural collections containing air
Iatrogenic, due to line placement or attempted
o Actual connection to bronchus may not be visible thoracentesis with bleeding
May be intermittent Traumatic, due to deceleration injury with rupture of
May vary with ventilatory cycle airway into pleural space
Site of fistula generally detected on bronchoscopy
o Pneumomediastinum may be present Esophagopleural Fistula
CECT Similar radiographic findings to BPF
o Parietal pleura will enhance Endoscopic evaluation generally required to locate
Intravenous (IV) contrast generally not needed esophageal tear
o Effacement of extrapleural fat Pneumomediastinum may also be present
o If contrast introduced into the pleural space via Oral contrast may be visible in pleural space
drainage tubes, may be visible in airways Usually occur within 6 weeks of surgery
Nuclear Medicine Findings Empyema Necessitatis
Various scintigraphic agents have been used to Empyema decompressing through chest wall
determine if BPF is present Common organisms: Tuberculosis, Actinomycosis
Injection through pleural drain with imaging to look May have rib destruction
for bronchial activity
Ultrasonographic Findings
Grayscale Ultrasound: Ultrasound can be useful for
I PATHOLOGY
guiding intervention, drainage of pleural collections General Features
Imaging Recommendations Etiology
o Occur in about 6% of pneumonectomy cases, 4%
Best imaging tool
overall after lung surgery
o Chest radiography often sufficient for diagnosis
More common after right than left
o Chest CT may be helpful for detection of specific
pneumonectomy
site of fistula
More common in patients requiring
Protocol advice
post-operative ventilatory support
o IV contrast not essential
Usually diagnosed within 2 weeks of initial
o Contrast administered into pleural collection may
surgery
be visible in airways
o More common in certain surgical situations
o Decubitus radiographs contraindicated: Pleural fluid
[n patients who receive preoperative radiation
may spill into lung
therapy
 BRONCHO-PLEURAL
1 In patients with incomplete resection for tumor,
positive margins
22 In patients with diabetes mellitus
In patients with emphysema
o Can also occur due to necrotizing pneumonia or
necrotizing tumor
Particularly common in tuberculosis and fungal
infections
o Can occur after placement of support lines
Feeding tubes with stiff internal stylettes are
particularly hazardous
If placed incorrectly into airway, can relatively
easily be forced far distally
May pierce small airways and enter pleural space
Trauma to central airways from endotracheal
tubes may also produce BPF
Associated abnormalities
o BPF may produce empyema
o Empyema may produce BPF
Gross Pathologic & Surgical Features
Changes of empyema in the pleural space
Thickening of pleura and adherence to underlying
lung
Fistulous connection generally visible on
bronchoscopy
Microscopic Features
Nonspecific inflammatory changes
I CLINICAL ISSUES
Presentation
Most common signs/symptoms
o Expectoration of purulent material
o Expectoration of serosanguineous material
o Falling fluid-level on chest radiograph
o Dyspnea, hypoxia
o Fever, chest pain
Demographics
Age: Mean age late 50s
Gender: Males much more common than females
Natural History & Prognosis
Mortality rate about 25% overall
o Higher if in the setting of pneumonectomy
Up to one third of BPFs may close spontaneously
Chronic empyema (> 5 years) may lead to malignancy
o Non-Hodgkin lymphoma, squamous cell carcinoma,
mesothelioma, malignant fibrous histiocytoma,
sarcomas, hemangioendothelioma
Treatment
Adequate drainage of pleural fluid collections and
antibiotic therapy are essential
Generally also require surgery
o If post-operative, bronchial stump revision
o Wrapping of stump with omentum or tissue flap
o May require open thoracic window, long term
drainage
Ventilatory support is problematic
FISTULA
o May require separate ventilation of each mainstem
bronchus
o Allows decreased pressure on side of fistula, to
facilitate closure
Newer treatments involve endoscopic methods to
attem pt to close fistula
o Stents
o One-way valves
o Coils, glue
Laser therapy may be helpful in small fistulae that are
not related to tumor
o Edematous reaction of surrounding tissues may help
to close off tract
May require muscle flap to fill pleural space if lung
does not re-expand
I DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Unequal lengths of air-fluid level on frontal and lateral
radiographs consistent with pleural collections
I SELECTED REFERENCES
1. Jones NC et al: Bronchopleural fistula treated with a
covered wallstent. Ann Thorac Surg. 81:364-6,2006
2. Cerfolio RJet al: Intercostal muscle flap to buttress the
bronchus at risk and the thoracic esophageal-gastric
anastomosis. Ann Thorac Surg. 80:1017-20, 2005
3. Chichevatov D et al: Omentoplasty in treatment of early
bronchopleural fistulas after pneumonectomy. Asian
Cardiovasc Thorac Ann. 13:211-6,2005
4. Lois M Noppen M et al: Bronchopleural fistulas: an
overview of the problem with special focus on endoscopic
management. Chest. 128:3955-65, 2005
5. Santini Met al: Use of a modified endobronchial tube for
mechanical ventilation of patients with bronchopleural
fistula: EurJ Cardiothorac Surg. 28:169-71, 2005
6. Athanassiadi Ket al: Late postpneumonectomy
bronchopleural fistula. Thorac Cardiovasc Surg.
52:298-301, 2004
7. Kacprzak G et al: Causes and management of
postpneumonectomy empyemas: our experience. EurJ
Cardiothorac Surg. 26:498-502, 2004
8. Kitami A et al: A case of post-upper lobectomy empyema
treated by serratus anterior muscle and pediculated
latissimus dorsi musculocutaneous flaps plombage via
open-window thoracostomy. Ann Thorae Cardiovasc Surg.
10:18-6,2004
9. Leone MAet al: Pneumocephalus from
bronchopleural-subarachnoid fistula. Eur Neurol. 52:253-4,
2004
10. Kiriyama M et al: Endobronchial
neodymium:yttrium-aluminum garnet laser for
noninvasive closure of small proximal bronchopleural
fistula after lung resection. Ann Thorac Surg. 73:945-8,
2002
11. Kempainen RRet al: Persistent air leaks in patients
receiving mechanical ventilation. Semin Respir Crit Care
Med. 22:675-84, 2001
12. Sirbu H et al: Bronchopleural fistula in the surgery of
non-small cell lung cancer: incidence, risk factors, and
management. Ann Thorac Cardiovasc Surg. 7:330-6, 2001
13. Sonobe M et al: Analysis of risk factors in bronchopleural
fistula after pulmonary resection for primary lung cancer.
EurJ Cardiothorac Surg. 18:519-23, 2000
 BRONCHO-PLEURAL FISTULA

IIMAGE GAllERY 1
23
Variant
(Left) Frontal radiograph
shows feeding tube in
abnormal position, following
the course of the right
mainstem bronchus (curved
arrows) and terminating low
in the right hemithorax
(arrows). (Right) Lateral
radiograph shows the
mercury-filled tip of the tube
coiled in the posterior right
costophrenic region, within
the pleural space (arrows).

Typical
(Left) Axial CECT shows
typical findings of right
pneumonectomy, with fluid
filling the entire right
hemithorax. There is a/so a
small left pleural effusion
(arrow). (Right) Axial CECT
shows the same patient,
several days later with new
air-fluid level in right
hemithorax (arrow) and
decrease in fluid indicating
development of a
bronchopleural fistula.

Variant
(Left) Frontal radiograph
shows development of a new
right apical pleural air
collection (arrows) in a
patient after resection of a
Pancoast tumor. A BPF was
diagnosed by endoscopy
fRight) Axial CECT shows air
in the ventricles and
subarachnoid spaces in the
same patien/. The BPF also
communicated via the
brachial plexus to the
subarachnoid space.
 METASTASIS, PLEURAL
1
24

Axial CECT shows a large pleural metastasis from Frontal radiograph shows increased hazy opacity at the
leiomyosarcoma with mass effect on the mediastinum left lung base with a mass overlying the cardiac
and right lung and chest waif invasion seen anteriorly silhouette from metastatic adenocarcinoma.
(arrow).

I TERMINOLOGY CT Findings
Bronchogenic carcinoma
Definitions
o CT scanning of limited predictive value in the cases
Hematogenous, lymphatic, or direct spread of of T3 and T4 tumors
malignancy to pleura o Occasionally only pleural effusion is seen
o Common occurrence, especially from o Presence of either ipsilateral pleural nodularity or
adenocarcinomas focal pleural thickening adjacent to or apart from
o May manifest as pleural thickening or mass the primary lung cancer is a significantly frequent
CT finding
o Presence of irregular pleural thickening and small
IIMAGING FINDINGS nodules at interlobar fissure on thin-section CT is
Genera~Features highly accurate for diagnosis
Pleural metastases
Best diagnostic clue: Unexplained pleural effusion in
o May demonstrate mediastinal pleural involvement
patient with malignancy
o Metastases may have variable enhancement
Radiographic Findings Invasive thymoma
Most common finding is pleural effusion o Thymoma seen as anterior mediastinal mass
o In adults, 2nd most common cause of pleural o Invasiveness often not distinguishable by CT
effusion (congestive heart failure number 1) Absence of invasiveness by imaging does not
o Usually moderate-sized, volume usually> 500 ml exclude the presence of a histologically invasive
Pleural masses or thickening uncommon on chest lesion
radiographs o Pleural metastases very common with invasive
lesions

DDx: Pleural Mass

Mesothelioma Splenosis Fibro/Hemothorax

 METASTASIS, PLEURAL

Key Facts
1
Terminology Fibrothorax or Loculated Pleural Effusion 25
Hematogenous, lymphatic, or direct spread of Localized Fibrous Tumor of the Pleura
malignancy to pleura Radiation Fibrosis

Imaging Findings Pathology

Best diagnostic clue: Unexplained pleural effusion in
patient with malignancy
Most common finding is pleural effusion
Pleural masses or thickening uncommon on chest
radiographs
CT scanning of limited predictive value in the cases
of T3 and T4 tumors
Metastases may have variable enhancement
CT procedure of choice to evaluate pleural disease
Top Differential Diagnoses
Mesothelioma
Pleural cavity a lymphatic space
Hematogenous, lymphatic, or direct spread to pleura
Visceral pleura more commonly involved
Adenocarcinoma most common tumor to metastasize
to pleura
Clinical Issues
Generally signifies advanced incurable disease; T4 or
Ml
Incomplete resection of T4 lung cancers does not
prolong the survival

o Invasive thymoma may extend through Diagnosis made with nuclear medicine liver spleen
diaphragmatic hiatus into abdomen or scan
retroperitoneum
Primary Intrathoracic Malignant Effusion
Imaging Recommendations Usually adenocarcinoma
CT procedure of choice to evaluate pleural disease Present with a malignant effusion
o Useful for planning biopsy No primary identified
6-15% of all malignant pleural effusions from an
unknown primary site
I DIFFERENTIAL DIAGNOSIS Demographics: Women (65%) and nonsmokers (noA
Poor prognosis
Mesothelioma
Adenocarcinoma more common Other Primaries
Pleural fluid 95% (vs. 50% for metastases) Synovial sarcoma of the pleura
10% have pleural plaques o Rare, epithelial and spindle cell components
Nearly always symptomatic (metastases maybe o Identical radiographic findings: Pleural mass and/or
asymptomatic) effusion

Fibrothorax or loculated Pleural Effusion Primary leiomyosarcoma

Spares mediastinal pleura Rare, spindle cell proliferation in cords and sheets
Pleural thickening not nodular Identical radiographic findings
Not circumferential in hemithorax
May be caicified
I PATHOLOGY
Epithelioid Hemangioendothelioma
Rare, old men General Features
Pleural effusion with nodularity of pleura General path comments
Similar to diffuse pleural metastases or mesothelioma o Pleural cavity a lymphatic space
o Exfoliation of cancer cells into the pleural cavity
localized Fibrous Tumor of the Pleura occurs when
Well-circumscribed, homogeneous, soft tissue mass The tumor exposed on the pleural surface or
related closely to the pleura Subpleural lymphatics invaded by the tumor
May be pedunculated o Pleural invasion occurs with
Angle with the chest wall may be acute or obtuse Hematogenous, iymphatic, or direct spread to
o Obtuse angle seen more often in small lesions pleura
Radiation Fibrosis Macroscopically detectable vascular invasion as
pulmonary arterial tumor emboli
History is highly pertinent
Microscopic vascular invasion occurring at the
Associated geographic parenchymal fibrosis aids in
level of the primary tumor
diagnosis
o Do not have to have blockage of the lymphatic
Splenosis drainage to mediastinal lymph nodes to have
History of trauma pertinent accumulation of pleural effusion
o Visceral pleura more commonly involved
 METASTASIS, PLEURAL
1 "Sedimentary" mechanism of pleural metastasis
Neoplastic implants from visceral pleura cast off
Other agents used in the past
o Catheter placement or repeat thoracentesis can be
26 cells to the pleural cavity used, but pleurodesis preferred
Results in secondary tumor implants to parietal
pleura and diaphragm
Etiology I DIAGNOSTIC CHECKLIST
o Adenocarcinoma most common tumor to
metastasize to pleura Consider
Lung cancer 40% Video-assisted thoracoscopic surgery (VATS)(with
Breast 20% biopsy) assuming an increasingly important role in
Lymphoma 10% diagnosis
Tumor of unknown origin 10% o Only modality that allows for complete visualization
High prevalence of pleural metastasis in women of pleural space
may be related to presence of adenocarcinoma
Image Interpretation Pearls
Gross Pathologic & Surgical Features Any subtle pleural abnormalities on pre-operative CT
Pleura large extensive lymphatic network scans in patients with primary lung cancer, such as
Visceral pleura more commonly involved than parietal nodularity or focal pleural thickening, should be
pleura considered as possible pleural metastasis, even if there
Metastases often flat, thin plaques, reason not seen on is only a small associated pleural effusion
imaging studies
Thymoma
o No pathologic features to separate invasive I SElECTED REFERENCES
thymoma from benign thymoma, invasiveness 1. Hwang)H et al: Subtle Pleural Metastasis without Large
dependent on imaging or surgical findings Effusion in Lung Cancer Patients: Preoperative Detection
Lymphoma on CT. Korean) Radiol. 6(2):94-10 I, 2005
o Usually secondary disease either recurrence or 2. )ung)1 et al: Thoracic manifestations of breast cancer and
associated with other nodal disease its therapy. Radiographies. 24(5):1269-85. 2004
o Usually will not cause volume loss 3. Lim Eet al: Intraoperative pleural lavage cytology is an
o Will grow around ribs rather than destroying them independent prognostic indicator for staging non-small
cell lung cancer. J Thorac Cardiovasc Surg. 127(4):1113-8,
Microscopic Features 2004
4. Nomori H et al: Fluorine 18-tagged fluorodeoxyglucose
Sensitivity of pleural fluid cytology 60% positron emission tomographic scanning to predict lymph
Separation of mesothelioma from adenocarcinoma node metastasis, invasiveness, or both, in clinical Tl NO
metastases difficult at light microscopy, requires MOlung adenocarcinoma.) Thorac Cardiovasc Surg.
special stains 128(3):396-401,2004
5. Lardinois D et al: Staging of non-small-cell lung cancer
with integrated positron-emission tomography and
ICLINICAllSSUES computed tomography. N Engl) Med. 348:2500-2507,
2003
Presentation 6. Rosado-de-Christenson MLet al: Localised fibrous tumors
of the pleura. Archives of the AFIP,RadioGraphies. 23 PI"
Most common signs/symptoms 759-783, 2003
o Most common symptom dyspnea 7. Asamura H et al: Thoracoscopic evaluation of
o May be asymptomatic (20%) histologically/cytologically proven or suspected lung
o Nonspecific, dull, aching chest pain, anorexia, cancer: a VATS exploration. Lung Cancer. 16:183-190,
weight loss, malaise 1997
o Complaint of persistent localized chest pain is a 8. Kjellberg 51et al: Pleural cytologies in lung cancer without
strong indication of parietal pleura and chest wall pleural effusions. Ann Thorac Surg. 64:941-944, 1997
involvement in non-small cell lung cancer 9. Hsu CP et al: Surgical experience in treating T4 lung
cancer: its resectability, morbidity, mortality and
Natural History & Prognosis prognosis. EurJ Surg Oncol. 22:171-176, 1996
10. Roviaro Get al: Videothoracoscopic staging and treatment
Generally signifies advanced incurable disease; T4 or
of lung cancer. Ann Thorac Surg. 59:971-974, 1995
M1
II. Yamada Ket al: The early detection of pleural
Incomplete resection of T4 lung cancers does not dissemination of lung cancer by thin-slice computed
prolong the survival tomography. Lung Cancer. 35:437-443, 1995
o Can contribute to a significant increase in hospital 12. White PG et al: Preoperative staging of carcinoma of the
mortality bronchus: can computed tomographic scanning reliably
identify stage III tumors? Thorax. 49:951-957,1994
Treatment 13. Kirby'lJ et al: Initial experience with video-assisted
Directed towards underlying malignancy thoracoscopic lobectomy. Ann Thorac Surg. 56:1248-125,
o Pleurodesis for symptomatic effusions 1993
Talc most common agent 14. Wain)C: Video-assisted thoracoscopy and staging of lung
cancer. Ann Thorac Surg. 56:776-778, 1993
Doxycycline and tetracycline used but not as
efficacious
 METASTASIS, PLEURAL
I IMAGE GALLERY 1
27
Typical
(Left) Axial CECT shows a
large left pleural metastasis
with chest wall invasion (rom
renal cell carcinoma. There
are small areas of pleural
thickening in the posterior
hemithorax as well (arrows).
(Right) PET in the same
patient with renal cefl
carcinoma shows multiFocal
disease with metastatic
disease involving the anterior
(arrows) and posterior left
hemithorax (curved arrows).

Typical
(Left) Frontal radiograph
shows an opacity overlying
the 7th posterior rib on the
right from a pleural
metastasis from melanoma
(arrows). The lesion has
ill-defined margins on the
lateral aspect. (Right) Axial
CECT shows a pleural
metastasis with rib and
vertebral involvement from
squamous cell carcinoma
(SCCA). This is a 23 year old
with h/o laryngeal
papillomatosis with
development of SCCA of
right lower lobe.

Typical
(Left) Axial CECT shows
multiFocal nodules in the
right lung, with a large cystic
lesion (arrow) in this patient
with a history of laryngeal
papillomatosis. There is
pleural thickening on the left.
(Right) Axial CECT shows
diffuse irregular pleural
thickening with pleural
enhancement and
calciFication in this patient
with a history of metastatic
osteosarcoma.
 MALIGNANT MESOTHELIOMA
1
28

Graphic shows typical features of mesothelioma. Pleural Frontal radiograph shows diffuse pleural thickening
thickening surrounding the right lung (arrows) and encasing the left hemithorax (arrows). Left hemithorax is
extending into the fissures (open arrows). Hemithorax is slightly smaller than the right hemithorax. Malignant
small. mesothelioma.

May be obscured by effusion

ITERMINOlOGY o Hemithorax usually small
Definitions Sometimes expanded due to massive pleural
Rare pleural tumor associated with asbestos exposure effusion
o Most common primary neoplasm of the pleura o Pleural plaques in uninvolved hemithorax (5%)
o Lymphangitic metastases or hematogenous
metastases rarely identified
IIMAGING FINDINGS CT Findings
General Features Surgical decisions depend on chest wall,
diaphragmatic, and mediastinal involvement
Best diagnostic clue
o CT sensitive but not specific for invasion, surgery
o Diffuse nodular pleural thickening, small
hemithorax necessary unless gross involvement detected
o Pleural effusion near universal, may be only Pleural effusion
o Fill 1/3rd of hemithorax (50%)
manifestation
o Fill 2/3rds of hemithorax (40%)
Location: Parietal and to a lesser extent visceral pleura
o Fill> 2/3rds of hemithorax (10%)
Radiographic Findings Lobulated pleural thickening
Radiography o Gravitational distribution
o Pleural effusion (95%), may be only finding Tumor thicker at base
Unilateral, rarely bilateral (10%) o Encases lobes as it grows along fissures (90%)
Moderate sized to large o Irregular interface between chest wall and tumor
o Lobulated pleural thickening unreliable predictor for chest wall invasion
Usually extensive, extends into fissures Contraction of hemithorax in 40%

DDx: Pleural Thickening

Metastatic Adenocarcinoma Malignant Thymoma Splenosis

 MALIGNANT MESOTHELIOMA
Key Facts
1
Terminology Asbestos Pleural Disease 29
Rare pleural tumor associated with asbestos exposure Pathology
Imaging Findings Long, thin asbestos fibers more likely to induce
Pleural effusion (95%), may be only finding mesothelioma
Lobulated pleural thickening Dose response relationship, latent period from
Hemithorax usually small exposure 30-45 years
Pleural plaques in uninvolved hemithorax (5%) Asbestos plaque not a precursor for mesothelioma
Tendency to extend along needle or chest tube 5% insulation workers die of mesothelioma
drainage tract (20%) Primarily involves parietal pleura, rapidly involves
the entire ipsilateral pleura
Top Differential Diagnoses
Metastatic Adenocarcinoma
Clinical Issues
History of occupational exposure to asbestos in 80%
Empyema
Dismal, median survival 12 months
Malignant Thymoma
Cytology usually not adequate for diagnosis
Splenosis
Lymphoma

a Enlargement of hemithorax (due to pleural effusion)

15%
Metastatic Adenocarcinoma
Contralateral pleural plaques 20% (versus 5% on chest May not have pleural effusion (50%)
radiographs) Shrinkage of hemithorax less common with metastatic
Tendency to extend along needle or chest tube adenocarcinoma
drainage tract (20%) Visceral pleura rather than parietal pleura
Mediastinal organ invasion Empyema
a Tumoral encasement of more than 50% Rarely involves the entire pleural space
circumference of a mediastinal structure
Diaphragm Malignant Thymoma
a Transdiaphragmatic extension: Soft tissue mass that Anterior mediastinal mass
encases hemidiaphragm Lobulated pleural masses from drop metastases
Clear fat plane between diaphragm and adjacent
abdominal organs, smooth diaphragmatic contour Splenosis
indicate tumor limited to thorax Left-sided pleural thickening
Diffuse hepatic calcification may be seen with liver History of remote trauma
metastases (rare) Absent spleen

MR Findings Lymphoma
Coronal imaging useful to evaluate Usually secondary to known disease
transdiaphragmatic extent Other nodal disease involvement
More sensitive than CT for chest wall and
Asbestos Pleural Disease
diaphragmatic invasion
Tumor: Slightly high T1 and moderately intense T2 Benign effusions diagnosis of exclusion
Usually occur earlier after exposure than
weighted imaging
mesothelioma
a Tumor enhances with gadolinium-based contrast
Nuclear Medicine Findings Tuberculous Dormant Empyema
PET: Useful in staging especially for mediastinal nodal Cicatricial scarring and nodules in upper lobes
involvement Pleura often diffusely calcified

Imaging Recommendations Hemangioendothelioma

Best imaging tool: CT to evaluate nature and extent of Rare, older men
Growth pattern similar to mesothelioma
pleural disease
Pleural thickening not as extensive

I DIFFERENTIAL DIAGNOSIS I PATHOLOGY

General Separation Benign vs. Malignant
General Features
Pleural Disease Etiology
Favors malignancy: Circumferential involvement, a Asbestos fiber induction depends on fiber aspect
nodular pleural thickening, parietal pleura> 1 cm ratio (Iength:width)
thick, mediastinal pleural involvement
 MALIGNANT MESOTHELIOMA
1 Long, thin asbestos fibers more likely to induce
mesothelioma
30 Higher aspect ratio the higher the prevalence of
mesothelioma
Crocidolite > amosite > chrysotile (rarely causes
mesothelioma)
o Dose response relationship, latent period from
exposure 30-45 years
Exposure may be incidental
o Asbestos plaque not a precursor for mesothelioma
o Erionite (asbestos like mineral) found in soil in
central Turkey (village of Karain)
High incidence of mesothelioma in local
population
Epidemiology
o Rare tumor, 10/1,000,000 men, typically 50-70 years
of age
o Prevalence depends on exposed population
5% insulation workers die of mesothelioma
Gross Pathologic & Surgical Features
Primarily involves parietal pleura, rapidly involves the
entire ipsilateral pleura
Microscopic Features
Three histologic types
o Epithelial (50%); best prognosis
Tubulopapillary pattern: Uniform cuboidal cells
with eosinophilic cytoplasm and distinct nucleoli
Similar to metastatic adenocarcinoma
o Sarcomatous (209,)
Spindle cells with nuclear atypia Demographics
Must be differentiated from true sarcoma
o Biphasic (both cell types) (30%)
Immunohistology useful to identify mesotheliomas
o Positive for cytokeratins and absence of other
positive antibodies
Staging, Grading or Classification Criteria
Tumor descriptors (T)
o Tl: (20%) resectable
A: Ipsilateral parietal pleural, no involvement
visceral pleura
B: Ipsilateral parietal and visceral pleura
o T2: (50%) resectable
Involvement of one of the following: Diaphragm, Treatment
fissure, or extension into the lung
a T3: (25%) resectable
Involvement of one of the following:
Endothoracic fascia, mediastinal fat, single focus
into chest wall, pericardium without penetration
o T4: 5%) unresectable
Involvement of one of the following: Diffuse or
multiple chest wall extensions, through
diaphragm into peritoneum, extension to
contralateral pleura, extension into mediastinal
organs or spine, through pericardium
Nodes (N) 1.
o NO: None
o Nl: lpsalateral hilar lymph nodes
o N2: Subcarinal or ipsilateral mediastinal lymph
nodes (including internal mammary lymph nodes)
o N3: Contralateral mediastinal or supraclavicular
lymph nodes
Metastases (M)
o Ml: Distant metastases
Stages TNM classification
o la: Tla, no nodes or distant metastases
o Ib: Tlb, no nodes or distant metastases
o II: T2, no nodes or distant metastases
o Ill: T3, with hilar or mediastinal metastases (at most
N2)
o IV: Any T4 or contralateral mediastinal node (N3) or
distant metastases (Ml)
Stages I-Ill resectable: Resectability criteria
o Preserved extra pleural fat planes
o Absence of extrapleural masses
o Smooth inferior diaphragmatic contour (best
assessed on multi planar reconstructions)
ICUNICAllSSUES
Presentation
Most common signs/symptoms
o Chest wall pain, dyspnea, fever, and weight loss
Symptoms precede diagnosis by 4-6 months
o History of occupational exposure to asbestos in SO'M)
Other signs/symptoms
o Pleural fluid
Bloody (30%)
Glucose decreased
Hyaluronic acid increased
o Course often complicated by thrombophlebitis
Age
o Latency period 35-40 years after initial exposure
o Seen rarely in childhood, peak in 60-70 years of age
Gender: Males 4:1 due to industrial exposure
Natural History & Prognosis
Dismal, median survival 12 months
Best prognosis for surgical candidates: 5 year survival
25%
o Negative surgical margins
o Epithelial cell type
o No metastases
Cytology usually not adequate for diagnosis
o Video-assisted thorascopic surgery (VATS) procedure
of choice for diagnosis
Potentially resectable: Stage I-III
o Extrapleural pneumonectomy: Major complications
in 25%
Palliative radiation therapy or chemotherapy
Local radiation prophylactic to chest wall needle tracts
I SELECTED REFERENCES
Benamore REet al: Use of imaging in the management of
malignant pleural mesothelioma. Clin Radial.
60(12):1237-47,2005
2. Flores RM: The roie of PET in the surgical management of
malignant pleurai mesothelioma. Lung Cancer. 49 5uppl
1(527-32,2005
 MALIGNANT MESOTHELIOMA
IIMAGE GALLERY 1
31

(Left) Frontal radiograph

shows subtle pleural
thickening in the left
hemithorax (arrows). Pleural
plaque right chest wall (open
arrow). Malignant
mesothelioma. (Right) Axial
CECT shows focal pleural
thickening involving the
mediastinal pleura. Subtle
extension into the chest wall
anteriorly (arrow). Calcified
pleural plaques right chest
wall (open arrows).

(Left) Axial CECT shows

tension hydrothorax
expanding the left
hemithorax. Pleural
thickening due to
mesothelioma is smooth
(arrows). Pleural plaque right
hemithorax (open arrow).
(Right) Axial NECT shows
smooth diffuse pleural
thickening due to malignant
mesothelioma (arrows).
Pleural effusion (open
arrow). Hemithorax is
contracted.

(Left) Axial NECT shows

lobulated diffuse pleural
thickening due to malignant
mesothelioma (arrows).
Hemithorax is contracted.
Calcified pleural plaques
(open arrows). (Right) Axial
NECT shows diffuse pleural
thickening (rom malignant
mesothelioma in the left
hemithorax. Tumor extends
along needle biopsy tract
(open arrows). Multiple
plaques and diffuse asbestos
thickening right chest wall
(arrows).
 PANCOAST TUMOR
1
32

Graphic shows characteristic features of Pancoast Frontalradiograph shows typical Pancoast tumor in the
tumor. From the lung apex, tumor usually extends left apex. Tumor has destroyed the first rib (open
through the chest wall extending into the brachial arrows). Apical cap is much thicker on the left (arrow).
plexus. Most common complaint is shoulder pain.

ITERMINOLOGY Radiographic Findings

Abbreviations and Synonyms
Superior sulcus tumor, apical lung tumor
Definitions
Pancoast syndrome: Neoplasm at the lung apex
(superior sulcus) accompanied by arm or shoulder pam
Described by Pancoast in 1932
I IMAGING FINDINGS
General Features
Best diagnostic clue
o Asymmetric apical pleural thickening
o Upper rib erosion
Location: Superior sulcus: Not a distinct anatomIc
structure but close proximity to nerves and vessels at
apex of lung gives rise to distinct symptomatology
Size: Tumor usually not large on chest radiograph
Morphology: May be either smooth or sharply
marginated from adjacent lung
Radiography
o Apical cap measuring> 5 mm in thickness (50%)
Asymmetrical apical caps> 5 mm in thickness
Apical cap with convex margin
. Progressive enlargement on sequential films
o Apical mass (45%)
o Bone destruction of upper ribs (33%)
o Apical lordotic projection better depicts lung apex
CT Findings
CECT
o Bulk of tumor may be extrathoracic
o Mediastinal lymph nodes staged as with other
bronchogenic carcinomas
Nodes considered positive if> 1 cm short-axis
.
diameter
Accuracy poor (70%)
o Chest wall invasion
Greater than 3 cm contact with pleural surface,
absent fat plane, obtuse angle
Definite invasion: Rib destruction, encasement
nerves or blood vessels
Complication post surgery

DDx: Pancoast Tumor

Apical Cap Neurofibromas

 PANCOAST TUMOR

Key Facts
1
Terminology Nerve Sheath Tumors 33
Superior sulcus tumor, apical lung tumor Pathology
Pancoast syndrome: Neoplasm at the lung apex Epidemiology: Accounts for < 5% of pulmonary
(superior sulcus) accompanied by arm or shoulder carcinomas
pain Most common cell types in apical tumors: Squamous
Imaging Findings cell carcinoma (50%), adenocarcinoma (25%)
Tumor is usually T3 (extension through pleura into
Apical cap measuring> 5 mm in thickness (50%)
chest wall)
Top Differential Diagnoses Stage lIB: T3NOMO typical Pancoast tumor stage
Apical Cap Clinical Issues
Reactivation Tuberculosis
Shoulder and arm pain (90%)
Radiation Fibrosis
Horner syndrome (25-50%) due to involvement of
Extrapleural Fluid
sympathetic chain and stellate ganglion
Pleural Effusion (Supine)
5 year survival of 50% with combined radiation and
Aneurysm Subclavian Artery
surgical resection of T3NOMO, Stage lIB
Thoracic Outlet Syndrome

o Cerebrospinal fluid (CSF) leak due to Bronchiectasis and cavitation common

subarachnoid-pleural fistula
Leak from nerve root most common
Pneumocephalus
Supratentorial subdural hematoma
Cerebellar hemorrhage
Radiation Fibrosis
Lung apex included in field for head & neck cancer,
Hodgkin lymphoma, and breast carcinoma
(supraclavicular therapy)

MR Findings Extrapleural Fluid

More accurate than CT for Most often associated with bleeding
o Vertebral body extension May be seen in aortic trauma
o Spinal canal extension May be seen in misplacement of central lines
o Brachial plexus involvement Transient
o Subclavian artery involvement Pleural Effusion (Supine)
Nuclear Medicine Findings Lung apex most dependent portion of the pleural
PET: Useful in lymph node staging of the mediastinum space in supine position
Transient
Imaging Recommendations
Aneurysm Subclavian Artery
Best imaging tool
o Chest radiography often 1st examination for Sharply defined mass at lung apex
detection Often related to trauma
o Chest CT useful to further characterize and stage CECT or MRI useful to exclude before biopsy
neoplasms Thoracic Outlet Syndrome
Limited accuracy in chest wall invasion and May have cervical rib
mediastinal nodal staging o Usually associated with arterial compression: Pallor,
o Chest MR extremely useful, multiplanar capabilities, absent pulses, ipsilateral decrease blood pressure
especially coronal and sagittal planes of the lung
apex, to evaluate brachial plexus Nerve Sheath Tumors
Protocol advice: With either CT or MR, coronal or Neurofibromas or schwannomas may extend from the
sagittal planes are most helpful spine over the apex of the lung
May widen neural foramen
Most common cause of posterior mediastinal mass
I DIFFERENTIAL DIAGNOSIS
Lipoma of Extrapleural Fat
Apical Cap Hypertrophy or excess deposition of extra pleural fat
Sharp, smooth or undulating margin < 5 mm thick Usually bilateral, enlarged body habitus
with concave margin Fat density at CT
Right apical cap more common than left
Aspergillus Superinfection
Normal process of aging
New pleural thickening adjacent to pre-existing
Reactivation Tuberculosis cavitary disease
Generally associated calcification Aspergilloma may not be radiographically evident
Often associated scarring and cicatricial atelectasis of (seen at CT)
upper lobe
 PANCOAST TUMOR
1 Peripheral Upper Lobe Collapse
Ptosis (drooping eyelid)
Miosis (constricted pupil)
34 More common in neonates Anhidrosis (loss of sweating)
Represents collapse of the apical-posterior segments Other signs/symptoms: Cord compression: 5% initially
with sparing of the anterior segment with tumor extension through the intervertebral
Pleural Metastasis foramen
Adenocarcinoma, lymphoma: Unusual as only site Demographics
Malignant thymoma may spread extrapleurally around Age: Typical of bronchogenic carcinoma, age> 40,
lung apex peak incidence in those 50-70
Gender: Bronchogenic cancer leading cause of death
in both men and women
I PATHOLOGY
Natural History & Prognosis
General Features 5 year survival of 50% with combined radiation and
General path comments surgical resection of T3NOMO, Stage liB
o Anatomic structures surrounding apex of the lung: o Stage iliA or stage llIB 5 year survival 15%
Anterior to posterior o Right-sided Pancoast tumor stage liB survival worse
Subclavian and jugular veins than left-sided Pancoast tumor stage liB
Phrenic and vagus nerves o Mediastinal adenopathy reduces median survival to
Subclavian and carotid arteries 9 months
Recurrent laryngeal nerve
8th cervical and 1st and 2nd thoracic nerve Treatment
Sympathetic chain and stellate ganglion Treatment for stage liB bronchogenic carcinoma
Ribs and vertebral bodies o Preoperative irradiation
Etiology o En bloc surgical resection of lung, chest wall, and
o Without effective screening, Pancoast syndrome is a lower brachial plexus
delay in diagnosis o Lung resection may be either wedge or lobectomy
Sym ptoms related to invasion of surrounding Contraindications for surgery
structures o Extensive invasion of the neck, upper brachial
o Risk factors for lung cancer: Smoking (90%), radon, plexus, vertebra or spinal canal
asbestosis o Mediastinal adenopathy (exception, right apical
Epidemiology: Accounts for < 5% of pulmonary tumors with right-sided paratracheal nodes which
carcinomas are considered N2 nodes)
o Subclavian venous obstruction
Microscopic Features o Distant metastases
Bronchogenic carcinoma Surgical mortality < 5%
o Most common cell types in apical tumors: Neurologic deficits are often temporary lasting a few
Squamous cell carcinoma (50%), adenocarcinoma months
(25%) Complication
o CSF leak from a subarachnoid-pleural fistula due to
Staging, Grading or Classification Criteria
trauma to nerve root or even spinal canal
Tumor is usually T3 (extension through pleura into
chest wall)
T4 if mediastinal invasion or vertebral body invasion
Stage liB: T3NOMO typical Pancoast tumor stage
I DIAGNOSTIC CHECKLIST
Consider
Pancoast tumor should be excluded in patients
IClINICAL ISSUES evaluated for thoracic outlet syndrome
Presentation
Most common signs/symptoms
o Pancoast original description
I SELECTED REFERENCES
Apical lung mass 1. Archie VC et al: Superior sulcus tumors: a mini-review.
Rib destruction Oncologist. 9(5):550-5, 2004
2. Huehnergarth KVet al: Superior pulmonary sulcus tumor
Horner syndrome
with Pancoast syndrome. Mayo Clin Proc. 79(10):1268,
Atrophy of hand muscles 2004
o Shoulder and arm pain (90%) 3. Alifano M et al: Surgical treatment of superior sulcus
Pain along the ulnar nerve distribution of the arm tumors: results and prognostic factors. Chest.
and hand 124(3):996-1003,2003
Nonspecific pain, patients often treated for 4. Jett JR: Superior sulcus tumors and Pancoast's syndrome.
bursitis for months prior to diagnosis Lung Cancer. 42 SuppI2(S17-21, 2003
o Horner syndrome (25-500/0) due to involvement of 5. Chiles C et al: Navigating the thoracic inlet. Radiographies.
sympathetic chain and stellate ganglion 19(5):1161-76,1999
 PANCOAST TUMOR

IIMAGE GALLERY 1
35

(Left) Frontal radiograph

shows asymmetrical sharply
defined apical pleural
thickening in the right apex
(arrow) from a Pancoast
tumor. (Right) Frontal
radiograph shows apical
asymmetry. Left apical cap
thicker than the right
(arrow). Margins are
indistinct. PancoasllUmor.

Typical
(Left) Axial CECT shows
tumor surrounding the left
subclavian artery (arrow)
and extending into the
mediastinum (open arrow).
Unresectable Pancoast
tumor. (Right) Axial CECT
shows Pancoast tumor at the
left apex. Tumor extends into
the vertebral body (arrow).

(Left) Sagittal T2WI FS MR

shows apical mass (arrows)
with extension into the
neural foramen (open
arrows) and destruction of
vertebral bodies.
Unresectable Pancoast
tumor. (Right)
Anteroposterior radiograph
shows post-operative en bloc
resection of the apiea/lung
tumor and chest wall. Two
chest tubes extend into the
neck. Pancoast tumors are
often amenable to aggressive
surgical resection.
 FIBROUS TUMOR OF PLEURA

36

Graphic shows a lypical spherical, somewhallobulaled Coronal T1WI MR shows a 31 cm mass in the righl
tumor displacing the lung. An atlachmenl by a shari hemithorax, compressing the diaphragm inferiorly
vascular pedicle is common. (arrows). Benign fibrous tumor of the pleura was
present at surgery.

Visceral pleural may be more common

ITERMINOLOGY Size
Abbreviations and Synonyms o Often large in size
Solitary (or localized) fibrous tumor of the pleura Usually grow slowly over years
o Benign mesothelioma is an inappropriate term and Radiographic Findings
should be avoided Peripheral, lobulated, sharply-marginated mass with
Definitions longitudinal axis paralleling chest wall
Uncommon primary mesenchymal neoplasm of pleura o Located anywhere along interlobar fissure,
o Reported cases in lung, mediastinum, abdomen and diaphragmatic, mediastinal or chest wall pleura
neck When present, the "incomplete border" sign and
Aries from pleural submesothelial connective tissue, obtuse margins helpful to localize outside lung
not from mesothelium parenchyma
80-85% benign, 15-20% malignant Variable sized but often> 7 em
o Large tumors often lack imaging features to localize
in pleural
IIMAGING FINDINGS o May mimic an elevated diaphragm or mediastinal
mass
General Features Pedunculated lesions change location with position, a
Best diagnostic clue characteristic imaging feature
o Large peripheral spherical or oblong mass o Rarely, pedicle may twist and tumor detaches as a
May change in position free intrapleural mass
Location Pleural effusions 20% (> common with malignant
o Pleura, often inferior hemithorax lesions)

DDx: Solitary Fibrous Tumor of the Pleura

Neurogenic Tumor Chest Wall/Pleural Metastasis

 FIBROUS TUMOR OF PLEURA
Key Facts
Terminology
Solitary (or localized) fibrous tumor of the pleura
Aries from pleural submesothelial connective tissue,
not from mesothelium
80-85% benign, 15-20% malignant
Imaging Findings
Large peripheral spherical or oblong mass
May change in position
Large tumors often lack imaging features to localize
in pleural
Tumors are vascular with enhancement often equal
to or > muscle
When present, a low T1W & T2W signal is
characteristic
Multiplanar imaging and post-processing help
identify its pleural origin
CT Findings
CECT
o Characteristic obtuse angle of pleural lesions often
absent with large tumors
Tumors displace structures rather than invade
o Calcification 5% [> with malignant lesions (20%)1
o Tumors are vascular with enhancement often equal
to or > muscle
Homogeneous enhancement with tumors < 6 em
Heterogeneous enhancement with larger tumors;
relates to myxoid degeneration or with malignant
tumors, necrosis
o No chest wall involvement
Pedicle uncommonly seen with large tumors
o Malignant and benign tumors are essentially
indistinguishable with imaging
MR Findings
Excellent for tissue characterization and tumor
boundary evaluation
When present, a low T1 W & T2W signal is
characteristic
o Low signal relates to extensive fibrous elements
Other tumors demonstrate high T2W signal
Patchy high T2W signal may occur with myxoid cystic
regions or necrosis
o This is particularly seen in large tumors (> 10 em)
Tumor usually demonstrates avid enhancement with
gadolinium
Pleural location and tissue planes well seen with
multi planar imaging
Imaging Recommendations
Best imaging tool
o Contrast-enhanced CT & MRI both have advantages
Pleural mass with low signal on Tl Wand T2W
allows a presumptive diagnosis of fibrous tumor
Consider surgical removal rather than biopsy in
this setting
Protocol advice
o Multiplanar imaging and post-processing help
identify its pleural origin
1
Clinical Issues 37
Asymptomatic
Hypertrophic osteoarthropathy seen in 17-30%,
especially tumors> 10 em
Hypoglycemia is a rare symptom (5%)
Recurrence in ipsilateral pleura; rarely lung
Presence of malignant components & complete vs
incomplete tumor resection determines prognosis
Surgical excision is curative in majority of cases
Video-assisted thoracotomy for smaller tumors
Resection of all recurrent lesions
Diagnostic Checklist
In one study, diagnosis not considered in 54% of
pre-operative radiology reports & only 20% stated its
pleural orgin
o May demonstrate tumor mobility by changing
patient's position on different scans
I DIFFERENTIAL DIAGNOSIS
Pleural Lipomas
Pleural nodule or mass, may be pedunculated and
even change position
o CT or MRI clearly depicts its dominant fat content
Thymoma
Solitary fibrous tumor along mediastinal pleural
resembles thymoma
o Eccentric and lopsided appearance are typical for
thymoma
Similar age of presentation and imaging characteristics
o However, thymoma has bright T2W signal
Pleural or Chest Wall Metastasis/Myeloma
Assess for rib destruction
Metastatic lesions usually multiple
Pleural Blastoma/Sarcoma
Large lesions with rapid doubling time
Often large areas of necrosis
Malignant features on histology
o High rate of mitosis, increased nuclear/cytoplasmic
ratio
Neurogenic Tumor
Posterior mediastinal location most common
o Occasional chest wall from intercostal nerves
May see localized scalloping of ribs
Calcification common with these tumors
I PATHOLOGY
General Features
General path comments
o Traditional teaching: Visceral pleural orgin (2/3) &
parietal (1/3)
 FIBROUS TUMOR OF PLEURA
1 Recent investigations demonstrate a SO/SO
distribution
38 a Parietal origin is more likely malignant
Etiology
a Unknown, no asbestos association
Case reports of tumor developing after chest wall
radiation therapy
a Not related to smoking
Epidemiology: Less than 5% of all pleural tumors
Gross Pathologic & Surgical Features
Large lobulated mass, often with pedicle (50%) and
adhesions to pleural
a Cysts, hemorrhage or necrosis seen with large or
malignant tumors
Pedicle more common with benign tumors
Microscopic Features
Originates from sub mesothelial mesenchymal cellular
layer of pleura
a Fibroblasts and connective tissue in a variety of
patterns and cellularity
Most common is random intermingling of
fibroblasts and collagen ("pattern-less pattern")
2nd most common is hemangiopericytoma-like
appearance with a network of irregular branching
vessels in hypercellular areas
Nuclear pleomorphism and mitoses essentially absent
Variable appearance on microscopy, resulting in a
large differential diagnosis
a Includes hemangiopericytoma (most difficult
differential), spindle cell carcinoma, melanoma,
sarcomatoid mesothelioma and sarcomas
Fine needle biopsy often insufficient for diagnosis,
even with large sample numbers
a In one series, only 32% of benign and 20% of
malignant tumors were initially diagnosed
accurately with needle biopsy
Staging, Grading or dassification Criteria
Malignant with broad-based attachment
a Highest rate of recurrence
Malignant with pedicle
Benign with broad-based attachment
Benign with pedicle
a Lowest rate of recurrence
ICLINICAL ISSUES
Presentation
Most common signs/symptoms
a Asymptomatic
Cough, dyspnea or chest pain less common
Other signs/symptoms
a Hypertrophic osteoarthropathy seen in 17-30%,
especially tumors> 10 cm
Tumor removal alleviates joint pain, but returns
with tumor recurrence
Greater association with fibrous tumor than lung
cancer
a Hypoglycemia is a rare symptom (5%)
Symptoms relieved with tumor removal
Called Doege-Potter syndrome
Numerous theories regarding pathophysiology:
Increased glucose consumption, increased insulin
receptors, secretion of insulin-like substances or
reduced growth hormone response to
hypoglycemia
a Clubbing of digits reported
Demographics
Age: Present at any age; mean is SO
Gender: Slightly more common in females
Natural History & Prognosis
Most demonstrate a benign growth pattern
a Recurrence after excision may occur, even if benign
on histology (15%)
a Tumors may recur years later
Require long term surveillance
Malignant transformation or metastasis rare
Recurrence in ipsilateral pleura; rarely lung
a Recurrence more likely with malignant tumors &
broad-based attachment
. Presence of malignant components & complete vs
incomplete tumor resection determines prognosis
Malignancy has poor prognosis, often survival < 2 year
a Extensive intrathoracic metastatic disease is often
responsible, extrathoracic disease is rare
Treatment
Surgical excision is curative in majority of cases
a Video-assisted thoracotomy for smaller tumors
a Open thoracotomy for large tumors
Vascular adhesions to adjacent tissues predispose
to intraoperative hemorrhage; need adequate
surgical exposure
Resection of all recurrent lesions
a Recurrence time interval decreases with each
successive tumor removal
Radiation and chemotherapy not helpful with benign
histology because of low cell count and mitotic rates
I DIAGNOSTIC CHECKLIST
Consider
In one study, diagnosis not considered in 54% of
pre-operative radiology reports & only 20% stated its
pleural orgin
I SELECTED REFERENCES
1. Lee SC et al: Solitary fibrous tumors of the pleura: clinical,
radiological, surgical and pathological evaluation. EurJ
Surg Oneal. 31(1):84-7, 2005
2. Mitchell JD: Solitary fibrous tumor of the pleura. Semin
Thorac Cardiovasc Surg. 15(3):305-9, 2003
3. Rosado-de-Christenson ML et al: From the archives of the
AFlP:Localized fibrous tumor of the pleura. Radiographies.
23(3):759-83,2003
4. Tateishi U et al: Solitary fibrous tumor of the pleura: MR
appearance and enhancement pattern. J Com put Assist
Tomogr. 26(2):174-9, 2002
5. England DM et al: Localized benign and malignant fibrous
tumors of the pleura. A clinicopathologic review of 223
cases. Am J 5urg Pathol. 13:640-58, 1989
 FIBROUS TUMOR OF PLEURA

I IMAGE GALLERY 1
39
Typical
(Left) Axial CECT in a 46
year old female with an
abnormal chest radiograph.
A 14 em enhancing mass
extending along the right
anterior mediastifwm with
mild compression of the right
atrium (arrow). (Right) Axial
CECT demonstrates the
inferior margin of mass.
Pre-operative diagnosis was
thymoma versus less likely,
lymphoma. Benign solitary
fibrous tumor of pleura was
found at surgery.

(Left) Frontal radiograph In a

6B year old female with
increasing dyspnea on
exertion. Opacification of the
right lower hemithorax is
suggestive {or an effusion or
elevated diaphragm. (Right)
Lateral radiograph
demonstrates an abnormal
lobulated contour with a
convex posterior elevation.
An underfying mass was
considered probable. A CT
was performed.

(Left) Axial CECT shows a

heterogeneously enhancing
mass, with some enlarged
vessels apparent (arrow). A
20 em benign localized
fibrous tumor of the pleural
was removed at surgery.
(RighI) Axial CECT In a 60
year old female with
dyspnea. A heterogeneous
27 em mass with areas of
necrosis/cystic degeneration
and large vessels (arrows) is
seen. This was a benign
fibrous pleural tumor.
 ERDHEIM CHESTER DISEASE, PLEURAL
1 ~/~II.:~~~ ~
,..... '. "
....

,. ---. tJ' ~ ""

. 'i. 1,,-' "
0(, ~-..
40
'/
;'. '~
. I'" -.
""

I~ II / \

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" '!i"
Graphic shows several major manifestations of EC
Bilateral symmetric sclerosis of clavicles. Bilateral
symmetric pleural thickening. Diffuse encasement of a
long segment aorta and both kidneys.
Axial HRCT left upper lobe. Smooth uniform septal
thickening, centrilobular nodules (curved arrow) and
mosaic ground glass attenuation. Mild thickening of the
major fissure.

Bilateral symmetric osteosclerosis of metaphyses

ITERMINOlOGY and diaphyses especially long leg bones: Virtually
Abbreviations and Synonyms pathognomonic
Polyostotic sclerosing histiocytosis o Perirenal
Perirenal fat effaced by soft tissue, soft tissue edge
Definitions fairly sharply demarcated from surrounding fat
Non-Langerhans cell histiocytosis of unknown origin Bilaterally symmetric
o Periaortic
Circumferential and nonocclusive
IIMAGING FINDINGS Generally involves long segments from the
ascending aorta to the iliac bifurcation
General Features MRI may distinguish the aortic wall from the
Best diagnostic clue: Triad of osteosclerotic bone surrounding periadventitial tissue
lesions, perirenal encasement, pleural thickening, and o Cardiac
diffuse septal interstitial lung disease May be endocardial, myocardial or pericardial
Location Pericardium most commonly affected either due
o Lung and pleural to smooth soft tissue thickening or effusion
Smooth thickening of visceral pleura and fissures, Myocardial predilection for atrial walls, in contrast
usually bilateral and relatively symmetric to other organs involvement is not uniform with
Smooth septal thickening, usually seen in development of focal masses
conjunction with visceral pleural thickening
Prominent centrilobular nodules Radiographic Findings
o Bone Diffuse interstitial thickening with prominent septal
lines (90%)

DDx: Erdheim Chester Disease

Lee Lung-Scapula Acute Pulmonary Edema Asbestos Pleural Disease

 ERDHEIM CHESTER DISEASE, PLEURAL
Key Facts
Terminology
Non-Langerhans cell histiocytosis of unknown origin
Imaging Findings
Smooth thickening of visceral pleura and fissures,
usually bilateral and relatively symmetric
Bilateral symmetric osteosclerosis of metaphyses and
diaphyses especially long leg bones: Virtually
pathognomonic
Encasement of the aorta and great vessels with soft
tissue thickening
Kidneys encased by soft tissue replacing perirenal fat
Osteosclerosis of skeletal structures
Smooth uniform interlobular septal thickening
Centrilobular nodules
Mosaic ground glass opacities
o Uniform through out the lung or preferentially
affecting either the upper or lower lobes
Mild to moderate pleural thickening (66%)
No adenopathy
Generalized cardiac enlargement
CT Findings
CECT
o Smooth pleural thickening extending into the
fissures with or without pleural effusions
o Ground glass opacities and smooth uniform
interlobular septal thickening
o Pericardial thickening or pericardial effusions
o Generalized cardiomegaly
o Encasement of the aorta and great vessels with soft
tissue thickening
Smooth uniform thickening involving long
segments
o Kidneys encased by soft tissue replacing perirenal fat
o Osteosclerosis of skeletal structures
HRCT
o Smooth uniform interlobular septal thickening
o Smooth pleural thickening extends into the fissures
o Centrilobular nodules
o Mosaic ground glass opacities
Imaging Recommendations
Best imaging tool: CECT and HRCT better detects and
characterizes lung, pleural, aortic and skeletal
abnormalities
I DIFFERENTIAL DIAGNOSIS
Cardiac
Congestive heart failure (CHF)
o Combination of enlarged heart, pleural thickening,
and septal thickening mimics CHF
o CHF will respond to diuretic and inotropic therapy,
Erdheim Chester (EC) will not change
Pulmonary
Edema, shares smooth septal thickening
1
Top Differential Diagnoses 41
Congestive heart failure (CHF)
Asbestos related pleural disease
Langerhans Cell Granulomatosis (LCG)
LCG and EC may coexist, suggesting a common
histiocytic stem cell line
Retroperitoneal fibrosis (RF)
Pathology
Macrophage-monocyte-Kupffer cell line gives rise to
EC
Symmetry: Long bones, pleural, perirenal, and lung
Diagnostic Checklist
CT useful modality to investigate unexplained pleural
thickening
o Centrilobular nodules not part of pulmonary edema
o Edema severity usually gravitationally dependent
Sarcoidosis
o Septal thickening usually nodular and preferentially
follows the bronchovascular bundles
o Adenopathy rare with EC
Lymphangitic tumor
o Septal thickening usually nodular and distribution
usually asymmetric: Sparing lobes or lungs
Pulmonary venoocclusive disease
o Similar septal thickening but central pulmonary
arteries are enlarged, absent with EC
Diffuse pulmonary lymphangiomatosis (DPL)
o Similar septal findings but bronchovascular bundle
thickened and mediastinal fat effaced
o Pleural thickening due to effusion, not soft tissue
(EC)
Pleura
Asbestos related pleural disease
o Often calcifies, EC will not have calcification
o Usually asymmetric in contrast to EC
Mesothelioma
o Usually unilateral, lobular rather than smooth, and
shrinks the hemithorax
Aortic Thickening
Takayasu aortitis
o Takayasu does not involve perirenal space which is
invariably abnormal with aortic involvement
o Takayasu does not involve pleura or lung
o EC usually more extensive, coating the entire aorta
Langerhans Cell Granulomatosis (LCG)
Triad of hypothalamic, bone, and pulmonary disease
o Hypothalamic involvement identical to EC
o LCG bone: Sharply defined lytic lesions
EC bone lesions sclerotic however 10% lytic
o LCG lung: centrilobular nodules and cysts
EC lung involvement smooth septal and pleural
thickening
o Associated periaortic or perirenal involvement only
with EC
 ERDHEIM CHESTER DISEASE, PLEURAL
1 LCG and EC may coexist,
histiocytic stem cell line
suggesting a common o Bone lesions usually asymptomatic
Mild continuous periarticular pain
(60%)

42 Most common lower extremities

Retroperitoneal o Diabetes insipidus due to involvement of the
Retroperitoneal fibrosis (RF) hypothalamus or posterior pituitary gland (20%)
o Does not involve the perirenal space like EC o Exopthalmos due to retroorbital intraconal mass
o EC circumferentially involves the aorta, RF spares (15%)
the posterior aortic wall Painless, symmetric
o RF may involve the inferior vena cava which is o Renal involvement may lead to renal failure (15')'(')
spared with EC o Xanthomas of the skin, particularly of the eyelid
o RF leads to hydronephrosis due to ureteral (10%)
occlusion, ureters spared with EC
Demographics
Age: > 40
I PATHOLOGY Gender: No gender predilection

General Features Natural History & Prognosis

Genetics: Nonfamilial Slowly progressive disease, prognosis depends on
Etiology: Unknown extent of extraosseous disease
Epidemiology Pleuro-pulmonary involvement has significant
o Histiocytes divided into 2 groups morbidity and mortality, 3 year survival 66%
Macrophage-monocyte-Kupffer cell line gives rise
Treatment
to EC
Langerhans cell-dendritic cell line gives rise to Corticosteroids
Vincristine and related chemotherapy
Langerhans cell histiocytosis (Hand
Radiotherapy for focal masses
-SchOller-Christian disease, Letterer-Siwe disease,
and Langerhans cell granulomatosis of the lung)

Gross Pathologic & Surgical Features I DIAGNOSTIC CHECKLIST

Tropism for pleural, perirenal, perivascular, and
meningeal tissue
Image Interpretation Pearls
Symmetry: Long bones, pleural, perirenal, and lung CT useful modality to investigate unexplained pleural
o Bone, pleural, perirenal and lung involvement never thickening
unilateral Findings of perirenal disease, aortic wall thickening or
bone sclerosis virtually pathognomonic for
Microscopic Features Erdheim-Chester
Lymphangitic expansion by inflammatory cells and
fibrosis
o Inflammatory cells consist of large foamy I SElECTED REFERENCES
histiocytes, lymphocytes, scattered plasma cells and 1. Allen TC et al: Pulmonary and ophthalmic involvement
Touton giant cells with Erdheim-Chester disease: a case report and review of
Granulomas rarely described the literature. Arch Pathol Lab Med. ]28(12):1428-31,2004
Electron microscopy: No Birbeck granules (seen 2. Dlon E et al: Imaging of thoracoabdominal involvement in
with LCG) Erdheim-Chester disease. A)R Am) Roentgenol.
CD68 stain positive, S-100 stain variably positive 183(5):1253-60,2004
(invariably positive with LCG) 3. Kenn W et al: Erdheim-Chester disease: evidence for a
disease entity different from Langerhans cell histiocytosis?
CD1a stain negative (invariably positive with
Three cases with detailed radiological and
LCG) immunohistochemical analysis. Hum Pathol. 31(6):734-9,
No emperipolesis 2000
o Fibrosis typically fine fibrillary mature collagen 4. Rush WL et al: Pulmonary pathology of Erdheim-Chester
without fibroblast proliferation disease. Mod Pathol. 13(7):747-54,2000
5. Wittenberg KH et al: Pulmonary involvement with
Erdheim-Chester disease: radiographic and CT findings.
IClINICAllSSUES A)R Am) Roentgenol. 174(5):1327-3],2000
6. Egan A) et al: Erdheim-Chester disease: clinical, radiologic,
Presentation and histopathologic findings in five patients with
interstitial lung disease. Am) 5urg Pathol. 23(]): 17-26,
Most common signs/symptoms ]999
o Slow onset cough and dyspnea 7. Veyssler-Belot C et al: Erdheim-Chester disease. Clinical
o Radiographic evidence of disease often precedes and radiologic characteristics of 59 cases. Medicine
clinical symptoms (Baltimore). 75(3):157-69,1996
o Pulmonary function tests
Moderate restriction
Decreased diffusion capacity
Other signs/symptoms
 ERDHEIM CHESTER DISEASE, PLEURAL
IIMAGE GALLERY 1
43

(Left) Frontal radiograph

mimics congestive heart
failure. Mild cardiomegaly.
Diffuse central (batwing)
interstitial thickening, Kerley
lines, and mild pleural
thickening right lateral chest
wall. (Right) Axial GCT
shows symmetric perirenal
encasement, mild
hydronephrosis, and
circumferential envelopment
of the abdominal aorta
(arrow).

Typical
(Left) Frontal radiograph
bilateral symmetric pleural
thickening (arrows) extends
from the mid hemithorax into
the costophrenic angles.
(Right) Axial NECT shows
bilateral pleural thickening.
Small right pleural effusion.
Note the circumferential
thickening on the right and
the involvement of the
mediastinal pleura (arrow).

Typical
(Left) Axial HRCT shows
diffuse smooth septal
thickening, centriacinar
nodules and ground-glass
opacities. Few scattered
cystic areas, predominantly
in the upper lobes. (Right)
Axial NECT shows bilateral
sclerosis or
the clavicles.
Sclerosis involved most of
the diaphyseal-metaphyseal
portion of the bone. Nodular
reticular interstitial changes
in the apices.
 TRANSUDATIVE PLEURAL EFFUSION
1
44

Axial graphic shows small dependent left pleural fluid Axial NECT in a paUent with prior myocardial infarctions
collecUon (arrows). and chronic CHF shows bilateral pleural effusions
(arrows). The pleural surfaces are smooth, thin and
partially imperceptible.

Separation of gastric bubble from diaphragm

ITERMINOLOGY (normal < 1.5 em)
Definitions On lateral: Diaphragm flat anteriorly then sharply
Rate of pleural fluid formation exceeds reabsorption descends at major fissure
Transudates are ultrafiltrates of plasma, characterized o Posterior costophrenic sulcus (only lateral view)
by low cell and protein content Blunting posterior costophrenic sulcus; average
quantity needed to blunt: 50 ml
o Lateral costophrenic sulcus (only PA view)
I IMAGING FINDINGS Blunting lateral costophrenic sulcus; average
quantity needed to blunt: 200 ml
General Features o Inversion hemidiaphragm
Best diagnostic clue: Blunted costophrenic angles Medial displacement of gastric air bubble
Location: Pleural space Seen with large effusions greater than 2,000 ml
Size: Small to massive Chest fluoroscopy to demonstrate paradoxical
Morphology: Variable respiration (pendelluft)
Inspiration: Inverted diaphragm ascends
Radiographic Findings Expiration: Inverted diaphragm descends
Radiography o Supine film poorest examination to detect fluid
o Sequence of accumulation upright film: Sensitivity 70%, up to 500 ml must accumulate for
Sub pulmonic > posterior angle> lateral angle reliable detection
o Subpulmonic Generalized increase in density hemithorax,
Flattening and "elevation" of hemidiaphragm meniscus often absent
Lateral shift of diaphragm apex Apical cap (apex most dependent portion of
supine hemithorax)

DDx: Transudative Effusion Mimics

Eventration Fibrous Tumor Empyema

 TRANSUDATIVE PLEURAL EFFUSION

Key Facts
1
Terminology Elevated Diaphragm 45
Rate of pleural fluid formation exceeds reabsorption Chronic Pleural Scarring
Primary or Metastatic Pleural Mass
Imaging Findings
Pathology
Sequence of accumulation upright film: Subpulmonic
> posterior angle> lateral angle Pleural effusions result from imbalance of Starling
Supine film poorest examination to detect fluid forces
CT: No reliable distinction between exudates and CHF, most common cause
transudates Clinical Issues
Pleural effusion vs. ascites Treated CHF: Days to weeks for fluid to resorb;
Grayscale Ultrasound: Anechoic effusions may be ex-vacuo effusions, resolve as lung expands
either transudates or exudates (50%) Remove < 1000 cc pleural fluid at a time
Layering fluid 1 cm thick, indicates an effusion> 200
mL that is amenable to thoracentesis Diagnostic Checklist
Effusions due to CHF especially when bilateral do not
Top Differential Diagnoses
requires thoracentesis, in most cases
Exudative Effusion

o Fissural accumulation helpful finding for the

presence of small effusions Imaging Recommendations
May preferentially accumulate air or fluid in Best imaging tool
CO PO patients o Lateral decubitus examination, will detect as little as
Minor fissure pseudotumor may be mistaken for 10 ml of fluid
pulmonary mass Defines a freely flowing effusion
Fluid in incomplete major fissure pitfall for Layering fluid 1 cm thick, indicates an effusion>
pneumothorax or pneumomediastinum 200 mL that is amenable to thoracentesis
Fluid in fissure has curvilinear edge concave CECT can provide information about quantity, pleural
toward hilum surfaces and underlying lung
o Mediastinal shift away from the effusion US useful for thoracentesis guidance
Observed with effusions> 1000 mL o Can be performed at bedside; can be used for chest
o Congestive heart failure (CHF): Bilateral effusions, tube placement
relatively equal size
Cardiomegaly, pulmonary vascular congestion,
interstitial and alveolar edema I DIFFERENTIAL DIAGNOSIS
o Hepatic cirrhosis: Right-sided, unilateral 70%;
Exudative Effusion
left-sided 15%; bilateral 15%
CT may show nodular thickened pleural surfaces or
Small to massive
heterogeneous fluid density
CT Findings
Elevated Diaphragm
CECT
o Can show effusions as small as 10 mL Costophrenic angles sharp, apex not shifted laterally
oCT: No reliable distinction between exudates and Chronic Pleural Scarring
transudates No layering on decubitus views; no fluid with
o Smooth thin pleural surfaces, extra pleural fat ultrasound
attenuation, homogeneous fluid density
o Ex-vacuo effusions: Effusions that develop following Primary or Metastatic Pleural Mass
bronchial obstruction by carcinoma or foreign body Enhancement with CT; solid appearance with
o Pleural effusion vs. ascites ultrasound
Pleural fluid peripheral, ascites central
Pleural fluid displaces crus anteriorly, posterior to
bare area of liver I PATHOLOGY
Pleural fluid interface with liver or spleen
indistinct, sharp with ascites General Features
Pitfall: Findings reversed for inverted diaphragm General path comments
Pleural fluid will get progressively smaller with o Normally, pleural fluid formed as an ultrafiltrate
caudal progression from capillaries in parietal pleura
Removed by lymphatics draining lower costal,
Ultrasonographic Findings mediastinal and diaphragmatic pleura
Grayscale Ultrasound: Anechoic effusions may be Removed by capillaries across visceral pleural
either transudates or exudates (50%) mesothelium
o Starling forces, balance between
 TRANSUDATIVE
1 Hydrostatic and oncotic forces in the visceral and
parietal pleural vessels
46 Lymphatic drainage
o Pleural effusions result from imbalance of Starling
forces
o Congestive left heart failure
Pulmonary venous hypertension essential for
pleural fluid development
Increased interstitial pressure in subpleural region
Edema fluid leaks from visceral pleura
o Isolated right heart failure: Pleural effusion, unusual
Etiology
o Congestive heart failure, associated with pulmonary
venous hypertension
o Cirrhosis, associated with transdiaphragmatic
movement of ascites
o Atelectasis, due to decrease in pleural pressure
o Peritoneal dialysis, dialysate moves from peritoneum
to pleural cavity across diaphragm
o Hypoalbuminemia, 1.5 gm/dl)
o Nephrotic syndrome, due to hypoalbuminemia,
hypervolemia and increased hydrostatic pressures
o Urinothorax, retroperitoneal leakage of urine enters
pleura through diaphragmatic lymphatics
o Post-partum, due to hypervolemia and high
intrathoracic pressures from Valsalva maneuvers
o Central line placement in pleural space
o Myxedema, most due to associated CHF or
pneumonia
Epidemiology
o Pleural effusions, common; 1.3 million pleural
effusions per year diagnosed in U.S.
o CHF, most common cause
3/4 of patients will have effusions at some time
Bilateral, 88%; unilateral right, 8%; unilateral left,
4%
Gross Pathologic & Surgical Features
Normal pleural fluid volume approximately 5 ml total
(2.5/hemithorax)
Normal pleura surface area 2000 cm2; no
communication between pleural spaces
Microscopic Features
Pleural membranes are intact
[CLINICAL ISSUES
Presentation
Most common signs/symptoms
o Dyspnea, mild non-productive cough, chest pain
o Large effusions may invert hemidiaphragm and
impair ventilation
o Asymptomatic effusions common due to CHF,
post-op, post-partum
Physical findings, do not usually manifest until pleural
effusions exceed 300 mL
D'Amato sign
o Displacement in dullness from vertebral area to
cardiac region due to free fluid
o When patient moves from sitting to lateral
decubitus position
Transudative effusion criteria
PLEURAL EFFUSION
o < 1,000 cells/mm3, lymphocytes, mesothelial cells
o Pleural fluid serum protein ratio < 0.5
o Pleural fluid serum lactate dehydrogenase (LDH)
ratio < 0.6
o Pleural fluid LDH < 2/3 of upper limit normal serum
value
Patients with CHF on diuretics, effusions incorrectly
diagnosed as exudative
Demographics
Age: Neonatal to elderly
Gender: M:F = 1:1
Natural History & Prognosis
Treated CHF: Days to weeks for fluid to resorb;
ex-vacuo effusions, resolve as lung expands
Treatment
For CHF: Diuretics, digitalis, afterload reduction
Thoracentesis
o May partially relieve symptoms
o Fluid analysis essential for differentiation of
transudative from exudative effusions
o Blind tap safe for fluid> 1 cm thickness lateral
decubitus examination
o Remove < 1000 cc pleural fluid at a time
Relative indications for thoracentesis, atypical
presentation
o Unilateral effusion, or effusions of disparate size
o Absence of cardiomegaly
o Fever, pleuritic chest pain
Relative contraindications for thoracentesis
o Effusions, < 1 cm thickness on a lateral decubitus
film; bleeding diathesis or on systemic
anticoagulation
o Mechanical ventilation; cutaneous disease over
puncture site
Complications of thoracentesis
o Pneumothorax, hemothorax, empyema, chest wall
hematoma, re-expansion pulmonary edema
Chest tube drainage for symptomatic effusions
Pleurodesis with doxycycline or talc, for refractory
large effusions
o CHF: May result in increased effusion on opposite
side
I DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Effusions due to CHF especially when bilateral do not
requires thoracentesis, in most cases
I SELECTED REFERENCES
1. Nandalur KRet al: Accuracy of computed tomography
attenuation values in the characterization of pleural fluid:
an ROC study. Acad Radiol. 12(8):987-9],2005
2. Kinasewitz GT:Transudative effusions. Eur RespirJ.
10(3):714-8,1997
3. Muller NL: Imaging of the pleura. Radiology. 186:297-309,
1993
4. Raasch BNet al: Pleural effusion: Explanation of some
typical appearances. AJR. 139:899-904, 1982
 TRANSUDATIVE PLEURAL EFFUSION

I IMAGE GAllERY 1
47

(Left) Frontal radiograph

shows a right effusion,
subpulmonic fluid with
blunting of right
costophrenic angle (arrow).
Note cardiomegaly and a left
ventricular aneurysm (open
arrow). (Right) Lateral
radiograph in same patient
shows opacification and
blunting at the right posterior
costophrenic sulcus
(arrows). Note calcified left
ventricular aneurysm (open
arrow).

(Left) Radiograph in right

side down decubitus position
shows layering of pleural
fluid in dependent right
hemithorax (arrows). > 1 em
width indicates sufficient
fluid for thoracentesis.
(Right) Frontal radiograph
shows effusion along major
fissure. Radiolucency medial
to the fissure (arrow)
represents the right lower
lobe with some volume 105s.
The appearance mimics a
medial pneumothorax.

Typical
(Left) Frontal radiograph
shows lateral deviation of the
dome of the diaphragm
(arrow) and mild blunting of
the costophrenic sulcus.
Subpulmonic effusion.
(Right) Frontal radiograph in
a patient undergoing
peritoneal dialysis shows
extensive opacification in
right hemithorax (arrow) due
to a large right effusion.
Transdiaphragmatic
extension of dialysate.
 HEMANGIOENDOTHELIOMA
1
48

Frontal radiograph shows a large pleural-based mass Axial CECTshows the mass to be heterogeneous, with
medially in the left apex producing deviation of the necrosis and coarse calcifications. The mass obstructs
trachea (arrows). The mass had gradually increased in the lelt brachiocephalic vein, leading to dilated
size over a 5 year period. subcutaneous vessels (arrow).

Location
ITERMINOLOGY o Most common in lung or pleura
Abbreviations and Synonyms o Many other sites
Intravascular bronchioloalveolar cell carcinoma Liver
Intravascular bronchoalveolar tumor (IVBAT) Skin and subcutaneous tissues
Intravascular sclerosing bronchoalveolar tumor Bone
Spleen
Definitions o Rare sites
Rare intermediate grade endothelial cell tumor Pericardium
May arise in lung or pleura as well as other sites in Thyroid
body Peritoneum
Probably on a spectrum with more malignant Chest wall
angiosarcoma Size
o Effusion may be small or large
o Pleural rind may be thin or lobular
IIMAGING FINDINGS o Lung nodules often small at presentation
Morphology
General Features o Pleural disease mimics mesothelioma
Best diagnostic clue o Lung disease most often multiple nodules
o Pleural form: Large unilateral loculated effusion with Rarely may present as solitary pulmonary nodule
diffuse lobular pleural thickening
May be misdiagnosed as mesothelioma, Radiographic Findings
sarcomatoid variant Radiography
o Pulmonary form: Multiple small nodules o Pleural form presents as small to moderate effusion

DDx: Pleural Masses

Mesothelioma Metastatic Disease Synovial Sarcoma

 HEMANGIOENDOTHELIOMA

Key Facts
1
Terminology Empyema 49
Intravascular bronchioloalveolar cell carcinoma Pleural Metastases
Rare intermediate grade endothelial cell tumor Other Primary Pleural Tumors
May arise in lung or pleura as well as other sites in Hematogenous Metastases
body Pathology
Imaging Findings Histologic features consistent with origin from
Pleural form: Large unilateral loculated effusion with endothelial cells
diffuse lobular pleural thickening Pleural fluid cytology often negative
Pleural disease mimics mesothelioma Fine needle aspiration can lead to bleeding
Pleural form presents as small to moderate effusion Clinical Issues
About 10% contain calcification Pleural disease usually symptomatic
Pulmonary form most often multiple small bilateral Pulmonary form more often asymptomatic
nodules
Diagnostic Checklist
Top Differential Diagnoses
When no known primary site outside chest
Mesothelioma Resembles mesothelioma but may be more indolent

Decreased lung volume on affected side

Fluid may be loculated
I DIFFERENTIAL DIAGNOSIS
Tumor may extend into fissures Mesothelioma
May produce lymphangitic appearance through Very similar appearance
invasion into interstitial septae Pleural fluid and pleural tumor in varying volume
About 10% contain calcification More often invades chest wall than
May have mediastinal adenopathy hemangioendothelioma
May have concomitant pulmonary nodules Less often involves mediastinal nodes
o Pulmonary form most often multiple small bilateral
nodules Empyema
Rarely can present as solitary pulmonary nodule May also produce loculated fluid and thickening of
pleura
CT Findings
Lobular masses less common
CECT May show gas pockets
o Pleural form resembles mesothelioma or pleural Clinical presentation generally characteristic
metastasis
Variable volume of fluid Pleural Metastases
Fluid often loculated Very similar in appearance to hemangioendothelioma
Variable volume of tumor along pleural surfaces and mesothelioma
May be smooth or lobular Frequent history of primary tumor
May invade mediastinum Common primaries include breast, ovary, lung
May produce lymphangitic spread along lung Immunohistochemistry can be helpful
septae
Chest wall invasion is relatively rare Other Primary Pleural Tumors
May have associated adenopathy Localized fibrous tumor, benign or malignant
About 10% contain calcification, may not be Synovial sarcoma
visible on radiography Angiosarcoma
o Pulmonary form resembles diffuse hematogenous
Hematogenous Metastases
metastases
Frequent history of primary tumor
May represent multifocal origin from lung rather
than metastases Sharply defined variable sized pulmonary nodules

Nuclear Medicine Findings

PET !PATHOlOGY
o Use of FOG PET may be indicated in multifocal
tumor General Features
Would allow easiest detection of many sites Genetics: Tumor of vascular origin
Etiology
Imaging Recommendations o May be related to asbestos exposure
Best imaging tool: CT more sensitive for pleural and o May be related to vinyl chloride exposure
pulmonary disease Possible link to silicone breast implants
Protocol advice: IV contrast optional
 HEMANGIOENDOTHELIOMA
1 Gross Pathologic & Surgical Features
50 Dense white rind of tissue
Closely adherent to underlying lung
May spread into fissures and lung septae
Microscopic Features
Histologic features consistent with origin from
endothelial cells
o Pinocytotic vesicles
o May form tubular arrays of cells
o May contain intracytoplasmic lumena containing
red cells or red cell fragments
o Immunoreactive to endothelial cell markers
o Not reactive to epithelial, muscle, mesothelial or
neural markers
o Myxoid and hyaline stromal matrix
Electron microscopy shows Weibel-Palade bodies
o Oval dense inclusions with striated appearance
o Found in normal endothelial cells and
well-differentiated endothelial tumors
Pleural fluid cytology often negative
o Similar to mesothelioma
Fine needle aspiration can lead to bleeding
I CLINICAL ISSUES
Presentation
Most common signs/symptoms
o Pleural disease usually symptomatic
Chest pain
Dyspnea
Shortness of breath
Cough
Fever
Weight loss
o May rarely present with hemoptysis or hemothorax
o Pulmonary form more often asymptomatic
Other signs/symptoms: May occasionally produce
hypertrophic pulmonary osteoarthropathy (HPO)
Demographics
Age
o Pleural form occurs in older patients
o Lung form may occur in younger patients
Gender
o Pleural form probably more common in men
Some variation from study to study
o Pulmonary form probably more common in women
Natural History & Prognosis
Prognosis not good for pleural form
o Often recurs years la ter
o Occasional cases with spontaneous partial remission
o Most do not respond well to chemotherapy
Prognosis better for pulmonary form
o Indolent disease with long survival
Treatment
Resection
Decortication
Extrapleural pneumonectomy
Lung transplant, for pulmonary form
o Presumed to represent multifocal origin rather than
metastases
I DIAGNOSTIC CHECKLIST
Consider
When no known primary site outside chest
Image Interpretation Pearls
Resembles mesothelioma but may be more indolent
o Less propensity for chest wall invasion than
mesothelioma
Pulmonary form resembles metastases
I SELECTED REFERENCES
1. Al-Shraim M et al: Primary pleural epithelioid
haemangioendothelioma with metastases to the skin. A
case report and literature review.J Clin Pathol. 58:107-9,
2005
2. Marsh Rde W et al: Breast implants as a possible etiology of
epithelioid hemangioendothelioma and successful therapy
with interferon-alpha2. Breast.J 11:257-61, 2oo5
3. Sakamoto N et al: High resolution CT findings of
pulmonary epithelioid hemangioendothelioma: unusual
manifestations in 2 cases. J Thorac Imaging. 20:236-8, 2005
4. Fagen Ket al: Detection of a pulmonary
hemangioendothelioma by FDG PETscan. Clin Nucl Med.
29:758-9,2004
5. Rest CC et al: FDG PETin epithelioid
hemangioendothelioma. Clin Nucl Med. 29:789-92, 2004
6. Attanoos RLet al: Malignant vascular tumours of the
pleura in 'asbestos' workers and endothelial differentiation
in malignant mesothelioma. Thorax. 55:860-3, 2000
7. Crotty EJet al: Epithelioid hemangioendothelioma of the
pleura: clinical and radiologic features. AJR.175:1545-9,
2000
8. Zhang PJ et al: Malignant epithelioid vascular tumors of
the pleura: report of a series and literature review. Hum
Pathol. 31:29-34, 2000
9. Kitaichi M et al: Pulmonary epithelioid
haemangioendothelioma in 2] patients, including three
with partial spontaneous remission. Eur RespirJ. 12:89-91,
1998
10. Yokoi Ket al: Epithelioid hemangioendothelioma
presenting as a chest wall tumor. Thorac Cardiovasc Surg.
45:254-6, 1997
11. Un BTet al: Malignant vascular tumors of the serous
membranes mimicking mesothelioma. A report of 14 cases.
Am J Surg Pathol. 20:1431-9, 1996
12. Struhar D et al: Alveolar hemorrhage with pleural effusion
as a manifestation of epithelioid haemangioendothelioma.
Eur RespirJ. 5:592-3, 1992
13. Yousem SAet al: Unusual thoracic manifestations of
epithelioid hemangioendothelioma. Arch Pathol Lab Med.
111:459-63,1987
 HEMANGIOENDOTHELIOMA

I IMAGE GALLERY 1
51

(Left) Frontal radiograph

shows marked elevation of
the right lung base with
lateralization o( the
diaphragmatic peak
suggesting subpulmonic fluid
(arrow) and shift of the
mediastinum to the left.
(Right) Axial CECT shows
large heterogeneous mass
producing marked shilt of
the heart to the left and
severe compression of the
right atrium (arrows). Pleural
hemangioendothelioma.

Variant
(Left) Frontal radiograph
shows relatively low lung
volumes and small vague
scattered lung nodules
bilaterally. (Right) Axial
NECT shows multiple small
round pulmonary nodules
predominantly in a
subpleural position.
Pulmonary
hemangioendothelioma.

(Left) Frontal radiograph

shows large right pleural
effusion extending over lung
apex and increase in
peripheral interstitial
markings bilaterally. (Right)
Axial NECT shows lung and
pleural disease in the same
patient, after drainage of the
pleural fluid. Fluid remains in
the fissure (arrow) and there
is bilateral interstitial disease
(curved arrows). Pleural
hemangioendothelioma.
 ASBESTOS RELATED PLEURAL DISEASE
1
52
Graphic shows typical plaques in parietal pleura and
diaphragms, sparing costophrenic angles and
mediastinal pleura. Plaques often calcify but do not
cause restrictive disease.
ITERMINOLOGY
Definitions
Benign pleural pathology related to asbestos exposure
o Entities include: Pleural plaques, benign pleural
effusions, diffuse pleural thickening and folded lung
I IMAGING FINDINGS
Radiographic Findings
Radiography
o Pleural plaques
Conspicuity on chest radiography dependent on
size, site and extent of calcification; visualization
improved by oblique radiographic views
Classical propensity for posterolateral, lateral,
diaphragmatic and pericardial pleura; sparing of
apices and costophrenic sulci
Invariably bilateral, when unilateral more
common in the left hemithorax
Rarely extend more than 4 rib interspaces; 2-5 mm
thick; relative symmetric involvement
DDx: Asbestos Related Pleural Disease
Empyema
Frontal radiograph shows bilateral calcified
diaphragmatic plaques (arrows) & chest wall plaques
(open arrows). Proper term is asbestos exposure & not
asbestosis (which refers to interstitial lung disease only).
Well demarcated but irregular elevations of pleura
(best seen in profile; more difficult when seen en
face)
Linear band of calcification when viewed in
profile, irregular "holly leaf" configuration en face
Shape: Individual plaques have squared shoulders
(butte shape) when viewed in profile
o Benign pleural effusions
Unilateral or bilateral and generally of small
volume 500 ml)
May eventually evolve into diffuse pleural
thickening
o Diffuse pleural thickening
Unilateral or bilateral smooth thickening of pleura
extending over at least one-quarter of chest wall
Ill-defined and irregular; calcification rare
Involvement of interlobar fissures and can involve
costophrenic angles (compare with pleural
plaques)
o Folded lung (also known as rounded atelectasis,
Blesovsky syndrome and atelectatic pseudotumor)
Peripheral, rounded mass
Associated parenchymal distortion/atelectasis
Adjacent pleural thickening
Mesothelioma Old TB Empyema
 ASBESTOS RELATED PLEURAL DISEASE

Key Facts
1
Terminology Folded lung (also known as rounded atelectasis, 53
Benign pleural pathology related to asbestos exposure Blesovsky syndrome and atelectatic pseudotumor)
Entities include: Pleural plaques, benign pleural Peripheral, rounded mass
effusions, diffuse pleural thickening and folded lung Associated parenchymal distortion/atelectasis
Adjacent pleural thickening
Imaging Findings CT more sensitive and specific in evaluation of
Pleural plaques pleural disease
Well demarcated but irregular elevations of pleura
(best seen in profile; more difficult when seen en
Top Differential Diagnoses
face) Previous Empyema
Benign pleural effusions Mesothelioma
Unilateral or bilateral and generally of small volume Previous Hemothorax
500 ml) Metastatic Adenocarcinoma
Diffuse pleural thickening Subpleural Fat
Unilateral or bilateral smooth thickening of pleura Peripheral Bronchogenic Carcinoma
extending over at least one-quarter of chest wall Rib Fractures
Serratus Anterior Muscle

o CT more sensitive and specific in evaluation of

CT Findings pleural disease
NECT Ultrasound sometimes of value in diagnosis of
o More sensitive than chest radiography in detection folded lung
of pleural disease
o Pleural plaques
Focal and discrete lesions I DIFFERENTIAL DIAGNOSIS
Separated from rib by thin layer of fat (seen as
lucent curvilinear line) Previous Empyema
Plaque calcification (seen in up to 15%) more Adjacent lung abnormal, scarring from previous
readily detected with CT than plain radiography pneumonia
Size of individual plaques and extent of Usually unilateral
calcification increases with time
o Benign pleural effusion Mesothelioma
CT generally more sensitive than radiography for Unilateral, nodular thickening (> 1 cm), creeping
detection of relatively small volumes of pleural mediastinal pleura and/or circumferential
fluid involvement
Fluid may have high attenuation if hemorrhagic Nearly always associated with pleural effusion
Evidence of pleural thickening at follow-up; Circumferential thickening markedly reduces volume
associated with "crow's feet" distortion of lung of involved hemithorax
parenchyma Previous Hemothorax
o Diffuse pleural thickening
Unilateral, multiple healed rib fractures
CT more sensitive than plain radiographs; also
distinguishes between true thickening and Metastatic Adenocarcinoma
(normal) extrapleural fat Lobulated unilateral pleural thickening in patient with
Continuous sheet of pleural thickening (> 5 cm is known malignancy; may be indistinguishable from
width, > 8 cm in cranio-caudal extent and> 3 mm mesothelioma
in thickness)
May be associated with underlying parenchymal Subpleural Fat
distortion ("crow's feet") Symmetric, mid-lateral chest wall 4th to 8th ribs; may
o Folded lung extend into fissures
Rounded or ovoid mass (forming acute angle with Associated with other fat deposition: Pericardial fat
chest wall pleural surface); may contain air pads, widened mediastinum
bronchograms No calcification
"Comet tail" appearance due to curved distortion
of bronchovascular structures towards mass; may Peripheral Bronchogenic Carcinoma
enlarge over time May mimic rounded atelectasis
Adjacent pleural thickening an invariable feature Spiculated outline (not "comet tail")
calcification Rib Fractures
Volume loss Abnormal rib contour, posterolateral location
Imaging Recommendations Hemidiaphragms normal
Best imaging tool
 ASBESTOS RELATED PLEURAL DISEASE
1 Serratus Anterior Muscle
54 Symmetric mid chest wall; between intercostal spaces
Triangular in shape with edge fading inferiorly and no
calcification
I PATHOLOGY
General Features
General path comments
o Pleural plaques
Arise almost exclusively from parietal pleura
Macroscopically comprising pale white and raised
regions of hyaline fibrosis with geographic outline
Smooth surface or showing fine nodularity
No evidence of malignant transformation or
association with mesothelioma
o Benign pleural effusion
Usually exudative but may be hemorrhagic or of
mixed cellularity
o Diffuse pleural thickening
Thickening and fibrosis of visceral pleura
(compare with pleural plaques) but then fusion
with parietal pleura
o Folded lung
Peripheral collapsed lung adjacent to region of
pleural thickening
Etiology
o Pleural plaques
More frequently associated with serpentine
asbestos (Le. chrysotile) than amphibole fibers;
latter tending to be retained in lung parenchyma
Exact pathogenesis uncertain but fibers probably
reach pleura via lymphatics leading to
inflammatory reaction
o Benign pleural effusion
Possible dose-response relationship between
severity of exposure and incidence of benign
effusions
o Diffuse pleural thickening
Preceded by benign pleural effusion in many
patients
True pathogenesis not clear
o Folded lung
Inflammation and fibrosis in superficial pleura
followed by contraction of pleura and atelectasis
of lung
May occur in context of any organizing pleural
exudate (e.g. following tubertulosis,
histoplasmosis, hemothorax, uremia)
Epidemiology
o Pleural plaques
Prevalence dependent on imaging test used for
detection (CT > chest radiography)
Very low prevalence in non-exposed subjects;
higher prevalence (up to 8%) in those
environmental exposure but highest (over 50%)
for occupational exposure
Long latency (> 20 years) between exposure and
appearance of plaques
Intensity of exposure less than that needed for
development of asbestosis
o Benign pleural effusion
Most common early (within 10 years of exposure)
manifestation of asbestos-related pleural disease
but latent period may be significantly longer
Incidence difficult to predict because many
subclinical
Generally resolve spontaneously within 6 months
(but may persist or recur); may be complicated by
diffuse pleural thickening
o Diffuse pleural thickening
Accurate prevalence unknown but may be as
frequent as pleural plaques
Latent period -15 years following exposure
More commonly associated with asbestosis than
plaque disease
o Folded lung
True prevalence unknown
Microscopic Features
Pleural plaques
o Either acellular or sparsely cellular collagen in
"basket weave" pattern
o Often calcified; increasing extent of calcification in
individual plaques with time
Benign pleural effusion
o Exudative, hemorrhagic, eosinophilic or mixed
cellularity fluid
Diffuse pleural thickening
o Similar to microscopic appearances to pleural
plaques with "basket weave" pattern of fibrosis and
scanty cellular infiltration
o Inflammation and fibrosis of visceral pleural
lymphatics followed by fusion of visceral and
parietal pleurae
o Large numbers of asbestos bodies may be present
and may be associated with asbestosis
Folded lung
o Inflammation and fibrosis in visceral pleura
IClINICAllSSUES
Presentation
Most common signs/symptoms
o Pleural plaques
Asymptomatic
Not associated with physiologic impairment per se
even when extensive
o Benign pleural effusions
Over 50% asymptomatic
Acute clinical features in some patients including:
Pleuritic chest pain, breathlessness, pyrexia and
elevation of white blood count
o Diffuse pleural thickening
Breathlessness, restrictive pulmonary physiology
o Folded lung
Generally asymptomatic but may be preceded by
symptoms related to underlying effusion
I SELECTED REFERENCES
1. Peacock C et al: Asbestos-related benign pleural disease.
Clinical Radiology. 55:422-32, 2000
 ASBESTOS RELATED PLEURAL DISEASE

IIMAGE GALLERY
1
55

(Left) Axial H RCT showing

bilateral discrete and
non-calcified pleural plaques
(arrows). (RighI) Axiall-IRCT
with a non-calcified plaque
in the left antero-Iateral
hemithorax (arrow).

(Lefl) Axial H RCT shows

bilateral smooth diffuse
pleural thickening (arrows).
There is an associated
"crow's feet" deformity o(
lung parenchyma (open
arrows) best appreciated on
the left. (RighI) Axial H RCT
with a bronchogenic
carcinoma in right lower
lobe (open arrow) in a
smoker with asbestos
exposure. A subtle,
non-calcified plaque is seen
in the left hemithorax
(arrows).

(Left) Axial H RCT with

bilateral folded lung. There is
parenchymal distortion
"comet tails" (open arrows)
and associated pleural
thickening (white arrows).
Note the pleural calcification
(black arrow). (RighI) Axial
H RCT showing discrete
bilateral pleural plaques and
subpleural ground-glass
opacification and fine
reticulation (arrows),
indicating asbestosis.
 Congenital
Pectus Deformity 11I-2-2
Kyphoscoliosis 11I-2-4
Poland Syndrome 11I-2-8

Infectious
Empyema Necessitatis 11I-2-10

Inflammatory - Degenerative
Ankylosing Spondylitis 11I-2-14
Elastoma, Fibroma, and Fibromatosis 11I-2-18

Neoplastic
Lipoma, Chest Wall 11I-2-22
Lymphoma, Chest Wall 11I-2-26
Askin Tumor, Chest Wall 11I-2-28
 PECTUS DEFORMITY

2
2

Frontal radiograph in an asymptomatic 20 year old Axial NECT shows compression and displacement of
woman shows leftward displacement of the heart and the heart by depressed sternum (arrows) causing
obscuraUon of the right heart border (arrows). obscuraUon of the right heart border on convenUonal
chest radiograph.

o Right heart border frequently obliterated because the

I TERMINOlOGY depressed sternum replaces aerated lung at the right
Abbreviations and Synonyms heart border
Pectus excavatum: Funnel chest o Heart displaced to the left and rotated (mitral
Pectus carinatum: Chicken breast or Pouter pigeon configuration), may cause spurious cardiomegaly
breast o Degree of depression best seen on lateral chest
radiograph
Definitions o In females, acute angle at medial superior margin of
Pectus excavatum: Sternum depressed so that anterior breasts
ribs protrude anteriorly more than sternum o Severity of defect can be quantified by CT or MR
Pectus carinatum: Anterior protrusion of the sternum; "Pectus index" = transverse diameter/ AP diameter
congenital or acquired Pectus index> 3.25 requires surgical correction
o Acquired: 50% of patients with atrial and ventricular Pectus carina turn: Three different types
septal defect have pectus carina turn o Chondrogladiolar protrusion (chicken breast):
o Acquired: Associated with cystic fibrosis and severe Anterior displacement of the sternum and
asthma (barrel chest) symmetrical concavity of the costal cartilages (most
common)
o Lateral depression of the ribs on one or both sides of
I IMAGING FINDINGS the sternum (frequently associated with Poland
syndrome)
General Features o Chondromanubrial prominence (Pouter pigeon
Pectus excavatum breast): Upper prominence with protrusion of the
manubrium and depression of the sternal body
(least common)

DDx: Obscuration of Right Heart Border

RML Collapse RML Pneumonia Morgagni Hernia

 PECTUS DEFORMITY

Key Facts
Terminology Heart displaced to the left and rotated (mitral
configuration), may cause spurious cardiomegaly
Pectus excavatum: Sternum depressed so that anterior
Severity of defect can be quantified by CT or MR
ribs protrude anteriorly more than sternum
Pectus carinatum: Anterior protrusion of the sternum; Top Differential Diagnoses
congenital or acquired Right Middle Lobe (RML) Collapse
Right Middle Lobe Pneumonia
2
Imaging Findings
Right heart border frequently obliterated because the Cardiophrenic Angle Mass 3
depressed sternum replaces aerated lung at the right
heart border

o Pectus excavatum: Mitral valve prolapse (20-60%)

I DIFFERENTIAL DIAGNOSIS o Pectus carinatum: Cyanotic congenital heart diseases
Right Middle Lobe (RML) Collapse
Wedge-shaped opacity overlying heart; lateral
radiograph - approximated minor and major fissure I CLINICAL ISSUES
Sternum normal Presentation
Right Middle Lobe Pneumonia Pectus ex cava turn and carinatum: Usually
Wedge-shaped opacity overlying heart; lateral asymptomatic; nonspecific chest or back pain
radiograph - no volume loss Pectus excavatum: Exercise induced decrease in
Sternum normal respiratory reserve or pain along the costal cartilages
Pectus excavatum: Occasionally cardiac (pulmonic
Cardiophrenic Angle Mass murmur, mitral valve prolapse, Wolff-Parkinson-White
Pericardia I fat pad syndrome)
Morgagni hernia
Demographics
Pericardial cyst
Adenopathy Gender
o M:F = 4:1
o Family history in 25-35% of cases
I PATHOLOGY Treatment
General Features Surgical correction
o Pectus index> 3.25
Epidemiology
o Respiratory or cardiovascular insufficiency
o Pectus excavatum: 1 in 300-400 births, most
o Psychosocial factors and cosmesis
common chest wall abnormality (90%)
o Pectus carinatum: Less frequent by a ratio of
approximately 1:5, 5-7% of chest wall deformities
Associated abnormalities
I SElECTED REFERENCES
o Musculoskeletal abnormalities: Marfan syndrome, I. Goretsky MJ et al: Chest wall anomalies: pectus excavatum
Noonan syndrome, and Osteogenesis imperfecta and pectus carinatum. Adolec Med Clin. 15:455-471,2004
o Scoliosis (15-20%)

I IMAGE GAllERY

(Left) Lateral radiograph shows a severe depression of the sternum (arrow) and a posterior displacement of the heart. (Center) Frontal
radiograph shows a bilateral ill-defined opacity with a paramediastinal distribution (arrows) in a 25 year old asymptomatic man with pectus
carina turn (chondromanubrial prominence). (Right) Lateral radiograph shows a marked prominence with protrusion of the sternal manubrium
(arrow) and depression of the sternal body.
 KYPHOSCOLIOSIS

2
4

Graphic shows S-curved scoliosis. Marked rotation is Frontal radiograph shows idiopathic right convex
evident al apices 0/ the curve and is reflected in rib scoliosisof the thoracic spine (arrows).
deformity Lungs will be restricted and de/armed in
thoracic cavity.

At their point of intersection, the angle is

ITERMINOLOGY measured
Abbreviations and Synonyms Radiographic Findings
Scoliosis, kyphosis, gibbus deformity Idiopathic kyphoscoliosis
Definitions o Usually convex to right
Complex three dimensional rotational curvature of the o Most cases, no kyphosis (hypokyphosis)
spine Actually represents rib "hump"
o Chest radiograph is difficult to interpret in severe
cases because of rotation of the thorax and heart
IIMAGING FINDINGS Neurofibromatosis type I, (Nfl, von Recklinghausen
disease)
General Features o Short segment angular scoliosis
Best diagnostic clue: Abnormal spinal curvature on Kyphosis more pronounced than scoliosis
anteroposterior and lateral radiographs Involves 5 vertebra or fewer in primary curve
Location: Cervical, thoracic, lumbar, sacral spine Nfl: Sharply angled at thoracolumbar junction
Size: Partial or entire spine involvement o Intervertebral foramina enlargement, due to
Morphology Nfl: Lateral thoracic meningocele
o Scoliosis: > 10 lateral deviation of the spine from Nfl: Neurofibromas, that may extend into spinal
the central axis canal
o Cobb angle: To indicate the scoliotic curve's angle o Lateral thoracic meningocele
Calculated by selecting the upper and lower end Herniation of meninges through intervertebral
vertebrae in a curve foramen
Erecting perpendiculars to their transverse axes Kyphoscoliosis, with meningocele on convex side

DDx: Kyphoscoliosis, Other Etiologies

Discitis Hemivertebra
 KYPHOSCOLIOSIS

Key Facts
Terminology Infectious spondylitis: Kyphosis, paraspinaI mass,
Complex three dimensional rotational curvature of bone destruction, disk space loss
the spine Echocardiogram: Mitral valve prolapse, in idiopathic
scoliosis (25%) and straight back syndrome (33%)
Imaging Findings Upright standing anteroposterior and lateral
Scoliosis: > 10 lateral deviation of the spine from the
0
radiographs, entire spine 2
central axis
Top Differential Diagnoses 5
Cobb angle: To indicate the scoliotic curve's angle
Usually convex to right Neurofibromatosis Type I
Neurofibromatosis type I, (Nfl, von Recklinghausen Infectious Spondylitis; Pott Disease
disease) Neuromuscular Etiology
Nfl: Sharply angled at thoracolumbar junction Clinical Issues
Intervertebral foramina enlargement, due to Restrictive lung disease
Nfl: Lateral thoracic meningocele Pulmonary artery hypertension; cor pulmonale
Nfl: Neurofibromas, that may extend into spinal Respiratory failure
canal

Round, well-defined, posterior mediastinal mass Peripheral rim-enhancement may occur

Right> left
Rib erosion and erosion of adjacent neural
foramen
10% multiple meningoceles
o Scalloping vertebral body
Anterior, posterior, lateral
MR Findings
o Wedge-shaped vertebra
o Hypoplastic or pressure remodeled pedicles
o Transverse process spindling
o Spondylolisthesis, spinal clefts, osteolysis
o Unstable spine, leading to subluxation or dislocation
o Spinal fusion, complicated by pseudoarthrosis, curve
progression
o Inferior rib notching, twisted ribbon-like upper ribs
o Pectus excavatum
Infectious spondylitis: Kyphosis, paraspinal mass, bone
destruction, disk space loss
Pott disease
o Ll vertebra corpus is the most common site; three or Echocardiographic
more contiguous vertebrae
o Erosive scalloping of the anterolateral surface of the
vertebral bodies (called gouge defect)
o Collapse of the intervertebral disk space Imaging Recommendations
o Progressive vertebral collapse with anterior wedging
o Angular kyphotic deformity and gibbus formation
o Paravertebral "cold" abscesses that may calcify
Congenital: Hemivertebra, fused ribs may lead to
scoliosis
Senile osteoporotic kyphosis: Compression fractures Protocol advice
multiple vertebra and cortical thinning
Ankylosing spondylitis
o Kyphosis, squared vertebral bodies
o Vertebral syndesmophytes, usually T9 to T12
o Interspinous ossification
o Ossification of costotransverse joints
o Manubriosternal joint erosion or fusions
CT Findings
CECT
o Lateral thoracic meningocele
Well-circumscribed, low-attenuation paravertebral
masses
CT myelography: Filling with intrathecally
injected contrast
o Neurofibromas
May show very low attenuation (10-20 HU)
Neurofibromatosis type I
o Dural dysplasia: Lateral meningoceles, dural ectasia
Shows cerebrospinal fluid content of
meningoceles
o Neurofibromas
Tl WI: Low to intermediate signal intensity
T2WI: Often heterogeneous
T2WI: High signal intensity regions, myxoid tissue
or cystic degeneration
T2WI: Central low signal intensity, collagen and
fibrous tissue
Tl C+: Enhance after contrast
Findings
Echocardiogram: Mitral valve prolapse, in idiopathic
scoliosis (25%) and straight back syndrome (33%)
Best imaging tool
o Radiography: Serial studies to assess for progression
To assess for skeletal maturity
o Complex cases: MR or CT with multiplanar
reconstructions
o Upright standing anteroposterior and lateral
radiographs, entire spine
Sitting radiographs for patients who cannot stand
Supine radiographs for patients who cannot sit
o Close surveillance during greatest growth, puberty
and early adolescence
o Radiation dose, approximately 140 microSv
MRI to detect peri/intraspinal abnormalities
 KYPHOSCOLIOSIS
Painful scoliosis
I DIFFERENTIAL DIAGNOSIS Clinical signs of dysraphism
Neurofibromatosis Type I Neck pain and headache, especially with exertion
Stigmata of neurofibromatosis Weakness, pes cavus, ataxia
o Neuromuscular disease: Poor cough reflex
Infectious Spondylitis; Pott Disease Susceptibility for pneumonia
Disc space involvement, vertebral destruction, sepsis Cardiac symptoms
2 Neuromuscular Etiology
o Pulmonic murmur
o Mitral valve prolapse
6 Upper motor neuron lesions: Cerebral palsy, o Syncope
syringomyelia, spinal cord trauma o Wolff-Parkinson-White syndrome
Lower motor neuron lesions: Poliomyelitis, spinal o Ankylosing spondylitis: Aortic valve stenosis
muscular atrophy o Neurofibromatosis type I
Myopathic conditions: Arthrogryposis, muscular Hypertension
dystrophy, and other forms of myopathies Coarctation aorta
Congenital Pulmonic valve stenosis
Atrial septal defect, ventricular septal defect
Hemivertebra, fused ribs, spina bifida, Klippel-Feil
Idiopathic hypertrophic subaortic stenosis
syndrome
Coronary artery disease
VATER(vertebral, anorectal, tracheal, esophageal,
Respiratory symptoms
renal) complex
o Kyphoscoliosis
Post Thoracotomy, Thoracoplasty Decreased compliance of lung and chest wall
Chest wall deformity, surgical history Restrictive lung disease
Hypoventilation, hypoxic vasoconstriction,
Complications after Radiation Treatment for hypercapnia
Childhood Malignancy Pulmonary artery hypertension; cor pulmonale
Hypoplasia ipsilateral pedicles in radiation port Respiratory failure
o Neurofibromatosis type I
Interstitial lung disease, basilar predominance
I PATHOLOGY Pulmonary artery hypertension, peripheral vessel
pruning
General Features Upper lobe bullae and honeycomb lung
Genetics Spontaneous pneumothorax/hemothorax
o Variety of diseases associated with scoliosis: o Ankylosing spondylitis
Friedrich ataxia, Morquio syndrome, Ehlers Danlos, Upper lobe fibrosis, mycetomas
Marfan syndrome, muscular dystrophy
Demographics
o Neurofibromatosis type I, autosomal dominant
Etiology: Majority are idiopathic Age
o Age at presentation: Congenital; infantile 3 years
Epidemiology
old)
o Idiopathic scoliosis
Juvenile (3-10 years); adolescent (> 10 years)
Prevalence, 1-3% for curves> 10
Gender: Idiopathic scoliosis: M:F = 1:4
80% of severe cases are idiopathic
o Neurofibromatosis type I: 50% of patients with Natural History & Prognosis
kyphosis Most severe in non-ambulatory patients
o Neuromuscular diseases Progression in neuromuscular disorders
90% of males with Duchenne muscular dystrophy Juvenile idiopathic scoliosis, nearly 90% of curves
60% of patients with myelodysplasia progress, almost 70% require surgery
20% of children with cerebral palsy Longstanding severe kyphoscoliosis
Associated abnormalities: Scoliosis associated with o Pulmonary artery hypertension
pectus excavatum or carinatum deformities o Respiratory failure, associated with a Cobb angle>
Gross Pathologic & Surgical Features 100
Pathophysiology: Not well understood Treatment
Scoliosis: Observation, orthosis, surgical correction
and stabilization
ICLINICAL ISSUES
Presentation
Most common signs/symptoms
I SELECTED REFERENCES
o Most patients are symptom free; many discovered at 1. Grissom LEet al: Thoracic deformities and the growing
school screening lung. Semin Roentgenol. 33(2): 199-208, 1998
o Indications for CT or MR imaging
2. Barnes I'D et al: Atypical idiopathic scoliosis: MR imaging
evaluation. Radiology. 186(1):247-53, 1993
Abnormal neurological examination
 KYPHOSCOLIOSIS

I IMAGE GALLERY

(Left) Frontal radiograph

shows severe right convex
scoliosis (arrows). Chest wall 2
deformity limits proper
evaluation of the lungs and
heart. (Right) Axial CECT in 7
same patient shows
counterclockwise rotation or
the vertebra (curved arrow).
This patient presented with
dyspnea, exacerbated by a
pneumothorax (arrow) that
is not visible on the
radiograph.

(Left) Frontal radiograph

shows right convex scoliosis
(arrows) of the thoracic
spine. (Right) Lateral
radiograph in same patient
shows peclus carinatum
deformity (arrow) and
kyphosis of the thoracic
spine (curved arrow).

(Left) Frontal radiograph

shows 5-shaped
thoracolumbar scoliosis
(arrows). Elevated right
diaphragm (curved arrow)
due to eventration. (Right)
Lateral radiograph shows
lordosis or
the thoracic spine
(arrows). Hypokyphosis, not
kyphosis, usually seen on the
lateral radiograph of scoliotic
patients.
 POLAND SYNDROME

2
8

Frontal radiograph shows increased radiolucency of Axial NEeT confirms the complete absence of the right
right hemithorax. In an asymptomatic patient, findings pectoral musculature (arrows).
strongly suggestive of congenital absence of the
pectoralis muscle.

ITERMINOLOGY I IMAGING FINDINGS

Abbreviations and Synonyms General Features
Pectoral aplasia-syndactyly syndrome Best diagnostic clue: Clinical suspicion: Syndactilism
with deformity of pectoral muscle
Definitions
Autosomal recessive condition Radiographic Findings
Congenital unilateral partial or total absence of Unilateral hyperlucency on chest radiograph
pectoralis major muscle Absence of the normal axillary fold on the affected
Rarely bilateral side
Associated anomalies Hypoplasia of the affected hand with hypoplastic
o Bony dysostoses affecting hand: middle phalanx
Brachymesophalangy with syndactyly, biphalangy, Rib deformities
and ectrodactyly
o Pectus excavatum CT Findings
o Anomalies of ipsilateral upper limb Absence or hypoplasia of pectoral girdle musculature
o Other anomalies: Absence of pectoralis minor, Hypoplastic breast
hypoplasia of latissimus dorsi and serratus anterior, Imaging Recommendations
hypoplasia or aplasia of nipple and breast, lung
Best imaging tool
herniation, and hypoplasia of hemithorax or ribs
o Chest radiograph usually sufficient to document
Increased incidence of: Leukemia, non-Hodgkin
thoracic abnormalities
lymphoma, lung cancer, and breast cancer
o CT and MR: More sensitive in detecting soft tissue
abnormalities

DDx: Unilateral Hyperlucency

Swyer-James Syndrome Lymphoma Breast Prosthesis

 POLAND SYNDROME

Key Facts
Terminology Imaging Findings
Autosomal recessive condition Unilateral hyperlucency on chest radiograph
Congenital unilateral partial or total absence of Rib deformities
pectoralis major muscle Absence or hypoplasia of pectoral girdle musculature
Associated anomalies
Bony dysostoses affecting hand: Brachymesophalangy
Top Differential Diagnoses 2
with syndactyly, biphalangy, and ectrodactyly Radiographic Artifact
Swyer-James Syndrome 9
Increased incidence of: Leukemia, non-Hodgkin
lymphoma, lung cancer, and breast cancer Radical Mastectomy/Prosthesis
Soft Tissue Mass of Chest Wall

I DIFFERENTIAL DIAGNOSIS I CLINICAL ISSUES

Radiographic Artifact Presentation
Malaligned grid Most common signs/symptoms: Asymptomatic,
Abnormal film density extends outside the thorax cosmetic deformity

Swyer-James Syndrome Demographics

Unilateral hyperlucent lung Gender: M > F
Pulmonary vasculature small in affected lung
Air-trapping on affected side with expiration
Treatment
Mosaic attenuation at HRCT Muscle flaps and breast implants: To correct muscle
deficiency and breast hypoplasia
Radical Mastectomy/Prosthesis Chest wall reconstruction if bony thorax is involved
Absen t or altered breast shadow o Homologous preservation of costal cartilage:
Often surgical clips in axilla Improves chest wall stability
History of breast cancer o Bone grafts or prosthetic mesh: Reconstruction of
aplastic ribs
Soft Tissue Mass of Chest Wall
Increased often asymmetric density of affected side
Extrapleural mass when extends into hemithorax I DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
I PATHOLOGY Look for occult lung cancer (increased incidence)

General Features
Genetics: Autosomal recessive I SElECTED REFERENCES
Epidemiology
1. Jeung MY et al: Imaging of chest wall disorders.
o True incidence/prevalence difficult to predict Radiographics. 19(3): 617-37,1999
Variable between groups (male vs. female) 2. Wright AR et al: MR and CT in the assessment of Poland
Prevalence: Ranges from 1/7000 to 1/100,000 live syndrome. J Comput Assist Tomogr. ] 6:442-447, ]992
births 3. Pearl M et al: Poland's syndrome. Radiology. 107:619-623,
197]

I IMAGE GALLERY

(Left) Anteroposterior radiograph shows an increased radiolucency of the hemithorax. Multiple rib deformities are also seen on the left chest wall
(arrow). Poland syndrome. (Cenler) Close-up view o( the left chest wall better shows the rib deformities. Also note an horizontal course of the
left anterior axillary fold as a result of the absence of the left pectoralis muscle (arrow). (Right) Frontal radiograph of the left hand shows
hypoplasia of the middle phalanx of the second finger (white arrow). Syndactyly is seen between the second and third fingers (black arrow).
 EMPYEMA NECESSITATIS

2
10

Graphic shows extension of empyema into the chest Frontal radiograph shows opacified left hemithorax
wall (arrow). Empyema necessitatis. (black arrow) and a calcific rind. Note marked
widening at the soft tissues of the chest wall (white
arrow). Tuberculous empyema necessitatis.

I TERMI NOlOGY Morphology

o Asymmetric chest wall soft tissues
Abbreviations and Synonyms o Obscured fascial planes
Empyema necessitans, cold abscess Radiographic Findings
Definitions Radiography
Chronic empyema attempting to decompress through o Soft tissue mass chest wall
chest wall o Pleural space widening, loculated pleural fluid
Common organisms o Pulmonary airspace opacification, atelectasis
o Tuberculosis, actinomycosis, staphylococcus, o Lung abscess containing air with extensions into
aspergillosis, mucormycosis, blastomycosis chest wall
Air may be visible in abscess and chest wall
o Pneumothorax, loculated
I IMAGING FINDINGS Bronchopleural fistula, subcutaneous emphysema
o Rib destruction, osteomyelitis, wavy periostitis
General Features o Tuberculosis
Best diagnostic clue Ribs often enlarge due to periostitis
o Loculated pleural fluid or mass with rib destruction Collection may be enclosed by a thick calcific rind
o Asymmetry of chest wall soft tissues CT Findings
Increased thickness of chest wall soft tissue
adjacent to parenchymopleural opacity CECT
o Loculated pleural fluid often admixed with air
Location: Chest wall abnormality, associated with
o Extension into chest wall or ribs
pleuropulmonary findings
o May extend into
Size: Variable, can be large
Paravertebral soft tissues

DDx: Chest Wall Mass

Necrotizing Fasciitis Staphylococcus Costochondritis Lung Cancer

 EMPYEMA NECESSITATIS
Key Facts
Terminology With or without peripheral calcification
Chronic empyema attempting to decompress through Best imaging too]: CT procedure of choice to
chest wall demonstrate chest wall and rib involvement
Common organisms Top Differential Diagnoses
Tubercu]osis, actinomycosis, staphylococcus,
aspergillosis, mucormycosis, blastomycosis
Tumors that Cross Fascial Planes
Lung cancer: Pancoast tumor traverses lung apex
2
Imaging Findings Lymphoma: May dissect into chest wall around 11
Locu]ated pleura] fluid or mass with rib destruction sternum or ribs without bony lysis
Asymmetry of chest wall soft tissues Primary Chest Wall Infection: Necrotizing Fasciitis
Increased thickness of chest wall soft tissue adjacent Pathology
to parenchymop]eural opacity Tuberculosis, most frequent cause
Tubercu]osis Responsible for about 3/4 of cases
Thick-walled, well encapsulated pleura] mass
Protrudes through chest wall or into abdomina] wall Clinical Issues
Low density abscess contents Tuberculous cold abscess: Lacks heat or redness on
Peripheral rim-enhancement the protruding mass

Vertebral column a Malignant mesothelioma

o Tuberculosis Tracks along biopsy or operative site
Thick-walled, well encapsulated pleural mass Chest wall tumor origin
Protrudes through chest wall or into abdominal a Askin tumor, malignant fibrohistiocytoma
wall a Desmoid tumor
Low density abscess contents III defined non-enhancing masses
Peripheral rim-enhancement a Elastofibroma
With or without peripheral calcification Benign, may be due to degenerating connective
Pleurocutaneous fistula, sinus tract formation, tissue between scapula and ribs
25% Rib origin
o Invasive aspergillosis a Chondrosarcoma, osteosarcoma
Pulmonary consolidation, pleural effusion, Large lobulated excrescent mass arising from rib
permeative osteolytic rib abnormalities, chest wall with scattered flocculent calcifications
mass, fistulas a Multiple myeloma, plasmacytoma
Well-defined punched out lytic lesions with
MR Findings associated soft tissue masses
Will show extent of chest wall involvement a Metastases from lung, kidney, breast, prostate
Tl C+ FS: Enhancement of peripheral rim, low signal
fluid/necrosis in abscesses Primary Chest Wall Infection: Necrotizing
Aspergillus: T2Wl, high signal intensity; T1 WI, Fasciitis
decreased signal intensity Infection of intermuscular fascial layers
Ultrasonographic Findings Spontaneous, or in patients with diabetes,
Grayscale Ultrasound: Can be used as a guide for immunosuppressed, post-trauma or surgery
biopsy or drainage Staphylococcus aureus, pseudomonas aeruginosa,
tuberculosis
Imaging Recommendations Loss of deep soft tissue planes
Best imaging tool: CT procedure of choice to Pyogenic osteomyelitis, periosteal elevation
demonstrate chest wall and rib involvement Soft tissue mass/abscess
Protocol advice: CECT: Standard technique
Costochondritis
Heroin addicts; Staphylococcus, streptococcus,
I DIFFERENTIAL DIAGNOSIS tuberculosis
Septic arthritis, sternoclavicular and sternochondral
Tumors that Cross Fascial Planes joints
Lung/pleural origin
a Lung cancer: Pancoast tumor traverses lung apex
May involve lower trunks of the brachial plexus I PATHOLOGY
May involve intercostal nerves, stellate ganglion,
General Features
adjacent ribs, vertebrae
General path comments: Extension of pleural
a Lymphoma: May dissect into chest wall around
infection into the chest wall with or without rib
sternum or ribs without bony lysis
destruction
May also originate from rib or chest wall
Etiology
 EMPYEMA NECESSITATIS
a Infection initially pneumonia with secondary o TB grows in 10-47% of cases
empyema with extension to ribs and chest wall Actinomycosis: Anaerobic culture, sulfur granules
a Mycobacterium tuberculosis: Acid-fast bacterium
Contiguous spread from underlying pleural or
pulmonary lesions ICLINICAllSSUES
a Actinomycosis: Rod-shaped bacterium, anaerobe,
sulfur granules Presentation
2 Oral colonization in patients with dental caries, Most common signs/symptoms
poor oral hygiene o Enlarging chest wall mass
12 Aspiration, pleuropulmonary infection Usually anterolateral chest
Pathogen produces proteolytic enzymes, creating Posterior trunk or abdominal wall
fistulas Breast
Traverses fascial planes from lung to pleura to Vertebral column
chest wall Brachial plexus involvement or cord compression
a Bacterial o Chest wall sinus drainage
Staphylococcus aureus o Mass may be painful or fluctuant
Streptococcus pneumoniae a Tuberculous cold abscess: Lacks heat or redness on
Post-operative complication of thoracotomy, the protruding mass
pneumonectomy or bypass surgery a Pyogenic: Hot and red skin
a Nocardia Fever, malaise, weight loss, pleuritic chest pain, night
Weakly acid-fast bacterium sweats, weight loss
Infection more likely in immunosuppressed Actinomycosis: Loose teeth, gingivitis, poor oral
patients hygiene
May uncommonly traverse tissue planes Immunosuppressed patients, leukemia, bone marrow
Must be treated because of potential for central transplant, heart transplant
nervous system (CNS) involvement o Fever, neutropenia
a Invasive aspergillosis a Angioinvasive fungi, chest wall involvement
Dimorphic fungus Diagnosis with fine needle aspiration biopsy
Mycelial form can invade vessels (angioinvasive) Specimens for smear and culture for aerobic and
and adjacent tissue anaerobic bacteria, fungi, and cytology
Immunosuppressed patients (e.g., leukemia,
transplant recipients, AIDS)
Demographics
Age: Childhood to elderly
Inhaled
Often fatal despite antibiotic treatment Gender: Actinomycosis, M:F = 4:1
a Mucormycosis Natural History & Prognosis
Fungus Tuberculous empyema, less common that pleuritis
Mycelial form can invade vessels (angioinvasive) a Chronic active infection
and adjacent tissue o Originates from pulmonary tuberculosis,
Often fatal despite antibiotic treatment lymphadenopathy, or hematogenous spread
a Blastomycosis o Large effusion with entrapped lung
Fungus, yeast form in tissue a Complications are bronchopleural fistula and/or
Rarely pleuropulmonary disease will progress to empyema necessitatis
involve chest wall, and ribs
Epidemiology Treatment
a Rare, was much more common in the pre-antibiotic Diagnosis with fine needle aspiration biopsy and
era than today microbiology
a Tuberculosis, most frequent cause Surgical drainage
Responsible for about 3/4 of cases Antibiotic treatment
Tuberculous chest wall involvement, uncommon Tuberculous empyema, and some bacterial infections
Associated abnormalities require chest tube drainage
a Rarely extends and involves retroperitoneum,
mediastinum
a May cause mastitis I SElECTED REFERENCES
Gross Pathologic & Surgical Features 1. Gotway MB et al: Thoracic complications of illicit drug use:
an organ system approach. Radiographies. 22 Spec
Collection of inflammatory tissue
No:S1l9-35,2002
Ruptures spontaneously through a weakness in the 2. Kim Y et al: Thoracic sequelae and complications of
chest wall into surrounding soft tissues tuberculosis. Radiographies. 21(4):839-58, 2001
Ordinarily, pleura is thin but difficult to traverse 3. Winer-Muram HT et al: Thoracic complications of
tuberculosis. J Thorac Imaging. 5(2):46-63, 1990
Microscopic Features 4. Bhatt GM et al: CT demonstration of empyema
Tuberculous (TB) empyema necessitatis. J Comput Assist Tomogr. 9(6):1108-9, 1985
o Purulent fluid, white blood cell (WBC) > 100,000
cells/mL, neutrophils
 EMPYEMA NECESSITATIS
I IMAGE GALLERY
Typical
(Left) Frontal radiograph in
same patient after
thoracentesis shows air
outlining the pleural and
chest wall space (open
2
arrows). The calcific rim is
13
often seen in tuberculous
empyema necessitaUs.
(Right) Axial CECT In same
patient shows calcified lung
i.e., auto-pneumonectomy
(curved arrow), a calcific
pleural rim due to
tuberculous empyema
(arrow), and chest wall
involvement (open arrow).

Typical
(Left) Frontal radiograph in a
patient with active
tuberculosis shows airspace
opacification in the right
lower lobe (arrow). (Right)
Axial CECT in same patient
shows right pleural effusion
(black arrow) and a complex
chest wall low density
collection with peripheral
enhancement (white arrows)
that represent "cold"
abscesses.

Typical
(Left) Axial NECT in a patient
with poor dentition shows
airspace opacification in the
right lower lobe (arrow).
(Right) Axial NECT in same
patient shows adjacent chest
wall mass (arrow). Smear
showed sulfur granules and
anaerobic culture of fine
needle aspirate grew
actinomycosis.
 ANKYLOSING SPONDYLITIS

2
14

Axial graphic shows typical parenchymal alterations in Axial HRCT shows corresponding alterations in
AS consisting of apical subpleural bullous and cystic advanced AS: Cysts (black arrows), interstitialmarkings
lesions with subtle interstitial thickening and mild (while arrow), traction bronchiectasis (open arrow).
traction bronchiectasis. Changes resemble IPFin advanced cases.

ITERMINOlOGY Radiographic Findings

Lung
Abbreviations and Synonyms
o Upper lobe symmetric fibrobullous disease, rare
Ankylosing spondylitis (AS) (1.25%)
Sacroiliitis o Cicatricial atelectasis and traction bronchiectasis
Thoracolumbar spondyloarthritis upper lobes
Ankylosis deformans o Stable or slowly progressive
Bechterew disease o Cysts and cavities, thick or thin-walled
Marie-Strumpell disease Fungus balls common
Definitions o Apical pleural thickening, pneumothorax, 8%
Chronic seronegative arthritis primarily involving the Skeletal changes
axial skeleton o Ankylosis (nearly always precedes lung disease)
o Extraspinal manifestations include iritis, pulmonary o Progression of vertebral changes
involvement, and aortitis Shiny corner sign (Romanus lesion): Small
erosions at corners of vertebral bodies surrounded
by reactive sclerosis
I IMAGING FINDINGS Squared vertebra body: Combination of corner
erosions and periosteal new bone along anterior
General Features vertebral body
Best diagnostic clue: Upper lobe fibrobullous disease Syndesmophytes: Ossification of outer fibers of
with spinal ankylosis annulus fibrosis
Calcification and ossification of paraspinous
ligaments

DDx: Ankylosing Spondylitis

Sarcoidosis Carcinoma
 ANKYLOSING SPONDYLITIS

Key Facts
Terminology Silicosis and Coal Worker's Pneumoconiosis
Chronic seronegative arthritis primarily involving the Pathology
axial skeleton Striking correlation with histocompatibility antigen
Extraspinal manifestations include iritis, pulmonary HLA-B27
involvement, and aortitis AS occurs in approximately 1 in 2,000 individuals
Aortic insufficiency, up to 10% of AS after 10 years of
2
Imaging Findings
Best diagnostic clue: Upper lobe fibrobullous disease disease 15
with spinal ankylosis Pleuropulmonary disease, 1-2% of AS
Upper lobe symmetric fibrobullous disease, rare Clinical Issues
(1.25%) Age: Disease onset: 15-35
Ankylosis (nearly always precedes lung disease) Gender: M:F = 8:1
Dilatation ascending aorta due to aortic insufficiency Usually, normal life span
Top Differential Diagnoses Most serious complication: Spinal fracture
Tuberculosis Bronchial artery embolization or surgery for life
threatening hemoptysis
Sarcoidosis

Complete fusion: Bamboo spine Lacks spinal ankylosis

o Fracture complications Aorta normal
Common locations: Cervicothoracic or Requires culture to exclude
thoracolumbar junctions
Chalk stick fracture: Typically transverse from Sarcoidosis
anterior to posterior through ossified disc space Lung involvement in bronchovascular distribution
Aorta Commonly accompanied by adenopathy early
o Aortic insufficiency Lacks spinal ankylosis
Dilatation ascending aorta due to aortic Aorta normal
insufficiency Silicosis and Coal Worker's Pneumoconiosis
Pleura Lung involvement in lymphatic pattern with
o Pleural effusion or thickening, rare centrilobular nodules and subpleural nodules
CT Findings May develop progressive massive fibrosis with
HRCT aggregation of nodules
o Apical fibrobullous disease Eggshell calcification in hilar and mediastinal lymph
Nonspecific appearance similar to tuberculosis nodes
Bronchial wall thickening or bronchiectasis Occupational history important
Tracheal dilatation from adjacent interstitial Lacks spinal ankylosis
fibrosis Aorta normal
Cystic change from paraseptal emphysema, Mediastinal Mass
cicatricial fibrosis, and cavities
Bronchogenic carcinoma
Mycetoma often found in cysts or cavities
Lymphoma
o Lymph nodes, borderline enlarged
Aortic aneurysm
o Non-apical interstitial lung disease (5%)
Identical pattern to idiopathic pulmonary fibrosis
(lPF): Ground-glass opacities, thickened
interlobular septa, honeycombing
I PATHOLOGY
Imaging Recommendations General Features
Best imaging tool Genetics
o Striking correlation with histocompatibility antigen
o Chest radiograph sufficient for diagnosis in most
HLA-B27
cases
o Occurs worldwide roughly in proportion to the
o CT may reveal subtle alterations at lung apex
undetected on chest radiograph prevalence of this antigen
o Association with B27 independent of disease severity
o CTA or MRA to investigate aorta
o One to 6% of adults inheriting B27 have been found
to have AS
o Susceptibility to AS almost entirely determined by
I DIFFERENTIAL DIAGNOSIS genetic factors
Tuberculosis Etiology: Lung disease unknown
Fibro-apical cavitary disease identical to ankylosing Epidemiology
spondylitis o AS occurs in approximately 1 in 2,000 individuals
 ANKYLOSING SPONDYLITIS
o Aortic insufficiency, up to 10% of AS after 10 years o Pulmonary function tests: Mixed restrictive and
of disease obstructive patterns
o Pleuropulmonary disease, 1-2% of AS o Acute anterior uveitis
Late onset, 15-20 years after spinal disease Most common extraarticular manifestation of AS
Associated abnormalities: Strongly associated with (25%)
inflammatory bowel disease Often precedes spondylitis
Typically unilateral ocular pain attacks
2 Gross Pathologic & Surgical Features
Pleuropulmonary
Photophobia, increased lacrimation
Secondary cataract and glaucoma
16 o Parenchymal destruction marked
Fibrobullous disease Demographics
Apical pleural thickening Age: Disease onset: 15-35
Mycetomas common Gender: M:F = 8:1
Bronchiectasis Ethnicity: Prevalence lower in African-Americans
Aorta
o Thickening of aortic valve cusps and/or aorta near Natural History & Prognosis
sinuses of Valsalva Initial involvement sacroiliac joint progressing up the
o Scar tissue can extend into ventricular septum, spine
resulting in functional heart block (atrioventricular Usually, normal life span
node in 95%) Mortality associated with
o Aortitis
Microscopic Features o Inflammatory bowel disease
Nonspecific fibrosis and chronic lymphocytic o Nephritis from amyloid deposition
infiltration Most serious complication: Spinal fracture
Elastic fragmentation and collagen degeneration o Can occur with even minor trauma due to rigid and
Primary site of pathology skeletal disease: Enthesis osteoporotic spine
(ligamentous attachment to bone) o Cervical spine most commonly involved
o Enthesitis characterized by edema of ligamentous o Fractures are often displaced and cause spinal cord
attachment injury
o Edema of adjacent bone marrow
o Formation of erosive lesions Treatment
No definitive treatment
Staging, Grading or Classification Criteria o Exercise to maintain functional posture and preserve
Diagnosis based on range of motion
o History of inflammatory back pain o Anti-inflammatory drugs for symptomatic pain relief
o Limitation of motion of lumbar spine in both o Total hip arthroplasty for severe arthritis and
sagittal and frontal plane stiffness
o Limited chest expansion, relative to standard Local glucocorticoid administration and mydriatic
reference values for age and gender agents for iritis
o Definite radiographic sacroiliitis Aortic valve replacement for valvulitis
Presence of B27 neither necessary nor sufficient for Bronchial artery embolization or surgery for life
diagnosis threatening hemoptysis
o B27 test can be helpful in patients with suggestive
clinical findings but normal radiographs
o Absence of B27 in a typical case of AS increases I DIAGNOSTIC CHECKLIST
probability for coexistent bone disease
Consider
Apical fibrous and bullous disease combined with
I CLINICAL ISSUES spinal ankylosis typical for A5

Presentation
Most common signs/symptoms I SELECTED REFERENCES
o Inflammatory back pain of AS distinguished by 1. Turetschek K et al: Early pulmonary involvement in
Insidious onset, age of onset below 40 ankylosing spondylitis: assessment with thin-section CT.
Duration greater than 3 months before medical Clin Radiol. 55(8):632-6, 2000
attention sought 2. Fenlon HM et al: Plain radiographs and thoracic
Morning stiffness, improvement with exercise or high-resolution CT in patients with ankylosing spondylitis.
activity AJR.168: 1067-72, 1997
Low grade fever and weight loss 3. Rosenow E et al: Pleuropulmonary manifestations of
Kyphosis common in advanced disease ankylosing spondylitis. Mayo Clin Proc 52:641-9,1977
4. Wolson AH et al: Upper lobe fibrosis in ankylosing
o Hemoptysis spondylitis. AJR124:466-71,1975
Mostly due to mycetomas
May be life-threatening
Other signs/symptoms
 ANKYLOSING SPONDYLI.TIS

I IMAGE GALLERY
Typical
(Left) Lateral radiograph of
cervical spine shows
demineralization of vertebrae 2
and calcification of anterior
ligament (arrows), giving the
impression of "flowing wax" 17
over the vertebral bodies.
(Right) Frontal radiograph of
the pelvis shows advanced
sacroiliitis with irregular
borders of sacroiliac joints,
demineralization, and
extensive erosions (arrows).

Typical
(Left) Axial HRCT shows
subtle subpleural
parenchyma/lesions
(arrows). Histological
diagnosis was nonspecific
interstitial pneumonia
(NSIP), lung function tests
were normal. Changes were
not visible on chest
radiograph. (Right) Axial
H RCT shows bilateral
subpleural disease (arrows).
Alterations are composed of
interstitial thickening,
ground-glass, subtle bullae,
and pleural irregularities.

Typical
(Left) Axial HRCT shows
apical bullous and cystic
disease in a patient wUh AS
(arrows). Subtle changes
were seen on chest
radiograph, but H RCT
reveals complete extent of
parenchymal alterations.
(Right) Axial H RCT shows
follow-up of the same patient
after three years. Note the
obvious progression of
bullous disease (arrows).
 ELASTOMA, FIBROMA, AND FIBROMATOSIS

2
18

Axial TlWI MR shows a well-defined slightly Corresponding axial T2WI MR image shows a high
heterogeneous mass (arrows). Subscapular signal intensity mass with interspersed linear and
elastofibroma, most of the mass has a signal intensity curvilinear areas of decreased signal (arrow).
higher than the adjacent musculature.

Intra-abdominal fibromatosis (intra-abdominal

ITERMINOLOGY desmoid tumor): Lesions occurring in the pelvis,
Abbreviations and Synonyms mesentery, and retroperitoneum
Benign fibroblastic proliferations
Fibromatosis (desmoid tumors)
Elastofibroma dorsi I IMAGING FINDINGS
Definitions General Features
Variety of benign fibroblastic proliferations and Best diagnostic clue: Solitary or multiple soft tissue
fibroblastic soft tissue tumors masses
Fibromatosis (desmoid tumors) Key concepts
o Superficial o Classification based on pathology, histology, clinical
Palmar fibromatosis (Dupuytren contracture) presentation, natural history, and patient age at
Plantar fibromatosis (Ledderhose disease) presentation
Penile fibromatosis (Peyronie disease) o Elastofibroma
Knuckle pads Considered a fibroelastic pseudotumor
o Deep (musculoaponeurotic) fibromatosis: Results from mechanical friction between the
Intermediate biologic behaviors between benign chest wall and the tip of the scapula
fibrous lesions and fibrosarcoma Manifest: Soft tissue mass with a characteristic
Extra-abdominal fibromatosis (extra-abdominal subscapular location (99%)
desmoid tumor) Bilateral in 10-66% of cases
Abdominal wall fibromatosis (abdominal desmoid Relatively common (autopsy incidence, 24% in
tumor) women; 11% in men)

DDx: Soft Tissue Tumors of Chest Wall

yoa. ,

I

\
"
, a
.J~~~
~,.-.
.
.
,
I

~-4
Fibrosarcoma
'",...
-;71 -1'\

Askin Tumor Malignant Schwannoma

 ELASTOMA, FIBROMA, AND FIBROMATOSIS

Key Facts
Terminology Top Differential Diagnoses
Benign fibroblastic proliferations Soft Tissue Sarcomas
Fibromatosis (desmoid tumors) Primitive Neuroectodermal Tumor (Askin Tumor)
Inflammatory and Infectious Processes
Imaging Findings
Best diagnostic clue: Solitary or multiple soft tissue Pathology 2
masses 18-20% of patients with Gardner syndrome develop
Classification based on pathology, histology, clinical desmoid tumor 19
presentation, natural history, and patient age at
presentation Clinical Issues
Soft tissue mass that may be hypodense, isodense or Wide surgical resection is the treatment of choice
hyperdense relative to muscle Diagnostic Checklist
Poorly defined margins Clinical presentation, natural history, and patient age
May enhance after injection of IV contrast material at presentation
Extra-abdominal desmoids are often hypervascular Usually solitary soft tissue lesions

Usually affect older individuals (mean age at o T2WI: Heterogeneous signal intensity
diagnosis, 70 years) approximating that of fat
o Deep (musculoaponeurotic) fibromatosis o T1 WI: Heterogeneous signal intensity
Benign tumor composed of fibrous elements approximating that of skeletal muscle
Rare: 0.03% of all neoplasms o Gd-DTPA: Moderate to marked enhancement; 10%
10-28% involve the chest wall (shoulder) of lesions do not enhance
Incidence: 3.7 new cases per million people per
year
Typically seen in the third and fourth decades of I DIFFERENTIAL DIAGNOSIS
life
Fibromatosis of the abdominal wall: Women of Soft Tissue Sarcomas
child-bearing age Common: Fibrosarcoma and malignant
Wide range of local aggressiveness (aggressive fibrohistiocytoma
fibromatosis) o Malignant fibrous histiocytoma: Most common
malignant soft tissue sarcoma in adults
CT Findings Others: Rhabdomyosarcoma, malignant schwannoma,
Elastofibroma hemangiopericytoma, and synovial sarcoma
o Poorly defined, crescent-shaped, heterogeneous soft Similar CT and MR appearances
tissue masses
o Contains linear low-attenuation streaks from fat Primitive Neuroectodermal Tumor (Askin
o Same attenuation as adjacent muscle Tumor)
Deep (musculoaponeurotic) fibromatosis Composed of small round cells with neural
o NECT differentiation
Soft tissue mass that may be hypodense, isodense Usually in children or young adults
or hyperdense relative to muscle Imaging findings: Large chest wall mass associated
Poorly defined margins with adjacent rib destruction, pleural thickening or
o CECT pleural effusion, and focal invasion of lung
May enhance after injection of IV contrast
material Inflammatory and Infectious Processes
Spontaneous or in association with diabetes,
Angiographic Findings immunosuppression, or trauma
Extra-abdominal desmoids are often hypervascular Empyema necessitatis: Tuberculosis and actinomycosis
MR Findings
Elastofibroma
o Lenticular well-defined mass with an intermediate
I PATHOLOGY
signal intensity General Features
o Areas of signal intensity similar to that of fat on Genetics
both T1 WI and T2WI o Deep (musculoaponeurotic) fibromatosis
o Heterogeneous enhancement after Gadolinium 30% show numerical chromosomal aberrations of
administration chromosome 8 (trisomy 8) or chromosome 20
Deep (musculoaponeurotic) fibromatosis (trisomy 20)
o Great variability of appearances Familial cases of fibromatosis have been reported
 ELASTOMA, FIBROMA, AND FIBROMATOSIS
Etiology o Abdominal and intra-abdominal desmoids: Women
o Elastofibroma more common
Mechanical friction between the chest wall and
the tip of the scapula Natural History & Prognosis
o Intra-abdominal fibromatosis (intra-abdominal Deep (musculoaponeurotic) fibromatosis
desmoid) o Prognosis is related to the age of patient
Exact cause is unknown Younger individuals 20 to 30 years): Higher
2 Usually rare recurrence rate
Locally aggressive growth pattern
Epidemiology: Elastofibroma: 2% incidence CT in
20 elderly Treatment
Associated abnormalities
Elastofibroma
o Intra-abdominal fibromatosis (intra-abdominal
o Surgery is curative
desmoid)
o Recurrence is rare (incomplete excision)
18-20% of patients with Gardner syndrome
Deep (musculoaponeurotic) fibromatosis
develop desmoid tumor
o Wide surgical resection is the treatment of choice
Exact cause is unknown o Adjuvant radiation therapy
Previous abdominal surgery (75% of cases) o Steroids, non-steroidal anti-inflammatory drugs
May be also associated with trauma and estrogen
therapy
Gross Pathologic & Surgical Features I DIAGNOSTIC CHECKLIST
Elastofibroma Consider
o Gray/white, well or poorly defined mass containing
entrapped adipose tissue Clinical presentation, natural history, and patient age
o Size: 5-10 em in dimension at presentation
Deep (musculoaponeurotic) fibromatosis Image Interpretation Pearls
o May have irregular or infiltrating borders Usually solitary soft tissue lesions
o White and coarsely trabeculated on the cut surface
o Usually larger than 5 em; may be larger than 15 em
Microscopic Features I SElECTED REFERENCES
Elastofibroma 1. Lee]C et al: Aggressive Fibromatosis: MRI Features with
o Fibroblasts, mature adipose tissue, and abundant Pathologic Correlation. A]R. 186:247-254, 2006
collagen and elastic fibrils 2. Mendenhall WM et al: Aggressive Fibromatosis. Am] Clin
Deep (musculoaponeurotic) fibromatosis Oncol. 28: 211-215, 2005
3. Schlemmer M: Desmoid Tumors and Deep Fibromatoses.
o Bland spindled or stellate fibroblastic cells Hematol Oncol Clin N Am. 19:565- 571, 2005
embedded in a collagenous stroma 4. Lindor NM et al: Desmoid tumors in familial adenomatous
o No evidence of muscular or neural differentiation polyposis: A pilot project evaluating efficacy of treatment
o Little or no inflammatory component with pirfenidone. Am] Gastroenterol. 98:1868-1874, 2003
o May infiltrate adjacent viscera and tissues at the 5. Robbin MR et al: Imaging of musculoskeletal fibromatosis.
periphery RadioGraphies. 21:585-600, 2001
6. Siegel M]: Magnetic resonance imaging of musculoskeletal
soft tissue masses. Radiol Clin North Am. 39:701-720,
2001
ICLINICAllSSUES 7. ]eung MYet al: Imaging of chest wall disorders.
RadioGraphies. 19:617-637, 1999
Presentation 8. Brandser EAet al: Elastofibroma dorsi: prevalence in an
Most common signs/symptoms elderly patient population as revealed by CT.A]RAm]
o Elastofibroma Roentgenol. 171:977-980, 1998
More than 50% of cases asymptomatic 9. Enzinger FMet al: Soft tissue tumors. 3rd edition. SI. Louis
Large lesions may ulcerate (MO), Mosby, 201-229, 1995
o Deep (musculoaponeurotic) fibromatosis 10. Hartman TE et al: MR imaging of extra abdominal
desmoids: differentiation from other neoplasms. Am]
Palpable mass Roentgenol. 158:58] -585, 1992
Abdominal pain (intra-abdominal desmoid) 11. Krandsdorf M] et al: Elastofibroma: MR and CT appearance
Demographics with radiologic-pathologic correlation. A]RAm]
Roentgenol. 159:575-579, 1992
Age 12. Casillas] et al: Imaging of intra- and extraabdominal
o Elastofibroma desmoid tumors. RadioGraphies. 11:959-968, 1991
Mean age 70 years 13. Quinn SFet al: MR imaging in fibromatosis: resuits in 26
o Deep (musculoaponeurotic) fibromatosis patents with pathologic correlation. Am] Roentgenol.
Between puberty and 40 years 56:539-542, 1991
Gender 14. Feld R et al: MRI of aggressive fibromatosis: frequent
o Elastofibroma: Females predominance 2:1 appearance of high signal intensity on T2-weighted
images. Magn Reson Imaging. 8:583-588, 1990
o Deep (musculoaponeurotic) fibromatosis: Women
more common
 ELASTOMA, FIBROMA, AND FIBROMATOSIS

I IMAGE GALLERY
Typical
(Left) Axial CECT of a 51
year old man with
extra-abdominal desmoid 2
tumor, shows a large 50ft
tissue mass with associated
deformity of the chest wall. 21
Peripheral enhancement is
seen (arrows). (Right)
Corresponding coronal T 1 WI
MR shows a well-defined 50ft
tissue mass with
heterogeneous areas of
marked/decreased signal
intensity (arrows).

Typical
(Left) Axial NEeT bilateral
elastofibromas in a 71 year
old man, shows bilateral
large subscapular masses
(arrows) with a density
similar of the adjacent
musculature. (Right) Axial
NECT of a 32 year old
woman with an
extra-abdominal (paraspinal)
desmoid tumor, shows a
large heterogeneous mass
with areas of slightly higher
attenuation (arrow) than
adjacent musculature.

Typical
(Left) Axial CECT shows a
large heterogeneous mass
with tow attenuation from
necrosis. Aggressive
fibromatosis in a 48 year old
woman. Note the intra- and
extrathoracic component
(arrow). (Right) Sagittal
T1 WI MR shows a large
mixed-signal intensity
polylobulated mass with
extension into
retroperitoneum and
paraspinal musculature
(black arrows). A second
mass is also seen (white
arrow).
 LIPOMA, CHEST WALL

2
22

Axial CECT demonstrates a typical chest wall lipoma, Axial T1WI MR shows a characteristic chest wall
presenting as a homogeneously fatty mass deep to right lipoma, appearing as a high signal, subcutaneous mass
latissimus dorsi muscle (open arrows). anterior to right pectoralis major muscle. Mass is defined
by a capsule (arrow).

Few or no septations
ITERMINOLOGY a Atypical
Abbreviations and Synonyms Calcification related to fat necrosis
Chest wall lipoma (CWL); liposarcoma (LS) Increased number & thickness of septations
Nonadipose areas
Definitions
Radiographic Findings
Lipoma = benign tumor composed of adipose tissue
Mass is radiolucent compared to other soft tissues
CT Findings
I IMAGING FINDINGS NECT: Mass is -SO to -100 HU
General Features CECT
a Septa can enhance slightly with iodinated contrast
Best diagnostic clue: Encapsulated mass with
material
composition identical to subcutaneous fat a Remainder of tumor does not enhance
Location: Back is most common chest wall location
Size: Most are 1-10 cm; if> 10 cm, worrisome for MR Findings
liposarcoma (LS) T1WI: High signal
Morphology T2WI: High signal
a Usually encapsulated, sometimes infiltrating T1 C+ FS
a Deep masses: Intramuscular, intermuscular or both a Signal suppresses with fat-saturation
a Typical a Septa can enhance with Gadolinium-based contrast
Soft & pliable material
Smooth, sharp margins; sometimes lobulated
Conforms to muscle, bone & fascial planes

DDx: Fatty Masses

Pleural Lipoma Liposarcoma Bochdalek Hernia

 LIPOMA, CHEST WALL
Key Facts
Imaging Findings Pathology
Best diagnostic clue: Encapsulated mass with Prevalence: 2% of population
composition identical to subcutaneous fat Soft, encapsulated, greasy, yellow-to-orange color
Location: Back is most common chest wall location Masses contain mature adipocytes that look very
similar to normal fat
Size: Most are 1-10 cm; if> 10 cm, worrisome for
liposarcoma (LS) 2
Deep masses: Intramuscular, intermuscular or both Clinical Issues
Soft, palpable mass 23
NECT: Mass is -SO to -100 HU
T1WI: High signal Small lesions can be left alone
T2WI: High signal Excision: Treatment of choice for indeterminate or
suspicious deep fatty tumors
Top Differential Diagnoses
Liposarcoma
Diagnostic Checklist
Pleural Lipoma Homogeneous mass identical to subcutaneous fat is
Spindle Cell Lipoma almost certainly benign
Lipoblastoma & Lipoblastomatosis Many fatty tumors are indeterminate by imaging
Hibernoma features

Variable
Nuclear Medicine Findings o Contours
PET o CT attenuation
o No uptake in lipomas o MR signal intensity
o FDG uptake ratio of tumor: Normal corresponds to o Enhancement
histological subtype of LS o Proportion of fat to soft tissue components
o False positive for LS: Other sarcomas, lymphoma & Increased concern
inflammation o Age> 60
o False negative for LS: Well-differentiated LS o Male
Imaging Recommendations o Mass> 10 cm
o Globular &/or nodular non-adipose areas
Best imaging tool
o Septal thickness> 2 mm
oCT
o Mass < 75% fat
Better for superficial masses
o Calcification
Excellent contrast sensitivity for characterizing fat
Quicker & less expensive Pleural Lipoma
More readily available Intrathoracic
o MR Arises from submesothelial layers of parietal pleura
Better for deep masses Encapsulated
Excellent contrast resolution CT: -SO to -100 HU
Better multiplanar imaging capability MR: Lipoma has high signal on Tl WI & T2WI; signal
Better definition of relationships to nerves & suppresses with fat-saturation
vessels
o FOG-PET Spindle Cell Lipoma
Can be helpful in primary tumors or looking for Benign, rare, middle-aged man
recurrence Posterior neck, chest & back
Protocol advice Well-defined, complex, fatty mass
o If septa are thickened or numerous, give contrast Intense enhancement of nonadipose component
material
oCT
Lipoblastoma & Lipoblastomatosis
Multiplanar reformations can be useful, Infants & children
particularly in deep tumors Usually does not recur after excision
o MR Lipoblastoma: Encapsulated
Select plane orientation that optimally displays Lipoblastomatosis: Infiltrative
relationships to important local anatomy Often appears indistinguishable from lipoma; young
T1 WI, T2WI, T2WI FS, STIR & T1 C+ FS sequences age suggests diagnosis
useful in differentiating lipomatous tumors Fat-containing mass in child < 2 years old likely a
lipoblastoma, even if nonadipose elements present
Hibernoma
I DIFFERENTIAL DIAGNOSIS Benign tumor of brown fat
Liposarcoma Adults
Shoulder, back, neck & chest
Chest wall LS make up 10% of all LS
 LIPOMA, CHEST WALL
Heterogeneous, enhancing tumor with fatty elements
Branching structures with serpentine high & low flow Treatment
vessels Small lesions can be left alone
Use image-guided biopsy to sample suspicious masses
Negative biopsy does not exclude tumor
I PATHOLOGY Removal can be indicated for cosmesis, relief of
symptoms or risk of malignancy
2 General Features o Nonexcision
Steroid injection: Causes fat atrophy, best for
Genetics
24 o Almost 60% of lipomas have clonal chromosomal lipomas < 1 cm
abnormalities Liposuction: Used when scar should be avoided;
o Most common: Translations & rearrangements small or large tumors
involving 12q13-q15 o Enucleation: Better for small lipomas
Etiology: Usually unknown o Excision: Treatment of choice for indeterminate or
Epidemiology suspicious deep fatty tumors
o Lipomas more common in obesity CWL's infiltrating muscle, tendon or nerve require
o Prevalence: 2% of population careful dissection & wide excision for removal
Associated abnormalities
o Multiple lipomas can be familial
o Bannayan-Zonana syndrome I DIAGNOSTIC CHECKLIST
Multiple lipomas, angiomas, macrocephaly
o Cowden syndrome Image Interpretation Pearls
Multiple lipomas, hemangiomas, hamartomas of Homogeneous mass identical to subcutaneous fat is
skin & bowel almost certainly benign
Increased risk of thyroid, breast & uterine tumors Many fatty tumors are indeterminate by imaging
o Frohlich syndrome features
Multiple lipomas, obesity & hypogonadotrophic CWL's & LS's can have a similar appearance
hypogonadism Indeterminate or suspicious fatty tumors should be
o Gardner syndrome referred for surgical evaluation
Lipomas, osteomas, abnormal dentition
Gastric polyps; large & small bowel polyps
Multiple benign & malignant tumors I SELECTED REFERENCES
o Proteus syndrome 1. Orevelegas A et al: Lipomatous tumors of soft tissue: MR
Multiple lipomas, hyperpigmentation of skin appearance with histological correlation. Eur J Radiol.
Fibroplasia of feet & hands, partial gigantism 50(3):257-67,2004
Vascular malformations, multiple nevi 2. Murphey MO et al: From the archives of the AFIP: benign
musculoskeletal lipomatous lesions. Radiographies.
Gross Pathologic & Surgical Features 24(5): 1433-66, 2004
Soft, encapsulated, greasy, yellow-to-orange color 3. Sandberg AA: Updates on the cytogenetics and molecular
genetics of bone and soft tissue tumors: liposarcoma.
Microscopic Features Cancer Genet Cytogenet. 155(1):1-24,2004
4. Bancroft LW et al: Imaging characteristics of spindle cell
Masses contain mature adipocytes that look very
lipoma. AJR Am J Roentgenol. 181(5):1251-4,2003
similar to normal fat 5. Tateishi U et al: Chest wall tumors: radiologic findings and
Tumor cells slightly larger than surrounding fat cells pathologic correlation: part 1. Benign tumors.
Radiographies. 23(6):1477-90, 2003
6. Tateishi U et al: Chest wall tumors: radiologic findings and
I CLINICAL ISSUES pathologic correlation: part 2. Malignant tumors.
Radiographies. 23(6):1491-508, 2003
Presentation 7. Gaerte SC et al: Fat-containing lesions of the chest.
Most common signs/symptoms Radiographies. 22 Spec No:S61-78, 2002
o Asymptomatic 8. Kransdorf MJ et al: Imaging of fatty tumors: distinction of
lipoma and well-differentiated liposarcoma. Radiology.
o Soft, palpable mass
224(1):99-104,2002
Other signs/symptoms: Lipoma in muscle is more 9. Salam GA: Lipoma excision. Am Fam Physician.
conspicuous when muscle contracts 65(5):901-4,2002
10. Schwarzbach MH et al: Assessment of soft tissue lesions
Demographics suspicious for liposarcoma by Fl8-deoxyglucose (FOG)
Age: Any, more common in 40-60 age group positron emission tomography (PET). Anticancer Res.
Gender 21(5):3609-14, 2001
o No clear gender difference 11. Weiss SW et al: Enzinger and Weiss's soft tissue tumors.4th
o Higher risk of liposarcoma for fatty tumors in males ed. St. Louis, Mosby. 571-639, 2001
12. Faer MJ et al: Transmural thoracic lipoma: demonstration
Natural History & Prognosis by computed tomography. AJRAm J Roentgenol.
Slow-growing 130(1):161-3,1978
 LIPOMA, CHEST WALL

I IMAGE GALLERY
Typical
(Left) Sagittal T7 WI MR
shows a lipoma, presenting
as an ovoid, high signal mass 2
in subcutaneous fat over
erector spinae muscles (open
arrows). Mass contains a few 25
fine septations, a normal
finding. (Right) Sagittal T2 WI
FS MR shows same
superficial chest wall lipoma
overlying erector spinae
muscles. Mass (arrows) has
low signal with fat
suppression, confirming
presence of fat within mass.

Variant
(Left) Axial T7 WI MR shows
a lipoma, appearing as a
large, high signal mass deep
to left serratus anterior
muscle (arrows). Dumbbell
component (open arrow)
insinuates through intercostal
space. (Right) Coronal T2WI
MR shows same large chest
wall lipoma, appearing as a
high signal mass. Dumbbell
components protrude
between ribs at two different
levels (open arrows).

Variant
(Left) Axial CECT
demonstrates a chest wall
lipoma, presenting as a fatty
mass with intra- and
extra-thoracic components
(arrows). (Right) Coronal
CECT shows same dumbbell
lipoma. Part of lipoma
causes a smooth, extrinsic
impression on lung (arrow) I

while another component

extends between ribs
(curved arrow).
 LYMPHOMA, CHEST WALL

2
26

Axial graphic shows an anterior mediastinal mass Frontal radiograph shows obscuration of right heart
causing mass effect on chest vessels (open arrow) and border (arrows) in a paUent with HD invading the
extending outside of right anterior chest wall (curved anteriorchest wall.
arrow).

!TERMINOlOGY CT Findings
Pleural mass extending into soft tissues of chest wall
Abbreviations and Synonyms Spinal involvement may extend into spinal canal
Non-Hodgkin lymphoma (NHL), Hodgkin disease Direct extension into anterior chest wall from anterior
(HD) mediastinal lymph nodes common
Definitions Isolated chest wall lesions without direct extension
Chest wall involvement by lymphoma secondary to can occur, especially in cases of recurrence
direct extension, secondary recurrence, or primary MR Findings
(rare) More sensitive than chest CT in detecting chest wall
involvement
o Twice as many lesions detected
I IMAGING FINDINGS MR more accurate at identifying parasternal soft tissue
involvement
Radiographic Findings
MR can identify possible spinal canal involvement
Chest wall mass with rib destruction
Lesions most evident using T2 weighted sequences
o Bone destruction may be lytic or sclerotic
o Lymphoma may grow around the ribs without Nuclear Medicine Findings
destroying them PET: FOG PET show increased uptake in chest wall
Other signs of thoracic lymphoma include
o Mediastinal nodal enlargement (most common), Imaging RecomlTlendations
pleural effusion, lung nodules, consolidated lung Best imaging tool
o CT usually sufficient for detection and
characterization

DDx: Chest Wall Tumors

Pancoast Askin Tumor Mesothelioma

 LYMPHOMA, CHEST WALL
Key Facts
Terminology Top Differential Diagnoses
Chest wall involvement by lymphoma secondary to Metastases
direct extension, secondary recurrence, or primary Stage IIIb or IV Bronchogenic Carcinoma
(rare) Askin Tumor
Imaging Findings Clinical Issues 2
Lymphoma may grow around the ribs without Chest wall involvement adverse prognostic factor
destroying them Typically treated by radiation therapy; for this reason 27
proper radiologic detection essential for treatment

Benefits: Biopsy localization, staging, radiotherapy o For HD, chest wall involvement 7%, rarely seen
planning without mediastinal involvement
o T2 weighted MR images most sensitive o For NHL, incidence of chest wall involvement is less
than in HD
Isolated chest wall lesions more common in NHL,
I DIFFERENTIAL DIAGNOSIS especially with the large cell lymphoma

Metastases
Common with adenocarcinomas ICLINICAl ISSUES
Multiple myeloma: Common cause of bony
destruction, though soft tissue masses (rare) Presentation
Most common signs/symptoms: May be asymptomatic
Stage IIlb or IV Bronchogenic Carcinoma or have painful palpable chest wall mass
Lung mass extending into ribs, vertebral body or bone
Natural History & Prognosis
Osteosarcoma or Chondrosarcoma Chest wall involvement adverse prognostic factor
Lesions can be osteolytic, osteoblastic, or both
Rib involvement common with chondrosarcoma, rare Treatment
osteosarcoma Typically treated by radiation therapy; for this reason
proper radiologic detection essential for treatment
Askin Tumor Chemotherapy regimens specific for their underlying
Malignant small cell tumors of neuroepithelial origin disease
Pleural or chest wall origin
Lipoma
Fat density
I SELECTED REFERENCES
1. Hodgson DC et al: Impact of Chest Wall and Lung
Invasion on Outcome of Stage 1-11Hodgkin's Lymphoma
I PATHOLOGY After Combined Modality Therapy. Int J Radiation
Oncology Bioi Phys. 57:1374-1381, 2003
General Features 2. Guermazi A et aI: Extranodal Hodgkin Disease: Spectrum of
Disease. Radiographies. 21:161-179, 2001
Epidemiology

IIMAGE GALLERY

(Left) Lateral radiograph shows nodular opacities along retrosternal chest wall (arrows). Subsequently, this patient was diagnosed with invasive
lymphoma. (Center) Axial CfCT shows large. heterogeneous anterior mediastinal mass in a patient with HD growing into left anterior chest wall
(arrows). (Right) Transverse CfCT shows large anterior mediastinal mass invading the chest wall and sternum (open arrow). Note the mass
effect on the airway and chest great vessels (curved arrow).
 ASKIN TUMOR, CHEST WALL

2
28

Graphic shows large mass filling the right hemithorax Axial CECT shows large Askin tumor (open arrow) with
(black arrows). White arrow shows chest waif compression atelectasis of right lung (black arrow).
involvement. Diagnosis: Askin tumor. Tumor involves pleura, multiple ribs (curved arrows)
and chest waif (white arrow).

!TERMINOlOGY Radiographic Findings

Radiography
Abbreviations and Synonyms a Large, unilateral, extra pulmonary mass
Ewing sarcoma family of tumors (ESFT) May be difficult to determine if mass originates
a Ewing sarcoma from chest wall or pleura
a Peripheral primitive neuroectodermal tumor May fill hemithorax
a Neuroepithelioma a With or without rib destruction (25-63%)
a Atypical Ewing sarcoma a Rapid growth
a Askin tumor a Lymphadenopathy
Definitions a Pleural effusion, frequent
Chest wall or pleural tumor: Askin tumor, primitive Smaller than mass
neuroectodermal tumor (PNET) a Lung and bone metastases
a Pathologic fractures
CT Findings
IIMAGING FINDINGS CECT
General Features a Heterogeneous mass due to hemorrhage and
necrosis
Best diagnostic clue: Large extrapulmonary mass in an
Calcification, rare
adolescent or young adult
a Pleural effusion> 90%
Location: Chest wall, pleural
a Local extension into chest wall, pleura, lung,
Size: Often very large
mediastinum
Morphology: Solid, compressing adjacent lung a Metastases (10%) at presentation to
Hilar/mediastinallymph nodes

DDx: Pleuropulmonary Chest Wall Mass

Fibrous Tumor Pleura Neuroblastoma Actinomycosis

 ASKIN TUMOR, CHEST WALL

Key Facts
Imaging Findings Top Differential Diagnoses
Best diagnostic clue: Large extrapulmonary mass in Ewing Sarcoma
an adolescent or young adult Rhabdomyosarcoma
May be difficult to determine if mass originates from Neuroblastoma, Ganglioneuroblastoma,
chest wall or pleura Ganglioneuroma
May fill hemithorax 2
With or without rib destruction (25-63%) Pathology
Positive staining for one or several neural markers 29
Rapid growth
Pleural effusion, frequent Electron microscopy: Presence of membrane-bound
Lung and bone metastases neurosecretory granules
Pathologic fractures Undifferentiated, small round blue cells
Unique metastases to sympathetic chaiu Clinical Issues
CT can best delineate bony involvement and
Palpable mass, with or without chest pain
metastatic disease
Askin: Overall survival poor
MRI best for soft tissue involvement
Diagnosis: Fine needle aspiration biopsy
Ewing sarcoma family of tumors treated similarly

Lung and bone

o Unique metastases to sympathetic chain Neuroblastoma, Ganglioneuroblastoma,
o After chemotherapy Ganglioneuroma
Extensive necrosis with pseudo-cystic appearance Involves sympathetic ganglion, adrenal medulla
Histology: Small round blue cells
MR Findings Neuroblastoma, most aggressive; ganglioneuroma,
Tl WI: Heterogeneous mass, high signal intensity benign
T1WI FS: Can best delineate soft tissue involvement, Adolescents and adults have well-differentiated tumors
extent of disease o Some tumors differentiate from neuroblastoma to
T2Wl: Heterogeneous mass, intermediate/high signal ganglioneuroma over time
intensity T2 75% < age 2 years
PD/lntermediate: Heterogeneous mass, high signal Posterior mediastinal paras pinal mass, often with
intensity calcifications
T1 C+: Enhances with gadolinium More likely to have intraspinal extension and cord
Nuclear Medicine Findings compression
Bone Scan: Tc-99m bone agent to assess for presence 1123/131-methyliodobenzylguanadine (MIBG)
or extent of bone metastases imaging
Thallium scan: 201 TI, to evaluate tumor response to o Accumulates in catecholaminergic cells
chemotherapy o Specific way of identifying primary and metastatic
disease
Imaging Recommendations
lymphoma
Best imaging tool
o CT can best delineate bony involvement and Pleural lymphoma usually a secondary manifestation
metastatic disease of known disease
o MRI best for soft tissue involvement Mass usually homogeneous without rib destruction
Protocol advice: MRI should be performed when spinal localized Fibrous Tumor of Pleura
involvement is suspected All age groups
Large lesions filling hemithorax, more likely to be
malignant
I DIFFERENTIAL DIAGNOSIS Pedunculated, may migrate with different positions
No chest wall involvement
Ewing Sarcoma
Paraneoplastic syndrome
Centered on bone (rib), otherwise similar radiographic
o Hypertrophic pulmonary osteoarthropathy (35%)
characteristics
and hypoglycemia (5%)
Histology: Small round blue cells
Osteosarcoma
Rhabdomyosarcoma
Adolescents, most common age
Most common soft tissue sarcoma in children Primary bone tumor may originate in thoracic cage,
Histology: Small round blue cells rib, scapula, spine
Thorax unlikely location Tumor may be lytic or blastic
Identical radiographic characteristics o Ossifying tumor
Chest wall and pleural extension
 ASKIN TUMOR, CHEST WALL
Pulmonary metastases
Staging, Grading or Classification Criteria
Osteomyelitis Localized or metastatic at presentation
Radiograph: Osteopenia, bone lysis, periosteal o 80% localized disease; 20% metastatic
reaction, soft tissue swelling
o Mass not as large as Askin tumor
Three-phase bone and Indium labeled neutrophil I CLINICAL ISSUES
2 scans: Positive with infection
Presentation
Empyema Necessitans Most common signs/symptoms
30
Infections that traverse tissue planes o Palpable mass, with or without chest pain
o Actinomycosis, tuberculosis, blastomycosis o Shoulder pain
Large mass or abscess involving lung, pleura, chest o Horner syndrome
wall o Dyspnea
Rib destruction o Cough
Loculated pneumothorax o Weight loss
o Cervical lymphadenopathy
o Pathologic fracture
I PATHOLOGY o Back pain
Other signs/symptoms
General Features o Fever and weight loss suggest metastatic disease
General path comments o Metastases to lung, pleura, bone, bone marrow
o Small round-to-oval blue cells and a lobulated o Thrombocytopenia with bone marrow involvement
stroma o Urinary catecholamine levels, normal
o Positive staining for one or several neural markers
o No significant differences with the immunomarkers Demographics
found in Ewing sarcomas Age: 20-30 years old, usual
o Electron microscopy: Presence of membrane-bound Gender: M: F = 1:1.3
neurosecretory granules Ethnicity: 9 times more common in Caucasians
Genetics
o Tumors show Natural History & Prognosis
Reciprocal translocation between chromosomes 11 Occasionally, it is a second malignancy
and 22, t(11;22) ESFT, 60-70% survival
o Families show increased incidence of Askin: Overall survival poor
neuroectodermal and stomach malignancies o 2 year rate of 38%; 6 year rate of 14%
Etiology Metastatic disease, < 25% survival
o Arises from the soft tissues of the chest wall Treatment
Probably from migrating embryonal cells of the
Diagnosis: Fine needle aspiration biopsy
neural crest
Ewing sarcoma family of tumors treated similarly
Possibly from post-ganglionic cholinergic neurons
o Based on localized versus metastatic disease
o May arise after radiation therapy for Hodgkin
Surgical excision
disease
Radiation therapy
Epidemiology
Chemotherapy
o Askin tumor: Rare
o Most common pleural mass in young adults
(especially women)
I SELECTED REFERENCES
o ESFT, incidence: 3 per million population/year
1. Schulman H et al: Thoracoabdominal peripheral primitive
Gross Pathologic & Surgical Features neuroectodermal tumors in childhood: radiological
Usually large, bulky tumors at diagnosis features. Eur Radiol. 10(10):1649-52,2000
2. Sallustio G et al: Diagnostic imaging of primitive
Microscopic Features neuroectodermal tumour of the chest wall (Askin tumour).
Undifferentiated, small round blue cells Pediatr Radiol. 28(9):697-702,1998
Similar to other ESFTs 3. Sabate JM et al: Malignant neuroectodermal tumour of the
chest wall (Askin tumour): CT and MR findings in eight
Routine stains and immunohistochemistry using patients. Clin Radiol. 49(9):634-8, 1994
antibodies 4. Winer-Muram HT et al: Primitive neuroectodermal tumors
o Positive for neuron-specific enolase of the chest wall (Askin tumors): CT and MR findings. AJR.
o Stains differentiate from rhabdomyosarcoma and 161:265-8,1993
lymphoma 5. Fink IJ et al: Malignant thoracopulmonary small-cell
Electron microscopy: Neurites and/or dense core ("Askin")tumor. AJRAmJ Roentgenol. 145(3):517-20, 1985
granules 6. Howman-Giles R et al: Gallium and thallium scintigraphy
Cytogenetic studies in pediatric peripheral primitive neuroectodermal tumor
(Askin tumor) of the chest wall. J Nucl Med. 36(5):814-6,
o To confirm a t(II;22) or related translocation
1985
 ASKIN TUMOR, CHEST WALL
I IMAGE GALLERY

(Left) Frontal radiograph

shows large mass (arrows)
filling right hemithorax with
pleural involvement (open
arrows) and a right effusion
2
(black curved arrow). Left 31
lung nodule (white curved
arrow) likely represents
metastasis. (Right) Lateral
radiograph in same patient
shows the mass (open
arrows) and a lower lobe
metastasis (arrow).

(Left) Axial CECT shows left

chest wall mass (arrow)
involving pectoralis muscles.
Diagnosis: Askin tumor.
(Courtesy B. Karmazyn,
MO). (Right) Axial CECT
shows pleural tumor
(arrows), pleural effusion
(black curved arrow) and
rib/chesl wall involvement
(while curved arrow).
Diagnosis: Askin tumor.

(Left) Frontal radiograph in a

patient with Askin tumor
shows a large mass in the
right hemithorax (arrows)
that resembles a pulmonary
mass. (Courtesy B.
Karmazyn, MO). (Right)
Axial NEeT in same patient
shows chest wall and rib
(arrows) involvement.
Anterior junction line is
displaced to the left (curved
arrow) indicating increased
volume of right hemithorax.
(Courtesy B. Karmazyn,
MO)
 Congenital
Eventration of Diaphragm 11I-3-2
Bochdalek and Morgagni Hernias 11I-3-6

Inflammatory - Degenerative
Phrenic Nerve Paralysis 11I-3-10
 EVENTRATION OF DIAPHRAGM

3
2

Lateral graphic shows eventration of the anterior portion /.;Jteralradiograph shows that the elevated portion of
of the left hemidiaphragm (arrow). Note that the the right hemidiaphragm involves only the anterior
posterior portion of the diaphragm is normally portion (arrows). Note that the posterior portion is
positioned (open arrow). normally positioned (open arrow).

More common on the right side

ITERMINOlOGY
Size
Abbreviations and Synonyms o On the frontal radiograph, typically reduces the
False diaphragmatic hernia projected lung height by one third
Diaphragmatic relaxation o Elevated anterior portion of hemidiaphragm can
Senescent diaphragm rarely project as high as the aortic arch on frontal
chest radiograph
Definitions Morphology: Lateral chest radiograph shows a
Non-paralytic weakening and thinning of anterior and "two-humped" hemidiaphragm, elevated anteriorly
dome of hemidiaphragm causing projected elevation
on frontal and lateral chest radiographs
CT Findings
Mild to markedly elevated anterior portion of
hemidiaphragm
IIMAGING FINDINGS Intact but thinned out muscle and tendon
Compressive atelectasis of the lower lung
General Features Contralateral shift of mediastinum, as the ipsilateral
Best diagnostic clue hemithorax volume can be markedly reduced
a Posterior portion of hemidiaphragm is normally Coronal or sagittal reformations can be helpful for
positioned diagnosis to confirm intact nature of hemidiaphragm
Best appreciated on lateral chest radiograph o Can be very thin, almost not discernible
a Typically unilateral Abdominal viscera are normally positioned below the
a Occasionally bilateral diaphragm
Location MR Findings
a Anterior portion and dome of hemidiaphragm
Similar to CT findings

DDx: Diaphragm Elevation Or Contour Abnormality

Traumatic Rupture Bochdalek Hernia Normal Variant

 EVENTRATION OF DIAPHRAGM

Key Facts
Terminology Pathology
Non-paralytic weakening and thinning of anterior Usually unilateral
and dome of hemidiaphragm causing projected
elevation on frontal and lateral chest radiographs
Clinical Issues
Usually asymptomatic
Imaging Findings Chronic atelectasis and chronic or recurrent
Posterior portion of hemidiaphragm is normally pneumonia are complications
positioned Acquired with advancing age, typically seen in elderly
Best appreciated on lateral chest radiograph age groups, particularly among women over the age
On the frontal radiograph, typically reduces the of 60
projected lung height by one third Typically benign course with good prognosis
Possibly more prone to rupture following minor
Top Differential Diagnoses trauma 3
Phrenic Nerve Injury With Paralysis of Surgical plication in complicated cases such as
Hemidiaphragm rupture or related respiratory failure 3
Diaphragmatic Rupture With Herniation of Viscera
Morgagni Diaphragmatic Hernia

Respiratory gating necessary for accurate

characterization
I DIFFERENTIAL DIAGNOSIS
Fluoroscopic Findings Phrenic Nerve Injury With Paralysis of
Upper GI: Normal position bowel loops, no Hemidiaphragm
constriction of bowel loops Fluoroscopic "sniff test" can distinguish
Fluoroscopic "sniff-test" Costophrenic angle often elevated with paralysis
o Used to determine diaphragmatic motion and
Diaphragmatic Rupture With Herniation of
function
o Can be used to distinguish eventration from a Viscera
paralyzed hemidiaphragm Antecedent history of high-energy blunt torso trauma
o Paralysis shows paradoxical movement with sniffing or penetrating trauma
o Eventration should show normal motion with Typically multiple associated injuries such as rib
sniffing fractures, pelvic fractures, hemo/pneumothorax,
o Uncommonly performed for this finding pulmonary contusion
Bowel loops constricted at the site of laceration
Nuclear Medicine Findings (kissing birds sign)
May be useful to distinguish herniation and associated
collar sign from normal visceral contours seen with Bochdalek Diaphragmatic Hernia
eventration Involves the posterior portion of the hemidiaphragm,
o Can be confusing on these studies if radiographs are best shown on lateral chest radiograph or CT
not compared Usually fat filled only
Occasionally, hernia contains viscera or kidney
Ultrasonographic Findings
Similar to CT findings Morgagni Diaphragmatic Hernia
Can be performed at bedside Occurs in the medial cardiophrenic angle, obscuring
Can be used to evaluate real-time, normal the right heart border
diaphragmatic motion Typically contains variable amounts of omental fat
Like CT, can provide a useful adjunct to conventional and bowel
chest radiography o Can be confused with fatty mediastinal tumors
o Accurately defines relationship to adjacent organs Much less common than Bochdalek hernias
Imaging Recommendations Ascites
Best imaging tool With or without associated pleural effusions
o Chest radiographs usually sufficient
Fluoroscopy or CT may be useful in problematic Subpulmonic Pleural Effusion
cases Lateralization (shouldering) of the dome of diaphragm
on frontal view
Upright frontal projection only, unless loculated
Decubitus views distinguish free-flowing effusions
from loculations
Entire diaphragm elevated on lateral
Wisps of fluid often extend into fissures
 EVENTRATION OF DIAPHRAGM

Hepatomegaly Treatment
CT scanning easily distinguishes Typically asymptomatic and needs no further
Typically uniform "elevation" of the hemidiaphragm evaluation or treatment
More closely mimics paralysis of hemidiaphragm or Surgical plication in complicated cases such as rupture
subpulmonic effusion or related respiratory failure
Exhalation
Bilateral very low lung volumes I DIAGNOSTIC CHECKLIST
Repeat examination with better inspiration, as needed
Consider
Tumor of Diaphragm (Sarcoma)
In the asymptomatic individual, consider normal
Also consider primary pleural tumor variation
Be sure to exclude a recent history of blunt trauma and
3 [PATHOLOGY associated traumatic injuries

4 Image Interpretation Pearls

General Features
Lateral costophrenic angle (frontal view) and posterior
General path comments gutter (lateral view) in normal location with no
o Usually unilateral blunting
o Occasionally bilateral
Genetics
o Can be congenital I SELECTED REFERENCES
o Rare association with Poland syndrome (ipsilateral
radial ray anomalies with absent pectoral muscles) 1. Haciibrahimoglu G et al: Video-assisted repair of an
eventrated left hemidiaphragm. Thorac Cardiovasc Surg.
Etiology: Overall position of the diaphragm related to
50(2):101-2,2002
a balance of the positive pressure in the peritoneum 2. Faheem M et al: Diaphragmatic rupture after epidural
and the negative pressure in the pleural space anaesthesia in a patient with diaphragmatic eventration.
Eur) Anaesthesiol. 16(8):574-6, 1999
Gross Pathologic & Surgical Features 3. Deslauriers): Eventration of the diaphragm. Chest Surg
Muscle is permanently elevated C1in N Am. 8(2):315-30,1998
Remains contiguous and has normal costal 4. Gierada DS et al: Imaging evaluation of the diaphragm.
attachments Chest Surg Clin N Am. 8(2):237-80, 1998
Thin muscle and tendon 5. Watanabe S et al: Large eventration of diaphragm in an
Normally no evidence of herniations elderly patient treated with emergency plication. Ann
Thorac Surg. 65(6):1776-7,1998
Lung does not typically show evidence of hypoplasia
6. Young TH et al: Isolated rupture of the right
hemidiaphragm with eventration of the liver demonstrated
by liver scan. Clin Nucl Med. 23(10):703-4, 1998
ICLINICAL ISSUES 7. Rais-Bahrami K et al: Right diaphragmatic eventration
simulating a congenital diaphragmatic hernia. Am)
Presentation Perinatol. 13(4):241-3, 1996
Most common signs/symptoms 8. Mitchell TE et al: Spontaneous rupture of a congenital
o Usually asymptomatic diaphragmatic eventration. Eur) Cardiothorac Surg.
o Chronic atelectasis and chronic or recurrent 8(5):281-2,1994
pneumonia are complications 9. Somers)M et al: Rupture of the right hemidiaphragm
following blunt trauma: the use of ultrasound in diagnosis.
Other signs/symptoms Clin Radiol. 42(2):97-101,1990
o Rarely can rupture (acquired or spontaneous) 10. Yeh He et al: Anatomic variations and abnormalities in the
leading to liver or bowel obstruction/strangulation diaphragm seen with US. Radiographies. 10(6):1019-30,
o Rarely can lead to acute progressive respiratory 1990
failure 11. Negre) et al: Hepatic coma resulting from diaphragmatic
rupture and hepatic herniation. Arch Surg. 121(8):950-1,
Demographics 1986
Age 12. Khan AN et al: The primary role of ultrasound in
o Acquired with advancing age, typically seen in evaluating right-sided diaphragmatic humps and
elderly age groups, particularly among women over juxtadiaphragmatic masses: a review of 22 cases. Clin
Radiol. 35(5):413-8, 1984
the age of 60
13. Okuda K et al: Age related gross changes of the liver and
o Congenital in children right diaphragm, with special reference to partial
o In one series, marked eventration was found in more eventration. Br) Radiol. 52(623):870-5,1979
than 1% of the women above 60 years 14. Salomon NW et al: Isolated rupture of the right
Gender: More common in women hemidiaphragm with eventration of the liver. )AMA.
241(18):1929-30,1979
Natural History & Prognosis 15. Armstrong RG et al: Liver scan in the diagnosis of ruptured
Typically benign course with good prognosis right hemidiaphragm with herniation of the liver. Ann
Possibly more prone to rupture following minor Thorac Surg. 6(5):480-3, 1968
trauma
 EVENTRATION OF DIAPHRAGM
I IMAGE GAllERY
Variant
(Left) Radiograph shows
markedly elevated left
hemidiaphragm (arrow). This
degree of elevation is less
common. Note normal
bowel gas in expected
position. Normal motion was
observed fluoroscopically.
(Right) CECT through the
lower chest shows the
elevated left hemi-diaphragm
(arrows) with normal
3
abdominal contents. Note
mass effect and shift of heart 5
and mediastinum.

(Left) Frontal radiograph

shows a slightly elevated
contour of the right
hemidiaphragm (arrow), as
compared to the normal left
side. Note the normal lateral
costophrenic angle. Lateral
view confirms diagnosis.
(Right) Lateral radiograph
shows that elevated portion
of right hemidiaphragm
again involves only anterior
portion. Note that posterior
portion is normally
positioned (arrow).

Typical
(Left) Frontal radiograph
again shows an abnormally
elevated contour of right
hemidiaphragm typical for
eventration (arrow). (Right)
Frontal view from a
hepatobiliary iminodiacelic
acid (/-IIDA) scan (same
patient as left) shows normal
liver activity conforming to
hump of elevated right
hemidiaphragm in this case
of eventration (arrows).
 BOCHDALEK AND MORGAGNI HERNIAS

3
6

Coronal graphic shows posterior diaphragmaUc defect Sagittal CECT shows large congenital Bochdalek hernia
with stomach and small bowel herniation into left containing numerous bowel loops. Diaphragmatic
hemithorax. Mediastinum shifted away from herniation. interrupUon (white arrow). Partially atelectatic lung
Both lungs are compressed (curved arrow). Left ventricle (black arrow).

Morphology: Usually smooth sharply marginated

ITERMINOlOGY
Radiographic Findings
Abbreviations and Synonyms
Congenital Bochdalek hernia
Congenital diaphragmatic hernia o Appearance depends on hernia contents and
Definitions whether air present within herniated bowel
Herniation of abdominal contents into the thorax o Left-sided 80%
through congenital or developmental diaphragmatic Initially: Hernia contents may be radiodense (prior
defects to air swallowing)
Bochdalek hernia: Through posterior pleuroperitoneal Hours later: Hernia contents contain cystic air
hiatus structures from small bowel loops
Morgagni hernia: Through anterior parasternal hiatus Marked contralateral shift of mediastinum and
compression of contralateral lung
o Right-sided hernia usually contains liver and
IIMAGING FINDINGS omentum and not bowel
o Decreased distension and decreased bowel gas in
General Features abdomen
Location o Catheter position clue to diagnosis
o Primarily right-sided Nasogastric (NG) tube descends into abdomen and
Morgagni hernia curves into stomach above hemidiaphragm
o Primarily left-sided NG tube in the mid-hemithorax deviated away
Congenital Bochdalek hernia from the hernia contents
Adult Bochdalek hernia o Adverse radiographic findings
Size: Small to large Lack of aerated ipsilateral lung

DDx: Bowel-Like Intrathoracic lucencies

Traumatic Hernia Gastric Volvulus In Hiatal Hernia Eventration

 BOCHDALEK AND MORGAGNI HERNIAS

Key Facts
Terminology Congenital Cystic Adenomatoid Malformation
(CCAM)
Herniation of abdominal contents into the thorax
Epiphrenic Diverticulum
through congenital or developmental diaphragmatic
defects Pathology
Imaging Findings If stomach in the herniated contents, implies that
herniation occurred earlier in utero
Appearance depends on hernia contents and whether
air present within herniated bowel Clinical Issues
Gastric volvulus sometimes associated with Morgagni Other signs/symptoms: Any hernia can present as
hernia acute abdominal emergency (acute obstruction or
Top Differential Diagnoses strangulation)
Hiatal or Paraesophageal Hernia Diagnostic Checklist 3
Eventration of Diaphragm Pleural effusions suggest strangulation of bowel
Traumatic Diaphragmatic Rupture 7
contents in hernia sac
Pericardial Cyst or Fat Pad
Posterior Mediastinal Mass

Lack of contralateral aerated lung Congenital Bochdalek hernia: CT usually not required
Contralateral pneumothorax for diagnosis
Severe mediastinal shift
MR Findings
Right-sided hernia
Herniated stomach: Stomach fixates early and it's Similar accuracy to CT
herniation infers earlier herniation in utero Lack of nonionizing radiation useful in infants and
o Adult Bochdalek hernia children
66% left-sided, 33% right-sided; bilateral in 15% Diaphragmatic motion limits image quality of
Smooth and sharply marginated posterior peridiaphragmatic lesions
diaphragmatic defect, more common medially Fluoroscopic Findings
o Morgagni hernia Useful to determine location of bowel and size of
Majority right-sided (heart prevents herniation on defect
left) Morgagni hernia
Smooth and sharply marginated anterior o Transverse colon situated high in the abdomen
retrosternal or parasternal mass, usually o Mid-transverse colon peaked towards the sternum
homogeneous unless it contains bowel virtually pathognomonic
If centered on heart, may be mistaken for Bowel hernia
cardiomegaly o In small defects (especially true of traumatic
o Bowel hernia diaphragmatic tear) bowel loops approximate
Pleural effusion: Suspect strangulation of bowel in ("kissing sign") as they traverse the defect
hernia sac
Pleural fluid should egress through open Imaging Recommendations
diaphragmatic defect into abdomen Best imaging tool: CT with multi planar
o Gastric volvulus sometimes associated with reconstructions commonly used to evaluate
Morgagni hernia peridiaphragmatic lesions and to characterize lesions
Double bubble sign: Air-fluid levels above and as hernias, pleural, mediastinum or lung in origin
below diaphragm
o Bowel obstruction
Dilated bowel in herniated sac with decompressed I DIFFERENTIAL DIAGNOSIS
distal bowel
Hiatal or Paraesophageal Hernia
CT Findings Retrocardiac air-containing masses
Adult Bochdalek hernia Common finding in adults
o Posteromedial (paraspinal) diaphragmatic defect
o Thickening of diaphragmatic edges from retraction Eventration of Diaphragm
o Majority contain fat only, less commonly kidney or Non-paralytic weakening and thinning of anterior
bowel dome of hemidiaphragm
o Common incidental finding Posterior portion of hemidiaphragm normally
Morgagni hernia positioned
o Majority contain omentum Bowel loops not contricted (no kissing birds sign)
o Omentum contains vessels (fat pads have sparse
Traumatic Diaphragmatic Rupture
vessels)
o May contain bowel, most commonly colon History o(trauma but may be remote
 BOCHDALEK AND MORGAGNI HERNIAS
Linear tear through posterolateral aspect of central Adult Bochdalek hernia contents
tendon o Development or congenital defect pleuroperitoneal
Generally left-sided hiatus
Size varies, contain stomach, colon, small bowel, o Retroperitoneal fat, rarely kidney or bowel
spleen and liver in that order Morgagni hernia contents
o Development defect between diaphragm muscle and
Pericardial Cyst or Fat Pad
ribs (space of Larrey)
No air o Omentum, liver, colon, or stomach
Generally right-sided
Fluid or fat density with cross-sectional imaging
Posterior Mediastinal Mass I CLINICAL ISSUES
Differential includes neurogenic tumors Presentation
(neurofibroma, schwannoma)
Most common signs/symptoms
3 Usually paraspinal, Bochdalek hernias usually more
lateral in location
o Congenital Bochdalek hernia: Neonatal respiratory
distress
8
Congenital Cystic Adenomatoid o Adult Bochdalek hernia: Asymptomatic, incidental
Malformation (CCAM) radiographic diagnosis
o Morgagni hernia
Multicystic, air-containing mass
Asymptomatic most common, may have
Herniated contents often change from film to film,
retrosternal pressure gastrointestinal symptoms
CCAM unchanging
Other signs/symptoms: Any hernia can present as
Epiphrenic Diverticulum acute abdominal emergency (acute obstruction or
Pulsion diverticulum strangulation)
Generally right-sided Demographics
May contain air or air-fluid level
Age: Any age
Communicates with esophagus
Natural History & Prognosis
Congenital Bochdalek hernia
I PATHOLOGY o Prognosis worse with larger volume of herniated
contents
General Features o Prognosis worse the earlier that herniation occurs
Genetics: Possible association of Morgagni hernias o Mortality 30%
with Down syndrome o Diagnosis rarely delayed, some present as adults
Etiology Adult Bochdalek hernia and Morgagni hernia
o Congenital Bochdalek hernia o Usually incidental finding of no clinical
Occurs around 10th week of gestation consequence
Herniated abdominal contents compress lung and
prevent normal development Treatment
Degree of hypoplasia major factor in prognosis Congenital Bochdalek hernia
If stomach in the herniated contents, implies that o Early surgical repair when stabilized
herniation occurred earlier in utero o In utero repair possible with prenatal sonographic
When bowel returns to the peritoneal cavity, the diagnosis
stomach is the first viscera to fixate in place Adult Bochdalek hernia and Morgagni hernia
Right-sided hernia (especially when delayed) o Surgical reduction for obstruction or strangulation
associated with group B streptococcal pneumonia
o Adult Bochdalek hernia
Probably acquired as incidence increases with age I DIAGNOSTIC CHECKLIST
o Morgagni hernia
Probably developmental as incidence increases Image Interpretation Pearls
with age and with conditions that increase Pleural effusions suggest strangulation of bowel
intra-abdominal pressure (obesity, straining) contents in hernia sac
Epidemiology
o Congenital Bochdalek hernia: 1 in 2,000 live births
o Adult Bochdalek hernia: 5-10% adults I SElECTED REFERENCES
o Morgagni hernia: Rare 2% of all diaphragmatic 1. Barut J et al: Intestinal obstruction caused by a strangulated
hernias morgagni hernia in an adult patient. J Thorac Imaging.
20(3):220-2, 2005
Gross Pathologic & Surgical Features 2. Eren 5 et ai: Diaphragmatic hernia: diagnostic approaches
Congenital Bochdalek hernia contents with review of the literature. Eur J Radial. 54(3):448-59,
o Congenital defect pleuroperitoneal hiatus 2005
o Multiple abdominal viscera, most commonly small 3. Anthes TB et al: Morgagni hernia: CT findings. CUff Probl
bowel, colon, stomach, spleen and liver Diagn Radial. 32(3): 135-6, 2003
 BOCHDALEK AND MORGAGNI HERNIAS
I IMAGE GALLERY

(Left) Frontal radiograph

shows mass-like density in
the right costovertebral angle
(arrows) from Morgagni
hernia. Differential includes
large pericardia I cyst or fat
pad. (Right) Lateral
radiograph shows that the
mass density is anteriorly
located (arrows). Mass is
homogeneous with no air 3
lucencies. CT recommended
for Further evaluation.
9

(Left) Coronal CECT MPR in

same patient shows that the
mass density is fat. Right
anterior diaphragmatic
defect (curved arrows)
contains omental vessels
(arrows). (Right) Coronal
CECT MPR in a diFFerent
patient shows a Morgagni
hernia containing colon.
Kissing birds sign with
colonic loops constricted at
the diaphragmatic site
(arrows).

(Left) Frontal radiograph

shows bowel-like /ucencies
in lower hemithorax
(arrows). Small leFtpleural
eFFusionblunts cp angle
suggesting bowel
strangulation. Sagittal
reconstruction shown
previously. Congenital
Bochdalek hernia. (Right)
Coronal CECT MPR shows
bowel herniation through
Bochdalek deFect. Esophagus
dilated and obstructed
(arrows). No pleural eFFusion
(meniscus on chest
radiograph from mesenteric
Fat).
 PHRENIC NERVE PARALYSIS

3
10

Graphic shows paralyzed elevated left hemidiaphragm. Frontal radiograph shows right hilar mass (arrow) and
Base of left lung parUally atelectatic due to mass effect of elevation of right hemidiaphragm (open arrow). Note
abdominal contents posiUon of right hemidiaphragm in relation to normal
left hemidiaphragm.

[TERMINOLOGY Radiographic Findings

Definitions Radiography
o Passive, compressive atelectasis is very common
Elevation of hemidiaphragm may be due to Mimics pneumonia
abnormalities in the diaphragmatic muscle, phrenic o Bilateral hemidiaphragm muscle weakness can be
nerve, or adjacent lung, pleura, mediastinum, and
difficult to assess as the radiographic findings are
abdomen
nonspecific, with low lung volumes being the only
manifestation
Elevated accentuated dome of diaphragm without
IIMAGING FINDINGS meniscus sign
General Features Costophrenic angles and posterior gutters are
deepened, narrowed and sharpened
Best diagnostic clue: Fluoroscopy useful to determine
motion Decubitus chest radiographs used to assess for
subpulmonic effusions that can mimic a true elevated
Morphology
hemidiaphragm
o True hemidiaphragm paralysis exhibits elevation of
the entire muscle Check prior films to determine chronicity
o Eventration involves only the anterior portion of o Can be present and asymptomatic for many years
the hemidiaphragm o Chronicity does not exclude the diagnosis
Does not show paradoxical motion when CT Findings
examined with the sniff test CTA: Multislice coronal reformations can better show
o Abdominal viscera are usually normally positioned the true nature of the "elevated" hemidiaphragm
unless ascites present CT of neck and chest

DDx: Phrenic Nerve Paralysis

Subpulmonic Effusion Ascites Hepatomegaly

 PHRENIC NERVE PARALYSIS

Key Facts
Terminology With unilateral hemidiaphragm paralysis, the
Elevation of hemidiaphragm may be due to affected muscle will move paradoxically compared
abnormalities in the diaphragmatic muscle, phrenic with the normal side
nerve, or adjacent lung, pleura, mediastinum, and Top Differential Diagnoses
abdomen Eventration of the Diaphragm
Imaging Findings Hernias
True hemidiaphragm paralysis exhibits elevation of Subpulmonic Effusion
the entire muscle Subphrenic Mass Effect
Eventration involves only the anterior portion of the Clinical Issues
hemidiaphragm
Bilateral paresis can lead to dyspnea, orthopnea,
Passive, compressive atelectasis is very common
Observe the hemidiaphragm motion under normal
respiratory failure, hypercapnia
Other signs/symptoms: Symptoms related to lung
3
tidal breathing cancer or other invasive intrathoracic malignancy
Compare the motion of hemidiaphragm under tidal 11
breathing with the hemidiaphragm motion during a
rapid sniff maneuver

o For mass invading the phrenic nerve or Sniffing focuses respiratory function more
peridiaphragmatic pathology exclusively on diaphragm contraction and much
o Thickened diaphragm muscle with traumatic less so on the effects of accessory muscle of
rupture respiration, such as the muscles of the chest wall
In patients with suspected phrenic nerve dysfunction With unilateral hemidiaphragm paralysis, the
who are having chest CT for any reason, obtaining affected muscle will move paradoxically compared
several dynamic images during forced exhalation can with the normal side
show the lack of motion artifact in the lung During a strong sniff, the paralyzed muscle will
parenchyma elevate while the normal muscle will depress
o Like the "sniff test", this may be a useful adjunct to
diagnosing phrenic nerve injury and
hemidiaphragm paralysis I DIFFERENTIAL DIAGNOSIS
MR Findings Eventration of the Diaphragm
Can best show anatomy of diaphragm with sagittal To distinguish eventration, look at the lateral chest
and coronal reconstructions radiograph
The eventration involves only the anterior portion of
Fluoroscopic Findings
the hemidiaphragm; posterior portion should be
Fluoroscopy can be used to assess for normal versus normally positioned
paradoxical hemidiaphragmatic motion
Hernias
Other Modality Findings
Foramen of Bochdalek (> 75% left side): Posterior
Barium studies: May show herniation of bowel Morgagni (most on right): Anterior and located at the
indicating hernia or traumatic rupture cardiophrenic angle
Imaging Recommendations Traumatic rupture: 90%, left side often contains bowel
Best imaging tool o Multiple associated injuries above and below the
o Fluoroscopic "sniff" test or ultrasound dynamically diaphragm
assess function of muscle lobar Atelectasis
Effective for unilateral paralysis only
Hilar displacement
Protocol advice ]uxtaphrenic peak (upper lobe)
o Observe the hemidiaphragm motion under normal
Mediastinal shift
tidal breathing
Even with bilateral diaphragm paralysis there will Infarct from Pulmonary Embolism
be some normal motion of the diaphragm as the Splinting elevates diaphragm
lungs ventilate secondary to accessory muscles of Humped-shaped area of consolidation (rare)
respiration
o Compare the motion of hemidiaphragm under tidal Scoliosis
breathing with the hemidiaphragm motion during a Apparent elevation on concave side of scoliosis
rapid sniff maneuver
Subpulmonic Effusion
Subpulmonic effusion can mimic an elevated
diaphragm
 PHRENIC NERVE PARALYSIS
Although the hemidiaphragm is apparently elevated, Pericardium (level of midcoronal plane through
the "dome" is shifted laterally the heart)
Lateralization of the dome causes "shouldering" of the Physiology
apparent diaphragmatic contour o Mean excursion of diaphragm 2.5 to 3.5 em (range 2
Fluid may extend into fissures - 8.5 em)
Decubitus chest radiographs can usually distinguish
pleural fluid from true elevation
!ClINICAlISSUES
Subphrenic Mass Effect
Multiple causes include hepatic tumors, hepatic Presentation
abscess, ascites, bowel obstruction, gastroparesis, Most common signs/symptoms
pancreatic pseudocyst, obesity, pregnancy, and o Usually no symptoms
abdominal compartment syndrome o Bilateral paresis can lead to dyspnea, orthopnea,
respiratory failure, hypercapnia
3 Large Epicardial Fat Pads
Homogeneous fat with few septa or vessels
Symptoms markedly accentuated in supine
position
12 Up to 50% reduction in vital capacity in supine
position
I PATHOLOGY o Can also lead to cardiac or gastrointestinal
General Features symptoms
Other signs/symptoms: Symptoms related to lung
Etiology
cancer or other invasive intrathoracic malignancy
o Unilateral paralysis
Most common cause of unilateral paralysis is hilar Demographics
lung cancer with phrenic nerve invasion (30%) Gender: No distinction other than increased
Muscular: SLE myopathy or muscular dystrophy prevalence of lung cancer in men
Idiopathic
Viral neuropathy usually involves right phrenic Natural History & Prognosis
nerve Typically permanent and stable, depending upon
Postcardiac surgery: Manipulation of injury during etiology
cardioplegia, previously up to 10%, now less Occasionally transient when the phrenic nerve is
common due to better surgical techniques injured peri-operatively
Diabetes mellitus
Subphrenic causes due to ascites, abdominal mass,
Treatment
massive obesity, pregnancy Usually none for unilateral paralysis
o Bilateral paralysis Bilateral paralysis
Neurologic etiologies due to cervical cord or o Ventilatory support with positive pressure
brainstem injury, multiple sclerosis, myasthenia ventilation
gravis o Diaphragmatic plication
Cervical spine surgery o Diaphragmatic pacing in quadriplegia, long term
Unilateral etiologies also results of pacing poor
Epidemiology: Incidence unknown
Associated abnormalities
o Shrinking lungs syndrome of systemic lupus I DIAGNOSTIC CHECKLIST
erythematosus
Consider
Characterized by unexplained dyspnea, a
restrictive pattern on pulmonary function test Bilateral paralysis in symptomatic patient with
persistent poor lung volumes
results, and an elevated hemidiaphragm
Cause remains controversial; some attribute the Image Interpretation Pearls
disorder to diaphragmatic weakness and others to Paradoxical motion on fluoroscopic sniff testing
chest wall restriction
Gross Pathologic & Surgical Features
Phrenic nerve
I SELECTED REFERENCES
o Arises from 3-5 cervical nerves 1. Kumar N et al: Dyspnea as the predominant manifestation
o Course right phrenic nerve of bilateral phrenic neuropathy. Mayo Clin Proc.
Lateral to right brachiocephalic vein and superior 79(12):1563-5, 2004
vena cava 2. Flageole H: Central hypoventilation and diaphragmatic
eventration: diagnosis and management. Semin Pediatr
Anterior to hilum Surg. 12(1):38-4S,2003
Pericardium left atrium (level of midcoronal plane 3. McCaul JA et al: Transient hemi-diaphragmatic paralysis
through the heart) following neck surgery: report of a case and review of the
o Course left phrenic nerve literature. J R Coil Surg Edinb. 46(3):186-8, 2001
Lateral to left brachiocephalic vein and aortic arch 4. Shanmuganaathan Ket al: Imaging of diaphragmatic
Anterior to hilum injuries. J Thorac Imaging. IS (2):104-11,2000
 PHRENIC NERVE PARALYSIS
I IMAGE GALLERY

(Left) Lateral radiograph

from the same patient as the
first case shows elevation of
right hemidiaphragm (white
arrow) ;n relation to normal
left hemidiaphragm (black
arrow). (Right) Axial GCT
(rom the same patient shows
large right lung mass with
extension to mediastinal
contour, where the phrenic 3
nerve is likely involved
(arrow).
13

Other
(Left) Axial CECT through
lower chest shows massive
hepatomegaly as etiology of
right hemidiaphragm
elevation. Note distinct
difference in height of the
right vs. left
hemidiaphragms. (Right)
Axial CECT through lower
chest shows ascitic fluid
around the liver (arrows) as
etiology of right
hemidiaphragm elevation.

Other
(Left) Frontal radiograph
shows apparent elevation of
right hemidiaphragm, in this
case due to a large hepatic
abscess, as shown in
companion CT scan to right.
(Right) Axial CECT shows a
large hepatic abscess
responsible for elevation of
right hemidiaphragm in
radiograph to left.
 Special Topics

Introduction and Overview [ill

Atelectasis W
Trauma rn
Lung Cancer rn
Portable ICU [i]
Physiology ~ 6
Special Patients ffiJ
 INTRODUCTION TO SPECIAL TOPICS
o
2

Frontill radiograph shows hyperinflared lungs but was IareraJ racfKJgraphshows a 2 an lung cancer (arrow)
otherwise normal. that was only apparent on the Iareral chest radiograph.
IareraJ radiographs often confirmalDly or the only
examination to show an abnormality.

o Single most Important reason why radiologists lose

ITERMINOlOGY
.. lawsuits over missed lung cancer
Chest Radiograph How should a chest radiograph be viewed? directed
Most common radiographic examination search (organ system or like a book) or a free global
(approximately 50% of all examinations) search?
Portable chest radiographs account for approximately o Directed search gives rise to more false positives
50% of all chest radiographs without Increase In true positives
o Free global search faster and more accurate
No matter the visual task, the eye randomly darts
IIMAGING ANATOMY across the Image In a random pattern
What Is the smallest solitary nodule visible on a chest
Perception radiograph?
Identification of abnormalIties not easy task o 3-4 mm sharply defined nodule
o Interobserver error 9-24% o Detection size Increases If margins of the nodule
o Intraobserver error 3-31% Indistinct
Errors due to faulty search, faulty recognition, faulty o Approximately 50:50 chance that a radiologist will
reasoning Identify a 9 mm nodule
o Search: 20% observer never focused on abnormality What factors affect detection?
o Recognition: 35% fixated on lesion but not brought o Image qualIty, lesion size, lesion consplcuity, lesion
to level of conscIousness of observer density and edge characteristics of lesion
o Reasoning: 45% lesion seen but dismissed as Is double reading worthwhile?
Insignificant o No guarantee of success, Mayo Clinic Lung Project
Satisfaction of search (lung cancer screening program) had many missed
o Premature termination of visual search due to most lung cancers despite two Independent readers
conspicuous abnormality 90% lung cancers visible In retrospect
o Common cause of error o More time consuming and costly
o Many patients have more than 1 abnormality o Computer-alded detection
Computers detect gray-level threshold, brightness,
lesion geometry
!ANATOMY-BASED IMAGING ISSUES Approved as second reader for chest radiography
and cr: Supplements radiologist Interpretation
Key Concepts or Questions How useful Is the lateral examination?
How long should a radiograph be vieWed? o In 25% the lateral examination confirms or clarifies
o Unknown although 70% of abnormalities the abnormalIty
recognized In 0.2 seconds o In 2% the lateral examination the only projection to
Nearly all nodules found within 4 seconds show the abnormality
o Long viewing times (minutes) may Increase false
positives without further increasing true positives
Imaging Pitfalls
What Is the most ImpQrtant method to reduce missed QualIty: Chest radiographs one of the most difficult
lung cancers? technical examinations because the chest contains
o Compare to previous radiographs tissues of such widely varying density (mediastinum
nearly all soft tissue and bone compared to the
air-filled lung)
 INTRODUCTION TO SPECIAL TOPICS

Differential Diagnosis
o
'ff . ,h h 0 Miliary tuberculosis 3
DI use Lung Disease Wit Poor C est 0 Respiratory bronchiolitis
Radiograph Sensitivity 0 Desquamative interstitial pneumonia

Disorders of aeration Disorders of vasculature

o Emphysema 0 Thromboembolism

o Asthma 0 Systemic lupus erythematosus

o Chronic bronchitis 0 Hepatopulmonary syndrome

o Bronchiolitis obliterans Disorders of diaphragm
Disorders of interstitium 0 Bilateral diaphragmatic paralysis

o Hypersensitivity pneumonitis

o Surveys have consistently shown that 40% of chest Does a normal chest radiograph rule out
radiographs are of poor quality pneumothorax?
o Yes,chest radiographs have high sensitivity for air
In the pleural space
I PATHOLOGY-BASED IMAGING American College of Radiology
ISSUES Recommendations
Key Concepts or Questions Indications for chest radiograph
What is the radiation dose of a chest radiograph? o Symptomatic patients with respiratory or cardiac
o 0.1 mSV;this dose compares to what an average symptoms
adult receives from natural background radiation In o Follow-up of known pulmonary disease
10 days o Evaluation of patients with known malignancy
o Pre-operatlve for thoracic surgery or pre-operative
for other operations where there is a history of
I CLINICAL IMPLICATIONS cardiopulmonary disease
o Daily Intensive care unit surveillance for monitoring
Function-Dysfunction tube and catheters
Radiograph not just an Image of pulmonary anatomy Contralndicatlons
and pathology o Routine screening of asymptomatic populations
o Radiograph also a point-In-time Image of dynamic o Screening for lung cancer
Information o Hospital admissions
o Employment physicals
Key Concepts or Questions
Does a normal chest radiograph rule out pneumonia?
o Yesfor practical purposes, normal chest radiograph I RELATED REFERENCES
obviates the need for antibiotics 1. SilvestriLet aI:Usefulnessof routine pre-operativechest
Does a normal mediastinum rule out aortic radiographyfor anaestheticmanagement:a prospective
transection? multicentrepilot study.EurJ Anaesthesia\.16(11):749-60,
o Yes,transection with normal mediastinal contours 1999
extremely rare

IIMAGE GALLERY
(I.eh) Frontal radiograph shows
normal lungs and a moderate
sized hiatal hernia (arrows).
Sensitivity of chest radiographs
depends on the clinical problem.
(RishtJ Axial HRCT shows diffuse
ground-glass opacities and focal
air-trapping (arrow) from
hypersensitivity pneumonitis.
en
Chest radiographs (and el'efl
have poor sensitivity for this
diagnosis.
Lobar Atelectasis IV-1-2
Subsegmental Atelectasis IV-1-6
Round Atelectasis IV-I-IO
 LOBAR ATELECTASIS
1
2

Graphic shows right upper lobe atelectasis resulting in Frontalradiograph shows bronchiectasis (arrow) in the
superior displacement of the minor fissure and right collapsed right lung, ipsilateral tracheal shift (open
hilum, bowing of the trachea to the right and arrow) and overexpanded left lung (curved arrow).
hyperaeration of the right,middle and lower lobes. Cicatrizing atelectasis.

Size
ITERMINOLOGY o Variable, less volume loss with more accumulation
Abbreviations and Synonyms of intra-alveolar fluid
Lobar volume loss, lobar collapse Least volume loss for right middle lobe
Lateral radiographs are presented as though sternum is Morphology
on left and spine on right o Atelectatic lobes pivot at the hilum by
Posteroanterior radiograph (PA), lateral radiograph bronchovascular structures
(lat) o Normal hilar relationships: Right hilum one
fingerbreadth lower than left (97%); both hila at
Definitions equal levels (3%)
Airspace volume loss of an entire lobe which may be o Normal diaphragm relationships: Right diaphragm
due to bronchial obstruction, cicatricial scarring, loss 1.S em higher than left; reversed in dextrocardia
of surfactant (adhesive), or compression from a space
Radiographic Findings
occupying mass
"Silhouette" sign - loss of air - soft tissue interface Lobar collapse
when collapsed lung abuts an adjacent soft tissue o Signs of atelectasis proportional to amount of
structure volume loss
o Airlessness of affected lobe, local increase in opacity
o Displacement of fissures, trachea, heart,
mediastinum and hilum towards the collapse
IIMAGING FINDINGS o Elevated ipsilateral diaphragm
General Features o Crowding of vessels and bronchi in affected lobe
Best diagnostic clue: Shift of fissures, mediastinum and o Overinflation of remaining lobes
hila toward collapse

DDx: Lobar Atelectasis

Pleural Effusion Aspiration Pneumonia Mediastinal Mass

 LOBAR ATELECTASIS
Key Facts
Terminology
"Silhouette" sign - loss of air - soft tissue interface
when collapsed lung abuts an adjacent soft tissue
structure
Imaging Findings
Best diagnostic clue: Shift of fissures, mediastinum
and hila toward collapse
Signs of atelectasis proportional to amount of volume
loss
Airlessness of affected lobe, local increase in opacity
Golden-S sign: Seen with obstructive atelectasis due
to a central hilar mass
]uxtaphrenic peak: Seen with upper lobe collapse
RML and RLL atelectasis, resembles pleural effusion
Central and peripheral air bronchograms in an
atelectatic lobe suggest cicatrizing atelectasis
o Golden-S sign: Seen with obstructive atelectasis due
to a central hilar mass
Fissure is S-shaped or reversed S-shaped curve
Convex centrally outlining the hilar mass and
concave peripherally
o ]uxtaphrenic peak: Seen with upper lobe collapse
Tenting of diaphragm (due to upward retraction
inferior accessory fissure)
Right upper lobe (RUL) atelectasis (average volume
RUL = 1140 ml)
o Lobe collapses superiorly and medially;
overexpansion of right middle and lower lobes
PA: Periphery at apical medial mediastinum; lat:
Periphery at apical anterior chest wall
Superior displacement of right hilum and right
main stem bronchus
o Silhouette: PA: Loss of SVC interface
o Fissure: PA: Minor fissure displaced superiorly
Left upper lobe (LUL) atelectasis (average volume LUL
= 1160 ml)
o Lobe collapses anteriorly; overexpansion of left
lower lobe
PA: Vague opacity upper lung and over left hilum;
lat: Periphery to full length of anterior chest wall
Superior displacement left hilum, horizontal left
mainstem bronchus
o Silhouette: Loss of left superior mediastinum (may
spare aortic arch) and left heart border interfaces
a Fissure: Lat: Major fissure shifted anteriorly
o Luftsichel sign: PA: Superior segment of lower lobe
causes a crescent lucency between aortic arch and
atelectatic upper lobe
a Lat: Retrosternallucency represents RUL
overexpansion leftward across ascending aorta
Right middle lobe (RML) atelectasis (average volume
RML= 670 ml)
o Lobe collapses medially toward the right heart
border; no overexpansion of other lobes or hilar
shift
PA: Vague opacity over right medial lung base; lat:
Periphery at anterior inferior chest wall
o Silhouette: PA: Obscuration of right heart border
1
Best imaging tool: Chest radiography usually 3
sufficient
Top Differential Diagnoses
Post Lobectomy
Pneumonia
Pleural Effusion or Tumor
Clinical Issues
Most common signs/symptoms: Asymptomatic
Atelectasis not a disease, treatment aimed at
underlying cause
Diagnostic Checklist
Complete atelectasis often missed, relationship of
hilar positions often key in recognizing atelectasis
o Fissure: Lat: Minor fissure and inferior major fissures
approximate
Lower lobe atelectasis (average volume LLL = 1550 ml,
RLL 2000 ml)
o Collapses posteriorly, medially and inferiorly,
inferior displacement of hilar vessels and mainstem
bronchi; overexpansion of remaining lobes
PA: Periphery at medial diaphragm; lat: Vague
opacity over lower thoracic spine
o Silhouette: PA: Right, medial diaphragm, right
paraspinal stripe, azygoesophageal recess
PA: Left, medial diaphragm, distal descending
aorta
o Fissure: PA: Major fissure courses from hilum
obliquely to diaphragm
o Upper triangle sign: Seen with RLL collapse,
rightward rotation of superior mediastinum
RML and RLL atelectasis, resembles pleural effusion
o Both lobes collapse as expected; overexpansion of
right upper lobe
PA: Periphery along entire diaphragm; lat:
Periphery along entire diaphragm
o Silhouette: PA: Obscuration of right diaphragm,
right heart border
o Fissures: PA: Major and minor fissures extend
obliquely from hilum to inferior lateral chest wall
RUL and RML atelectasis
o Identical to left upper lobe atelectasis except on
right side; overexpansion of RLL
Total lung atelectasis: Shift of heart/mediastinum to
opacity, hyperinflation of contralateral lung
CT Findings
NECT
o Can identify the bronchus feeding atelectatic lobe
o RUL and LUL atelectasis: Posterior margin, major
fissure; shift of trachea to ipsilateral side
a RML atelectasis: Posterior margin, major fissure;
anterior margin, minor fissure
o Lower lobe atelectasis: Anterior margin, major
fissure
CECT can help identify cause (Le., bronchial
obstructing lesion)
 LOBAR ATELECTASIS
1 o Differential enhancement of a central bronchial
mass
4 o Mucoid impactions: Mucus-filled dilated bronchi
due to secretions beyond obstructing lesion
Central and peripheral air bronchograms in an
atelectatic lobe suggest cicatrizing atelectasis
No imaging can predict if the atelectatic lobe is sterile
or infected
Imaging Recommendations
Best imaging tool: Chest radiography usually sufficient
Protocol advice
o CT useful when the chest radiographic findings are
difficult to interpret and to establish cause
Contrast-enhancement may show an enhancing
central lesion
I DIFFERENTIAL DIAGNOSIS
Post Lobectomy
History of lung surgery, clips and suture material at
hilum; CECT shows clipped lobar draining vein
Pneumonia
Clinical presentation of fever, cough, abundant
purulent sputum, elevated white blood cell count
Pleural Effusion or Tumor
Small amount of pleural fluid tends to occur adjacent
to lobar atelectasis
Decubitus radiograph may show shifting fluid but if
loculated CT may differentiate
Large pleural effusion (opaque hemithorax):
Contralateral mediastinal shift
CECT to show solid enhancing pleural tumor
Mediastinal Widening, Lipomatosis or Mass
No shift of fissures, no hilar displacement
I PATHOLOGY
General Features
General path comments
o Lobar obstruction, collapse in 18-24 hours if
breathing room air
Nitrogen very slowly absorbed, delays
development of atelectasis
o Lobar obstruction, collapse in < 5 minutes if
breathing 100% oxygen
o Persistent aeration of obstructed lobe due to
ventilation across pores of Kohn canals of Lambert
and incomplete fissures
Etiology
o Types of atelectasis
Obstructive: Central bronchial obstruction
resulting in lobar atelectasis, as with bronchial
neoplasm, mucus plug, foreign body, broncholith
Compressive: Compression of adjacent lung, as
with large pleuropulmonary mass or bulla
Passive: Retraction of lung away from the chest
wall (elasticity) as with pneumothorax or effusion
Cicatrizing: Pulmonary fibrosis with volume loss
(decreased lung compliance), as with sequela of
tuberculosis
Adhesive: Loss of alveolar lining surfactant
(decreased lung compliance), as with adult
respiratory distress syndrome
Epidemiology
o Variable, LLL collapse common in ventilated
patients, post-op and ICU setting
o RML syndrome: Cicatrizing atelectasis of RML due to
prior pneumonia and poor collateral drift
Small bronchus diameter, susceptible to
compression from adjacent lymphadenopathy
Gross Pathologic & Surgical Features
RML and RLL collapse: Pathology involving bronchus
intermedius
RUL and RML collapse: Usually due to two distinct
endobronchial lesions
o Bronchogenic carcinoma most common cause
o Borrie sump: Nodes between RUL and RML
bronchus compress both lobar bronchi
ICLINICAllSSUES
Presentation
Most common signs/symptoms: Asymptomatic
Other signs/symptoms: Dyspnea, chest pain, cough
Demographics
Age: Neonate to elderly
Gender: M = F
Natural History & Prognosis
Determined by underlying cause
Treatment
Atelectasis not a disease, treatment aimed at
underlying cause
I DIAGNOSTIC CHECKLIST
Consider
CECT for patients who have unexplained lobar
atelectasis to evaluate for an obstructing lesion
Image Interpretation Pearls
Complete atelectasis often missed, relationship of hilar
positions often key in recognizing atelectasis
ISElECTED REFERENCES
1. Gupta P. The Golden 5 sign. Radiology. 233(3):790-1, 2004
2. Ashizawa K et al: Lobar atelectasis: diagnostic pitfalls on
chest radiography. Br J Radiol. 74(877):89-97, 2001
3. Blankenbaker DG et al: The luftsichel sign. Radio]ogy.
208(2):319-20, 1998
4. Gurney JW et al: Atypical manifestations of pulmonary
atelectasis. J Thorac Imaging. 11(3):165-75, 1996
5. Proto AV et al: Radiographic manifestations of lobar
collapse. Semin Roentgenol. 15:117-73, ]980
 LOBAR ATELECTASIS

I IMAGE GALLERY 1
5
Typical
(Left) Frontal radiograph
shows retrocardiac opacity
silhouetting descending aorta
and medial diaphragm
(arrows). Lateral margin is
major fissure (curved arrow).
Left lower lobe atelectasis.
(Right) Lateral radiograph
shows opacity over lower
thoracic spine (arrows)
representing left lower lobe
atelectasis. No silhouetting
dome of left diaphragm
because collapse is medial to
the dome.

(Left) Frontal radiograph

shows triangular opacity
silhouetting the right heart
border (arrows). Diaphragm
interface is preserved and
there is no shift of hilum or
mediastinum. (Right) Lateral
radiograph in same patient
shows approximation of
minor (arrow) and inferior
major (open arrow) fissures.
Chronicity suggests RML
syndrome.

(Left) Frontal radiograph

shows opacity extends to
lateral chest wall (arrow),
RML and RLL atelectasis
caused by mucus plugging of
bronchus intermedius.
Appearance resembles
pleural effusion. (Right)
Frontal radiograph shows
luftsichelsign (arrows),
overexpanded superior
segment of LLL between
collapsed LUL and aorta.
Note Golden-S sign (curved
arrow) indicating central
hilar mass.
 SUBSEGMENTAL ATELECTASIS
1
6

Frontal radiograph in a patienl with asciles shows small Frontal radiograph same paUent CWoweeks later shows
volume lungs, elevated diaphragms and bilaleral small volume lungs, elevated diaphragms and residual
subsegmental alelectasis (arrows). subsegmental alelectasis (arrows).

o Usually abuts pleura and is perpendicular to the

ITERMINOlOGY pleural surface
Abbreviations and Synonyms o Usually posterior lower lobes
Plate atelectasis, linear atelectasis, discoid atelectasis, o 45% obliquely oriented
Fleischner lines o May cross entire lobe
o Single or multiple; unilateral or bilateral
Definitions o Presence of plate atelectasis usually infers more
Atelectasis involving less than a segment extensive underlying atelectasis
o Subsegmental atelectasis adjacent to large bullae;
pseudomass
I IMAGING FINDINGS Central, sharply marginated, mass-like opacities
that are oblong, lenticular, or triangular
General Features Upper lobes and right middle lobe
Best diagnostic clue o Associated findings
o Disc or plate shaped atelectasis Surgery: Thoracic, abdominal, head and neck,
o Usually horizontally oriented spinal surgery
o Crossing segmental boundaries Lines and tubes: Endotracheal tube, tracheostomy,
Location: Mid and lower lungs, most common chest tubes
Size: 1-3 mm thick, 4-10 cm long Thoracic: Pneumothorax, pleural effusion,
Morphology: Linear or band-like opacity pulmonary mass, large bulla, rib fractures,
pulmonary edema
Radiographic Findings
Abdominal: Organomegaly, ascites,
Radiography pneumoperitoneum, abscess

DDx: linear Opacity

Interlobular Septa
 SUBSEGMENTAL ATELECTASIS
Key Facts
1
Imaging Findings Pulmonary thromboembolism, venous air embolism 7
Disc or plate shaped atelectasis Epidemiology: Common in hospitalized intensive
Usually horizontally oriented care unit patients
Crossing segmental boundaries Clinical Issues
Size: 1-3 mm thick, 4-10 em long Atelectasis not a disease, treatment aimed at
Usually posterior lower lobes underlying cause
Nordenstrom sign: Lingular subsegmental atelectasis
in patients with left lower lobe atelectasis Diagnostic Checklist
CECT: May show endobronchial abnormalities CTA to evaluate for pulmonary embolism
In patients with radiographic findings of
Top Differential Diagnoses subsegmental atelectasis and acute onset of dyspnea
Fibrotic Bands and chest pain .
Pathology Assess for associated abnormalities of lung, pleura,
General anesthesia, mechanical ventilation; surgery: diaphragm and abdomen
Abdominal, thoracic, head and neck
Children: Asthma, viral pneumonia

Elevated hemidiaphragms: Phrenic nerve o Asbestosis: Curvilinear subpleural bands, short and
paralysis, paresis, eventration, Bochdalek or long linear bands
traumatic rupture o Healed pneumonia
o Pulmonary laceration: History of penetrating trauma
CT Findings
CTA: Can help identify cause (I.e., pulmonary Comet Tail Sign of Round Atelectasis
embolism) Linear opacities that arc or whorl toward the round
NECT atelectasis
o Linear opacities, horizontal or oblique crossing lobar Adjacent focal (plaques) or diffuse pleural disease
segments History of asbestos exposure, common
o Interlobular septal thickening bordering linear
atelectasis
Septal Thickening (Kerley B and A Lines)
o Nordenstrom sign: Lingular subsegmental atelectasis Pulmonary edema
in patients with left lower lobe atelectasis o Subpleural edema with fissural thickening
o May show associated pulmonary, pleural, o Transient linear opacities, as edema clears, the
diaphragmatic, chest wall or subphrenic disease opacities disappear
CECT: May show endobronchial abnormalities Lymphangitic carcinomatosis
o HRCT: Beading of septal lines, centrilobular nodules,
HRCT
o Plate-like atelectasis in the corticomedullary circumferential thickening and irregular nodularity
junction of the lung of bronchovascular bundles
Curvilinear subpleural irregular linear opacity o Associated pleural effusions, hilar and mediastinal
Confluence of honeycomb subpleural cysts lymphadenopathy, common
o Prone HRCT: Subsegmental atelectasis may o Progressive disease
disappear Intrapulmonary Fissures and Ligaments
Fluoroscopic Findings Superior accessory fissure (5%)
Chest Fluoroscopy: Fluoroscopy, sniff test: To evaluate o Oblique linear opacity, more common at right lower
diaphragmatic motility lobe
o Separates the superior segment from the remaining
Imaging Recommendations basal segments of lower lobe
Best imaging tool: Found incidentally with o Frontal radiograph: Similar appearance to minor
radiography and CT fissure
Protocol advice: Incidentally found on standard CT o Lateral radiograph: Horizontal fissure located
performed for other indication; no additional imaging posterior to major fissure
required Azygos fissure (1 %)
o Accessory fissure between azygos and right upper
lobe
I DIFFERENTIAL DIAGNOSIS o Azygos vein always seen at inferior aspect of fissure
Inferior accessory fissure (9%)
Fibrotic Bands o Accessory fissure between the medial basal segment
Prior radiographs or CT scans help differentiate; linear of right lower lobe and other basal segments
scarring is chronic and unchanging o Juxtaphrenic peak: Tenting of diaphragm
Seen with upper or middle lobe collapse
 SUBSEGMENTAL ATELECTASIS
1 Traction of inferior accessory fissure, medial
septum or another accessory fissure
Microscopic Features
Right side, more common Not generally seen as specimens often inflated prior to
8
Left minor fissure (6%) fixation
a Accessory fissure between left upper lobe and lingula
a Obliquely oriented in contrast to horizontal minor
fissure ICLINICAL ISSUES
Vertical fissure of Davis Presentation
a Parallels chest wall in the lateral costophrenic angle;
seldom> 1 mm width Most common signs/symptoms
a Usually of little clinical importance
a Represents the major fissure in profile with either
a Signifies more widespread peripheral atelectasis than
partial atelectasis of a lower lobe or middle lobe
radiologically apparent
a More common on right
Once considered a cause for post-operative fever,
Pleural fluid tracking along fissure
now debatable
a Decubitus radiograph may show shifting pleural
Other signs/symptoms: May indicate serious other
effusion
abnormality in the chest or abdomen
a CT to show the fluid in the fissure
Demographics
Age: Variable, neonate to elderly
I PATHOLOGY Gender: No preference
General Features Natural History & Prognosis
General path comments Determined by underlying cause
a Develops at sites of pre-existing pleural invagination
or at incomplete fissure Treatment
Surface of the lung invaginated with subpleural Atelectasis not a disease, treatment aimed at
atelectasis underlying cause
Etiology
a Hypoventilation or decreased diaphragmatic
excursion I DIAGNOSTIC CHECKLIST
General anesthesia, mechanical ventilation;
surgery: Abdominal, thoracic, head and neck Consider
Trauma, splinting from pleurisy CTA to evaluate for pulmonary embolism
Acute cerebral infarction a In patients with radiographic findings of
Eventration, phrenic nerve paralysis, systemic subsegmental atelectasis and acute onset of dyspnea
lupus erythematosus, quadriplegia, myasthenia and chest pain
gravis Abdominal ultrasound or CT to evaluate for
Hepatomegaly, splenomegaly, ascites, subphrenic subphrenic process
abscess Fluoroscopy, sniff test to assess for diaphragmatic
a Small airways disease motion
Accumulation of bronchial secretions, obstruction Image Interpretation Pearls
of small airways
Assess for associated abnormalities of lung, pleura,
Collateral air drift impaired by poor bronchial
diaphragm and abdomen
clearance and obstructed canals of Lambert and
pores of Kohn
Children: Asthma, viral pneumonia
Influenza, mycoplasma pneumonia
I SELECTED REFERENCES
Aspiration foreign bodies 1. Davis SD et al: Obliquely oriented superior accessory fissure
Pulmonary edema of the lower lobe of the lung: CT evaluation of the normal
a Decreased surfactant production (adhesive appearance and effect on the distribution of parenchymal
and pleural opacities. Radiology. 216(1):97-106, 2000
atelectasis) 2. Glerada DSet al: Pseudomass due to atelectasis in patients
Pulmonary thromboembolism, venous air with severe bullous emphysema. AJRAm J Roentgenol.
embolism 168(1):85-92,1997
a Pulmonary compression 3. PriceJ: Linear atelectasis in the lingula as a diagnostic
Large pulmonary mass or bulla feature of left lower lobe collapse: Nordenstrom's sign.
Large pleural effusion or mass Australas Radial. 35(1):56-60, 1991
Diaphragmatic abnormalities 4. Westcott JL et al: Plate atelectasis. Radiology. 155(1):1-9,
1985
Epidemiology: Common in hospitalized intensive care
5. Kubota H et al: Plate-like atelectasis at the corticomedullary
unit patients junction of the lung: CT observation and hypothesis.
Gross Pathologic & Surgical Features Radiat Med. 1:305-310, 1983
Surface of the lung invaginated due to invaginated
pleura and subpleural atelectasis
 SUBSEGMENTAL ATELECTASIS
IIMAGE GALLERY 1
9

(Left) Frontal radiograph

shows a horizontal linear
opacity in the right lower
lobe, indicating
subsegmental atelectasis
(arrow). She aspirated
particulate maleria/two days
ago during general
anesthesia. (Right) Lateral
radiograph in same patient
shows the subsegmental
atelectasis in the right lower
lobe (arrow).

(Left) Axial CECT shows right

middle lobe subsegmental
atelectasis (arrows). (Right)
Axial eTA in same patient
shows right middle lobe
pulmonary embolism
(arrow).

Typical
(Left) Axial CECT shows
elevated right
hemidiaphragm (open
arrow). Note adjacent
subsegmental atelectasis
(curved arrow). (Right) Axial
CECT shows bilateral lower
lobe curvilinear opacities
that represent dependent
subsegmentaf alelectasis
(arrows).
 ROUND ATELECTASIS
1
10

Coronal graphic shows round atelectasis in the left Axial CECT shows curvilinear bronchovascular markings
lower lobe. Atelectasis has developed adjacent to focal entering the mass (curved arrows). Round atelectasis
pleural thickening (curved arrow). A comet tail is associated with calcified asbestos-related plaque
present medially (arrow). (arrow).

Size: Variable, 2-7 cm in diameter, but may reach up to

ITERMINOlOGY lOcm
Abbreviations and Synonyms Morphology: Crowding together of bronchi and blood
Folded lung, Blesovsky syndrome, atelectatic vessels that extend from the lower border of the mass
pseudotumor, asbestos pseudotumor, pleuroma, toward the hilum
shrinking pleuritis with atelectasis Radiographic Findings
Definitions Radiography
A form of peripheral atelectasis that develops in o Subpleural homogeneous mass
patients with pleural disease Oval, wedge-shaped, round or irregular mass with
smooth margins
Distortion of vessels and bronchi in involved lobe
I IMAGING FINDINGS with crowding of markings
Usually separated from the diaphragm by
General Features interposed lung
Best diagnostic clue: Asbestos-related pleural plaques o Single or multiple, unilateral or bilateral
with associated mass-like opacity o Volume loss often, but not invariably present
Location o Costophrenic angles usually blunted or obliterated
o ]uxtapleural, may occur in any lobe o Pleural thickening is always present and is
Posterior, posteromedial lower lobes, most frequently greatest near the mass
common site Asbestos-related calcified pleural plaques or diffuse
Upper lobes, lingula and middle lobe may be pleural thickening
involved Focal or diffuse pleural thickening from any cause
Pleural effusion (rare)

DDx: Pleural Based Mass

Pleural Fibrous Tumor Bronchogenic Carcinoma PE/lnfarction

 ROUND ATELECTASIS

Key Facts
1
Terminology Top Differential Diagnoses 11
A form of peripheral atelectasis that develops in Lung cancer, metastatic neoplasm to lung
patients with pleural disease Pleural tumor
Pulmonary Embolus with Infarction
Imaging Findings
Posterior, posteromedial lower lobes, most common Clinical Issues
site Radiographic or CT observation to show stability and
Morphology: Crowding together of bronchi and confirm diagnosis
blood vessels that extend from the lower border of
the mass toward the hilum Diagnostic Checklist
Oval, wedge-shaped, round or irregular mass with PET if carcinoma is suspected; rounded atelectasis is
smooth margins most commonly metabolically inactive
Acute angles with pleura Convergence of bronchovascular markings is the best
Air bronchograms, 60% discriminator of round atelectasis from other pleural
Comet tail sign; best seen with CT based masses

o Structures within and surrounding the round

CT Findings
NECT
o Single or more than one lesion in a lung or lobe;
unilateral or bilateral
o Wedge-shaped, lentiform, or (less often) irregular
opacity; small lesion may be appreciated only with
CT
Acute angles with pleura
Perpendicular to pleural surface
Sharp lateral margins with a poorly defined
central margin
Irregular margins toward incoming
bronchovascular markings
Air bronchograms, 60%
Calcifications, uncommon
Involvement of entire lobe, rare
o Comet tail sign; best seen with CT
Also referred to as vacuum cleaner effect, helical
effect, IU sign, cords of a parachute, crows feet,
talon sign
Distortion, displacement and convergence of
vessels into the atelectatic mass
Curvilinear relationship of bronchovascular
structures with mass
Bronchovascular structures diverge and arc before
merging with pulmonary surface of the mass
Comet tail to round atelectasis on the diaphragm,
not well seen with axial images
o Volume loss of affected lobe, often with
hyperlucency of adjacent lung
o Pleural disease: CT may show more extensive pleural
disease than radiography
CECT
o Homogeneous enhancement of atelectatic lung,
usually not helpful
Can also be seen with bronchogenic carcinoma
MR Findings
T1WI
o Round atelectasis: Signal intensity higher than
muscle and lower than fat
atelectasis can be clearly depicted
o Pulmonary vessels and bronchi converging toward
the atelectasis (comet tail sign) on sagittal or oblique
sagittal planes
o Infolded visceral pleura: Low signal intensity line
T2WI
o Round atelectasis: Similar to or lower than fat on T2
weighted images
o Shows thickened pleura or effusion
Tl C+: Homogeneous enhancement
Nuclear Medicine Findings
PET
o Not metabolically active on FOG-PET imaging
Rarely may be positive
o Can be helpful to differentiate from bronchogenic
carcinoma
Ultrasonographic Findings
Highly echogenic line extending into the mass from
the pleural surface
o May represent scarred invaginated pleura
Imaging Recommendations
Best imaging tool: CT findings can be definitive when
most characteristic features are present
Protocol advice: Standard CT without intravenous
contrast
I DIFFERENTIAL DIAGNOSIS
Neoplasm
Lung cancer, metastatic neoplasm to lung
o May have history of asbestos exposure
o Not always juxta pleural or associated with pleural
disease
o Usually no comet sign with radiography and CT
o May be PET positive
Pleural tumor
o Mesothelioma, associated with asbestos exposure
 ROUND ATELECTASIS
1 o Pleural metastasis from lung, head and neck,
gastrointestinal neoplasms
12 o CT shows pleural mass(es) with adjacent lung
compression, obtuse angles, not perpendicular to
visceral pleura
o No comet tail sign
o May be PET positive
Pulmonary Embolus with Infarction
Acute symptoms of dyspnea, hypoxia, chest pain
Peri pheral wedge-shaped opacity, volume loss of
hemithorax due to splinting
I PATHOLOGY
General Features
General path comments: Retracting pulmonary scar
causing invagination of parietal pleura and infolding
of lung
Etiology
o Pleural effusion causing passive atelectasis of lung
Infolding of visceral pleura elevating and tilting
lung
Development of fibrinous parietal pleural
adhesions maintain the infolding and tilting of
lung
As pleural fluid resolves, the lung reexpands
except for the sequestered round atelectasis
Pleuropulmonary fibrosis with organization and
contraction causes additional distortion
o Asbestos related: Contraction of diffuse visceral
pleural thickening
Retraction of parenchymal fibrosis
o Pleural/parenchymal disease caused by
History of asbestos inhalation, 70% of cases
Congestive heart failure, post-operative coronary
artery bypass graft surgery, Dressler syndrome
Tuberculosis and other infections
Therapeutic pneumothorax for tuberculosis
Pleurisy: Uremic, nonspecific
Pulmonary infarction
Epidemiology: 65% have had asbestos exposure
Associated abnormalities: Co-existence with lung
carcinoma is rare, but has been reported
Gross Pathologic & Surgical Features
White, irregular, firm, pleural plaque; infolding of
visceral pleura, perpendicular into parenchyma; firm,
fibrotic, atelectatic lung
Microscopic Features
Band-like zone of fibrosis containing bronchioles or
zone of collapsed alveoli with fibrotic thickening,
nonspecific
IClINICAllSSUES
Presentation
Most common signs/symptoms
o Almost always asymptomatic, chest wall pain may
be related to pleurisy or effusions
o Usually discovered incidentally on a radiograph or
CT scan performed for other reasons
Other signs/symptoms: Dyspnea may be related to
pleural disease or asbestos related pulmonary fibrosis
Demographics
Age: Usually> 50 years old, (range, 20-92 years old)
Gender: M:F = 4:1
Natural History & Prognosis
May develop and progress over months or years
May be stable, grow very slowly, shrink or disappear
May arise years after resolution of pleural effusion
Radiographic or CT observation to show stability and
confirm diagnosis
Treatment
Atelectasis not a disease, treatment aimed at
underlying cause
Fine needle aspiration biopsy
o Usually not necessary, reserved for atypical cases
o When performed should not result in
pneumothorax
Lung and pleura are adhesed and biopsy is
through non-aerated lung
Excisional biopsy rarely performed
I DIAGNOSTIC CHECKLIST
Consider
PET if carcinoma is suspected; rounded atelectasis is
most commonly metabolically inactive
Image Interpretation Pearls
Convergence of bronchovascular markings is the best
discriminator of round atelectasis from other pleural
based masses
I SElECTED REFERENCES
1. Hakomaki Jet al: Contrast enhancement of round
atelectases. Acta Radiol. 43(4):376-9, 2002
2. Roach HD et al: Asbestos: when the dust settles an imaging
review of asbestos-related disease. Radiographics. 22 Spec
No:SI67-84, 2002
3. McAdams HP et al: Evaluation of patients with round
atelectasis using 2-[18F]-nuoro-2-deoxy-D-glucose PET.J
Comput AssistTomogr, 1998
4. O'Donovan PBet al: Evaluation of the reliability of
computed tomographic criteria used in the diagnosis of
rounded atelectasis. J Thorac Imaging. 12:S4-S8, 1997
5. Yamaguchi T et al. Magnetic resonance imaging of
rounded atelectasis. J Thorac Imaging. 12(3):188-94, 1997
6. Woodring JH et al: Types and mechanisms of pulmonary
atelectasis. J Thorac Imaging. 11(2):92-108,1996
Doyle TC et al: CT features of rounded atelectasis of the
7.
lung. AJR.143:225-228, 1984
8. Schneider HJ et al: Rounded atelectasis. AJR. 134:22S-232,
1980
 ROUND ATELECTASIS
I IMAGE GALLERY 1
13
Typical
(Left) Axial NECT shows a
small wedge-shaped pleural
based opacity (arrow). The
axis is perpendicular to the
pleura. Comet tail enters the
mass (curved arrow). (Right)
Axial NECT shows round
atelectasis Foci in lefl/ower
lobe (arrows). Curved
arrows show diaphragmatic
calcified plaques.

(Left) Frontal radiograph

shows right inFra-hilarmass
(arrow) that represents
round ateleclasis. (Right)
Axial NECT in same patient
shows whorled
bronchovascu/ar structures
with air bronchograms
(curved arrow) entering the
round atelectasis. Adjacent
pleural plaques (arrow).

Variant
(Left) Axial NECT in same
patient shows whorting
calcification in the round
atelectasis (arrow).
Peripheral air bronchograms
are seen (curved arrow).
(Right) Axial NECT in same
patient shows adjacent cyst
formation or honeycombing
(arrow).
Pneumomediastinum IV-2-2
Pneumothorax IV-2-6
Tracheobronchial Tear IV-2-10
Lung Trauma IV-2-12
Rib Fracture and Flail Chest IV-2-16
Aortic Transection IV-2-20
Spinal Fracture IV-2-24
Sternal Fracture IV-2-28
Diaphragmatic Tear IV-2-30
Esophageal Tear IV-2-34
Splenosis IV-2-38
Air Embolus, Pulmonary IV-2-42
Thoracic Duct Tear IV-2-46
 PNEUMOMEDIASTINUM

2
2

Frontal radiograph shows extensive CECT shows extensive pneumomediastinum and soft
pneumomediastinum decompressing into soft tissuesor tissue emphysema. Note air dissecting throughout all
neck and chest wall. Air was secondary to asthma tissue planes, including vascular bundles, trachea,
exacerbation. esophagus and {ascial planes.

o Lateral radiograph can be most helpful in diagnosis:

ITERMINOlOGY Look for streaky lucencies overlying anterior clear
Abbreviations and Synonyms space
Pulmonary interstitial emphysema (PIE) Ring around the artery sign: Circumferential air
Barotrauma lucency around the pulmonary arteries
Location
Definitions o Anterior mediastinum, thoracic inlet, hila
Extra-alveolar air dissecting within pulmonary o Usually more conspicuous on lateral view
interstitium and mediastinum o On CT, look for air within the interlobular septa:
Essentially, black Kerley B lines
Air can be followed, tracking medially around the
IIMAGING FINDINGS pulmonary arteries into the mediastinum
Size: Subtle to striking
General Features Morphology: Streaky extra-alveolar air lucencies
Best diagnostic clue
o Air outlining the left heart border and mediastinal Radiographic Findings
vessels on frontal radiograph Radiography
Be careful not to confuse with o Differentiate from other air collections
pneumopericardium Pneumomediastinum does not shift with
Streaky lucencies can be followed up mediastinum decubitus position
out thoracic inlet into neck and chest wall soft o Lateral radiograph more sensitive than frontal
tissues radiograph
Signs
o Thymic sail

DDx: Thoracic Air Collections

Medial Pneumothorax Pneumopericardium Pneumoperitoneum

 PNEUMOMEDIASTINUM

Key Facts
Terminology Top Differential Diagnoses
Extra-alveolar air dissecting within pulmonary Pneumopericardium
interstitium and mediastinum
Pathology
Imaging Findings Sustained Valsalva leading to alveolar rupture:
Air outlining the left heart border and mediastinal Asthma, cough, weight lifting, straining, marijuana 2
vessels on frontal radiograph use
Lateral radiograph can be most helpful in diagnosis: 3
Look for streaky lucencies overlying anterior clear Clinical Issues
space Soft tissue "swelling" of the neck, chest, and face
Usually more conspicuous on lateral view leading to Hamman sign (precordial crunching at
On CT, look for air within the interlobular septa: auscultation)
Essentially, black Kerley B lines May lead to pneumothorax, pneumoperitoneum,
Streaky air collections within mediastinal fat and in retroperitoneal air
connective tissue sheaths around tubular structures Morbidity and mortality related to etiology
such as trachea and pulmonary arteries None, observe for pneumothorax

o Ring around the artery

Relatively common finding
I DIFFERENTIAL DIAGNOSIS
o Tubular artery sign Pneumothorax
o Double bronchial wall Air will shift with position, visceral pleural line
o Continuous diaphragm: Look for air between the smooth, extra pleural line not smooth and usually
pericardium and diaphragm thicker
Can be similar to pneumoperitoneum Usually unilateral
o V sign of Naclerio
Costovertebral air adjacent to hemidiaphragm and Pneumopericardium
spine Less common in adults
Air confined to pericardium key feature
CT Findings
o On chest radiographs, should see air around the
CECT bottom of heart for confident diagnosis
o More sensitive than chest radiograph o Best appreciated with CT
o Streaky air collections within mediastinal fat and in
connective tissue sheaths around tubular structures Mach Band
such as trachea and pulmonary arteries Due to retinal inhibition, obscuring one edge with
o Soft tissue air can extend through almost any and all finger or hand will make mach band disappear
fascial planes, from head to toe Convex soft tissue density into concave lung density =
Subcutaneous tissues black Mach band, example heart border
Mediastinum Convex lung density into concave soft tissue density =
Muscle bundles white Mach band, example paraesophageal stripe
Retroperitoneum
Fat planes Skin Fold
o Look for air within the interlobular septa Gradation of gray that fades medially, with a sharp
Best seen in the lung periphery mach line laterally
Air can often be tracked back to the mediastinum Follows non-anatomic distribution
via the pulmonary arteries
Air Distended Esophagus
Imaging Recommendations Achalasia, can mimic extraluminal mediastinal air
Best imaging tool Pneumonia in a Lateral Regions of Lower
o Chest radiograph
CT helpful to confirm in difficult cases Lobes when Delimited by Inferior Accessory
CT also helpful when etiology can be perforated Fissure
viscus Dense lung opacity lateral to sharp interface with
Protocol advice: Conventional protocols except aerated medial basilar segment may mimic
consider using oral contrast when hollow viscus pneumopericardium or pneumomediastinum
perforation suspected clinically

I PATHOLOGY
General Features
General path comments
 PNEUMOMEDIASTINUM
o When alveoli rupture, air tracks through the
pulmonary interstitium, decompresses into the
mediastinum, and then out the thoracic inlet into
the soft tissues of the chest wall and neck
This implies an intact visceral and parietal pleura
In infants, pneumomediastinum may not
decompress into neck leading to tension
2 pneumomediastinum
o PIE can lead to pneumothorax
4 o Pneumothorax does not lead to PIE or
pneumomediastinum
Etiology
o Macklin effect
Alveolar rupture leads to
Pulmonary interstitial emphysema: PIE in infants;
rarely seen in adults leads to pneumomediastinum
o Common causes
Spontaneous
Sustained Valsalva leading to alveolar rupture:
Asthma, cough, weight lifting, straining,
marijuana use
Blunt chest trauma leading to pulmonary
laceration and alveolar rupture
Dental extraction
Mechanical ventilation: "Volutrauma" as opposed
to barotrauma
o Less common causes
Bronchial or tracheal fracture: Consider if
progressively worsens
Esophageal tear, perforation, or puncture
Sinus fracture
Duodenal ulcer or sigmoid diverticulitis
Epidemiology: Depends upon underlying etiology
I CLINICAL ISSUES
Presentation
Most common signs/symptoms
o Soft tissue "swelling" of the neck, chest, and face
leading to Hamman sign (precordial crunching at
auscultation)
o May be fatal in neonates: Tension
pneumomediastinum
Other signs/symptoms
o Occasionally there is associated pneumothorax,
especially in the setting of blunt chest trauma
o May lead to pneumothorax, pneumoperitoneum,
retroperitoneal air
o Chest pain
o Dyspnea
o In past, was common in mechanically ventilated
patients, especially those with acute respiratory
distress syndrome with poor lung compliance, as a
result of barotrauma
Less common now with modern ventilator
strategies and management
o For isolated pneumomediastinum, consider
underlying causes such as
Asthma, look for hyperinflated lungs in younger
non-smoking patients
Chronic obstructive pulmonary disease
Acute viral infections causing small-airways
air-trapping
Crack cocaine smoking
Minor blunt chest trauma
o For pneumomediastinum evident with other
morbidities, consider underlying causes such as
Boerhaave syndrome: Typical clinical presentation
Ventilator-associated barotrauma
Penetrating trauma such as knife and gun shot
wounds
Foreign bodies, such as fishbones
Mediastinitis, either post-sternotomy or extension
from the soft tissues of the neck such as dental or
sinus disease
Demographics
Age: All age groups
Gender: No gender predilection
Natural History & Prognosis
Benign course, except in neonates
Morbidity and mortality related to etiology
Treatment
None, observe for pneumothorax
Treat underlying cause
I DIAGNOSTIC CHECKLIST
Consider
Intrathoracic (Valsalva) and extrathoracic (sinus,
dental, duodenal) causes
Trauma: Blunt chest trauma, bronchial tear,
esophageal tear
Image Interpretation Pearls
Typically benign finding, so clinical history important
to unearth more medically important disease
Lateral radiograph more sensitive than frontal
radiograph
I SELECTED REFERENCES
1. Chapdelaine J et al: Spontaneous pneumomediastinum: are
we overinvestigating? J Pediatr Surg. 39(5):681-4, 2004
2. Howton JC: Boerhaave's syndrome in a healthy adolescent
male presenting with pneumomediastinum. Ann Emerg
Med. 43(6):785, 2004
3. Langwieler TE et al: Spontaneous pneumomediastinum.
Ann Thorac Surg. 78(2):711-3,2004
4. Mihos P et al: Sports-related spontaneous
pneumomediastinum. Ann Thorac Surg. 78(3):983-6, 2004
5. Momin AU et al: Childhood asthma predisposes to
spontaneous pneumomediastinum. Emerg Med J.
21(5):630-1,2004
6. Putukian M: Pneumothorax and pneumomediastinum.
Clin Sports Med. 23(3):443-54, x, 2004
7. Wang YC et al: Pneumomediastinum and subcutaneous
emphysema as the manifestation of emphysematous
pyelonephritis. Int J Urol. 11(10):909-11,2004
8. Asplund CA et al: Spontaneous pneumomediastinum in a
weightlifter. Curr Sports Med Rep. 2(2):63-4, 2003
9. Gardikis S et al: Spontaneous pneumomediastinum: is a
chest X-ray sufficient? Minerva Pediatr. 55(3):293-6, 2003
 PNEUMOMEDIASTINUM

I IMAGE GAllERY

(Left) Radiograph shows

typical appearance of simple
pneumomediastinum with
lucent air collection along 2
left mediastinal con lOur
(arrow). (Right) Axial CEeT 5
shows typical features of
simple pneumomediastinum.
Note air around the
esophagus and pulmonary
arteries, and within
mediastinal fat (arrows).

(Left) Radiograph shows

streaky lucencies along lefl
mediastina! border (curved
arrow), coursing into sort
tissues of neck (arrows) via
Ihoracic inlel. (Right) Fronlal
radiograph shows streaky
/ucencies within confines or
mediastinal contours
(arrows).

(Left) Lateral radiograph

shows streaky air collections
(arrows) within anterior
mediastinal fal. (Right) Axial
CECT shows pulmonary
interstitial emphysema. Note
air in inter/obular septa
(black arrow) and along
pulmonary vein (white
arrow).
 PNEUMOTHORAX

2
6

Anteroposterior radiograph shows typical left-sided PTX Anteroposterior radiograph shows large left PTX in
with 3 em pleural separatjon (arrows), in a patient that supine-positioned patient (arrow). Note "deep sulcus"
is in semi-upright position. sign (curved arrow) on left with costophrenic angle
depressed off field of vie..

Radiographic deep sulcus sign grossly

ITERMINOlOGY underestimates the true size of PTX
Abbreviations and Synonyms Size
Pneumothorax (PTX) o Report size in amount of separation of the visceral
and parietal pleura
Definitions Example: State "12 mm of apical pleural
Air in pleural space separation" not just "small"
Primary typically spontaneous Morphology
Secondary associated with blunt or penetrating o With an otherwise normal pleural space, the pleural
trauma, iatrogenic causes, and diffuse lung diseases air forms around the normal lung shape,
conforming to the principal of "hot air rises"
o When pleural fluid, adhesions, or other underlying
IIMAGING FINDINGS lung diseases present, unusually shaped pleural air
collections collect in non-gravitationally related
General Features regions of the pleural space
Best diagnostic clue: Observation of thin pleural line
parallel to chest wall with no lung markings projecting Radiographic Findings
beyond Visceral pleural line usually parallels chest wall
Location Supine, much less sensitive and underestimates size
o Upright view, look in apex o Deep sulcus (anterolateral hemithorax most
o Supine radiographs, look for deep sulcus nondependent in supine position)
Most non-dependent portion of pleural space, in o Relative transradiancy hemithorax
supine position, is inferior lateral hemithorax o Mediastinal contours or heart border sharper than
uninvolved side

DDx: Pseudopneumothorax

Overlying Body Brace Pneumomediastinum Skin Folds

 PNEUMOTHORAX

Key Facts
Terminology Pathology
Air in pleural space Spontaneous PTX associated with apical bulla
Imaging Findings Clinical Issues
Best diagnostic clue: Observation of thin pleural line Size less important than patient's physiologic status
parallel to chest wall with no lung markings Absorption of air in pleural space: 1.5% per day on 2
projecting beyond room air
Report size in amount of separation of the visceral Increased absorption with supplemental oxygen 7
and parietal pleura Re-expansion pulmonary edema 1%)
Supine, much less sensitive and underestimates size Failure to expand lung may be due to chest tube
malposition, underlying tracheal, bronchial, or
Top Differential Diagnoses esophageal tear, or due to trapped lung with pleural
Skin Fold, Scapula, Hair, Extraneous Monitoring or metastases
Support Lines
Bullous Emphysema Diagnostic Checklist
Pneumomediastinum Don't confuse with skin folds which are a gradation
of grey that fades medially, not a sharp pleural line

o Visualization pericardial tags or fat pads which

become "mass-like" Ultrasonographic Findings
o Air in minor fissure us is more sensitive and confident method for
Expiratory exam diagnosing a pneumothorax, compared to supine
o Increases the relative size of the pleural space bedside chest radiography
compared the overall lung volume o US is as sensitive as CT in detection of
o Should aid detection but not shown to more pneumothoraces
sensitive than full inspiratory examination Imaging Recommendations
o Low lung volumes of expiratory exam often
Best imaging tool: Upright chest radiograph, or CT
misinterpreted as interstitial lung disease or
congestive heart failure
Tension pneumothorax: Radiographic findings +
hemodynamic compromise
I DIFFERENTIAL DIAGNOSIS
o Contralateral shift of mediastinum Skin Fold, Scapula, Hair, Extraneous
o Ipsilateral hyperexpansion of ribs and flattening of
hemidiaphragm
Monitoring or Support lines
o Ipsilateral lung compressed An edge rather than a line, often extends outside chest
May be little compression when the lung wall
compliance decreased as in adult respiratory o Skin fold: Gradation of grey with a black Mach line
distress syndrome laterally that fades off medially, compared to
Pneumothorax ex vacuo distinct visceral pleural line of PTX
o Collection of air in pleural space adjacent to Bullous Emphysema
collapsed lobe Air will not change location with position
o May resolve with reexpansion of lobe No visceral pleural line
o Considered a vacuum phenomenon CT scanning much better at making the important
o Conversely a large pneumothorax may collapse an distinction between large bulla and presence or
upper lobe due to bronchial kinking from weight of absence of PTX
the lung, lobe will reexpand with chest tube
drainage Pneumomediastinum
Can mimic medially loculated PTX
CT Findings
Can also dissect parietal pleura away from thoracic
NECT fascia mimicking an apical PTX
o Air in pleural space, separating lung from chest wall
o Very useful to distinguish pneumothorax from
bullous emphysema or cystic lung disease
Important to distinguish prior to chest tube
I PATHOLOGY
placement to avoid creating a bronchopleural General Features
fistula General path comments
o More sensitive than chest radiography for pleural air o Spontaneous PTX associated with apical bulla
o More sensitive than chest radiography for o Congenital abnormalities leading to apical bulla
distinguishing apical bulla from PTX
 PNEUMOTHORAX
Connective tissue disorders: Marfan;
Ehlers-Danlos, cutis laxa, pseudoxanthoma
elasticum
Etiology
o Primary spontaneous pneumothorax
Height a risk factor; lungs of tall asthenic
individuals may be subject to more gravitational
2 stress
o Secondary pneumothorax
8 Centrilobular pulmonary emphysema
Paraseptal em physema
Interstitial lung disease, especially sarcoidosis
Cystic lung diseases Iymphangioleiomyomatosis
and Langerhans cell histiocytosis
Catamenial: Recurrent PTX associated with
menses and endometrial implants in the pleura
Traumatic: Blunt or penetrating
Neoplastic, especially osteosarcoma metastases
Post-infectious pneumatoceles, typically
Pneumocystis jiroveci or Staphylococcus aureus
pneumonia
Chronic infections, especially tuberculosis, can
also lead to bronchopleural fistula and chronic
pneumothorax
o Complication of intrathoracic interventional
procedures such as lung biopsy and line placements
Gross Pathologic & Surgical Features
Subpleural apical bulla or paraseptal emphysema
commonly identified at CT in patients with
spontaneous pneumothorax
Humans have separate pleural space for each lung
o Transient communication immediately after median
sternotomy
o Long term communication in heart-lung transplants
o Unilateral insult can result in bilateral
pneumothorax, known as "buffalo chest" (buffalo
normally have common pleural space)
Single chest tube will drain both pleural spaces
Microscopic Features
Air in pleural space can induce eosinophilia within
pleural fluid
Staging, Grading or Classification Criteria
Best to describe size in terms of millimeters of apical
pleural separation
Avoid use of small, medium, or large descriptors
Judging percentage size of PTX notoriously inaccurate
ICLINICAL ISSUES
Presentation
Most common signs/symptoms
o Sudden dyspnea, may be life-threatening
o Tall, asthenic individuals have slightly increased risk
of primary (spontaneous) pneumothorax
Other signs/symptoms: Chest pain, may be
asymptomatic
Demographics
Age
o Younger adults prone to spontaneous as well as
trauma-related PTX
o Frequent complication of lung biopsy, especially in
older patients with underlying pulmonary
emphysema
Gender: Spontaneous pneumothorax more common
in younger men
Natural History & Prognosis
Will resolve with observation if air leak not present
Prognosis generally good
Treatment
Chest tube drainage common for larger pneumothorax
o Size less important than patient's physiologic status
o Small pneumothorax can be problematic in patients
with little reserve (COPD)
o Large pneumothorax may not be clinically
significant in young patient with large
cardiopulmonary reserve
o Absorption of air in pleural space: 1.S% per day on
room air
Increased absorption with supplemental oxygen
oRe-expansion pulmonary edema 1%)
Develops within hours of drainage of
long-standing pneumothorax
Transient, resolves over several days
More likely with large chronic pleural space
collections, either air or fluid
o Failure to expand lung may be due to chest tube
malposition, underlying tracheal, bronchial, or
esophageal tear, or due to trapped lung with pleural
metastases
Techniques to decrease pneumothorax incidence post
lung biopsy
o Patient positioned biopsy site down
o Consider injection of autologous thrombus in
biopsy tract
Pleurodesis or bullectomy for recurrent spontaneous
pneumothorax
I DIAGNOSTIC CHECKLIST
Consider
Be sure of patient inclination when reporting exam as
supine exams underestimate size and presence
Image Interpretation Pearls
Don't confuse with skin folds which are a gradation of
grey that fades medially, not a sharp pleural line
Avoid temptation to estimate size as percentage
(notoriously inaccurate)
Report size in terms of distance of pleural separation
CT scan patients with severe bullous disease and
sudden dyspnea best to distinguish PTX from bulla
I SELECTED REFERENCES
1. Chung MJet al: Value of high-resolution ultrasound in
detecting a pneumothorax. Eur Radiol. 2004
2. Waitches GM et al: Usefulness of the double-wall sign in
detecting pneumothorax in patients with giant bullous
emphysema. AJRAmJ Roentgenol. 174(6):1765-8, 2000
 PNEUMOTHORAX

I IMAGE GAllERY
Variant
(Left) Anteroposterior
radiograph shows large right
pleural effusion just before
thoracentesis. (Right) Frontal 2
radiograph just after
thoracentesis shows right 9
PTX with unusual distribution
(arrows). Decrease in size or
right effusion, yet lung has
not re-expanded; so-called
pneumothorax ex vacuo.

(Left) Anteroposterior
radiograph shows shift of
mediastinum to right from
large left PTX physiologically
under tension. (Right)
Sagittal oblique CTA shows a
PTX (arrow) in supine
patient. Note how air
collects in highest portion of
hemithorax. This results in
deep sulcus sign evident on
supine radiographs.

Variant
(Left) Frontal radiograph
shows right PTX with
adhesions (arrow) yielding
an usual loculated
appearance. (Right) Axial
CECT shows same right PTX
with adhesion (arrow) that
results in unusual
appearance.
 TRACHEOBRONCHIAL TEAR

2
10

Sagittal eTA shows asymmetric shape of an overinflated Frontal radiograph shows a large right pneumothorax
endotracheal bal/oon cuff (arrow) from a partial and a "fal/en lung" due to rupture of right mainstem
thickness trachea/laceration. bronchus. Note also, pneumomediastinum (open
arrow) and suocutaneous emphysema in neck (arrow).

Lung falls away from hilum into a gravitationally

ITERMINOlOGY dependent position
Abbreviations and Synonyms o Endotracheal tube
Distended cuff beyond expected tracheal borders
Fallen lung
CT Findings
CTA
I IMAGING FINDINGS o Look for associated injuries to great vessels,
including traumatic aortic rupture and
General Features
pseudoaneurysm
Best diagnostic clue: Persistent or progressive o 10% have no direct signs (tracheal defect, extensive
pneumothorax despite thoracostomy tube drainage air leak, or fractured cartilage)
Location: Most within 2.5 cm of the tracheal carina, o Chronic: If unrecognized acutely, can heal with
where airway is most fixed and subject to shearing
resulting airway stricture
Size: Partial thickness to complete disruption
Imaging Recommendations
Radiographic Findings
Protocol advice: In severe blunt chest trauma, airways
Radiography can be imaged along with aorta and great vessels, as
o Subcutaneous emphysema
part of routine CTA aorta protocol
o Pneumomediastinum, large and progressive
o Pneumothorax (often tension)
o Fractured ribs, clavicles, scapula, sternum often
I DIFFERENTIAL DIAGNOSIS
associated but nonspecific
o "Fallen lung" sign Pneumomediastinum/Pneumothorax
Multiple common etiologies

DDx: Tracheal Laceration

Esophageal Rupture Esophageal Rupture Pneumomediastinum

 TRACHEOBRONCHIAL TEAR

Key Facts
Imaging Findings Esophageal Rupture
Best diagnostic clue: Persistent or progressive Esophageal Intubation
pneumothorax despite thoracostomy tube drainage Clinical Issues
Location: Most within 2.5 em of the tracheal carina, Must be repaired promptly with surgery in most cases
where airway is most fixed and subject to shearing
Pneumomediastinum, large and progressive Diagnostic Checklist
Pneumothorax (often tension) Other life-threatening co-injuries such as aortic
laceration
Top Differential Diagnoses
Pneumomediastinum/Pneumothorax

Esophageal Rupture ICLINICALISSUES

From emetic injury, or blunt/penetrating trauma
Presentation
Esophageal Intubation Most common signs/symptoms
Frontal radiograph shows balloon hyperinflation o Respiratory distress
superimposed over the tracheal air column; lateral o Continuous air leak despite chest tube drainage
radiograph shows endotracheal tube posterior to Other signs/symptoms: Extensive
trachea pneumomediastinum and subcutaneous emphysema:
Confirmation of diagnosis with bronchoscopy
Natural History & Prognosis
I PATHOLOGY Delayed diagnosis due to lack of specific signs; leads to
General Features bronchostenosis
Etiology Treatment
o Direct compression between sternum and spine Must be repaired promptly with surgery in most cases
o Sudden deceleration of lung with fixed trachea
o Forced expiration against closed glottis
o Penetrating trauma I DIAGNOSTIC CHECKLIST
Gunshot and stabwounds; anywhere along airway
Epidemiology Consider
o Uncommon; occurs in 3% of patients who die from Other life-threatening co-injuries such as aortic
trauma laceration
o Delayed diagnosis common: 70% not identified first
24 hours, 40% delayed more than 1 month
Associated abnormalities: Aortic or other great vessel I SElECTED REFERENCES
lacerations, esophageal rupture
1. Kuhlman JE et al: Radiographic and CT findings of blunt
Gross Pathologic & Surgical Features chest trauma: aortic injuries and looking beyond them.
Radiographies. ]8(5):1085-106; discussion 1107-8; quiz 1,
Tears occur commonly at the attachment of the
]998
membranous posterior membrane with cartilage rings
2. Van Hise ML et al: CT in blunt chest trauma: indications
and limitations. Radiographies. 18(5):1071-84, 1998

I IMAGE GALLERY

(Left) Axial CECT shows large right hydropneumothorax despite chest tube (arrow), fallen right lung (curved arrow), pneumomediastinum. and
severe anterior chest wall fracture (open arrow). (Center) Frontal radiograph shows a markedly enlarged endotracheal balloon cuff (arrows) and
otherwise normal exam as the only finding of a partiallhicknes5 trachea/laceration. (Right) Axial CECT shows asymmelric shape of an
overinflated endotracheal balloon cuff (arrow) from a partial thickness tracheal laceration. Also note subcutaneous emphysema.
 LUNG TRAUMA

2
12

Graphic shows multiple left rib fractures, flail chest with Anteroposterior chest radiograph after blunt chest
pulmonary contusion and hemorrhage. trauma shows bilateral peripheral pulmonary contusions
(arrows), beneath multiple bilateral rib fractures, and
right pneumothorax (open arrow).

To avoid semantic confusion, general term

ITERMINOlOGY laceration preferred
Abbreviations and Synonyms Contusion: Torn capillaries and small blood vessels
Contusions without frank disruption of alveoli
Pulmonary lacerations o Marker of rather severe kinetic energy absorption --
o Hematomas not just a bruise of the lung
o Pneumatoceles
Definitions IIMAGING FINDINGS
Injury to lung parenchyma occurring after blunt or
penetrating lung trauma General Features
Typically a combination of contusion, hemorrhage Best diagnostic clue
and laceration o For contusion, peripheral lung opacity with
Laceration is disruption of alveoli leading to radial overlying acute rib fractures
retraction of parenchyma Does not conform to normal bronchovascular
o In blunt trauma, linear tear results in a spherical anatomic distributions
hole in the lung parenchyma o For laceration, look for an air-fluid level within a
o In penetrating trauma, the laceration conforms to complex cystic space, surrounded by hemorrhage
the path of the penetrating object, commonly Location
bullets and knives o Contusions occur directly under the point of energy
o Pulmonary hematoma: Blood filled laceration absorption
o Pneumatocele: Air-filled laceration Common in peripheral lower lung, away from
o Lacerations: Typically filled with variable amounts overlying chest wall musculature
of air and blood

DDx: lung Trauma

Aspiration Lung Abscess Pneumonia

 LUNG TRAUMA
Key Facts
Terminology
Injury to lung parenchyma occurring after blunt or
penetrating lung trauma
Laceration is disruption of alveoli leading to radial
retraction of parenchyma
Contusion: Torn capillaries and small blood vessels
without frank disruption of alveoli
Marker of rather severe kinetic energy absorption u
not just a bruise of the lung
Imaging Findings
Contusions occur directly under the point of energy
absorption
Lacerations occur in four distinct locations,
depending upon the mechanism of injury
Radiographic appearance can be quite variable over
time
Contusions do not respect anatomic boundaries
such as fissures and do not follow
bronchovascular distributions
Can be bilateral
Can occur as contrecoup contusions
o Lacerations occur in four distinct locations,
depending upon the mechanism of injury
Lacerations will conform to the track of a
penetrating object
Small peripheral lacerations occur from rib
fractures
Large central pulmonary lacerations occur after
forced chest compression against a closed glottis
Paravertebral lacerations occur as a shearing injury
when the lung is squeezed over the spine during
an anterior/posterior compression
Can be multiple lacerations in multiple locations
Size
o Contusions can be very small 1 cm) to quite
massive (whole lung) depending upon the extent of
injury
Larger contusions can cause profound hypoxia
o Lacerations are quite variable in size, from < 1 cm to
up to 20 cm
Can be multiple
Morphology
o In blunt trauma, lacerations are approximately
spherical, but can be multiloculated or complex in
appearance
o In penetrating trauma, the laceration conforms to
the path of the penetrating object
o Contusions typically involve the periphery of the
lung in a rim-like distribution
Radiographic Findings
Radiography
o Contusions and lacerations can all be obscured by
surrounding hemorrhage, atelectasis,
pneumothorax, hemothorax
Become more apparent as other confounding
abnormalities clear, heal, or are otherwise treated
o Radiographic appearance can be quite variable over
time
Contusions typically clear within the first few days of
injury
Lacerations heal over several weeks leaving only
minimal scarring
Hematomas can appear as spiculated lung masses as
they heal, mimicking malignancy, sometimes called 2
vanishing lung tumors
13
Top Differential Diagnoses
Aspiration
Pneumonia
Lung Abscess
Clinical Issues
Supportive therapy, surveillance for other major
organ injuries, observe for complications
o Initially, radiographs usually nonspecific patchy or
homogeneous airspace opacities
History of trauma paramount for accurate
diagnosis
Anterior or posterior peripheral opacities can
appear to be diffuse lung injury
o Contusions typically clear within the first few days
of injury
Complete clearing within 10 days
Exception is interval development of adult
respiratory distress syndrome (ARDS)
Contrecoup contusions are very unusual
o Lacerations are present at time of initial injury
May not become apparent for hours or even days
after trauma, as can be obscured by surrounding
atelectasis, contusion, and hemorrhage
Lacerations can change their appearance over days
to weeks, initially being air-filled but becoming
blood-filled, or vice-versa
Lacerations heal over several weeks leaving only
minimal scarring
Hematomas can appear as spiculated lung masses
as they heal, mimicking malignancy, sometimes
called vanishing lung tumors
Opacities appear soon after trauma, < 6 hours
Adjacent to ribs and vertebral bodies
Irregular patchy areas of airspace consolidation (mild)
Perihilar increased interstitial markings
o Hemorrhage and edema in peribronchovascular
interstitium
Diffuse extensive homogeneous consolidation (severe)
CT Findings
CECT: CT: Best to show small lacerations or
hemopneumothorax
More sensitive than radiography for the
characterization of injury and for distinction of
superimposed injuries
May see small lacerations with air-fluid levels
Hematoma
o Slight increased attenuation centrally
o Enhancing rim
o May be confused with nodule
 LUNG TRAUMA
Hemopneumothorax, especially from type 3 injury
Natural History & Prognosis
Imaging Recommendations Contusions will typically clear without residua, unless
Best imaging tool: CT there is superimposed ARDS
Chest radiographs usually sufficient to follow course of Lacerations will take a bit longer to clear (several
blunt trauma weeks) and can leave residual scarring
Extent of pulmonary parenchymal injuries playa
2 [DIFFERENTIAL DIAGNOSIS
pivotal role in determining mortality
o > 20% pulmonary contusion at initial evaluation
14 can be predicted to go on to develop ARDS with
Aspiration 90% specificity
In patients with loss of consciousness, aspiration may
be superimposed Treatment
Follows a bronchovascular distribution; perihilar Supportive therapy, surveillance for other major organ
predominant instead of peripheral injuries, observe for complications
Severe contusions can lead to profound hypoxia
Pneumonia requiring mechanical ventilation
Can have similar radiographic findings, but develop o Lung injury with flail chest requires prolonged
later in hospital course mechanical ventilation
If contusion worsens after 48 hours, consider Severe lacerations with massive hemorrhage may
superinfection require lobectomy
Lacerations rarely become secondarily infected, and o Pulmonary vein laceration, potential for systemic air
therefore should not be confused for a lung abscess embolism
Lung Abscess
Non-traumatic clinical scenario
I SELECTED REFERENCES
1. Mirvis SE: Diagnostic imaging of acute thoracic injury.
I PATHOLOGY 2.
Semin Ultrasound CT MR. 2S(2):156-79, 2004
Mullinix AJ et al: Multidetector computed tomography and
General Features bluntthoracoabdominaltrauma. J Comput Assist Tomogr.
28 Suppll:S20-7, 2004
General path comments 3. Shanmuganathan K: Multi-detector row CT imaging of
o Contusions very common with blunt chest trauma blunt abdominal trauma. Semin Ultrasound CT MR.
o Etiology pathogenesis 25(2):180-204,2004
Sudden deceleration tearing capillaries and small 4. Miller PR et al: ARDS after pulmonary contusion: accurate
blood vessels measurement of contusion volume identifies high-risk
Direct impaction or impalement (fractured rib) patients. J Trauma. 51(2):223-8; discussion 229-30, 2001
CT staging of pulmonary lacerations 5. Ketai Let al: Nonaortic mediastinal injuries from blunt
Type 1: From blunt trauma and sudden compression chest trauma. J Thorac Imaging. ]5(2):120-7,2000
6. LeBlang SD et al: Imaging of penetrating thoracic trauma. J
of pliable chest
Thorac Imaging. ]5(2):128-35,2000
Type 2: Lung compressed and lacerated between chest 7. Mirvis SE et al: MR imaging of thoracic trauma. Magn
wall and vertebra Reson Imaging CIin N Am. 8(1):91-104, 2000
Type 3: Punctured lung by fractured rib 8. Wicky S et al: Imaging of blunt chest trauma. Eur Radiol.
Type 4: Pleural adhesions tear at lung when chest wall 10(10):1524-38,2000
compressed 9. Zinck SE et al: Radiographic and CT findings in blunt chest
trauma. J Thorac Imaging. 15(2):87-96, 2000
Gross Pathologic & Surgical Features 10. Shanmuganathan K et al: Imaging diagnosis of nonaortic
Air spaces filled with blood thoracic injury. Radiol CIin North Am. 37(3):533-5], vi,
1999
Microscopic Features 11. Kuhlman JE et al: Radiographic and CT findings of blunt
Contusion is tearing of capillaries without frank chest trauma: aortic injuries and looking beyond them.
disruption of alveoli Radiographics. 18(5):1085-]06; discussion] 107-8; quiz 1,
1998
]2. Van Hise ML et al: CT in blunt chest trauma: indications
and limitations. Radiographics. 18(5):1071-84, ]998
ICLINICAL ISSUES 13. Kang EY et al: CT in blunt chest trauma: pulmonary,
tracheobronchial, and diaphragmatic injuries. Semin
Presentation Ultrasound CT MR. 17(2):114-8, 1996
Most common signs/symptoms: Nonspecific dyspnea ]4. Reuter M: Trauma of the chest. Eur Radiol. 6(5):707-16,
and chest pain 1996
Other signs/symptoms: Hemoptysis 1S. Mirvis SE et al: hnaging in acute thoracic trauma. Semin
Roentgenol. 27:184-210, 1992
Demographics ]6. Wagner RB et al: Classification of parenchymal injuries of
Age: Young adult men suffer blunt chest trauma more the lung. Radiology. 167(1):77-82, 1988
commonly than other demographic groups
Gender: M > F
 LUNG TRAUMA

I IMAGE GALLERY

Typical
(Left) Frontal radiograph
shows nonspecific patchy
lung opacities. (Right) Axial
CECT shows right-sided
paravertebral laceration and
2
left-sided subcostal 15
laceration (arrows), each
with surrounding
hemorrhage. Bilateral
pneumothoraces and chest
tubes.

(Left) Frontal radiograph

shows large left lung
laceration with surrounding
hemorrhage (arrow) on day
2 after injury. (Right) Frontal
radiograph shows large left
lung blood-filled laceration
with clearing of surrounding
hemorrhage (arrow) on day
7 after injury. Without
specific history easily
mistaken for malignancy.

(Left) Axial CECT shows

large left lung blood-filled
laceration (arrow) on day 7
after injury. (Right) Axial
CECT {rom a man who
suffered a small caliber
gunshot wound shows the
lacerated path of the
projectife (curved arrow)
through the lung
parenchyma (arrows), with
surrounding hemorrhage.
 RIB FRACTURE AND FLAIL CHEST

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,
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, ~~
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;

f',
" ' . ,(
,
\ .

2 J ~ '",;' J~r~I'"
16 r/j~"'l
,
~ ,
"

\
\
Graphic shows multiple displaced mid left rib fractures,
flail chest deformity with underlying pulmonary
contusion, hemorrhage, & hemothorax/pneumothorax.
Frontal radiograph shows bilateral chest tubes, multiple
displaced left rib fractures with flail deformity (arrows),
and underlying peripheral lung contusion.

Morphology
ITERMINOlOGY o Traumatic fractures are often multiple and in
Definitions alignment
Fracture of rib varying from hairline cortical break to May have underlying pulmonary contusion,
shattering of bones hemothorax, or pneumothorax
o Injury to intercostal structures, including nerves, o Pathologic fractures more likely isolated
tendons, ligaments, cartilage and blood vessels, and Expansile and lytic
overlying soft tissues are common Radiographic Findings
Flail chest: 3 or more segmental rib fractures (more Radiography
than 2 fractures within the same rib) or more than 5 o Chest radiograph usually sufficient
adjacent rib fractures o Dedicated rib series occasionally helpful, especially
o That portion of chest wall shows paradoxical when documentation of fracture important
motion with breathing
Medical-legal cases
When pain management will change
Sternum
IIMAGING FINDINGS o Direct trauma: Posterior displacement lower sternal
General Features fragment
o Indirect trauma: Posterior displacement upper
Best diagnostic clue: Cortical break and step off
sternal fragment
Location
Spinal flexion "buckles" the sternum
o Dependent on site of energy absorption
o Most common in the lower ribs, laterally, away from Ribs
overlying musculature o 30% sensitivity for non-displaced fracture by chest
radiography ("normal" to miss rib fractures)
Size: Hairline cortical break to comminution

DDx: Pseudo Rib Fractures

Blastic Metastases Thoracotomy Defect Lytic Metastases

 RIB FRACTURE AND FLAIL CHEST

Key Facts
Terminology Pathology
Flail chest: 3 or more segmental rib fractures (more Paradoxical motion flail segment with ventilation (in
than 2 fractures within the same rib) or more than 5 with inspiration, out with expiration)
adjacent rib fractures
Clinical Issues
Imaging Findings Large segment may result in respiratory impairment 2
Most common in the lower ribs, laterally, away from Rarely, sharp edges of extremely displaced rib
overlying musculature fractures can puncture or lacerate viscera such as the 17
Fractures may only become evident after healing diaphragm, aorta, airway or heart
begins and callous forms
Ribs 4 through 9 most commonly fractured
Diagnostic Checklist
Fractures usually multiple Search for sequela such as pneumothorax or
underlying lung parenchymal injury such as
Top Differential Diagnoses laceration
Pathologic Fracture Lower rib fractures are marker for abdominal visceral
injury

o Fractures may only become evident after healing

begins and callous forms
Imaging Recommendations
Initial radiographs may not show fractures if they Best imaging tool: In most cases, chest radiography
are not displaced suffices
Repeat radiographs taken 4 or more days after
injury usually reveal fractures
Early treatment for uncomplicated rib fracture is I DIFFERENTIAL DIAGNOSIS
same as for bruised ribs; delay in diagnosis does
Pathologic Fracture
not hinder treatment
Typically not aligned
o Ribs 4 through 9 most commonly fractured
Do not present with co-morbidities such as
o Fractures usually multiple
pneumothorax
o Common following blunt chest trauma
May present with minimal trauma only
o Isolated first rib fracture
Lytic and expansile
In proper clinical setting, isolated first rib fracture
can be avulsion injury, especially from throwing Thoracostomy Tube
motion, rowing, or related to whiplash On CT scan, thoracostomy tubes can be confused with
Avulsion from scalene muscle attachment ribs and displaced fractures
o 1st rib fracture, when associated with other rib
fractures, in proper clinical setting, is marker of
high-energy chest trauma I PATHOLOGY
Protected by clavicle, scapula
2% have bronchial tear and 10% have aortic General Features
transection Etiology
Non-traumatic 1st rib fractures have a very low o Direct blow
incidence of major vascular injury o Crush or compression injuries, such as contact
o Flail chest (up to 20% of patients with major sports
trauma) o Severe coughing
3 or more adjacent ribs with segmental fractures o Flail chest
or more than 5 adjacent rib fractures Paradoxical motion flail segment with ventilation
Costal hook sign: Elephant trunk shaped ribs (in with inspiration, out with expiration)
(rotation of segmental fractures) o Rib stress fractures are uncommon
Ultrasound can detect rib fractures Typical locations are the first rib anterolaterally,
o Does not significantly increase the detection rate the fourth through ninth ribs laterally, and the
o Too time-consuming to justify routine use posteromedial upper ribs
Bone scintigraphy useful and sensitive in detecting Occurs in golfers (Duffer fracture), canoeists,
stress fractures and bone metastases, and in child rowers, swimmers, weightlifters, ballet dancers
abuse o Minor trauma if bones are osteoporotic
CT Findings
Primarily used to investigate skeletal integrity and to
evaluate for underlying visceral injuries
 RIB FRACTURE AND FLAIL CHEST
IClINICAllSSUES
Presentation
Most common signs/symptoms
o Chest wall pain or pain with deep breathing,
sneezing, or coughing, accompanied by severe local
2 rib tenderness and swelling
Differential diagnosis for post-traumatic chest wall
pain includes severe rib contusion, costochondral
18
separations, muscle strains and pneumothorax
o Rib fractures very common after blunt chest injury
and after cardiopulmonary resuscitation (CPR)
Associated with thoracic spine and sternum
fractures
Fractures associated with CPR are underreported,
occur in up to 30%
o Cough-induced rib fractures occur primarily in
women with chronic cough
Middle ribs along the lateral aspect of the rib cage
are affected most commonly
Also occurs in patients with Pertussis infection
and post-nasal drip
Sequela include pneumothorax, hemothorax,
pulmonary laceration, and lung contusion
In proper clinical setting, isolated first rib fracture can
be sports-related avulsion injury, especially from
throwing motion or related to whiplash
Flail chest
o May not be clinically evident in 1/3
o Large segment may result in respiratory impairment
o Requires early intubation
o Traumatic extra thoracic intracostallung herniation
is a rare extraordinary associated injury
Rarely, sharp edges of extremely displaced rib fractures
can puncture or lacerate viscera such as the
diaphragm, aorta, airway or heart
Patients with rib fractures should be watched for
development of delayed hemothorax
The greater the number of fractured ribs, the higher
the mortality and morbidity rates
Atelectasis a common sequela of rib fractures
Demographics
Age
o More common with advancing age
Result in a longer duration of pain
Admission of elderly for observation and
treatment, for isolated rib fractures, is justified and
beneficial
o Overall trauma-related mortality is higher in elderly
patients with multiple rib fractures than younger
patients
Gender: Cough-induced rib fractures occur primarily
in women with chronic cough
Natural History & Prognosis
Typically heal with callous
Rarely non-union and pseudoarthrosis
Multiple bilateral healed rib fractures commonly seen
in alcoholiCS
Treatment
Symptomatic pain management
o In trauma patients, epidural analgesia associated
with decreased nosocomial pneumonia and shorter
duration of mechanical ventilation
Surgical fixation very uncommon
Short period of mechanical ventilation and positive
pressure ventilation to stabilize flail segment
I DIAGNOSTIC CHECKLIST
Consider
Other causes of chest pain such as pleuritis
Search for sequela such as pneumothorax or
underlying lung parenchymal injury such as laceration
Image Interpretation Pearls
Lower rib fractures are marker for abdominal visceral
injury
Dedicated rib radiographs only, without a chest
radiograph, may miss associated pneumothorax
I SElECTED REFERENCES
1. Hanak V et al: Cough-induced rib fractures. Mayo Clin
Proc. 80(7):879-82, 2005
2. Sharma OP et al: Prevalence of delayed hemothorax in
blunt thoracic trauma. Am Surg. 71(6):481-6,2005
3. Bulger EM et al: Epidural analgesia improves outcome after
multiple rib fractures. Surgery. 136(2):426-30, 2004
4. Hurley ME et al: Is ultrasound really helpful in the
detection of rib fractures? Injury. 35(6):562-6, 2004
5. Stawicki SPet al: Rib fractures in the elderly: a marker of
injury severity. JAm Geriatr Soc. 52(5):805-8, 2004
6. Holcomb JB et al: Morbidity from rib fractures increases
after age 45. J Am Coli Surg. 196(4):549-55,2003
7. Kara M et al: Disclosure of unnoticed rib fractures with the
use of ultrasonography in minor blunt chest trauma. EurJ
Cardiothorac Surg. 24(4):608-13, 2003
8. Karmakar MKet al: Acute pain management of patients
with multiple fractured ribs. J Trauma. 54(3):615-25, 2003
9. Sirmali M et al: A comprehensive analysis of traumatic rib
fractures: morbidity, mortality and management. EurJ
Cardiothorac Surg. 24(1):133-8, 2003
10. Victorino GP et al: Trauma in the elderly patient. Arch
Surg. 138(10):1093-8,2003
11. Bamea Yet al: Isolated rib fractures in elderly patients:
mortality and morbidity. Can J Surg. 45(1):43-6, 2002
12. Velmahos GC et al: Influence of flail chest on outcome
among patients with severe thoracic cage trauma. Int Surg.
87(4):240-4,2002
13. Weyant MJ et al: Severe crushed chest injury with large
flail segment: computed tomographic three-dimensional
reconstruction. J Trauma. 52(3):605, 2002
14. Collins J: Chest wall trauma. J Thorac Imaging.
15(2):112-9,2000
15. Westcott J et al: Rib fractures. American College of
Radioloh'Y.ACRAppropriateness Criteria. Radiology. 215
Suppl:637-9, 2000
16. el-Khoury GYet al. Trauma to the upper thoracic spine:
Anatomy, biomechanics, and unique imaging features.
AJR.160:95-102, 1993
17. Krischer JP et al: Complications of cardiac resuscitation.
Chest. 92(2):287-91,1987
18. DeLuca SAet al. Radiographic evaluation of rib fractures.
AJR.138:91-2, 1982
 RIB FRACTURE AND FLAIL CHEST

I IMAGE GAllERY

(Left) Axial CECT after car

crash shows multiple
displaced left rib fractures 2
(arrows), bilateral
pneumothorax, left
hemothorax, and 50(( tissue 19
emphysema. (Right) 30
reconstruction from a eTA of
the chest (scapula removed)
shows typical flail chest
deformity of the upper right
posterior chest wall (arrows).

Variant
(Left) Axial bone CT shows
non-displaced fracture of left
first rib (arrow). This is a
common sequelae of
"whiplash", from avulsion
after strong contraction of
scalene muscle attachment
(Right) Axial CECT shows
sequelae of a severe chest
injury with multiple
fractures: Herniation of lung
parenchyma (arrow). Note
similarity in appearance of
chest tube (open arrow) to a
rib.

(Left) Axial CECT shows a

single right rib fracture
(arrow) suffered after a
minor fall. Note severe
subsequent complications:
Pneumothorax,
pneumomediastinum, and
subcutaneous emphysema.
(Right) 3D reconstruction
from a CTA of the chest
(scapula removed) shows
typical flail chest deformity
of the mid left posterior chest
waif (arrows).
 AORTIC TRANSECTION

2
20

Frontal radiograph shows a widened mediasUnum and Axial CECT in same patient shows an intimal flap
enlargement of the aortic contours (curved arrows) (arrow) at the site of aortic injury in the proximal
from aortic transection. Note the tracheal deviation to descending aorta with a mediasUnal hematoma at the
the right (arrow). typical site for aortic rupture.

ITERMINOLOGY Radiographic Findings

Radiography
Abbreviations and Synonyms
o Will not visualize tear, indirect signs only from
Traumatic aortic injury (TAl), acute traumatic aortic hemorrhage
injury (ATAI), blunt traumatic aortic rupture (BTAR), o Signs of transection sensitive but not specific
blunt aortic trauma (BAT) o Most signs present, frequency of 30-70%
Definitions Widened superior mediastinum
Disruption or tear of aortic wall, usually result of Abnormal contour aortic arch, obscuration AP
traumatic injury from motor vehicle accident (MVA) window
or serious fall Tracheal shift to right
o 20% of cases injury may be limited to partial Nasogastric (NG) tube shift to right
circumferential tear intima and or the media Widening paraspinal stripe
Right para tracheal stripe thickening
Left apical cap
I IMAGING FINDINGS Depressed left mainstem bronchus
o 1st rib fracture direct indicator severity of trauma
General Features and likelihood of aortic transection
Best diagnostic clue Protected by clavicle and scapula, requires
o Trauma chest radiograph: Widened mediastinum considerable force to break
o Intimal flap, irregular contour aortic wall by eTA Frequency of finding less than 30%
Location (approximately 15%)
o 90% occur at aortic isthmus o Any above signs require further investigation to rule
o 7-8% occur aortic root, 2% diaphragmatic out transection

DDx: Ductal Dilatation

Fusiform ~ilatation Atherosclerotic Ulcer

 AORTIC TRANSECTION

Key Facts
Terminology Top Differential Diagnoses
Disruption or tear of aortic wall, usually result of Ductus Diverticulum (Type III Ductus)
traumatic injury from motor vehicle accident (MVA) Normal Variant Fusiform Enlargement Proximal
or serious fall Descending Aorta
Atherosclerotic Ulceration
Imaging Findings 2
Protected by clavicle and scapula, requires Pathology
considerable force to break Deceleration injury: Stretching injury; aorta fixed at 21
Signs centered at aortic arch, most common location ligamentum arteriosum
for transection 90% at aortic isthmus
Chronic aneurysm (2% survivors)
CTA directly demonstrates aortic tear, markedly
Clinical Issues
reducing need for aortography Multiple associated injuries: Diaphragm rupture, lung
Periaortic hematoma contusion, rib fractures, head injury
Pseudo diverticulum or irregular contour aortic wall 15% of mortalities in MVA
Delayed repair may be acceptable in many cases
Intimal flap
Endovascular stent grafts very promising

o Signs centered at aortic arch, most common location

for transection
MR Findings
o Normal chest radiograph previously considered rare MR generally no role in evaluation of acute trauma
CTA has shown injures in up to 15% with no o Limited transporting and monitoring critically
signs aortic transection on radiograph (false injured patients
negative chest radiograph) Echocardiographic Findings
o Chronic aneurysm (2% survivors) Transesophageal echocardiography
Calcified mass aorticopulmonary window o Demonstrate intimal tears, transection
CT Findings o More difficult in severely injured patients
o Limited availability
CTA
o Now modality of choice Imaging Recommendations
o CTA directly demonstrates aortic tear, markedly Best imaging tool: CTA for direct evaluation of aorta
reducing need for aortography Protocol advice: CTA with thin-reconstructions and
o Direct signs multi planar reformations
Periaortic hematoma
Pseudo diverticulum or irregular contour aortic
wall I DIFFERENTIAL DIAGNOSIS
Intimal flap
o Requires intravenous contrast and helical scanning Widened Mediastinum
o Accuracy: Sensitivity 85-100%, specificity 80% False positives from rotation, supine positioning,
o False positives expiration
Motion or streak artifact
Atherosclerotic plaque Ductus Diverticulum (Type III Ductus)
Ductus diverticulum Anteromedial outpouching of isthmus
Adjacent bronchial artery Smooth, gently sloping shoulders
NECT: May only visualize hematoma No intimal tear
CECT: Initially used to reduce false-positive chest
radiographs by demonstrating other causes of Normal Variant Fusiform Enlargement
mediastinal widening Proximal Descending Aorta
Similar appearance, no intimal flap
Angiographic Findings
Angiography Aortic Spindle
o Considered gold standard for evaluating aorta and Congenital narrowing ligamentum arteriosum
great vessels
o Using chest radiograph as guide, perform 10 Atherosclerotic Ulceration
negative angiograms for each tear Ulcerated plaque
o Small risk of rupture More common in older patients
o Rapidly being replaced by CT angiography Look for other aortic plaques
o False negatives and false positives low Infundibulum of Bronchial-Intercostal Trunk
o CT angiography: 5% angiographic miss rate for
Take off may show bump in aortic contour
aortic injury
 AORTIC TRANSECTION
I PATHOLOGY I DIAGNOSTIC CHECKLIST
General Features Consider
General path comments Look for signs of aortic transection in any blunt
o Etiology: Theories of pathogenesis trauma chest radiograph
Deceleration injury: Stretching injury; aorta fixed
Image Interpretation Pearls
2 at ligamentum arteriosum
Osseous pinch: Manubrium and first ribs rotate Consider chronic pseudoaneurysm in any patient with
and impact spine causing shear injury vascular calcification in the aorticopulmonary window
22
Multivariate hypothesis likely: Shearing, torsion,
stretching, hydrostatic forces
Epidemiology: Accounts for 15% fatalities in MVA I SELECTED REFERENCES
I. Anakwe RE. Traumatic aortic transection. Eur J Emerg Med.
Gross Pathologic & Surgical Features 12(3): 133-5, 2005
90% at aortic isthmus 2. Pacini DA et al: Traumatic rupture of the thoracic aorta:
o From origin left subclavian artery to ligamentum ten years of delayed management. J Thorac Cardiovasc
arteriosum, often anteromedially 5urg. 129(4):880-4,2005
Other 7-8% ascending aorta or 2% descending aorta at 3. Takahashi K et al: Multidetector CT of the thoracic aorta.
diaphragmatic hiatus IntJ Cardiovasc Imaging. 21(1):141-53, 2005
4. Hatem A et al: Vascular Emergencies of the Thorax after
Ascending aorta 20% coroner's cases, rarely survive to
Blunt and Iatrogenic Trauma: Multi-Detector Row CT and
reach hospital Three-dimensional Imaging. RadioGraphics. 24: 1239-1255,
Transverse circumferential tear: Intima and media tear 2004
with intact adventitia (60%) 5. Kondo N et al: Surgical repair for chronic traumatic
May involve layers to varying degrees thoracic aneurysm after 12-year follow-up. Jpn J Thorac
o In survivors, pseudoaneurysm usually contained by Cardiovasc 5urg. 52(12):586-8, 2004
adventitia or occasionally mediastinal structures 6. Neuhauser B et al: 5teol-graft repair for acute traumatic
o Complete tear through intima, media and adventitia thoracic aortic rupture. Am 5urg. 70(12):1039-44, 2004
7. Stamenkovic SA et al: Emergency endovascular stent
usually leads to exsanguination
grafting of a traumatic thoracic aortic dissection. Int J Clin
Noncircumferential tears more common posteriorly Pract. 58(12):1165-7, 2004
8, Wong H et al: Periaortic Hematoma at Diaphragmatic
Crura at Helical CT: Sign of Blunt Aortic Injury in Patients
ICLINICAL ISSUES with Mediastinal Hematoma. Radiology. 231(1): 185- 189,
2004
Presentation 9. Czermak BV et al: Placement of endovascular stent-grafts
Urgent diagnosis, 50% expire 24 hours if untreated for emergency treatment of acute disease of the descending
Majority have no signs or symptoms, nonspecific chest thoracic aorta. AJR Am J Roentgenol. 179(2): 337-45, 2002
10. Richens 0 et al: The mechanism of injury in blunt
pain, dyspnea
traumatic rupture of the aorta. Eur J Cardiothorac Surg
o Acute coarctation syndrome rare
21 :288-293, 2002
Upper extremity hypertension II. Thompson CS et al: Acute traumatic rupture of the thoracic
Decreased femoral pulses aorta treated with endoluminal stent grafts. J Trauma
Multiple associated injuries: Diaphragm rupture, lung 52(6): 1173-7, 2002
contusion, rib fractures, head injury 12. Dyer OS et al: Thoracic aortic injury: how predictive is
mechanism and is chest computed tomography a reliable
Natural History & Prognosis screening tool? A prospective study of 1,561 patients.J
] 5% of mortalities in MVA Trauma 48(4): 673-82; discussion 682-3, 2000
85% mortality prior to reaching hospital 13. Fishman JE: Imaging of blunt aortic and great vessel
Survival dependent on time from injury to trauma. J Thorac Imaging 15(2): 97-103, 2000
14. Dyer OS et al: Can chest CT be used to exclude aortic
intervention
injury? Radiology 213:195-202,1999
Treatment I S. Fishman JE et al: Direct versus indirect signs of traumatic
aortic injury revealed by helical CT: performance
Surgical repair characteristics and interobserver agreement. AJR Am J
o Delayed repair may be acceptable in many cases Roentgenoll72(4): 1027-31, 1999
o Other injuries increase mortality of immediate repair 16. Mirvis SE et al: Use of spiral computed tomography for the
o 70-85% surgical survival quoted (up to 20% surgical assessment of blunt trauma patients with potential aortic
mortality) injury. J Trauma 45(5): 922-30, 1998
o Paraplegia 10% (directly related to cross-clamp time) 17. Patel NH et al: Imaging of acute thoracic aortic injury due
o Circulatory assistance techniques decrease incidence to blunt trauma: A review. Radiology 209:335-48, 1998
of ischemia 18. Ahrar K et al: Angiography in blunt thoracic aortic injury. J
Trauma 42(4): 665-9, 1997
Beta-adrenergic blocking agents decrease wall stress 19. Mirvis SE et al: Traumatic aortic injury: diagnosis with
Endovascular stent grafts very promising contrast-enhanced thoracic CTu five-year experience at a
o Especially with multiple other associated injuries major trauma center. Radiology 200(2): 413-22, 1996
o Complete pseudoaneurysm resolution reported at 3 20. Gavant ML et al: Blunt traumatic aortic rupture: detection
months with helical CT of the chest. Radiology 197:125-133, 1995
o Long term follow-up not available
 AORTIC TRANSECTION

I IMAGE GALLERY
Typical
(Left) Sagittal oblique CTA
shows the aortic tear seen on
the prior axial CECT (open
arrow). The tear is currently 2
contained. This shows the
proximity to the adjacent 23
ligamentum arteriosum
(arrow). (RighI) Coronal
CECT shows the level of the
ligamentous arteriosum
(arrow). This patient also
had an aortic injury which is
not shown in the field of
view, although there is a
mediastinal hematoma.

(Left) Coronal CECT shows

an image at a more posterior
level demonstrating the
aortic tear (arrows). (RighI)
Sagittal oblique OSA shows a
contained transection of the
descending aorta (arrows).
This severe of an injury is at
a very high risk for imminent
rupture. (Courtesy /. Caridi,
MO).

Typical
(Left) Axial CTA shows
pseudoaneurysm {ormation
from a concentric aortic tear
(arrows) with a large
mediastinal hematoma. As in
most images here, the CTA
findings preclude the need
for OSA. (RighI) Sagittal
oblique OSA shows tear of
both the ascending aorta,
near the left main coronary
artery (arrow), as well and
proximal descending aorta
(open arrow). (Courtesy J.
Caridi, MOJ.
 SPINAL FRACTURE

2
24

Sagittal graphic shows fracture dislocation in the Sagittal T2WI FS MR shows two column compression
thoracolumbar spine. In blunt chest trauma and flexion, fracture with retropulsion and spinal canal stenosis
the most common location for fractures is the (arrow). Spinal cord normal.
thoracolumbar junction .

Location: Most common location thoracic spine injury

I IMAGING FINDINGS at thoracolumbar junction
General Features Radiographic Findings
Best diagnostic clue Radiography
o Anterior compression fracture o Anterior compression fracture
Most common type of thoracic spine fracture due Paraspinous hematoma apparent
to blunt trauma Distinct fracture line usually not visible
Wedge-shaped vertebral body Kyphosis common
May occur at multiple levels, contiguous or Almost always involves superior endplate, < 5%
noncontiguous involve inferior endplate only
o Flexion-distraction (Chance) fracture < 40-50% loss of height in patients with normal
Injury involving compression of anterior column bone density; if greater loss of height, probably
with distraction of middle and posterior columns Chance fracture
o Burst fracture Osteoporotic patients may develop vertebra plana
Compressed thoracic vertebral body with fractured o Chance fracture
endplates, widened pedicles Usually more than 40-50% loss of height of
o Facet-Lamina fracture vertebral body
Cortical disruption/discontinuity through Focal kyphosis
laminae, facet joints of thoracic vertebrae 5eparation of facet joints
Uncommon in upper/mid-thoracic spine; TI-TIO Increased interspinous distance
stabilized by ribs Listhesis and retropulsion of posterior vertebral
o Lateral compression fracture body cortex absent
Lateral wedge deformity o Burst fracture

DDx: Thoracic Spine Fracture

" '--., . '" -.:--.r.=

Kyphoscoliosis
" ~~
Pott Disease
-'
Lytic Metastasis
 SPINAL FRACTURE
Key Facts
Imaging Findings
Anterior compression fracture
Most common type of thoracic spine fracture due to
blunt trauma
Flexion-distraction (Chance) fracture
Injury involving compression of anterior column
with distraction of middle and posterior columns
Location: Most common location thoracic spine
injury at thoracolumbar junction
Top Differential Diagnoses
Spinal Abscess
Spinal Metastasis
Kyphoscoliosis
Pathology
Thoracic spine fractures, 15% have multiple levels
Widened pedicles on AP view, wedge-shaped
vertebral body on lateral view
May see malalignment
o Facet-lamina fracture
Cortical disruption/discontinuity through
laminae, facet joints of thoracic vertebrae,
increased interspinous distance indicates ruptured
posterior ligamentous complex often with
concomitant neural arch fractures
o Lateral compression fracture
Lateral wedge deformity
Intact posterior body wall, no fragment
retropulsion
Thoracic spine
o Pedicle thinning with a slight increase in the
interpediculate distance at the level of thinning
normally seen at the thoracolumbar junction in 7%
o Rule of 2's: 2 mm is the normal upper limits for
difference in
Interspinous or interlaminar distance
Interpedicular distance (transverse and vertical)
Anterolisthesis or retrolisthesis with flexion and
extension
Facet joint width
Height of anterior and posterior vertebral bodies
o Normal bone integrity
Anterior height < posterior height: Ratio 0.80
males, 0.87 females
o Instability if one of the following
Displaced vertebra
Widened interlaminar or interspinous distance
Perched or dislocated facet joints
Increased interpedicular distance
Disrupted posterior vertebral body line
CT Findings
Bone CT
o Primarily used to investigate skeletal integrity
o Anterior compression fracture
Multiple fracture lines often visible
Posterior cortical displacement absent
Fractures of posterior elements absent
< 5% have both aortic transection and cord injury
Spinal canal smallest in thoracic spine
Diagnostic Checklist
Widened pedicles relative to vertebral body above is
strong clue to burst and instability of vertebral body
Compression fracture involving inferior end plate
2
with normal superior end plate raises suspicion for 25
pathologic fracture
Consider Chance fracture whenever radiography
shows severe compression fracture in patient with
normal bone density
Look for paraspinal soft tissue hematoma as clue to
spinal fractures on CT
Up to 20% of compression fractures are multiple;
screen entire spine
Alignment of posterior elements normal on
reformatted images
o Chance fracture
Fracture vertebral body, often comminuted
Separation of facet joints
Increased interspinous distance
Focal kyphosis
o Burst fracture
Soft tissue swelling surrounding vertebral body
May see blood in canal
Stellate pattern of fractures
o Facet-lamina fracture
Widened, comminuted neural arch
Fracture through facet joints
"Naked facet" sign: Partially or completely
uncovered articulating processes on axial imaging
Locked facets
Vertebral body comminution; retropulsed bony
fragments within spinal canal
"Double body" sign: Overlapping vertebrae on
axial imaging
o Lateral compression fracture
Intact posterior body wall, no fragment
retropulsion
Arc of irregular bone fragments circumferentially
from vertebral body
MR Findings
Primarily used to investigate spinal cord, disc, and
ligaments
Optimal timing for cord imaging: 24-72 hours
following injury
Anterior compression fracture
o T1 WI: Low signal intensity fracture line mayor may
not be seen
o Posterior cortex intact
o Paraspinous hematoma mimics tumor
Chance fracture
o TIWI: Discrete fractures as for CT or amorphous low
signal in vertebral body
o T2WI: Disruption of posterior longitudinal ligament,
interspinous ligaments
 SPINAL FRACTURE
Anterior longitudinal ligament usually intact, may
be stripped from vertebra inferior to fracture Kyphoscoliosis
May see cord contusion 3 contiguous levels
o Syringomyelia may develop chronically Undulation of vertebral endplates
Burst fracture
o Tl WI: Low signal within wedged vertebral body
o T2Wl: High signal within disrupted vertebral body, I PATHOLOGY
2 surrounding soft tissue
General Features
May see cord contusion
26 Facet-lamina fracture Etiology
o Paraspinal and intraspinal hematoma o Thoracic spine flexion
o Herniated disc Results in compression fractures (50% of all
o Tl WI: Acute intraspinal hemorrhage hyperintense fractures)
o Axial compression
Hypointense fracture lines
Separated osseous fragments Results in burst fracture (15% of all fractures)
o T2Wl: Hyperintense marrow edema o Hyperflexion
Results in flexion-distraction (seat belt) fracture
Posterior soft tissue edema
(15% of all fractures)
Ligamentous tear with widened interspinous gap
o Shearing results in fracture-dislocation (5% of all
Cord edema, compression, distraction
Lateral compression fracture fractures)
o TlWI Epidemiology
Acute: Hypointense marrow edema o Thoracic spine fractures, 15% have multiple levels
o < 5% have both aortic transection and cord injury
Chronic: Marrow signal normalizes with time
Paraspinal, epidural enhancing tissue suggests Gross Pathologic & Surgical Features
malignant pathology Spinal canal smallest in thoracic spine
o STIR o Limited leeway for fragments to cause cord injury
Acute: STIR best visualizes hyperintense marrow
edema which can extend into pedicles
Acute: May reveal fluid filled intravertebral cleft = ICLINICAl ISSUES
"fluid sign"
Presentation
Imaging Recommendations
Osteoporotic patients may have progressive fracture
Best imaging tool
o Anterior compression fracture Treatment
CT best to differentiate from Chance fracture, Surgical fixation thoracic spine fractures canal
burst fractures decompression
o Chance fracture
CT scan for surgical planning
o Burst fracture I DIAGNOSTIC CHECKLIST
MR vital if neurologic symptoms/signs present
o Facet-lamina fracture Image Interpretation Pearls
Thin-section bone CT most effective in Widened pedicles relative to vertebral body above is
characterizing posterior column fractures strong clue to burst and instability of vertebral body
MR best for cord evaluation Compression fracture involving inferior end plate with
o Lateral com pression fracture normal superior endplate raises suspicion for
CT best to differentiate from Chance fracture, pathologic fracture
burst fractures Consider Chance fracture whenever radiography
shows severe compression fracture in patient with
normal bone density
I DIFFERENTIAL DIAGNOSIS Look for paraspinal soft tissue hematoma as clue to
spinal fractures on CT
Spinal Abscess Up to 20% of compression fractures are multiple;
Marrow edema in facet articular processes & adjacent screen entire spine
laminae on MR
Surrounding soft tissue edema, enhancement and
abscess I SELECTED REFERENCES
Spinal Metastasis 1. el-Khoury GY et al. Trauma to the upper thoracic spine:
More likely to involve inferior cortex of vertebral body Anatomy, biomechanics, and unique imaging features.
AJR. 160:95-102, 1993
Metastases often involve posterior elements as well as
2. DeLuca SA et al. Radiographic evaluation of rib fractures.
vertebral body AJR. 138:91-2,1982
Cortical destruction
 SPINAL FRACTURE
I IMAGE GALLERY

(Left) Frontal radiograph

shows fracture dislocation of
T 11-12 vertebral bodies
(arrows). Note the lateral 2
displacement and angulation
at the site o( injury. (Right) 27
Axial NECT shows unstable
fracture dislocation of the
thoracolumbar spine. Note
the displaced fracture
fragments within the spinal
canal (arrow).

Typical
(Left) Sagittal reformatted CT
shows unstable fracture
dislocation of the
thoracolumbar spine. Note
the displaced fracture
fragments within the spinal
canal (arrow). (Right) Axial
NECT shows a stable, single
column burst fracture of the
lower thoracic spine.

Typical
(Left) Axial NECT from a
patient injured in a car crash
shows a comminuted
flexion/distraction fracture
(arrows) of lower thoracic
vertebra with anterior
compression and posterior
distraction. (Right) Sagittal
reformatted CT shows
comminuted
flexion/distraction Chance
fracture of T 9-10 thoracic
vertebra with anterior
compression and posterior
distraction, with widened
interspinous distance
(arrow).
 STERNAL FRACTURE

2
28

Sagittal oblique MDCT shows 3D reconstruction of a Multiplanar reconstruction. Origin of the displaced
manubrial transverse fracture (arrows). Fracture fragment from the manubrium nicely demonstrated on
fragment displaced posteriorly against the aortic arch the reconstructed views (arrows).
(open arrow).

o Posteroanterior chest radiographs: Fractures usually

ITERMINOlOGY not identified
Nonspecific findings: Mediastinal widening
Definitions
o Lateral radiograph: Useful to demonstrate fractures
Most sternal fractures are caused by blunt anterior
chest trauma CT Findings
Other causes MDCT with sagittal, coronal and 3D reconstructions
o Stress fractures improves the diagnostic accuracy for sternal injuries
o Insufficiency (spontaneously in patients with
osteoporosis or osteopenia) MR Findings
o Pathologic fractures: Underlying neoplastic lesions Useful in diagnosing insufficiency fractures
either primary or metastatic o Tl WI: Intermediate signal intensity; T2 weighted
fat-suppressed images: High signal intensity

I IMAGING FINDINGS
I DIFFERENTIALDIAGNOSIS
General Features
Location Pathologic Fractures
o Most of fractures are localized in the body of the Underlying neoplastic lesions with bone destruction
sternum History of malignancy
Nondisplaced and transverse
Osteomyelitis
Radiographic Findings Soft tissue mass usually prominent
Radiography Constitutional symptoms: Fever, chills, malaise

DDx: Non-Traumatic Sternal Fracture

Osteomyelitis Tuberculous Osteomyelitis Metastases

 STERNAL FRACTURE

Key Facts
Imaging Findings After thoracic compression during cardiopulmonary
Posteroanterior chest radiographs: Fractures usually resuscitation (CPR) .
not identified Clinical Issues
Lateral radiograph: Useful to demonstrate fractures Death and morbidity are related to associated injuries
Pathology Diagnostic Checklist 2
Motor-vehicle accidents, high-speed sports activities 3D reconstructions improves diagnostic accuracy
Osteoporosis: Often have associated thoracic 29
MR useful in diagnosing insufficiency fractures
compression fractures

o Palpitations (cardiac contusion)

I PATHOLOGY
Demographics
General Features
Age: Uncommon in children
Etiology Gender: Slightly more common in females (shoulder
o Direct trauma: Posterior displacement lower sternal restraint positioning)
fragment
Motor-vehicle accidents, high-speed sports Natural History & Prognosis
activities Death and morbidity are related to associated injuries
o Indirect trauma: Posterior displacement upper o Mediastinal hematoma ranging in size from a few
sternal fragment (spinal flexion "buckles" the mm to 2 cm
sternum) o Traumatic aortic rupture occurs in 2% of cases
o Osteoporosis: Often have associated thoracic o Myocardial contusion (often clinically silent)
compression fractures
o After thoracic compression during cardiopulmonary Treatment
resuscitation (CPR) Directed toward associated injuries
Epidemiology
o Motor vehicle accidents account for 60-90% of
sternal fractures I DIAGNOSTIC CHECKLIST
o 8% of patients with blunt chest trauma have sternal
fractures Consider
Associated abnormalities: Thoracic and cardiac injuries Sternal fracture not an indication for aortography
are more frequent in displaced fractures of the body of 3D reconstructions im proves diagnostic accuracy
the sternum MR useful in diagnosing insufficiency fractures

ICLINICAL ISSUES I SELECTED REFERENCES

1. Rashid MA et al: Cardiovascular injuries associated with
Presentation sternai fractures. Eur J Surg. 167:243-248,2001
Most common signs/symptoms: Localized sternal pain 2. Franquet T et al: Imaging findings of sternal abnormalities.
Other signs/sym ptoms Eur Radial. 7: 492-497, 1997
o Dyspnea in 15-20%

I IMAGE GALLERY

(Left) Lateral chest view shows transverse sternal fracture with displacement of fragments (arrows). Stress fracture. (Center) Corresponding axial
NECT shows transverse sternal fracture with associated dehiscence of fragments (arrows). (Right! Axial NECT in a post-traumatic patient shows a
longitudinal sternal fracture (arrows). Note associated subcutaneous emphysema or
the right chest wall.
 DIAPHRAGMATIC TEAR

2
30

Graphic shows typical appearance of left Sagittal oblique eTA shows herniation of stomach
hemidiaphragm rupture with herniation of portion of above ruptured left hemidiaphragm. Arrows indicate the
stomach into pleural space. point of disruption to the diaphragm.

Clinical manifestation (visceral herniation) much

I TERMI NOLOGY more common on left side (70-90%)
Abbreviations and Synonyms Liver less likely to herniate through smaller
Hemidiaphragm rupture or laceration right-sided lacerations
Size
Definitions o Tear in hemidiaphragm is quite variable in size
Post-traumatic laceration of hemidiaphragm Prevalence of visceral herniation increases with
frequently resulting in herniation of abdominal viscera larger tears
into thorax Morphology: Linear or radial tears typically at dome of
o Blunt trauma more common than penetrating hemidiaphragm where tendon is thinnest
trauma
Radiographic Findings
Chest radiography usually abnormal but often
nonspecific because of associated lower lobe
IIMAGING FINDINGS atelectasis, contusion, and other more compelling
General Features injuries such as hemothorax or pneumothorax
Best diagnostic clue o Specific signs: Air-filled viscus in hemithorax
o Air-filled bowel above the hemidiaphragm Abnormal 90% but diagnostic in only 50%
Even more accurate when nasogastric (NG) tube Abnormal diaphragmatic contour
present above expected position of Air-filled bowel in hemithorax
hemidiaphragm Tip of NG tube in hemithorax
Location o Tear usually spares esophageal hiatus
o Occur on right and left sides with equal frequency o NG tube will course normally into abdomen and
then traverse into hemithorax if stomach herniated

Bochdalek Hernia Eventration Hepatomegaly

 DIAPHRAGMATIC TEAR

Key Facts
Terminology Top Differential Diagnoses
Post-traumatic laceration of hemidiaphragm Eventration of Diaphragm
frequently resulting in herniation of abdominal Diaphragm Paralysis
viscera into thorax Enlarged Liver
Pleural Effusion
Imaging Findings
Clinical Issues
2
Air-filled bowel above the hemidiaphragm
Clinical manifestation (visceral herniation) much Most common signs/symptoms: High-energy blunt 31
more common on left side (70-90%) torso injury with associated chest wall, pleural,
Morphology: Linear or radial tears typically at dome vascular, and visceral in juries
of hemidiaphragm where tendon is thinnest Intubated patient on positive pressure may prevent
Tip of NG tube in hemithorax herniation
Dependent viscera sign very accurate, especially for High index of suspicion important throughout the
left-sided rupture and herniation of bowel hospital course of trauma patients
Best imaging tool: CT best for global recognition of
associated injuries Diagnostic Checklist
Must have a high index of clinical suspicion

Elevated diaphragm> 7 cm o Approximation and narrowing of afferent and

Diaphragmatic contour changes shape with change in
position
Contralateral mediastinal shift
Strangulation of bowel
o With open communication, pleural fluid should not
accumulate
o Pleural effusion in patient with hernia should
suggest strangulation
o Omental fat may simulate pleural effusion,
including layering on decubitus examination
CT Findings
Dependent viscera sign very accurate, especially for
left-sided rupture and herniation of bowel
o Liver or bowel in contact with posterior lower ribs,
in expected location of pleural fluid (pleural space)
Visceral herniation with focal constriction of bowel or
liver (collar sign)
Diaphragmatic discontinuity, thickening, segmental
abscence, and combined hemothorax and
hemoperitoneum strong predictors of blunt
diaphragmatic rupture
Left diaphragmatic tear: Sensitivity approaching 100%,
specificity 100%
Right diaphragmatic tear: Sensitivity 70-800,,),
specificity 100%
Reformats in coronal and sagittal plane can increase
diagnostic confidence, especially multidetector CT
scanning technology
efferent bowel loops (pinched limbs) through the
diaphragmatic defect (collar sign or kissing birds
sign)
Liver-spleen colloid scans have been used to diagnose
diaphragmatic tears (collar sign)
Imaging Recommendations
Best imaging tool: CT best for global recognition of
associated injuries
Protocol advice: Coronal or sagittal reformats can be
helpful
I DIFFERENTIAL DIAGNOSIS
Eventration of Diaphragm
No dependent viscera sign
Hemidiaphragm should appear intact
No associated injuries
Typically seen in elderly females without a history of
recent trauma
Bowel loops will not be approximated in eventration
Can be difficult to distinguish if pre-existing condition
is associated with recent blunt torso trauma
Diaphragm Paralysis
Paradoxical motion at fluoroscopy (sniff test)
No recent history of trauma
o Idiopathic or secondary to malignancy

MR Findings Enlarged Liver

Similar to CT, more difficult to perform in acute No collar sign for liver
traumatic setting Pleural Effusion
Other Modality Findings Subpulmonic or loculated
Bedside emergency ultrasonography can be safe and No abnormally positioned air-filled bowel
accurate Crus intact
Barium gastrointestinal findings Paraesophageal Hernia
o Historical method to demonstrate herniation Tear rare at esophageal hiatus
Subphrenic Abscess
Diaphragm intact, separate from bowel
 DIAPHRAGMATIC TEAR
Clinical presentation of chronic infection, not trauma o Strangulation of bowel
85% strangulation within 3 years; however, cases
have been undiagnosed for decades
I PATHOLOGY Morbidity and mortality strangulation 30%
o Obstructive symptoms, fever, chest pain
General Features
General path comments Natural History & Prognosis
2 o Kinetic energy absorption does not respect anatomic
borders
Excellent
Morbidity and mortality higher with strangulation
32 Thus, blunt torso trauma frequently results in o New pleurai effusion in patient with hernia heralds
multiple simultaneous injuries above and below the onset of strangulation
the hemidiaphragm Treatment
o Spontaneous healing uncommon, herniated
Surgical repair
abdominal contents prevent approximation of edges
of tear
o Most commonly herniated organs are stomach,
colon, omentum, liver, and spleen
I DIAGNOSTIC CHECKLIST
Etiology: High-energy blunt torso trauma Consider
Epidemiology: Prevalence 5% blunt chest trauma Must have a high index of clinical suspicion
Associated abnormalities: Chest wall, pleural,
abdominal visceral, and vascular injuries Image Interpretation Pearls
Dependent viscera sign
Gross Pathologic & Surgical Features
Radial tear extending from central tendon
posterolaterally I SELECTED REFERENCES
> 2 em long, most more than 10 em long
CT diaphragmatic defects 5% 1. Nchimi A et al: Helical CT of blunt diaphragmatic rupture.
o Normal process of aging A]RAm] Roentgenol. 184(1):24-30, 2005
2. Blaivas M et ai: Bedside emergency ultrasonographic
o More common in women diagnosis of diaphragmatic rupture in blunt abdominal
trauma. Am] Emerg Med. 22(7):601-4, 2004
3. lochum 5 et al: Imaging of diaphragmatic injury: a
ICLINICAl ISSUES diagnostic challenge? Radiographies. 22 Spec No:S103-16;
discussion S116-8, 2002
Presentation 4. Karmy-]ones R et al: The impact of positive pressure
Most common signs/symptoms: High-energy blunt ventilation on the diagnosis of traumatic diaphragmatic
torso injury with associated chest wall, pleural, injury. Am Surg. 68(2):167-72, 2002
vascular, and visceral injuries 5. Bergin D et al: The "dependent viscera" sign in CT
diagnosis of blunt traumatic diaphragmatic rupture. A]R
Other signs/symptoms Am] Roentgenol. 177(5):1137-40,2001
o Thoracic splenosis can rarely occur years after an 6. Hagman TF et al: Intrathoracic splenosis: superiority of
injury technetium Tc 99m heat-damaged RBCimaging. Chest.
o Rupture with intrapericardial herniation occurs 120(6):2097-8,2001
rarely 7. Nau T et al: The diagnostic dilemma of traumatic rupture
Acute of the diaphragm. Surg Endosc. 15(9):992-6, 2001
o Multiple associated injuries 8. Shanmuganathan K et al: Imaging of diaphragmatic
Rib fractures 40% injuries.] Thorac Imaging. 15(2):104-11,2000
9. Killeen KLet al: Helical CT of diaphragmatic rupture
Pelvic fractures 50% caused by blunt trauma. AJRAm J Roentgenol.
Liver or spleen laceration very common 173(6):1611-6, 1999
Aortic tear in 5% 10. Sadeghi N et al: Right diaphragmatic rupture and hepatic
Head injury commonly associated hernia: an indirect sign on computed tomography. Eur
o Diagnosis delayed 25% Radiol. 9(5):972-4, 1999
Often other more compelling injuries such as 11. Israel RSet al: Diaphragmatic rupture: use of helical CT
aortic iaceration scanning with multiplanar reformations. A]RAm J
o Intubated patient on positive pressure may prevent Roentgenol. 167(5):1201-3, 1996
12. Shapiro MJ et al: The unreliability of CT scans and initial
herniation chest radiographs in evaluating blunt trauma induced
o Herniation may be obscured by other injuries diaphragmatic rupture. Clin Radiol. 51(1):27-30,1996
Latent 13. Guth AAet al: Pitfalls in the diagnosis of blunt
o Asymptomatic or mild epigastric discomfort diaphragmatic injury. Am J Surg. 170(1):5-9, 1995
o Spontaneous respiration (negative intrapleural 14. Gelman R et al: Diaphragmatic rupture due to blunt
pressure) trauma: sensitivity of plain chest radiographs. AJRAm J
Gradient for progressive herniation of abdominal Roentgenol. 156(1):51-7, 1991
contents
High index of suspicion important throughout the
hospital course of trauma patients
Obstructive
 DIAPHRAGMATIC TEAR

I IMAGE GALLERY
Typical
(Left) Frontal radiograph

" shows apparent elevation of

. . "..'. e . .. '~ left hemidiaphragm after

high,energy blunt torso
2
' ~.""':~-:"' ~ " trauma strongly suggestive of

,
ruptured hemidiaphragm. 33

&~ '~~ ... x (Right) Axial CECT shows

, '.
..
constrictive narrowing of the
stomach as it passes through

,
~.r defect in left
hemidiaphragm. Note the
' .. . jJ.''. 't.
.. , dependent viscera sign with
stomach abutting the
"~ .. ~, ..',"~~ . posterior ribs (arrow).

'-- ' .. 'I'

Typical
(Left) Frontal radiograph
shows irregular contour of
left hemidiaphragm after a
motor vehicle crash
suggestive of tear with
herniation of abdominal
viscera. (Right) Axial CECT
shows corresponding
herniation of stomach and
omentum (arrows).

(Left) Frontal radiograph

shows dilated gastric bubble
above expected position of
left hemidiaphragm. Also
note wide mediastinum
suggesting vascular injury.
(Right) Axial CTA shows a
typical traumatic aortic
pseudoaneurysm (arrow) as
a common associated injury.
 ESOPHAGEAL TEAR

2
34

Graphic shows classic locaUon of esophageal tear in Esophagram shows spontaneous perforation of the
Boerhaave syndrome. Small transverse laceraUon of the esophagus (Boerhaave syndrome) with a massive leak
left-lateral posterior wall of the distal esophagus farrow) of barium to the mediastinum. Tear in the typical left
]-] em proximal to gastroesophageal fGE) junction. posterolateral distal wall just prior to the GEjunction.

o Areas of anatomic narrowing

ITERMINOLOGY Site of extrinsic compression by aortic arch of left
Abbreviations and Synonyms main bronchus
Esophageal laceration; esophageal perforation o Post-surgical
At or above benign or malignant strictures; with
Definitions biopsies or dilatation procedures
Transmural esophageal tear Anastomotic sites after esophageal surgery
Boerhaave syndrome: Rupture of esophagus after Morphology: Tear usually linear
forceful emesis
Radiographic Findings
Mallory-Weiss: Partial thickness tear after forceful
Radiography
emesis
o May be completely normal (10%), early
o Diagnosis suspected in less than 20'M,
o Air
IIMAGING FINDINGS Subcutaneous emphysema in the neck and upper
General Features thorax (60%)
Best diagnostic clue Pneumomediastinum, especially in the left
o Diagnosis depends on high degree of suspicion and costovertebral angle (V-sign of Naclerio) (25%)
recognition of clinical features Occasional pneumothorax (always associated with
o Pneumomediastinum in the left costovertebral angle pleural fluid - hydropneumothorax)
Location o Mediastinal widening
o Boerhaave syndrome or blunt chest trauma o Lung
Left lateral wall of distal esophagus 2 to 3 cm Consolidation or atelectasis of the lung adjacent
above gastroesophageal (GE) junction to tear (80%)

DDx: Esophageal Fistulas

Esophagitis Esophageal Cancer Hodgkin Disease

 ESOPHAGEAL TEAR

Key Facts
Terminology Esophageal Fistula
Esophageal laceration; esophageal perforation Clinical Issues
Transmural esophageal tear Sudden onset excruciating substernal or lower
Imaging Findings thoracic chest pain
May be completely normal (10%), early Clinical diagnosis confused with acute myocardial
infarction, aortic dissection, perforated peptic ulcer
2
Pneumomediastinum, especially in the left
costovertebral angle (V-sign of Naclerio) (25%) Prognosis directly related to interval between 35
Pneumomediastinum, pleural effusion, and opacified perforation and initiation of treatment
lung usually occur together and although nonspecific Diagnostic Checklist
should raise suspicion for esophageal tear Esophageal tear often overlooked, must have high
Top Differential Diagnoses index of suspicion
Pneumomediastinum may be subtle, localization
Hiatal Hernia
around esophagus (CT) or in left costovertebral angle
Diaphragmatic Rupture
(chest radiography) should raise suspicion for
Paraesophageal Hernia
esophageal tear
Epiphrenic Diverticulum

In Boerhaave syndrome or blunt chest trauma: oCT

Medial basal segment left lower lobe More sensitive in detecting mediastinal
o Hydropneumothorax (90%) complications
Tear mid or upper esophagus mainly right Protocol advice
hemithorax (5%) o Esophagography
Tear distal esophagus mainly left hemithorax Start with non-ionic water soluble contrast
(75%) If no leak, follow with barium
o Abdomen Water-soluble contrast agent may fail to detect
Pneumoperitoneum occasional with distal tears ] 5-25% of esophageal tears
o Pneumomediastinum, pleural effusion, and Barium may detect small leak not visible initially
opacified lung usually occur together and although
nonspecific should raise suspicion for esophageal
tear I DIFFERENTIAL DIAGNOSIS
Due to caustic nature of gastric and ingested fluid;
signs evolve rapidly within hours of perforation Hiatal Hernia
Pneumomediastinum absent
CT Findings Extravasation contrast absent
NECT
o Useful to evaluate esophageal and mediastinal Diaphragmatic Rupture
complications Also occurs in setting of blunt chest trauma
Tiny gas collections centered on esophagus (90%) Usually left-sided
Extravasation of oral contrast Stomach most common herniated organ
Esophageal thickening due to intramural Nasogastric (NG) tube may course through
hematoma or esophageal dissection esophagogastric hiatus normally and then extend into
Periesophageal fluid collections hemithorax with herniated stomach
Mediastinal abscess No extra-alimentary air or pneumomediastinum
Pleural effusion or hydropneumothorax, usually No pleural effusion unless bowel strangulated
small but with time enlarges Paraesophageal Hernia
Does not show the size of the tear
Organoaxial gastric torsion most common
o Protocol advice: Oral administration of non ionic
Double air-fluid level; above and below diaphragm
water-soluble agent to find small leaks
Usually no pleural effusion, unless bowel strangulated
Other Modality Findings
Epiphrenic Diverticulum
Esophagram findings
No free mediastinal gas or inflammation
o Procedure of choice to determine site and extent of
Extraluminal contrast, fluid + gas in mediastinal
tear
pocket may mimic diverticulum or even stomach
o Possible perforation should be evaluated with
non ionic contrast Esophageal Fistula
o Gastrografin should be avoided because of the risks Usually secondary to erosion from esophageal
of aspiration carcinoma
Imaging Recommendations Fistula may develop into trachea, mediastinum, aorta,
pleura
Best imaging tool
 ESOPHAGEAL TEAR
Classically vomiting but also straining during
I PATHOLOGY weight lifting, childbirth, severe coughing
General Features Other signs/symptoms
o After instrumentation
General path comments
o Weakest part of esophageal wall: Left posterolateral May not have symptoms initially and present
wall just above gastroesophageal hiatus several hours after endoscopy

2 Thinnest musculature, lack of adjacent supporting

structures
Demographics
Gender: Boerhaave syndrome more common in males
Anterior angulation at diaphragmatic crus
36 Natural History & Prognosis
Etiology
o Penetrating trauma (90%) Most serious and rapidly fatal type of perforation in
Iatrogenic following endoscopic procedures: Most gastrointestinal tract
common cause of esophageal tear Life-threatening; associated with high morbidity and
Post-surgical without intervention, high mortality
Ingested foreign bodies (chicken and meat bones) o Mortality 30-50%
o Spontaneous (15%) o Delayed> 90%
Boerhaave syndrome: Increased intraluminal Prognosis directly related to interval between
pressure due to retching against a closed glottis perforation and initiation of treatment
Child abuse, in young children o After 24 hours; 70% mortality rate
o Blunt chest trauma (10%) o Untreated perforation; mortality rate nearly 100%
Increased intraluminal pressure due to due to fulminant mediastinitis
compression of esophagus against a closed glottis
o Neoplasms: Esophageal carcinoma; lymphoma, rare Treatment
Epidemiology Drainage, antibiotics, surgical closure
o Esophagoscopy (60% of ruptures) Conservative
1.7% incidence during procedure o For small tears especially in cervical esophagus
More common in those with pre-existing disease: o NG tube drainage, antibiotics, parenteral fluids
Tumors, achalasia, strictures, or surgical Surgical
anastomosis o For large tears ideally performed within first 24
o Boerhaave syndrome: Incidence 1 in 6,000 (15% of hours
ruptures) o Thoracotomy and primary closure, mediastinal
o < 1% in blunt chest trauma (10% of ruptures) drainage
o Pneumatic dilatation for achalasia: 2-6% o For well-defined abscess or mediastinal
Associated abnormalities: From blunt or penetrating fluid-collection: Percutaneous drainage and then
trauma other injuries common: Aortic transection, primary closure
bronchial fracture, spinal trauma Esophageal stents
o Bridge the esophageal tear
Gross Pathologic & Surgical Features o May be useful in delayed diagnosis prior to
Full-thickness linear tear of left lateral wall of distal definitive repair
esophagus just above GE junction
Mallory-Weiss: Irregular linear tear or laceration in
long axis of esophagus near GE junction involving I DIAGNOSTIC CHECKLIST
mucosa only, does not penetrate the wall
Consider
Esophageal tear often overlooked, must have high
ICLINICAL ISSUES index of suspicion

Presentation Image Interpretation Pearls

Most common signs/symptoms Pneumomediastinum may be subtle, localization
o Sudden onset excruciating substernal or lower around esophagus (CT) or in left costovertebral angle
thoracic chest pain (chest radiography) should raise suspicion for
Clinical diagnosis confused with acute myocardial esophageal tear
infarction, aortic dissection, perforated peptic CT does not necessarily substitute for simple
ulcer esophagram
o Dysphagia, hemoptysis or hematemesis (50%)
o Subcutaneous crepitus neck (subcutaneous
emphysema) I SELECTED REFERENCES
o Mackler triad: Vomiting, lower thoracic pain, and 1. Fadoo FD et al: Helical CT esophagography for the
subcutaneous emphysema evaluation of suspected esophageal perforation or rupture.
o Hamman sign: Crunching sound on auscultation AJR Am J Roentgenol. 182: 1177 -1179, 2004
(20%) 2. Gimenez A et al: Thoracic complications of esophageal
o Boerhaave syndrome typically occurs after drinking disorders. RadioGraphies. 22 Spec No:S247-2S8, 2002
and eating binge
 ESOPHAGEAL TEAR

I IMAGE GALLERY
Typical
(Left) Axial NECT shows an
esophageal carcinoma
(white arrow). Mediastinal
air bubbles and a small
amount of contrast material
2
due to formation of a fiswla 37
(black arrow) are also seen.
(Right) Axial NEeT in a
patienl with esophageal
carcinoma shows a large
esophagopulmonary fiswla
(arrows) secondary to
radiation therapy.

(Left) Anleroposterior
radiograph shows
nonspecific features of
esophageal tear
Pneumomediastinum with
largest collection in the left
costovertebral angle (open
arrow). Bibasilar opacities
and pleural effusions
(arrows). (Right) Axial CECT
shows extravasation of air
and contrast surrounding
esophagus (arrows). Chicken
bone (open arrow) lodged in
distal esophagus. Moderate

t t bilateral pleural effusions

(curved arrows).

Typical
fLeft) Anteroposterior
radiograph shows air
collection in lower left
hemithorax (arrows) thought
to be herniated stomach
following blunt chest trauma.
Lower lobes are opacified
(open arrows). (Right)
Esophagram shows NC wbe
in stomach. Air collection
now contains contrast
(arrow). Second focus of
contrast extravasation in the
right costovertebral junction
from ruptured esophagus
(open arrow). CT was
interpreted as diaphragmatic
rupture.
 SPLENOSIS

2
38

Typical features of splenosis. Multiple small pleural Axial NEeT shows mulUple pleural masses both within
implants in the lower lelt hemithorax farrows). the hemithorax (arrows) and abdomen (curved arrow).
Diaphragmatic injury (open arrow). Spleen absent. Old rib fracture (open arrow).

Indistinct margin en face

ITERMINOLOGY Different radiographic characteristics in
Definitions orthogonal views
Autotransplantation of splenic tissue following Homogeneous
traumatic or surgical disruption of the spleen Convex to lung
Ectopic splenic tissue Obtuse angle to chest wall
a Accessory spleens (splenunculi) Consecutive examinations may have different
a Splenosis radiographic characteristics due to slight changes
in obliquity (changing tangential margins)
a Splenosis
I IMAGING FINDINGS Single or multiple pleural masses in lower
posterior left hemithorax
General Features Most < 3 cm in diameter with sharp, smooth
Best diagnostic clue: Multiple pleural masses in left contours
hemithorax in patient with history of remote trauma Associated with rib fractures
Location: Inferior posterior left hemithorax Absent splenic imprint on stomach bubble
Size Bullet fragments or shrapnel important clues
a Multiple pleural masses < 3 cm in diameter CT Findings
May be up to 6 cm in diameter
CECT
Radiographic Findings a Multiple pleural masses or pleural thickening in the
Radiography left hemithorax
a Typical radiographic features of pleural mass Located anywhere within the pleural space
Sharp margin in profile including the mediastinal pleura

DDx: Multiple Pleural Masses

Metastatic Adenocarcinoma Invasive Thymoma Asbestos Exposure

 SPLENOSIS
Key Facts
Terminology Pathology
Autotransplantation of splenic tissue following Splenic implants parasitizes blood supply from
traumatic or surgical disruption of the spleen adjacent serosal surface
Ectopic splenic tissue functional
Imaging Findings Sepsis may be less common in those with splenosis
Best diagnostic clue: Multiple pleural masses in left Grow slowly with time 2
hemithorax in patient with history of remote trauma Epidemiology: May develop in up to 15% with
Location: Inferior posterior left hemithorax splenic trauma and diaphragmatic laceration 39
Multiple pleural masses < 3 cm in diameter
Clinical Issues
Top Differential Diagnoses Asymptomatic, incidentally discovered on chest
Pleural Metastases radiographs
Asbestos Pleural Plaques Interval between trauma and diagnosis averages
Invasive Thymoma nearly 20 years
Malignant Mesothelioma Surgery may lead to second splenectomy and loss of
Fibrin ball splenic function

Predominately clustered in the posterior inferior

hemithorax along the costophrenic sulcus Invasive Thymoma
Noncalcified Anterior mediastinal mass (may be small)
Pleural masses may enhance with contrast Pleural masses represent drop metastases
Similar masses may be located in subcutaneous May be unilateral or bilateral
tissue and abdominal cavity Malignant Mesothelioma
o Spleen absent
Diffuse circumferential pleural thickening
o May have other evidence of trauma
Often cicatricially shrinks the involved hemithorax
Healed rib fractures Often associated with chest wall pain
MR Findings Usually unilateral
Signal intensity on both T1 and T2 weighted images Fibrous Tumor of Pleura
comparable to spleen
Large solitary tumors inhomogeneous, often show
Nuclear Medicine Findings contrast-enhancement
Tc-99m Sulfur Colloid May be associated with hypoglycemia or hypertrophic
o Uptake in splenic tissue (specifically osteoarthropathy
reticuloendothelial tissue) Multiple Pulmonary Nodules
Occasional false-negative Pleural nodules mimic true intrapulmonary nodules
Tc-99m Labeled Red Cell Scintigraphy
CT useful for separating pleural from parenchymal
o Uptake specific for splenic tissue
abnormalities
o More sensitive than Tc-99m sulfur colloid scans
Mediastinal Mass
Imaging Recommendations
Pleural masses adjacent to the mediastinum mimic
Best imaging tool: CT procedure of choice to evaluate anterior, mediastinal, or posterior mediastinal masses
and characterize intrapleural abnormalities
CT useful for separation of pleural from mediastinal
disease
I DIFFERENTIAL DIAGNOSIS Mobile Pleural Masses
Lipoma
Pleural Metastases o Oval or lenticular shaped, fat density on computed
Multiple pleural masses or diffuse pleural thickening tomography
o Usually unilateral o May grow slowly
Often associated with pleural effusions o Large lesions may be pedunculated and change
Usually history of known malignancy, especially location with positional maneuvers
adenocarcinoma o Smaller lesions may change shape with respiration
Asbestos Pleural Plaques (lipomas are soft)
Bilateral discrete flattened pleural masses Foreign bodies
o When unilateral, more commonly left-sided only o History of trauma
o Foreign bodies (such as bullets) usually radio-opaque
Plaques usually calcified
Fibrous tumor of pleura
History of asbestos exposure
o Usually pedunculated, change location with
positional maneuvers
 SPLENOSIS
Fibrin ball Surgery may lead to second splenectomy and loss of
o End result of exudative effusions splenic function
o Usually only a few mm in size, rarely detected
radiographically
I DIAGNOSTIC CHECKLIST
I PATHOLOGY Consider
2 General Features
Splenosis for multiple
hemithorax
pleural masses in the left

40 General path comments

o Splenic implants parasitizes blood supply from
Image Interpretation Pearls
adjacent serosal surface Evaluate the spleen in any patient with multiple
Visceral or parietal pleura pleural masses
o Implants are sessile or pedunculated
o Ectopic splenic tissue functional
Sepsis may be less common in those with I SELECTED REFERENCES
splenosis 1. Alaraj AM et al: Thoracic splenosis mimicking thoracic
o Grow slowly with time schwan noma: case report and review of the literature. Surg
Also reported to regress Neurol. 64(2): 185-8; discussion 88, 2005
Etiology 2. Khosravi MR et al: Consider the diagnosis of splenosis for
soft tissue masses long after any splenic injury. Am Surg.
o Follows penetrating or less commonly blunt
70(11):967-70,2004
traumatic injury 3. Young JT et al: Splenosis: a remote consequence of
o Splenic tissue could gain access across normal traumatic splenectomy. J Am Coli Surg. 199(3):500-1,2004
diaphragmatic hiatus or congenital defects 4. Bizekis C5 et al: Thoracic splenosis: mimicry of a
Epidemiology: May develop in up to IS% with splenic neurogenic tumor. J Thorac Cardiovasc 5urg.
trauma and diaphragmatic laceration 125(5):1155-6,2003
5. Horger M et al: Improved detection of splenosis in patients
Gross Pathologic & Surgical Features with haematological disorders: the role of combined
Normal splenic tissue containing red and white pulp transmission-emission tomography. Eur J Nucl Med Mol
Implants can be found in peritoneum, omentum, Imaging. 30(2):316-9, 2003
serosal surfaces of bowel and liver, pelvic organs and 6. Yammine IN et al: Radionuclide imaging in thoracic
splenosis and a review of the literature. Clin Nucl Med.
subcutaneous tissue
28(2):121-3,2003
Diaphragmatic tears usually not found at surgery 7. Pekkafali Z et al: Intrahepatic splenosis: a case report. Eur
Rare in lung, requires lung laceration Radiol. 12 5uppl 3(562-5, 2002
8. Castellani M et al: Tc-99m sulphur colloid scintigraphy in
the assessment of residual splenic tissue after splenectomy.
I CLINICAL ISSUES Clin Radiol. 56(7):596-8, 2001
9. Hagman TF et al: Intrathoracic splenosis: superiority of
Presentation technetium Tc 99m heat-damaged RBC imaging. Chest.
Most common signs/symptoms 120(6):2097-8,2001
10. 5arda R et al: Pulmonary parenchymal splenosis. Diagn
o Asymptomatic, incidentally discovered on chest
Cytopathol. 24(5):352-5, 2001
radiographs 11. 5yed 5 et al: Thoracic splenosis diagnosed by fine-needle
o History of traumatic injury aspiration cytology: a case report. Diagn Cytopathol.
Other signs/symptoms 25(5):321-4,2001
o Red blood cell Howell-Jolly bodies may be present 12. Fidvi SA et al: Posttraumatic thoracic splenosis and chronic
suggesting that the splenic tissue may not be aortic pseudoaneurysm. J Thorac Imaging. 14(4):300-2,
functional 1999
o Fine needle biopsy and cytology can be used for 13. Naylor MF et al: Noninvasive methods of diagnosing
thoracic splenosis. Ann Thorac Surg. 68(1):243-4,1999
problematic cases
14. O'Connor JV et al: Thoracic splenosis. Ann Thorac 5urg.
Demographics 66(2):552-3, 1998
IS. Tsunezuka Y et al: Thoracic splenosis; from a thoracoscopic
Age: Any age viewpoint. Eur J Cardiothorac Surg. 13(1):104-6, 1998
Natural History & Prognosis 16. Bordlee RP et al: Thoracic splenosis: MR demonstration. J
Thorac Imaging. 10(2):146-9, 1995
Interval between splenic rupture and splenosis varies 17. Madjar S et al: Thoracic splenosis. Thorax. 49(10):1020-2,
from months to years 1994
o Mean 7 years 18. Normand JP et al: Thoracic splenosis after blunt trauma:
Interval between trauma and diagnosis averages nearly frequency and imaging findings. AJR Am J Roentgenol.
20 years 161(4):739-41,1993
19. Carr NJ et al: The histological features of splenosis.
Treatment Histopathology. 21(6):549-53,1992
None, must be differentiated from more sinister 20. Roucos 5 et al: Thoracic splenosis. Case report and
process literature review. J Thorac Cardiovasc 5urg. 99(2):361-3,
1990
 SPLENOSIS

I IMAGE GALLERY
Typical
(Left) Frontal radiograph
shows nodule in the left
costophrenic angle (curved
arrow). Splenic imprint on
stomach absent (open
arrow). (Right) Axial CECT
shows multiple pleural-based
masses in the lower left
hemithorax, some of which
enhance with contrast
administration (arrows).
Splenosis.

Typical
(Left) Axial CECT multiple
pleural masses in the
posterior pleural space
(arrows). History of
traumatic injury 15 years
ago. (Right) Axial liver spleen
scan shows uptake in the
posterior left hemithorax
(arrows), corresponding to
the pleural masses in the
pleural space. Spleen also
absenl.

Typical
(Left) Axial CECT shows
absent spleen. Splenic
implant also evident in the
posterior chest wall (arrows).
(Right) Axial NECT shows
large anterior mediastinal or
pleural mass in the left
hemithorax (arrow). Splenic
implants may occur
anywhere in the pleural
space.
 AIR EMBOLUS, PULMONARY

2
42

Graphic shows venous air bubbles passing through a Frontal radiograph shows air in left jugular vein (arrow)
patent foramen ovale (arrow), resulting in systemic and main pulmonary artery (open arrow). Venous air
arterial air embolism. Patent foramen ovale is present in embolism.
up to 75% ofindividuals.

Size: Amount of air varies by the rate and amount of

I TERMI NOlOGY
air emboli zed
Abbreviations and Synonyms Morphology: Pulmonary edema that has a basilar
Venous air embolism predominance
Non-thrombotic pulmonary embolism Radiographic Findings
Systemic arterial air embolism Radiography
Definitions o Venous air embolism
Entry of air into the venous system Radiographs may be normal
Venous air embolism: Entry of air via systemic veins Air in axillary, subclavian, innominate, hepatic,
Systemic arterial air embolism: Entry of air usually via jugular veins or cavernous sinus
pulmonary veins Bell-shaped air in main pulmonary artery
Focal pulmonary oligemia (Westermark sign)
Subsegmental atelectasis
IIMAGING FINDINGS Pulmonary edema: Interstitial edema
Followed by bilateral peripheral alveolar opacities
General Features Basilar predominance
Best diagnostic clue Enlargement of the central pulmonary arteries or
o Venous air embolism: CT demonstration of air in superior vena cava
right heart, systemic veins, cavernous sinus No cardiomegaly
o Systemic arterial embolism: CT demonstration of air o Systemic air embolism
in left heart, aorta, cerebral arteries Air in left atrium, left ventricle, aorta, cerebral
Location: Heart, lung, brain arteries

DDx: Mediastinal Air

Pneumomediastinum Mediastinitis Pneumopericardium

 AIR EMBOLUS, PULMONARY

Key Facts
Terminology Pathology
Venous air embolism: Entry of air via systemic veins Iatrogenic causes of venous air embolism
Systemic arterial air embolism: Entry of air usually via Manipulation or placement of subclavian or central
pulmonary veins venous catheters
Air inadvertently injected with CT power injector,
Imaging Findings common 2
Venous air embolism: CT demonstration of air in Iatrogenic causes of systemic artery air embolism
right heart, systemic veins, cavernous sinus Transthoracic needle aspiration and biopsy 43
Systemic arterial embolism: CT demonstration of air
in left heart, aorta, cerebral arteries Clinical Issues
Radiographs may be normal Treatment: 100% inspired oxygen
To trap air in right heart heart and prevent
Top Differential Diagnoses embolization to lungs
Pneumomediastinum Left lateral decubitus position
Mediastinitis Trendelenburg position
Pneumopericardium

CT Findings I DIFFERENTIAL DIAGNOSIS

CECT Pneumomediastinum
o More sensitive than chest radiography
o Venous air emboli in 11% of CECT studies Air tracks in mediastinal fat, subcutaneous
Inadequate flushing of line prior to injection emphysema
Small emboli (3 air bubbles < 1 cm, 10%); Air not intravascular
microbubbles usually inconsequential Mediastinitis
Moderate emboli in 1% Air tracks in mediastinal fat, subcutaneous
o Venous air emboli in emphysema
Axillary, subclavian, internal jugular, Mediastinal abscesses
brachiocephalic veins Sepsis, fever, leukocytosis
Superior vena cava
Right heart: Right ventricle or atrium Pneumopericardium
Main pulmonary artery Confined to pericardia! recesses
o Multiple locations, uncommon
o Pulmonary edema in unobstructed areas of Pulmonary Embolism
pulmonary circulation Occurs in hospitalized, post-operative patients
o Focal oligemia, more frequent in upper lobes Radiographs show similar findings
Not as low density as air
Non-Vascular Interventions Deep venous thrombosis suggests thrombotic
Transthoracic needle biopsy (co-axial technique) pulmonary embolism
o Instruct patient to stop breathing when the stylet is
pulled from the guide needle Fat Embolism
Inhalation without the stylet may predispose Also seen following orthopedic surgery
patient to air embolism Radiographs show similar findings
Needle tip may be located in a pulmonary vein No demonstration of air in vessels
Pressure gradient favors air to flow into the vein Fat in urine, conjunctivae, retinal vessels
causing systemic air embolism
Ultrasonographic Findings I PATHOLOGY
Transthoracic or transesophageal echocardiography
o Air bubbles observed in right ventricular outflow General Features
tract or pulmonary veins Etiology
o Air enters low pressure venous system
Imaging Recommendations Pressure gradients favoring air embolus: Positive
Best imaging tool: CT demonstration of nondependent extravascular pressure and negative intravascular
air bubbles in intrathoracic veins, heart or arteries pressure
Traumatic or procedural communication with the
venous system
Venous air embolism, to right heart and lungs
Right to left shunt, air may embolize from
systemic veins to systemic arteries
 AIR EMBOLUS, PULMONARY
Patent foramen ova Ie, approximately 15% o Pulmonary injury
Systemic artery air embolism, from pulmonary o Right ventricular dysfunction
veins to systemic arteries, rare o Right ventricular outflow obstruction
o Iatrogenic causes of venous air embolism o Increased central venous pressure
Frequent during neurosurgery with patient in o Increased pulmonary artery pressure
sitting position o Drum-like or "mill wheel" cardiac murmur
Neck or craniofacial injury o Cardiogenic shock
2 Orthopedic or cardiac surgery o Cyanosis
Manipulation or placement of subclavian or o Circulatory arrest
44 central venous catheters
Hemodialysis catheters
Demographics
Air inadvertently injected with CT power injector, Age: Neonates to elderly
common Gender: M = F
o Iatrogenic causes of systemic artery air embolism Natural History & Prognosis
Transthoracic needle aspiration and biopsy
Risk of death
Barotrauma from positive pressure ventilation o Affected by amount of air and speed of introduction
Asthma o Reports of death with injection of 100 mL air
Neonates with respiratory distress Minimum lethal injection volume, 300-500 mL
Cardiac bypass surgery, mismanagement of line Minimum lethal injection rate, 100 mL/sec
flushing In patients who survive, opacities disappear rapidly
o Venous or systemic arterial air embolism
Open or closed chest trauma Treatment
Scuba divers: Decompression injury, gas bubble Clam p suspect central line
formation in the blood Restore cardiopulmonary circulation
Scuba divers: Due to rapid reduction in the Treatment: 100% inspired oxygen
ambient pressure o To decrease partial pressure of nitrogen in the air
Scuba divers: Occurs during diver's ascent bubbles
Scuba divers: Embolization to lungs and systemic Venous air embolism
arteries o To trap air in right heart and prevent embolization
Epidemiology to lungs
o True incidence, unknown o Left lateral decubitus position
o Subclinical air embolism in hospitalized patients, o Trendelenburg position
may be common Hyperbaric oxygen therapy
o Venous air embolism after central venous line Intraoperative needle aspiration
placement, < 2%
o Venous air embolism with CT studies,
approximately 11% I DIAGNOSTIC CHECKLIST
Gross Pathologic & Surgical Features Consider
Mechanical obstruction of pulmonary vasculature Air embolism during transthoracic lung biopsy if the
o Pre- and post-capillary pulmonary vasoconstriction patient starts to seize or gets very agitated
o Pulmonary hypertension
Turbulent blood flow, platelet aggregation, fibrin Image Interpretation Pearls
formation During lung biopsy, if air embolism is suspected, do a
Liberation of oxygen radicals from neutrophils CT of the head to look for air bubbles in intracranial
Disruption of capillary endothelium arteries
Macromolecules, proteins, blood cells enter interstitial
and alveolar spaces
Microscopic Features
I SELECTED REFERENCES
I. Imai 5, et al: Iatrogenic venous air embolism caused by CT
Mild interstitial edema, hemorrhagic airspace
injector-from a risk management point of view. Radiat
consolidation Med. 22(4):269-71, 2004
2. Han D et al: Thrombotic and non thrombotic pulmonary
arterial embolism: spectrum of imaging findings.
ICLlNICAllSSUES Radiographies. 23(6):1521-39, 2003
3. Aurora T et al: Iatrogenic venous air embolism. J Emerg
Presentation Med, 18:255-256,2000
Most common signs/symptoms 4. Rossi SE et ai: Nonthrombotic pulmonary emboli. AJR Am J
o Sudden onset dyspnea, chest pain, tachypnea Roentgenoi. 174(6):1499-508,2000
o Tachycardia, hypotension 5. Kodama F, et ai. Fatal air embolism as a complication of
CT-guided needle biopsy of the iung. J Comput
o Agitation
AssistTomogr, 23:949-951,1999
o Disorientation
o Seizures, coma
Other signs/symptoms
 AIR EMBOLUS, PULMONARY

IIMAGE GALLERY
Typical
(Left) Frontal radiograph in a
patient with tachypnea and
chest pain due to venous air
embolism shows mild basilar
opacification (arrows).
2
Patient recovered without
45
consequence. (Right) Axial
CECT shows air collections
in main pulmonary artery
(arrows) as a result of entry
of air into a systemic vein.
Diagnosis: Venous air
embolism

(Left) Axial CECT shows air

in non-dependent right
ventricle (arrow) as a result
of venous air embolism.
(Right) Frontal radiograph
shows air in main pulmonary
artery (arrow) and bibasilar
lower lobe pulmonary
edema (open arrows).
Diagnosis: Venous air
embolism.

(Left) Frontal radiograph

shows air in left ventricle
(open arrow), aorta (curved
arrow), and axillary arteries
(black arrows). Air may have
dissected into pulmonary
veins adjacent to cavities
with mycetomas (white
arrows) during positive
pressure ventilation. (Right)
Axial NECT shows air
bubbles in middle cerebral
artery branches (arrows).
This patient developed
seizures during transthoracic
fine needle aspiration lung
biopsy. Diagnosis: Systemic
arterial air embolism.
 THORACIC DUCT TEAR

2
46

Coronal graphic shows a thoracic duct tear (insert) with Coned down radiograph from a Iymphangiogram
chylous fluid filling the right pleural space. The right side shows the normal course of the thoracic duct.
is the most common location of a thoracic ducl tear.

o Location of effusion depends on anatomic level of

I TERMINOlOGY thoracic duct tear
Abbreviations and Synonyms o Right
Traumatic chylothorax Most common
Occurs when duct is torn between its
Definitions diaphragmatic entry site at the aortic hiatus to
Disruption of the thoracic duct or its tributaries where it veers left around the level of the fifth
Chylothorax thoracic vertebrae after traveling superiorly in the
o The presence of chylus (lymph of intestinal origin) posterior mediastinum along the right side of the
in the pleural space vertebral column
o Confirmed by pleurai fluid triglyceride levels> 110 o Left
mg/dl Duct damage around the area of the aortic arch
o Can also be confirmed by the presence of o Bilateral
chylomicrons Duct is damaged as it travels from left to right at
the level of the fifth thoracic vertebrae
Size
IIMAGING FINDINGS o Size of effusion varies
Tends to be large
General Features Morphology
Best diagnostic clue o Chylous fluid is usually indistinguishable from
o Chylous effusion on thoracentesis pleural effusions on standard imaging exams
o Recent history of surgery/trauma Exhibits same imaging characteristics as pleural
Location fluid
Blunts the lateral costophrenic angles

DDx: Pleural Fluid

Chylous Effusion Malignant Effusion

 THORACIC DUCT TEAR

Key Facts
Terminology Pathology
Traumatic chylothorax Thoracic duct injury causes 25% of all cases of
Disruption of the thoracic duct or its tributaries chylothorax
Rupture of duct secondary to obstruction also
Imaging Findings possible
Chylous effusion on thoracentesis Whitish, creamy appearance of fluid 2
Recent history of surgery/trauma Fluid with a triglyceride level of> 110 mg/dL
Chylous fluid is usually indistinguishable from 47
Presence of chylomicrons in fluid
pleural effusions on standard imaging exams
Contrast-enhanced CT may be helpful in determining Clinical Issues
density of fluid Dyspnea is common with large chylous effusions
Conservative management: Drainage & dietary
Top Differential Diagnoses restriction (low-fat diet)
Lymphangioleiomyomatosis (LAM) High output leak lasting greater than 7 days - surgery
Pleural Effusion (thoracic duct ligation or pleurodesis)
Pseudochylothorax

o Chylomas
Extremely rare
Pleural Effusion
Complication of penetrating or blunt trauma Indistinguishable from chylothorax on plain film
Also a complication of neck surgery - most radiographs
common in left neck (75%) Contrast-enhanced CT may help differentiate fluid
Sign of chylous fistula densities
Innumerable causes
Imaging Recommendations o Size variable
Best imaging tool
o Usually not necessary to identify the exact location Malignant Effusion
of the tear lymphangiography rarely performed Massive effusions
CT useful to delineate surrounding mass or o Most commonly related to malignancy
vertebral body injuries Chylothorax most commonly caused by malignancy
Lymphangiography rarely performed o Lymphoma
Protocol advice o Non-small cell lung carcinoma
o Contrast-enhanced CT may be helpful in o Breast carcinoma
determining density of fluid o GYN neoplasms: Ovarian, uterine, etc.
Often hyperdense in chylous effusions Mediastinal shift may be absent due to chest wall
Attenuation density of chylous fluid similar to fixation
pleural effusion Pseudochylothorax
Chylous effusions are rarely hypodense Occurs with long-standing fluid in a fibrotic pleura
o Due to high fat content, can show high intensity on
o Cholesterol pleurisy
T1 weighted MR
Clinically also produces triglyceride-rich milky pleural
effusion
Chylomicrons are absent
I DIFFERENTIAL DIAGNOSIS Cholesterol crystals often seen in chyliform fluid
Lymphangioleiomyomatosis (LAM) Lymphangiography rarely performed
Diffuse, cystic changes in the lung parenchyma
Occurs almost exclusively in women of reproductive
age I PATHOLOGY
Associated with pneumothorax General Features
Chylous effusion common as smooth muscle nodules
Etiology
invade lymphatics causing obstruction
o Thoracic duct injury causes 25% of all cases of
Tuberous Sclerosis chylothorax
Inheritable multiorgan hamartomatosis o Iatrogenic surgical injury is number one cause of
Pulmonary involvement uncommon thoracic duct tear
o Typically manifests as cysts or interstitial opacities o Complicates up to 4% of esophageal resections
on imaging o Reportedly seen with
Chylous effusion very rare - but reported Cardiac surgery
Pneumonectomy
Lung transplantation
 THORACIC DUCT TEAR
Spinal surgery o Some patients respond to conservative measures
Esophagoscopy Diet and tube drainage
o Nonsurgical trauma (penetrating trauma) and o Many patients require one or more surgeries to
Childbirth repair ductal lacerations
Emesis o Mortality rate of postsurgical thoracic duct tear may
Blunt trauma be up to 50% in cancer patients
Stretching (rare)
2 o Rupture of duct secondary to obstruction also Treatment
Conservative management: Drainage & dietary
possible
48 Epidemiology restriction (low-fat diet)
o Chyle leak secondary to surgical or non-surgical tear o Tube thoracostomy or pleuroperitonea I shunts may
in thoracic duct be used for drainage
o Rupture of duct following obstruction o Pleuroperitoneal shunts are more invasive but
Associated abnormalities reduce the risk of malnourishment
o Recent surgery/trauma High output leak lasting greater than 7 days - surgery
o Obstruction-related chylothorax (thoracic duct ligation or pleurodesis)
Malignancy (especially lymphoma) o Chylous output> 800 ml/day
Lymphangiomyomatosis Should be considered life-threatening if tube
thoracostomy used
Gross Pathologic & Surgical Features o Thoracotomy or thorascopic approach can be used
Whitish, creamy appearance of fluid o Site of leak occasionally identified during surgery
Following centrifugation supernatant is creamy white Pre-operative lymphangiogram can help localize
Fatty meal can increase chylous flow up to a factor of Pre-operative patient consumption of heavy cream
10 helps to intraoperatively visualize leak
o Allows for visualization of leak at time of surgery o Thoracic duct may be ligated slightly above the level
of the diaphragm or repaired at the site of leakage
Microscopic Features
Fluid with a triglyceride level of> 110 mg/dL
Presence of chylomicrons in fluid I SELECTED REFERENCES
Low serum albumin worrisome for malnourishment
1. Kumar S et al: Thoracoscopic management of thoracic duct
injury: Is there a place for conservatism? J Postgrad Med.
50(1):57-9,2004
ICLINICAllSSUES 2. Ziedalski TM et al: Chylothorax after heart/lung
transplantation. J Heart Lung Transplant. 23(5):627-31,
Presentation 2004
Most common signs/symptoms 3. Chinnock BF:Chylothorax: case report and review of the
o Dyspnea is common with large chylous effusions literature. J Emerg Med. 24(3):259-62, 2003
Due to lung compression 4. RyuJH et al: Chylothorax in lymphangioleiomyomatosis.
o Chyloma development Chest. 123(2):623-7,2003
5. Osman Ket al: A chylous rupture. J RSoc Med.
Dyspnea 95(12):616-7,2002
Chest pain 6. Patten RMet al: Isolated traumatic rupture of the cisterna
Tachycardia chyli: CT diagnosis. J Comput AssistTomogr. 23(5):701-2,
Can rupture into pleural space 1999
o Greater than one week of external drainage causes 7. Hillerdal G: Chylothorax and pseudochylothorax. Eur
hypoproteinemia, hyponatremia, and lymphopenia RespirJ. 10(5):1157-1162, 1997
Weakness 8. Sachs PBet al: Diagnosis and localization of laceration of
Dehydration the thoracic duct: usefulness of lymphangiography and CT.
AJRAmJ Roentgenol. 157(4):703-5,1991
Edema 9. De Hert S et al: Current management of traumatic
Emaciation chylothorax. Acta Anaesthesiol Belg. 39(2):101-7,1988
o Onset of symptoms is typically delayed due to time 10. Tocino 1Met al: Mediastinal trauma and other acute
necessary for chylus accumulation mediastinal conditions. J Thorac Imaging. 2(1):79-100,
Other signs/symptoms 1987
o Causes a significant loss of lymphocytes and 11. Ferguson MKet al: Current concepts in the management of
protein-rich fluid postoperative chylothorax. Ann Thorac Surg. 40(6):542-5,
Leads to immunosuppression 1985

Demographics
Age: Due to traumatic nature, can occur in any age
group
Gender: Due to traumatic nature, affects both genders
equally
Natural History & Prognosis
Variable course
 THORACIC DUCT TEAR

I IMAGE GALLERY
Typical
(Left) Normal appearance of
the thoracic duct.
Lymphogram shows the
termination of the thoracic 2
duct at the left
subclavian-jugular venous 49
anastomosis at the base of
the neck (arrow). (Right)
Axial CECT following
lymphangiography shows
normal position of the
thoracic duct (arrow).

(Left) Coned down

radiograph from a
Iymphangiogram shows a
thoracic duct tear with
extravasation of contrast
(arrow). (Right) Frontal
radiograph shows
embolization coils coursing
along left paramediastinum
(curved arrows) after repair
of a thoracic duct tear.

(Left) Lateral radiograph of

the previous patient shows
embolization coils in the
thoracic duct (curved
arrows) in a patient who has
undergone thoracic duct
repair. (Right) Axial CECT
shows bilateral pleural
effusions in a 36 year old
patient with LAM.
Thoracentesis revealed
chylous fluid. CT fluid
density nonspecific for chyle.
Non-Small Cell Lung Cancer IV-3-2
Small Cell Lung Cancer IV-3-6
Staging of Lung Cancer IV-3-10
Regional Lymph Node Classification IV-3-14
Solitary Pulmonary Nodule IV-3-16
Lung Cancer Screening IV-3-20
Missed Lung Cancer IV-3-24
 NON-SMALL CELL LUNG CANCER

3
2

Frontal radiograph shows right upper lobe collapse with Frontal radiograph shows large mass in right upper lobe
minor fissure outlining central mass (arrows), S-sign of (arrows) without rib destruction extending to the right
Colden. Cut off of right upper lobe bronchus (curved hilum; diagnosed as adenocarcinoma at biopsy.
arrow). Diagnosis: Squamous cell carcinoma.

o Squamous cell carcinoma: More frequently central

I TERMI NOLOGY lesion
Abbreviations and Synonyms Size
Non-small cell lung cancer (NSCLC), bronchogenic o Average size at detection approximately 2.5 cm by
carcinoma chest radiography
o Tumors detected incidentally and at screening
Definitions usually 8-15 mm by computed tomography
A group of primary lung neoplasms which have Morphology
similar staging system and therapy o Borders: Spiculated, lobular, smooth
o Includes: Adenocarcinoma (35-40%), squamous cell o Density: Solid, part solid part ground glass, pure
carcinoma (25-30%), large cell carcinoma (10-15%) ground glass
Cavitation more often associated with squamous
cell carcinoma than other histologies
IIMAGING FINDINGS Ground glass component associated with
adenocarcinoma and bronchioloalveolar
General Features carcinoma
Best diagnostic clue: Solitary pulmonary nodule or
mass, often spiculated with or without mediastinal Radiographic Findings
and hilar adenopathy Radiography
Location o Peripheral nodule or mass ranging in size from 1-10
o Upper lobes more common than lower lobes cm
o Adenocarcinoma: More frequently located in lung Nodules under 1 cm rarely detected by
periphery conventional radiography
o Central tumor with hilar or mediastinal enlargement

DDx: Non-Small Cell Lung Cancer

Atypical Mycobacteria Hamartoma Carcinoid Tumor

 NON-SMALL CELL LUNG CANCER

Key Facts
Imaging Findings 170,000 cases diagnosed annually; 150,000 deaths in
U.S.
Best diagnostic clue: Solitary pulmonary nodule or
Most common cause of cancer death in U.S.
mass, often spiculated with or without mediastinal
and hilar adenopathy Clinical Issues
Size used for probability of lymph node metastasis; > Cough, hemoptysis, dyspnea, fever, 10%
1 cm short axis considered abnormal asymptomatic
Top Differential Diagnoses Symptoms much more common with advanced
unresectable disease
Tuberculoma and Histoplasmoma
Majority of patients diagnosed with advanced stage
Carcinoid Tumor
disease
Hamartoma
Pathology
Diagnostic Checklist
If CT morphology is suspicious for cancer, biopsy is
3
General path comments: Sputum cytology helpful for
diagnosing central tumors; false negative rate 40% strongly suggested even with negative PET 3
Smoking> 85%

o Rib or spine destruction with chest wall invasion Best for detection of brachial plexus involvement by
superior sulcus tumors
CT Findings
NECT Nuclear Medicine Findings
o Usually adequate for evaluation of primary tumor PET
and mediastinal adenopathy o Activity greater than mediastinal background or
Advantage: No contrast reactions or standard uptake value (SUV) of> 2.5 associated with
nephrotoxicity, better characterization of adrenal greater chance of malignancy
nodule False positive: Infection, sarcoidosis
Disadvantage: Difficult to detect hilar lymph False negative: Lesions < 1 cm, low grade
nodes and invasion of vascular structures; adenocarcinomas, bronchioloalveolar carcinoma,
decreased sensitivity for liver lesions carcinoid tumor
o Peripheral lesions o Mediastinum: SUV > 2.5 or above background
Cavitation more frequent in squamous cell considered abnormal
histology Specificity 80%; positive result mllst be confirmed
Peripheral adenocarcinomas may be solid, mixed pathologically
solid and ground glass or pure ground glass o Avoids nontherapeutic thoracotomy in 20% by
density detection of distant disease
o Thin-section CT to delineate tumor features:
Density, margins, calcification, air bronchograms
Imaging Recommendations
and bronchiolograms Best imaging tool
o HRCT to detect lymphangitic carcinomatosis o Computed tomography and whole body PET
o Pulmonary metastases in non-tumor lobe, multiple MRI of brain when clinically appropriate
and bilateral Protocol advice
o Synchronous or metachronous primary neoplasm o CT should extend inferiorly through the adrenal
2%)
glands
o Screening studies should be performed without
CECT
o Central lesions contrast and lowest possible mA
Increased conspicuity for small endobronchial
lesions
Bronchial obstruction with collapse of lobe or I DIFFERENTIAL DIAGNOSIS
post-obstructive pneumonitis Tuberculoma and Histoplasmoma
o Mediastinal lymph nodes
May have central calcification and calcified hilar and
Size used for probability of lymph node
mediastinal lymph nodes
metastasis; > 1 cm short axis considered abnormal
Subcarinallymph nodes use size cut-off of 1.2 cm Carcinoid Tumor
short axis Located at bronchial branch points; small
o Identification of soft tissue pleural metastases in endobronchial component
pleural effusion
Hamartoma
MR Findings May contain fat and/or "popcorn" calcification
Rarely necessary; slightly more sensitive than CT for
chest wall invasion
 NON-SMALL
Pulmonary Infarct
Peripheral, wedge shaped; rapid decrease in size
I PATHOLOGY
General Features
General path comments: Sputum cytoiogy helpful for
diagnosing central tumors; false negative rate 40%
Genetics: Adenocarcinoma: 30% have mutation of ras
oncogene; associated with worse prognosis
Etiology
o Smoking> 85%
3 50% of lung cancers develop in former smokers
May be related to airflow and deposition of
4 particulate matter from smoking
o Environmental carcinogens: Asbestos, radon
Epidemiology
o 170,000 cases diagnosed annually; 150,000 deaths in
U.S.
o Most common cause of cancer death in U.S.
Associated abnormalities: Smoking related disease,
predominately chronic obstructive pulmonary disease
Gross Pathologic & Surgical Features
Adenocarcinoma: Peripheral nodule; may have
features of bronchioloalveolar cell carcinoma
Squamous cell carcinoma: Small endobronchial lesion;
peripheral tumors may be cavitary
Large cell carcinoma: Usually greater than 4 em at
time of diagnosis, more frequently peripheral
Microscopic Features
Adenocarcinoma
o Forms glands; mucus production frequent
Squamous cell carcinoma
o Cells have irregular nuclei and large nucleoli,
intercellular bridging, stain positive for keratin
Large cell carcinoma
o Difficult to identify; features of adenocarcinoma
squamous cell carcinoma absent
o May have neuroendocrine features
Staging, Grading or Classification Criteria
Mediastinal and hilar lymph nodes
o Hilar nodes (NI) usually found at vessel branch
points
o N2 lymph nodes include those ipsilateral to the
primary tumor and in the midline
o N3 lymph nodes include those contralateral to
primary tumor and supraclavicular fossa
I CLINICAL ISSUES
Presentation
Most common signs/symptoms
o Cough, hemoptysis, dyspnea, fever, 10%
asymptomatic
o Symptoms much more common with advanced
unresectable disease
Other signs/symptoms
o Chest pain and pleuritis from chest wall invasion
CELL LUNG CANCER
o Recurrent pneumonia in same lobe of lung
o Neuropathic pain from brachial plexus invasion:
Pancoast tumor
o Signs and symptoms of metastases: Bone pain,
pathologic fracture, neurologic symptoms
o Paraneoplastic syndromes: Hypercalcemia, clubbing
of finger nails, hypertrophic pulmonary
osteoarthropathy
Demographics
Age: Most frequently in people over 50
Gender
oM>F=2:1
Mortality rate higher in males; increasing
incidence and mortality in females
Ethnicity
o Second most common cancer in African-Americans
African-American males 50% more likely to
develop lung cancer than Caucasian males
Natural History & Prognosis
Majority of patients diagnosed with advanced stage
disease
Adenocarcinoma: Most common histology in non
smokers and women
o Stage for stage worse prognosis than squamous cell
carcinoma except for TINO lesions
Treatment
Stage I and 2: Surgery followed by adjuvant
chemotherapy in select cases
o Radiation therapy or radiofrequency ablation may
be considered for medically non-operable
Stage 3A: Neoadjuvant chemotherapy and radiation
therapy followed by surgery in select cases
Stage 3B: Chemotherapy and radiation therapy
o Surgery may be performed in select T4NO tumors
Stage 4: Chemotherapy with palliative radiation
therapy in select cases
o Solitary brain metastasis: Resection of brain lesion;
and followed by resection of primary tumor if possible
I DIAGNOSTIC CHECKLIST
Consider
NSCLC as most likely diagnosis in tobacco users with
focal lesion on chest radiograph
NSCLC as etiology for a non resolving pneumonia due
to bronchial obstruction
Image Interpretation Pearls
PET and CT are complimentary and thus should be
interpreted together
o If CT morphology is suspicious for cancer, biopsy is
strongly suggested even with negative PET
I SElECTED REFERENCES
1. Ravenel JG: Lung cancer staging. Semin Roentgenol.
39(3):373-85,2004
2. Vansteenkiste Jet al: Positron-emission tomography in
prognostic and therapeutic assessment of lung cancer:
systematic review. Lancet Oneal. 5(9):531-40, 2004
 NON-SMALL CELL LUNG CANCER

I IMAGE GALLERY

(Left) Axial NECT shows an

endobronchial squamous
cell carcinoma (open black
arrow) with collapse of right
upper lobe. Note
displacement of the minor
(arrows) and major (open
white arrow) fissures. (Right)
Axial CECT shows mixed
density nodule with punctate
regions of ground glass
attenuation centrally (arrow)
3
and spiculated margins.
Biopsy proven 5
adenocarcinoma.

(Left) Axial CECT shows

large mass invading the
mediastinum (arrows) with
metastatic left
tracheobronchial lymph
node (open arrow)
diagnosed as large cell
carcinoma at histology.
(Right) Axial CECT shows
large lobulated right upper
lobe mass (white arrows)
with extensive contact with
parietal pleura (black
arrows). Small pleural
effusion seen.

Variant
(Left) Axial NECT shows
well-circumscribed right
upper lobe nodule with
peripheral and eccentric
punctate calcification
(arrow). Initially thought
benign, lesion resected due
to growth. Diagnosis:
NSCLC. (Right) Axial CECT
shows large necrotic mass in
left lower lobe (white
arrows) with invasion of the
inferior pulmonary vein and
tumor thrombus within the
left atrium (black arrow).
 SMALL CELL LUNG CANCER

3
6

Frontal radiograph shows large mediastinal and hilar Axial CECT shows large mediastinal mass encasing the
mass in aorto-pulmonary window (arrows) as a result of left main bronchus (curved arrow). The mass envelopes
lymph node metastasis from small cell lung cancer. and attenuates main and left pulmonary artery (arrows).

Abdominal organs: 60% (especially adrenal)

ITERMINOLOGY Bone: 35%
Abbreviations and Synonyms Brain: 10%
Small cell lung cancer (SCLC), oat cell carcinoma, Size: Usually quite large at time of diagnosis
Kulchitsky cell tumor, neuroendocrine tumor Morphology: Bulky mediastinal or hilar mass

Definitions Radiographic Findings

Lung cancer: Thought to arise from Kulchitsky Radiography
(neuroendocrine) cells o Discrete pulmonary nodule or mass (5-15%) may
not be visible (cavitation distinctly rare)
o Large mediastinal mass extending to at least one
IIMAGING FINDINGS hilum (85%)
o Endobronchial obstruction
General Features May cause lobar or whole lung atelectasis
Best diagnostic clue: Large mediastinal mass Lobar consolidation, 30-50%
Location o Solitary pulmonary nodule, 5%
o Arises within proximal airway Variable size, noncalcified
Primary pulmonary lesion often not detected o Elevation of hemidiaphragm due to phrenic nerve
radiographically involvement or pleural effusion
o Large mass (adenopathy) involving mediastinum CT Findings
and at least one hilum
May collapse lobe or entire lung NECT
o Bulky mediastinal mass
o Solitary pulmonary nodule: Less than 5%
o Post-obstructive pneumonitis or collapse
o Hematogenous metastases
o Bronchial encasement, compression, or obstruction

DDx: Small Cell Carcinoma

Metastatic Breast Cancer Non-Small Cell Carcinoma Non-Hodgkin Lymphoma

 SMALL CELL LUNG CANCER

Key Facts
Imaging Findings
Best diagnostic clue: Large mediastinal mass
Arises within proximal airway
Discrete pulmonary nodule or mass (5-15%) may not
be visible (cavitation distinctly rare)
Large mediastinal mass extending to at least one
hilum (85%)
Endobronchial obstruction
Solitary pulmonary nodule, 5%
Mass enveloping pulmonary arteries, aorta, great
vessels
Compression/invasion of superior vena cava, 10-15%
Top Differential Diagnoses
Lymphoma
Non-small cell lung carcinoma (NSCLC)
Metastases from extra-thoracic primary
Benign Adenopathy
Pathology
General path comments: Aggressive behavior: Rapid
growth and early metastatic spread
Strong relationship to tobacco use
Epidemiology: 15-20% of lung cancers
Hilar mediastinal mass usually bulky
Clinical Issues
Most common signs/symptoms: Cough, chest pain,
hemoptysis, dyspnea
Age: 5th-7th decade most common
Gender: M > F
3
Most rapidly growing of the lung carcinomas 7
Overall poor prognosis, 5 year survival 4%

o Pleural effusion Non-small cell lung carcinoma (NSCLC)

o Pulmonary nodule(s) o Primary tumor usually visible, less bulky mediastinal
CECT disease
o Mass enveloping pulmonary arteries, aorta, great Metastases from extra-thoracic primary
vessels o History of breast cancer, head and neck cancer, renal
o Compression/invasion of superior vena cava, cell cancer, melanoma
10-15%
Collaterals may not be present due to rapid tumor
Benign Adenopathy
growth Infection, particularly tuberculosis
o Generally smaller, discrete lymph nodes with
MR Findings necrosis
Rarely utilized for intrathoracic disease Sarcoidosis, atypical
Test of choice for imaging brain metastases o Symmetric hilar adenopathy
May consider for imaging of liver metastases and Fibrosing mediastinitis
adrenal metastases in certain circumstances o Partially calcified mass

Nuclear Medicine Findings Solitary Pulmonary Nodule

PET Non-small cell lung cancer
o Role remains controversial o Indistinguishable
o Upstage limited disease to extensive disease in 15% o Cavitation rare with untreated small cell lung cancer
compared with conventional work-up Solitary metastasis
Bone Scan: Metastases typically show areas of o Indistinguishable
increased uptake o Cavitation rare with untreated small cell lung cancer

Imaging Recommendations
Best imaging tool I PATHOLOGY
o CECT to determine extent of disease in chest and
abdomen General Features
o CECT or MR with gadolinium of brain for General path comments: Aggressive behavior: Rapid
metastases growth and early metastatic spread
o Bone scintigraphy for osseus metastases Etiology
FOG-PET may be considered for staging distant o Strong relationship to tobacco use
metastases o Radiation exposure
Protocol advice: CT should include entire liver Effect is synergistic with tobacco
o Exposure to Bis-chloromethyl ether
Epidemiology: 15-20% of lung cancers
I DIFFERENTIAL DIAGNOSIS Gross Pathologic & Surgical Features
Other Neoplasms Hilar mediastinal mass usually bulky
Lymphoma o Lymphatic and vascular invasion
o Discrete lymph nodes o Hematogenous metastasis to lung, adrenals, liver,
o Usually nonobstructive, no atelectasis or superior kidneys, brain, and bone
vena cava obstruction
 SMALL CELL LUNG CANCER
Microscopic Features
Cells two-three times the size of lymphocytes
o Finely stippled chromatin, scant cytoplasm, small or
absent nucleoli, crush artifact
High rate of mitotic figures
Necrosis may be extensive
May have mixed non-small cell carcinoma cell
populations
Staging, Grading or Classification Criteria
Limited disease
o Disease involving mediastinum, hila, supraclavicular
fossa
o Can be included in tolerable radiation port
Extensive disease: All others
8 o Extra-thoracic disease
o Disease confined to chest but cannot be confined
within tolerable radiation port
Pulmonary metastases
Pleural effusion
Enlarged axillary lymph nodes
Tumor, node, metastasis (TNM) classification, rarely
applicable
o May be appropriate when considering surgery
ICLINICAL ISSUES
Presentation
Most common signs/symptoms: Cough, chest pain,
hemoptysis, dyspnea
Other signs/symptoms
o Usually systemic disease at presentation including
bone marrow suppression
Weight loss, anorexia
o Hypertrophic pulmonary osteoarthropathy rare
compared to NSCLC
o Symptoms due to compression of mediastinal
structures
Superior vena cava syndrome
Dysphagia from esophageal compression
Hoarseness from involvement of recurrent
laryngeal nerve
o Sym ptoms due to metastatic disease
Headache, mental status changes, seizures, ataxia
from brain metastases
Bone pain
Pruritis and jaundice due to liver metastases
o Nonendocrine paraneoplastic syndromes
Eaton-Lambert-Myasthenia syndrome
Limbic encephalitis
Cerebellar degeneration
Anti-Hu encephalomyelitis
Dermatomyositis/polymyositis
o Endocrine paraneoplastic syndromes
Syndrome of inappropriate antidiuretic hormone
Ectopic ACTH causing Cushing syndrome
Hypercalcemic hyperparathyroidism
Demographics
Age: 5th-7th decade most common
Gender: M > F
Natural History & Prognosis
Most rapidly growing of the lung carcinomas
Without treatment, usually fatal within 4 months
Often complete radiographic response but recurs in <
2 years
Overall poor prognosis, 5 year survival 4%
o Limited disease
Median survival 14-16 months
2 year survival 10%
o Extensive disease
Median survival 8-11 months
2 year survival 2%
o Screening
Rapid growth not amenable to screening, previous
chest radiograph screening studies, small cell lung
carcinomas discovered as interval cancers,
previous chest radiographs usually normal (even
in retrospect)
Treatment
Limited disease
o Combination chemotherapy and radiation therapy
o Prophylactic cranial irradiation
o Surgery rarely performed for solitary pulmonary
nodule
Extensive disease
o Chemotherapy
o Radiation therapy reserved for palliation of
symptoms
o Cerebral metastases usually treated with radiation
therapy, occasionally surgery
I DIAGNOSTIC CHECKLIST
Consider
Small cell carcinoma in patients with paraneoplastic
syndrome
Image Interpretation Pearls
Best imaging clue: Bulky mediastinal mass without
obvious primary tumor
Carefully evaluate extra thoracic sites and all
abdominal organs, particularly adrenals and liver
I SELECTED REFERENCES
1. Buccheri G et al: Prognostic factors of small cell lung
cancer. Hematol Oneal Clin North Am. 18(2):445-60, 2004
2. Irshad A et al: Imaging of small-cell lung cancer. Curr Probl
Oiagn Radial. 33(5):200-11, 2004
3. Stupp R et al: Small cell lung cancer: state of the art and
future perspectives. Lung Cancer. 4S(1):1OS-17, 2004
4. Kamel EM et al: Whole-body (18)F-FOG PET improves the
management of patients with small cell lung cancer. J Nuel
Med. 44(12):1911-7, 2003
5. Zakowski MF: Pathology of small cell carcinoma of the
lung. Semin Oneal. 30(1):3-8, 2003
6. Chin RJr et al: Whole body FOG-PET for the evaluation
and staging of small cell lung cancer: a preliminary study.
Lung Cancer. 37(1): 1-6,2002
7. Adjei AA et al: Current guidelines for the management of
small cell lung cancer. Mayo Clin Proc. 74(8):809-16, 1999
8. PearibergJL et al: Small-cell bronchogenic carcinoma: CT
evaluation. AJR Am J Roentgenol. 150(2):265-8, 1988
 SMALL CELL LUNG CANCER

I IMAGE GALLERY

(Left) Frontal radiograph

shows large right
para tracheal mass extending
from thoracic inlet to right
hilum (arrows). No primary
tumor is visualized, typical
for small cell carcinoma.
(Right) Axial CECT shows
right paratracheal mass
(arrow) with obstruction of
superior vena cava with
absent collateral vessels from
3
rapid growth of small cell
carcinoma. Also note pleural 9
effusion (open arrows).

aeft)Ax~/CECTshows
necrotic tumor in left upper
lobe (arrow) with associated
paraesophageal mediastinal
adenopathy (open arrow).
Primary tumor may not be
seen in small cell carcinoma.
(Right) Axial CECT shows
necrotic lymph node mass in
aorta-pulmonary window
with probable invasion of
descending aorta (arrow) in
small cell carcinoma.

Variant
(Left) Axial NECT shows
peripheral spiculated mass in
the left upper lobe (arrows)
without air bronchograms.
This appearance is more
often associated with NSCLC
than small cell carcinoma.
(Right) Axial H RCT shows
well-circumscribed
peripheral nodule (black
arrow) in right lower lobe.
Note also traction
bronchiectasis (white
arrows) and dilated
esophagus (curved arrow)
related to scleroderma.
 STAGING OF LUNG CANCER

3
10

Graphic shows noncalcified irregular nodule in right Coronal PET shows hypermetabolic primary tumor in
lung measuring < 3 cm. The ipsilateral right hilar nodes right upper lobe (arrow), right paratracheal adenopathy
are enlarged with metastatic tumor. Stage lIa non-small (open arrow) and right pleural metastasis (curved
cell lung carcinoma. arrow). Stage IV disease.

Adrenal: Contour abnormality; > 10 HU

ITERMINOlOGY unenhanced CT
Abbreviations and Synonyms Liver: Hypodense lesion with peripheral
Mountain classification enhancement
NPV: Negative predictive value, PPV: Positive Bone: Lytic lesion
predictive value Brain: Ring enhancing lesion
Location
Definitions o Staging non-small cell carcinoma
Staging non-small cell lung cancer determines o Tl: Solitary pulmonary nodule < 3 cm diameter
anatomic extent, treatment and prognosis o T2
o Defined by primary tumor (T), mediastinal lymph Mass> 3 cm
nodes (N) and distant metastases (M): TNM Invasion of visceral pleura
classification Involves major bronchus> 2 cm from carina
Lobar atelectasis
o T3
IIMAGING FINDINGS Any tumor extending into chest wall, diaphragm,
mediastinal fat, or pericardium
General Features Whole lung atelectasis
Best diagnostic clue < 2 cm from carina
o Primary tumor: Dominant nodule or mass in lung o T4
parenchyma Any tumor invading heart, great vessels, trachea,
o Mediastinal nodes: Considered abnormal if size esophagus, vertebral body, carina
greater than 1 cm short axis Malignant pleural effusion
o Metastasis Satellite nodule in ipsilateral tumor lobe

DDx: Mediastinal Adenopathy

Congestive Heart Failure Tuberculosis

 STAGING OF LUNG CANCER
Key Facts
Terminology
Staging non-small cell lung cancer determines
anatomic extent, treatment and prognosis
Imaging Findings
Primary tumor: Dominant nodule or mass in lung
parenchyma
Mediastinal nodes: Considered abnormal if size
greater than 1 cm short axis
Adrenal adenomas < 10 HU due to lipid content
Accuracy of CT nodal staging
Sensitivity (57%), specificity (82%), NPV (56%), PPV
(83%)
Use of PET prevents unnecessary thoracotomy in up
to 20% of patients
Sensitivity of PET for malignancy> 95% for tumors>
1 cm; specificity < 80%
a NO: No nodes
a N1: Ipsilateral hilar nodes
a N2: Ipsilateral mediastinal nodes; includes
subcarinal and midline nodes
a N3: Contralateral mediastinal, ipsilateral scalene, or
supraclavicular nodes
a MO: No distant metastases
a M1: Distant metastases
a Staging small cell carcinoma
Limited: Tumor, mediastinal and supraclavicular
nodes can be confined to single radiation port
Extensive: All others
Radiographic Findings
Radiography
a No longer appropriate for tumor staging
Inaccurate measurement of primary tumor size
Insensitive for mediastinal adenopathy and
distant metastases
CT Findings
NECT
a Suitable for staging lung cancer
Equivalent to CECT for staging mediastinum
Provide definitive evaluation of indeterminate
adrenal lesions
Less sensitive than CECT for detecting liver lesions
a Adrenal evaluation
Up to 10% normal population have incidental
adrenal lesion
Adrenal adenomas < 10 HU due to lipid content
Nonenhanced CT 98% specific, 70% sensitive to
rule-in adrenal adenoma
Abnormal nodes> 1 cm short axis diameter
a Subcarinal nodes may be up to 12 mm in short axis
diameter
Accuracy of CT nodal staging
a Sensitivity (57%), specificity (82%), NPV (56%), PPV
(83%)
Causes of false positive lymph nodes: Reactive
adenopathy, congestive heart failure, sarcoidosis,
granulomatous infection, collagen vascular disease
a Frequency of nodal metastases (20-50%)
Best imaging tool: CT and PET in combination
Clinical Issues
Patient should be given the benefit of proof, stage
should be confirmed pathologically
Diagnostic Checklist
Non-neoplastic causes of enlarged lymph nodes
including reactive nodes due to infection, heart
failure and sarcoidosis
Benign adrenal lesion when adrenal nodule present,
particularly adenoma
Benign liver lesion when focal hypodensity present,
particularly cyst and hemangioma
3
Biopsy should be directed at finding that will confirm 11
highest stage
a Frequency of metastases nodes over 3 cm (66%)
a Frequency of metastases nodes over 4 cm (100%)
Predictors of chest wall invasion
a Greater than 3 cm contact with pleural surface,
absent fat plane, obtuse angle
Two of three present: Sensitivity 87%; specificity
59%
Chest wall pain: Sensitivity 67%; specificity 94%
Predictors of aortic invasion
a Greater than 90 degrees contact with aortic wall
Adrenal evaluation
a Enhanced CT
a % Enhancement washout = (enhanced CT
attenuation - delayed CT attenuation)/(enhanced CT
attenuation - unenhanced CT attenuation) x 100
Adenomas> 60% washout
Sensitivity 86%, specificity 92%
o % Relative washout = (enhanced CT attenuation -
delayed CT attenuation)/enhanced CT attenuation x
100
Adenomas> 40% washout
Sensitivity 82%, specificity 92%
MR Findings
Overall accuracy similar to CT
o Slightly more sensitive than CT for chest wall
invasion
o Coronal plane advantageous for superior sulcus
tumors
Adrenal Imaging
o Perform in phase and out of phase imaging
Adenoma: Signal drop out on out of phase image
Sensitivity 81-100%; specificity 94-100%
CNS imaging
o Not routinely necessary for T1 tumors
o Perform in otherwise asymptomatic potentially
resectable lesions> 3 cm
o Asymptomatic metastases 3-10%
Adenocarcinoma and large cell most common
subtypes to metastasize to brain
Nuclear Medicine Findings
PET
 STAGING OF LUNG CANCER
o Use of PET prevents unnecessary thoracotomy in up Stage 3A (Tl-3N2, T3Nl) 10% 5-year survival
to 20% of patients Stage 3B (Tl-4N3, T4NO-3) 5% 5-year survival
o Primary tumor Stage 4 (Ml) 1% 5-year survival
Sensitivity of PET for malignancy> 95% for
tumors> 1 cm; specificity < 80% Treatment
False positive: Focal pneumonia, active granuloma Patient should be given the benefit of proof, stage
False negative: Bronchioloalveolar carcinoma, low should be confirmed pathologically
grade adenocarcinoma, carcinoid tumor Stage 1 and 2: Surgical resection
Standard uptake value (SUV) correlates with o Adjuvant chemotherapy appropriate when
prognosis recurrence risk is high
o Mediastinum o Radiation therapy and radiofrequency ablation can
Sensitivity and specificity linked to CT result be considered for medically inoperable stage 1
CT positive mediastinum: Sensitivity approaches disease
100%; specificity 78% Stage 3A: Neoadjuvant chemotherapy and radiation
3 CT negative mediastinum: Sensitivity 82%; therapy
specificity 93% o May be performed as definitive treatment or
12 o Distant metastases followed by surgery
Adrenal: Sensitivity 94-100%; specificity 80-91% o Primary resection of 3A disease when malignant
Bone: Sensitivity and accuracy similar to bone lymph nodes are detected at thoracotomy; should
scintigraphy; better specificity receive adjuvant chemotherapy
Stage 3B and 4 considered unresectable disease
Ultrasonographic Findings o Stage 3B disease may be treated with chemotherapy
Endoscopic ultrasound: Trans-esophageal (EUS) and and radiation therapy with curative intent
trans-bronchial (EBUS) o Occasionally T4NO tumors with limited invasion of
o Fine needle aspiration can be performed in same vertebral body, mediastinum or left atrium may be
setting resectable
o EUS best for levels 4L, 5, 6, 7, and 8 o Stage 4 disease treated primarily with chemotherapy;
o EBUS: Allows access to right mediastinal lymph radiation therapy reserved for palliation of
nodes symptoms
Imaging Recommendations
Best imaging tool: CT and PET in combination I DIAGNOSTIC CHECKLIST
Consider
I PATHOLOGY Non-neoplastic causes of enlarged lymph nodes
including reactive nodes due to infection, heart failure
General Features and sarcoidosis
General path comments Benign adrenal lesion when adrenal nodule present,
o Non-small cell carcinoma, in order of frequency particularly adenoma
Adenocarcinoma> squamous cell carcinoma> Benign liver lesion when focal hypodensity present,
large cell carcinoma particularly cyst and hemangioma
o Prevalence of metastases in normal-sized nodes: 15%
Image Interpretation Pearls
Staging, Grading or Classification Criteria Suspicious imaging findings should be confirmed
Stage 1 pathologically before denying curative surgery
o A: TlNOMO Biopsy should be directed at finding that will confirm
o B: T2NOMO highest stage
Stage 2
o A: TlNIMO
o B: T2NIMO; T3NOMO I SELECTED REFERENCES
Stage 3
o A: T3NIMO; Tl-3N2MO 1. Ravenel JG: Lung cancer staging. Semin Roentgenol.
39(3):373-85,2004
o B: Tl-3N3MO; T4NI-3MO 2. Gould MK et al: Test performance of positron emission
Stage 4 tomography and computed tomography for mediastinal
o Any T; any N; Ml staging in patients with non-small-cell lung cancer: a
meta-analysis. Ann Intern Med. 139(11):879-92,2003
3. Silvestri GA et al: The noninvasive staging of non-small cell
I CLINICAL ISSUES lung cancer: the guidelines. Chest. 123(1 Suppl): 147S-1 S6S,
2003
Natural History & Prognosis 4. Toloza EM et al: Noninvasive staging of non-small cell lung
Stage lA (TlNO) 65% 5-year survival cancer: a review of the current evidence. Chest. 123(1
Suppl):137S-146S, 2003
Stage IB (T2NO) 40% 5-year survival 5. Mountain CF et al: Regional lymph node classification for
Stage 2A (TlN]) 35% 5-year survival lung cancer staging. Chest. 111 (6): 17] 8-23, 1997
Stage 2B (T2N 1, T3NO) 25% 5-year survival
 STAGING OF LUNG CANCER
I IMAGE GAllERY
Typical
(Left) Axial CECT shows
heterogeneous right upper
lobe mass with invasion of
mediastinum posterior to
trachea and mass effect on
the esophagus (arrows). T4
tumor. (Right) Coronal CECT
shows left upper lobe mass
extending through chest wall
(arrow) and contacting left
subclavian artery (curved
arrow); PancoasllUmor. T3 3
tumor by TNM classification
system. 13

(Left) Axial CECT shows two

enlarged left hilar lymph
nodes (N7 - arrows) and
cluster of enlarged left
tracheobronchial and AP
window lymph nodes fN2 -
curved arrow). Stage is at
least iliA. (Right) Axial CECT
shows left supraclavicular
adenopathy (N] - arrows)
lateral to left common
carotid and left internal
jugular vein. At least stage
IIIB.

(Left) Axial CECT shows left

adrenal nodule with central
low attenuation (arrow)
shown to be metastatic
adenocarcinoma at biopsy.
Stage IV lung cancer. (Right)
Axial T7 C+ FS MR shows
two rim~enhancjng cystic
metastases in left cerebellar
hemisphere (arrows) with
mass effect on fourth
ventricle (open arrow) (rom
adenocarcinoma of lung.
 REGIONAL LYMPH NODE CLASSIFICATION

3
14

Anatomic distribution and labeling of mediastinal and Aorta-pulmonary window from left anterior oblique
hilar lymph nodes (with exception of levels 5 and 6) as perspective. Level 5 nodes are lateral to ligamentum
specified by American Thoracic 50ciety (AT5). Modified arteriosum and level 6 nodes are lateral to transverse
with permission ATS. aortic arch. Modified with permission ATS.

o Nodes lateral to ligamentum arteriosum and medial

ITERMINOLOGY to first branch of left pulmonary artery
Abbreviations and Synonyms Level 6: Para-aortic lymph nodes (ascending aortic or
American Joint Committee on Cancer (AJCc) lymph phrenic)
node stations o Nodes anterior, lateral and inferior to transverse
aortic arch
Definitions Level 7: Subcarinallymph nodes
Level 1: Highest mediastinal o Inferior to tracheal carina between main bronchi
o Nodes superior to the left brachiocephalic vein Level 8: Paraesophageallymph nodes
Level 2: Upper paratracheal o Nodes adjacent to esophageal wall to the right (8R)
o Nodes inferior to left brachiocephalic vein but above or left (8L) of midline
aorta, lateral to trachea Level 9: Pulmonary ligament
Level 3: Pre-vascular and retro-tracheal o Nodes within the inferior pulmonary ligament
o Nodes anterior to great vessels and superior to aortic Level 10: Hilar
arch (3A) o Nodes inferior to superior aspect of main bronchus
o Nodes posterior to trachea superior to azygous arch and medial to bronchus intermedius (lOR) or left
(3P) upper lobe bronchus (lOL)
Level 4: Lower para tracheal lymph nodes Level 11: Interlobar
o Nodes inferior to transverse arch, lateral to trachea o Nodes between and adjacent to proximal lobar
and superior to right main bronchus (4R) bronchi
o Nodes inferior to transverse arch, superior to left Level 12: Lobar
main bronchus and medial to ligamentum o Nodes adjacent to distal lobar bronchi
arteriosum (4L) Level 13: Segmental
Level 5: Subaortic lymph nodes (A-P window) o Nodes adjacent to segmental bronchi

DDx: Lymph Nodes Mimics

Superior Pericardia I Recess Dilated Collateral Vein Anomalous Pulmonary Vein

 REGIONAL LYMPH NODE CLASSIFICATION

Key Facts
Terminology Clinical Issues
American Joint Committee on Cancer (AJCc) lymph EUS-FNA best for levels 4L, 5, 7, 8
node stations EBUS allows access to sites not evaluable by EUS
Cervical mediastinoscopy can reach levels 1, Z, 3, 4,
Imaging Findings anterior 7
CT is primary modality for anatomic detection of Extended cervical mediastinoscopy reaches levels 5
lymph nodes and 6 as well
Allows for selection of appropriate route to attain VATSmay be used to reach levels 4,5,6, 7, 8, and 9
tissue in selected cases

Level 14: Subsegmental

o Nodes adjacent to subsegmental bronchi and in
FNA performed under direct ultrasound guidance
EUS-FNA best for levels 4L, 5, 7, 8
3
lung parenchyma Sensitivity 85-90% 15
o Trans-bronchial ultrasound (EBUS) with FNA
EBUS allows access to sites not evaluable by EUS
IIMAGING FINDINGS Can perform FNA on hilar nodes
o Mediastinoscopy
CT Findings Cervical mediastinoscopy can reach levels 1, Z, 3,
CT is primary modality for anatomic detection of 4, anterior 7
lymph nodes Extended cervical mediastinoscopy reaches levels
Allows for selection of appropriate route to attain 5 and 6 as well
tissue o Anterior mediastinotomy (Chamberlain procedure)
Right lung tumor Via right thorax reaches level ZR, 3, 4R
o NIlymph nodes: lOR, llR, IZR, 13R, 14R Via left thorax reaches level 5 and 6 only
o NZlymph nodes; IR, ZR, 3, 4R, 7, 8R, 9R o Video assisted thoracoscopic surgery (VATS)
o N3 lymph nodes; IL, ZL, 4L, 5, 6, 8L, 9L VATSmay be used to reach levels 4, 5, 6 , 7, 8, and
Left lung tumor 9 in selected cases
o NIlymph nodes; lOL, IlL, IZL, 13L, I4L
o NZlymph nodes; IL, ZL, 3, 4L, 5, 6, 7, 8L, 9L
o N3 lymph nodes; IR, ZR, 4R, 8R, 9R I SELECTED REFERENCES
1. Toloza EM et al: Invasive staging of non-small cell lung
cancer; a review of the current evidence. Chest. 123(1
I CLINICAL ISSUES Suppl):157S-166S, 2003
2. Ko JP et al; CT depiction of regional nodal stations for lung
Treatment cancer staging. AJR Am J Roentgenol. 174(3):775-82,2000
Methods for pathologic confirmation 3. Mountain CF et al: Regional lymph node classification for
o Bronchoscopy and biopsy lung cancer staging. Chest. 111(6):1718-23, 1997
Operator dependent; sensitivity for mediastinal
nodes approximately 75%
o Trans-esophageal ultrasound (EUS) with fine needle
aspiration (FNA)

I IMAGE GAllERY

(Left) Axial CECT shows mediastinal and hilar adenopathy with lymph nodes at station 77 L (arrows), 4L (open arrow), and 5 (curved arrow).
With a left lung primary tumor these are NI and N2 nodes. (Center) Intra-operative photograph During VATSshows enlarged lymph node
(curved arrow) just inferior to the aorta (open arrow) in aorta-pulmonary window (level 5). (Courtesy Carolyn Reed, MD). (Right! Transverse
transesophageal endoscopic ultrasound shows enlarged homogeneous 4L lymph node (curved arrow) anterior to the aorta (open arrows) and
medial to the left pulmonary artery (arrows).
 SOLITARY PULMONARY NODULE

3
16

Graphic shows hamartoma. CT detecUon of fat and Axial NECT shows 7.5 cm solid nodule (arrow) in the
"popcorn" calcification in a lobulated 50ft tissue nodule posterior segment right upper lobe. FNA showed no
< 2.5 cm in diameter suggests diagnosis. 510w growing malignant cells, a nonspecific benign diagnosis. Further
and usually detected in 4th or 5th decade of life. CT observation or intervention is indicated.

Upper lobe, most common

ITERMINOLOGY 1.5 times more likely on right side
Abbreviations and Synonyms With pulmonary fibrosis, lower lobes more
Solitary pulmonary nodule (SPN), fine needle common
aspiration biopsy (FNA) Size
a < 3 cm; > 90% of nodules < 2 cm are benign
Definitions a Nodules approaching 3 cm, more likely to be
Round or oval opacity, < 3 cm in diameter malignant
Completely surrounded by pulmonary parenchyma Morphology: Mixed solid/part-solid attenuation: 50%
Not associated with lymphadenopathy, atelectasis, or malignant
pneumonia
Radiographic Findings
Radiography
I IMAGING FINDINGS a Seldom detect nodules < 9 mm
a 50% chance to detect 7 mm non-calcified nodule
General Features a Up to 90% lung cancers visible retrospectively on
Best diagnostic clue prior radiographs
a Common radiographic problem: Goal is to separate a Radiography: Site of most missed cancers, right
benign from malignant nodules upper lobe
For benign disease: Benign patten of calcification a Prior radiographs critical for nodule detection
or presence of fat a Misses due to
For malignant disease: Positive PET, 94% sensitive Faulty search pattern
Location Decreased conspicuity (overlapping structures)
a Lung cancer a Calcification

DDx: Pulmonary Nodule Mimics

Old Rib Fracture Costosternal Spur Nipple Shadow

 SOLITARY PULMONARY
Key Facts
Terminology
Round or oval opacity, < 3 em in diameter
Imaging Findings
< 3 em; > 90% of nodules < 2 em are benign
Nodules approaching 3 em, more likely to be
malignant
Radiography: Site of most missed cancers, right upper
lobe
Prior radiographs critical for nodule detection
Benign calcification: Central nidus, laminated,
popcorn, diffuse
Hamartomas, 1/3 show popcorn calcification Diagnostic Checklist
Diffuse calcification in osteogenic sarcoma,
chondrosarcoma metastases
1/2 hamartomas contain fat
Nodule < 9 mm seen on radiograph likely calcified
and benign
o Growth
2-Year rule: No change in 2 years implies
benignity
Case control CT lung cancer screening studies
have shown tumors with extremely long doubling
times (1700 days)
Typical malignancy, 100 day doubling time
Tumors double in volume from 1 to 18 months
CT Findings
CTA: Arteriovenous malformation: To show feeding
artery and draining vein
NECT
o To determine if nodule pulmonary or
extrapulmonary
o Solitary or other nodules
o Calcification, benign
lOx more sensitive technique to detect
calcification
> 200 HU considered calcified
> ] 0% calcification of cross sectional area
Benign calcification: Central nidus, laminated,
popcorn, diffuse
55% of granulomas are calcified
Hamartomas, 1/3 show popcorn calcification
Carney's triad: Multiple chondromas,
gastrointestinal stromal tumors, extra-adrenal
paraganglioma
o Calcification, malignant
Eccentric calcification, seen in benign or
malignant nodules
2% of lung cancers < 3 em have calcification
1/3 carcinoids calcified, central lesions may have
central nidus
Diffuse calcification in osteogenic sarcoma,
chondrosarcoma metastases
Psammomatous calcifications: Colon or ovarian
metastases, rarely primary bronchogenic
carcinoma
o Fat, benign
1/2 hamartomas contain fat
NODULE
Growth: Much overlap between benign and
malignant nodules
Mixed solid/part solid, up to 50% < 1.5 em in
diameter are malignant
Top Differential Diagnoses
1st costochondral junction osteophytes
Nipple shadow
Pathology
90% represent (in order) granuloma, bronchogenic
carcinoma, hamartoma, solitary metastasis, carcinoid
3
Some lung cancers grow so slowly that growth may
17
not be detected with 2 year follow-up
Lipoid pneumonia
o Fat, malignant
Liposarcoma, renal cell carcinoma metastases,
uncommon
o Growth: Much overlap between benign and
malignant nodules
Software available for semiautomated
measurement
Malignant nodules: CT doubling times, 32 days to
> 730 days
Benign nodules: CT doubling time < 7 days to no
growth
CECT
o Advantage for lymph nodes, pleural, chest wall,
liver, adrenal involvement
o Contrast enhancement of SPN, sensitive not specific
for malignancy
o Not performed in nodules < 1 em, cavitary or with
central necrosis
o Densitometry following IV contrast bolus
o Related to angiogenesis and blood flow
o Enhancement < 15 HU, benign
o Maximal enhancement> ]5 HU, indeterminate;
carcinoid, striking contrast-enhancement
HRCT
o Nodule, margin and shape
Overlap of benign and malignant features
Margin: Benign = sharp, smooth; malignant =
ill-defined, spiculated coronal radiata, halo
Shape: Benign = spherical, slightly lobulated,
satellite nodules; malignant = irregular, lobulated
o Nodule attenuation
Non-solid (ground glass), 34% are malignant
Mixed solid/part solid, up to 50% < 1.5 em in
diameter are malignant
Solid, 15% < ] em are malignant; as diameter
increases cancer risk increases
Most lung cancers and metastatic nodules solid
o Air bronchograms
More common in pulmonary carcinomas than
benign nodules
30% of malignant nodules; 6% of benign nodules
 SOLITARY PULMONARY NODULE
Bubble-like lucencies (pseudocavitation): Up to
55% of bronchioloalveolar cell carcinomas Gross Pathologic & Surgical Features
Due to small airway distortion Depends on pathology
o Cavitation
Microscopic Features
Malignant cavity: Variable wall thickness,
Adenocarcinoma, most common malignant SPN
maximal wall thickness> 16 mm
Benign cavity: Smooth wall, maximal wall Staging, Grading or Classification Criteria
thickness < 4 mm Goal to detect Stage IA lung cancer, TlNOMO
Nuclear Medicine Findings o Nodule < 3 cm, no pleural, central airway, nodal
involvement
PET o No distant metastases
o Measures glucose metabolism
o For nodules> 1 cm to < 3 cm
o Sensitivity 94%, specificity 83%
3 o False negatives: Small 7 mm diameter), carcinoid,
ICLINICAL ISSUES
bronchioloalveolar cell carcinoma, cavitary or Presentation
18 necrotic nodules
Most common signs/symptoms: Asymptomatic,
o False positives: Tuberculosis, histoplasmosis, usually incidental finding
sarcoidosis, rheumatoid
o Shows extra pulmonary involvement Demographics
Imaging Recommendations Age: Neonatal to elderly; lung cancer, usually> age 40;
exception HIV
Best imaging tool
Gender: M:F = 1.2:1
o NECT with sequential thin cuts for presence of
calcification or fat Natural History & Prognosis
o PET for nodules with high likelihood for malignancy Early detection (CT screening) may reduce lung
Protocol advice cancer-specific mortality
o NECT sequential thin cut images, single breathold Not proved; clinical trials in progress
o MIP increases conspicuity for nodules
Treatment
Benign nodules: None
I DIFFERENTIAL DIAGNOSIS Low likelihood for malignancy
o Observation: Radiography or 3, 6, 12, 24 month
Mimics serial CT scans
1st costochondral junction osteophytes o No growth for 2 years, benign
o Inferior aspect 1st rib, more common right o Small changes in growth in small nodules 1 cm)
(right-handed) may be difficult to detect
Nipple shadow High likelihood for malignancy
o Bilateral, outer edge sharp, inner edge indistinct o PET scan: Positive, intervene
Skin lesions o FNA
o Neurofibromas, moles Useful for indeterminate nodules, need good
Pulmonary vein confluence cytopathologist
o Upper aspect right heart border Goal to obtain benign specific or malignant
diagnosis
Pneumothorax rate, 25%
I PATHOLOGY o Surgery
Malignancy: Resection: 5 year survival up to 70% stage
General Features I lung cancer
Etiology
o 90% represent (in order) granuloma, bronchogenic
carcinoma, hamartoma, solitary metastasis, I DIAGNOSTIC CHECKLIST
carcinoid
o Lung cancer: Increased risk with smoking, increasing Image Interpretation Pearls
age, pulmonary fibrosis, asbestosis, HIV Some lung cancers grow so slowly that growth may
o Cigarette consumption, direct dose (tar) relationship not be detected with 2 year follow-up
with lung cancer
o Solitary metastasis: Colon, breast, renal, melanoma,
osteosarcoma, testicular I SELECTED REFERENCES
Epidemiology 1. Erasmus JJ et al: Solitary pulmonary nodules: Part I.
o SPN noted on up to 0.2% of all chest radiographs Morphologic evaluation for differentiation of benign and
o Prevalence of granulomas depends on indigenous malignant lesions. Radiographies. 20:43-58, 2000
fungi 2. Erasmus JJ et al: Solitary pulmonary nodules: Part II.
o Granulomas calcify earlier in young individuals Evaluation of the indeterminate nodule. Radiographies.
o Up to 40% of SPNs may be curable lung cancer 20:59-66, 2000
 SOLITARY PULMONARY NODULE

I IMAGE GALLERY

(Left) Axial CECT shows

calcified nodules (arrow) at
the left lower lobe. NECT
with thin cuts is the preferred
method to show
calcification. (Right) Axial
CECT shows calcified right
upper lobe nodule (arrow)
that is a new finding in a
patient with
chondrosarcoma. Diagnosis: 3
Chondrosarcoma metastasis.

19

(Left) Axial CECT in a patient

with melanoma shows a
solitary metastatic nodule
(arrow) in the right lower
lobe. Solitary metastases are
seen with kidney, colon,
testicular, breast and primary
bone tumors. (Right) Axial
H RCT shows a I cm right
lower nodule (arrow) that
showed fat (-7 74 HU).
Diagnosis: Hamartoma.

(Left) Axial NECT shows left

upper lobe nodule (arrow)
with mixed solid and
part-solid attenuation. FNA
showed malignant cells.
(Right) Axial CECT shows a
feeding artery (open arrow)
supplying the left lower lobe
nodule (arrow). A draining
vein was seen (nolshown).
Arteriovenous malformation.
 LUNG CANCER SCREENING

3
20

Frontal radiograph shows subtle increased opacity of Axial CECT shows lobulated 3.5 em mass in right lower
right hilum (arrow). Lateral radiograph normal (not lobe from non-small cell carcinoma. Chest radiography
shown). has never been shown to be an adequate screening test
for lung cancer.

o On chest radiography most missed cancers are in the

ITERMINOlOGY
Definitions
For screening to be effective, test must predict
morbidity far enough in advance that something can
be done but the test must also be widely available and
cost effective
Screening with sputum cytology or chest radiography
has not been shown to reduce mortality
CT screening for lung cancer has shown promise but
should be considered experimental
CT detects more cancers and smaller cancers compared
to chest radiography
Endpoint of screening studies is reduction in
mortality, not increase in 5-year survival
Currently no modality has been shown to decrease
lung cancer mortality
I IMAGING FINDINGS
General Features
Best diagnostic clue
upper lobes
o On CT most missed cancers are centrally located
Location
o Most common location of lung cancer is upper lobes
o Most common location of missed lung cancer is
upper lobes (chest radiograph) or central (CT)
Size
o Larger nodules more likely malignant
Nodules less than 5 mm very low likelihood of
malignancy 1%)
Morphology
o Can be smooth, lobulated or spiculated
Spiculated nodules have highest likelihood of
malignancy (90%)
Radiographic Findings
Radiography
o Observer has 50% chance to detect a nodule 1 cm in
diameter
Satisfaction of search affects detection: Fixation
on larger more obvious abnormalities and missing
small nodules
o Factors that limit detection

DDx: Solitary Pulmonary Nodule

Bronchogenic Carcinoma Carcinoid Granuloma

 LUNG CANCER SCREENING
Key Facts
Terminology
For screening to be effective, test must predict
morbidity far enough in advance that something can
be done but the test must also be widely available
and cost effective
Imaging Findings
Most common location of lung cancer is upper lobes
Most common location of missed lung cancer is
upper lobes (chest radiograph) or central (CT)
Nodule attenuation on CT influences likelihood of
malignancy
Likelihood of malignancy - semisolid> ground-glass
attenuation> solid
Nodules less than 5 mm very low likelihood of
malignancy 1%)
Therefore can be followed with yearly screening CT
Poor film quality
Small lesion size
Ill-defined edges
Overlap of vessels and chest wall structures
o Hierarchy of errors
45% poor decision making: Lesion seen but
dismissed as insignificant
35% recognition error: Observer fixated on lesion
but not brought to level of consciousness
20% scanning error: Observer never focused on
abnormality
o In screening studies, of those with lung cancer, up
to 90% of tumors were visible in retrospect on
previous radiographs
CT Findings
NECT
o Observer still will miss approximately 25% of lung
nodules
Most missed nodules are < 5 mm in diameter
o Nodule attenuation on CT influences likelihood of
malignancy
Likelihood of malignancy - semisolid>
ground-glass attenuation> solid
o Nodules less than 5 mm very low likelihood of
malignancy 1%)
Therefore can be followed with yearly screening
CT
o Margins: Spiculated nodules have highest likelihood
of malignancy
Smooth margins can still be malignant: 20% of
lung cancers have smooth margins
o Ground-glass nodules more likely to be
bronchioloalveolar cell carcinoma (BAC) than other
cell types
CECT
o Nodule enhancement study can be done on nodules
> 7 mm in diameter
Enhancement < 15HU has 98% probability of
being benign
Margins: Spiculated nodules have highest likelihood
of malignancy
Smooth margins can still be malignant: 20% of lung
cancers have smooth margins
Best imaging tool: No screening modality has been
proven to decrease lung cancer mortality
Top Differential Diagnoses
Granuloma
Hamartoma
Solitary Metastases
Carcinoid
Clinical Issues
3
Gender: CT screening studies show excess 21
nonsmoking females developing lung cancer
5-year survival for stage I lung cancer (SPN) 70-80%
Imaging Recommendations
Best imaging tool: No screening modality has been
proven to decrease lung cancer mortality
Screening
o Endpoint of screening is reduction of mortality, not
increase 5-year survival
o Survival subject to biases
Lead time bias
o Example: 2 identical smokers, one screened the
other not, both develop lung cancer at the same
time
o Screened individual has lung cancer diagnosed, he
dies 6 years later of metastases, survival is 6 years
o Non-screened individual is discovered to have lung
cancer (symptomatic) 5 years after the screened
individual and dies 1 year later, survival 1 year
o The difference in survival is lead time, mortality is
the same
Overdiagnosis bias
o Cancers that individual will die with and not die
from
22% of autopsies in one series showed renal cell
carcinoma that was not related to cause of death
o Other tumors or co-morbid conditions that will
decrease life expectancy
Length time bias
o Growth so slow or indolent as not to affect the
health of the individual
o Indolent growth documented for some prostate and
thyroid carcinomas
o Autopsy evidence for occult lung cancer (1%)
o However, in NCI lung cancer detection program: 45
unresected stage I lung cancers only 2 survived 5
years (4.4%)
NCI Lung Cancer Detection Programs
o Johns Hopkins OH), Memorial Sloan Kettering
(MSK), Mayo Clinic (MC)
6 year screen
JH and MSK both control and interventional
groups had chest radiograph, only variable was
sputum cytology
 LUNG CANCER SCREENING
MC, interventional group had chest radiograph
every 4 months, control group advised to have
chest radiograph
Approximately 50% of the control group followed
advice had chest radiography, contaminating the
effect of radiographic screening
In none was mortality decreased in the
interventional group (actually mortality increased
but not statistically significant)
CT screening
o Lung cancer screening with low dose CT
Low dose
40 mA multislice, 80 mA single slice
Dose 1/10 that of conventional CT
3 o In comparison to chest radiography
Average size primary tumor size decreased by 50%
22 Proportion of stage I tumors nearly doubled
4x as many tumors detected compared to chest
radiography
o Early Lung Cancer Action Project (ELCAP)
1,000 smokers older than 60
85% (23/27) stage I lung cancers
2nd incidence scan 70% (5/7) stage I lung cancer
Nonrandomized, results may be biased
13% (4/30) of cancers missed on prior CT
o Mayo Clinic nonrandomized study
70% of screened individuals have indeterminate
nodules
98% of detected nodules will be benign
False positive rate is high leading to increased
screening expense for nodule work-up
Some benign nodules undergo unnecessary
surgery
I DIFFERENTIAL DIAGNOSIS
Granuloma
Most common cause of solitary pulmonary nodule
(SPN)
Endemic dependent on geography and prevalence of
tuberculosis and fungi
When calcified: Central nidus, laminated or diffuse
Hamartoma
Lobulated contour
May contain fat (33%) or calcification (25%)
"popcorn" type
Solitary Metastases
Usually history of malignancy
Most common tumors: Renal cell, colon, breast,
sarcomas, melanoma
Round and sharply defined
Carcinoid
Central lesion adjacent to lobar or segmental bronchi
may have central calcified nidus
May have striking contrast-enhancement
I PATHOLOGY
General Features
General path comments
o Average doubling time of bronchogenic carcinoma
100 days (40-400 days)
o CT screening studies show excess numbers of
adenocarcinomas (which includes BAC) compared
to current and historical statistics
Etiology
o Smoking accounts for 85% of all bronchogenic
carcinoma
Risk directly related to dose and time
o Other known carcinogens
Radon, asbestos, radiation exposure, uranium
Epidemiology: Overall lifetime risk of smokers
developing lung cancer 1 in 10
ICLlNICAl ISSUES
Presentation
Most common signs/symptoms
o For screening to be effective must detect lesion
before it becomes symptomatic
o Missed lung cancer significant medical-legal
problem
Missed lung cancer 2nd most common
malpractice litigation (breast cancer #1)
Demographics
Age: Most screening studies start at age SO or 60
Gender: CT screening studies show excess nonsmoking
females developing lung cancer
Natural History & Prognosis
Current 5-year survival for lung cancer 13%, hasn't
changed in decades
Treatment
5-year survival for stage I lung cancer (SPN) 70-80%
Smoking cessation programs should always
accompany screening
Public health control of tobacco more important than
screening
Lung cancer screening with CT currently undergoing
randomized trial
I SELECTED REFERENCES
]. Swensen SJ et al: CT screening for lung cancer: five-year
prospective experience. Radiology. 235(]):259-65, 2005
2. Henschke CI et al: CT screening for lung cancer:
suspiciousness of nodules according to size on baseline
scans. Radiology. 23](]):164-8, 2004
3. Swensen SJ et al: Lung cancer screening with CT: Mayo
Clinic experience. Radiology. 226(3):756-61, 2003
4. Henschke CI et al: CT screening for lung cancer: frequency
and significance of part-solid and non solid nodules. AJR
Am J Roentgenol. ] 78(5):1053-7, 2002
5. Swensen 5J et al: Lung nodule enhancement at CT:
multicenter study. Radiology. 214(1):73-80, 2000
6. Henschke CI et al: Early Lung Cancer Action Project:
Overall design and findings from baseline screening.
Lancet. 354:99-]05, ]999
 LUNG CANCER SCREENING
I IMAGE GALLERY
Typical
(Leh) Axial NECT shows that
small lung cancers can be
easily overlooked at cr 2
mm nodules adjacent to the
descending aorta was missed
(arrow). (Right) Axial NECT
II months later shows
growth of the nodule to 1 cm
in diameter. This still
represents a stage I
non-small cell bronchogenic
carcinoma.
3
23

Typical
(Left) Axial CECT shows
missed central bronchogenic
carcinoma. Subtle narrowing
of right lower lobe segmental
bronchus (arrow) was
missed. (Right) Axial CECT
18 months later shows right
lower lobe mass (arrow)
obstructing the segmental
bronchus with distal
atelectasis or pneumonia
(curved arrow). Central
carcinomas are more difficult
to detect than peripheral
carcinomas.

(Left) Axial HRCT shows 2

carcinomas, the largest is
mostly ground glass with tiny
solid focus (arrow) and the
smaller is predominantly
solid. Mixed ground-glass
and solid nodules highly
malignant characteristic.
(Right) Axial NECT shows 12
mm by 7 mm spiculated
nodule (non-small cell
carcinoma) in the left upper
lobe (open arrow). Enlarged
pre-tracheal lymph node
(arrow) proven to be
malignant. Small primary
tumors does not necessarily
equate with curability.
 MISSED LUNG CANCER

24

Fronta' radiograph shows a right hilar mass (arrow) not Axia' CECT shows a right hilar mass (arrow) that proved
recognized on initial interpretation. to be lung cancer

Morphology
ITERMINOLOGY o Lesions typically inconspicuous amid complex
Abbreviations and Synonyms adjacent/overlying shadows
Missed lung cancer (MLC) o Most common location of lung cancer is upper lobes
o Typical bronchogenic carcinoma has ill-defined
Definitions edges and soft tissue density, factors which decrease
Missed lung cancer includes failure to diagnose and conspicuity
failure to communicate findings
o Missed lung cancer on CT most likely to increase
Radiographic Findings
with lung cancer screening Bronchogenic carcinoma often subtle lesion in the
o Computer-aided-detection may reduce missed lung upper lobes
cancer Observer has 50% chance to detect a nodule 1 cm in
diameter on CXR
Hierarchy of observer errors
IIMAGING FINDINGS o 45% poor decision making: Lesion seen but
dismissed as insignificant
General Features o 35% recognition error: Visual fixatation on lesion
Best diagnostic clue: Most missed bronchogenic but not brought to level of consciousness of observer
carcinomas located in the upper lobes due to both o 20% scanning error: Observer never focused on
frequent location and problems of conspicuity abnormality
Location: Missed cancers predominantly in right upper Satisfaction of search error
lobe on chest radiograph (CXR) o Observer distracted by interesting but unrelated
Size: Average CXR size of MLC is 1.6 cm, 0.5-1.2 cm on finding
CT o Common cause of missed lung cancer

DDx: lung Cancer Mimics

Hamartoma Sequestration Hematoma

 MISSED LUNG CANCER
Key Facts
Terminology
Missed lung cancer includes failure to diagnose and
failure to communicate findings
Missed lung cancer on CT most likely to increase with
lung cancer screening
Imaging Findings
Best diagnostic clue: Most missed bronchogenic
carcinomas located in the upper lobes due to both
frequent location and problems of conspicuity
Size: Average CXR size of MLC is 1.6 cm, 0.5-1.2 cm
onCT
Most common location of lung cancer is upper lobes
Observer has 50% chance to detect a nodule 1 cm in
diameter on CXR
All missed lung cancers not necessarily regarded as
malpractice
Lesional factors affecting detection
o Lesion size and shape
Smallest visible lesion in retrospect is 4 mm
30% of missed lung cancers> 2 cm
Edges of cancers usually ill-defined and more
difficult to detect
o Lesion conspicuity
Refers to nodule characteristics compared to
surrounding or overlapping structures
Missed lung cancers often obscured by
overlapping rib, clavicles, and vascular structures
Technical factors
o Poor film quality (over- or underpenetrated)
degrades detection
o Lesion more likely to be overlooked on AP portable
film
Poorer contrast due to scatter radiation from
smaller grid ratio
Patient factors such as shallow inspiratory effort,
patient motion decrease conspicuity
o Computed radiography and direct radiography
reduce technical variability
Medicolegal
o Negligence is the standard in tort law: Three
elements
Breach of standard of care (defined as action of
reasonable physician faced with similar
circumstances)
Proximate cause of injury
Substantial injury
o All missed lung cancers not necessarily regarded as
malpractice
Evidence from Mayo lung cancer screening project
suggesting most lesions visible in retrospect (90%)
Courtroom often contest of competing expert
witnesses
Lesion conspicuity often key factor in jury
deliberation
High conspicuity associated with adverse outcome
CT Findings
Missed lung cancer
Only 50% of lung nodules 3 mm or less detected at
CT
Top Differential Diagnoses
First Costochondral] unction
Granuloma
Hamartoma
Cystic lung cancer
Clinical Issues
Assuming constant growth: Typical bronchogenic
tumor life span approximately 50 doublings
Diagnostic Checklist 3
If lungs are hyperinflated, take an extra look around,
25
especially in the upper lobes
o Only 50% of lung nodules 3 mm or less detected at
CT
o Routine clinical studies: Misses
Mixture of central and peripheral lesions
Some lesions> 1 cm
More likely to be advanced stage
Lesion misses often endobronchial
Distractors often present (e.g., aortic aneurysm)
o Screening studies: Misses
As many as 30% of nodules missed on screening
CT
Missed lung cancer usually central in location
Many lesions> 1 cm
Most stage lA
Factors include detection failure and
misidentification of nodule as normal structure;
misidentification misses usually larger nodules
o Medicolegal
5-10% of missed lung cancer result in lawsuits
Plaintiff awards have been given in screening lung
cancer setting
Imaging Recommendations
Best imaging tool: Chest radiography sensitivity for
Stage I lung cancer poor
Protocol advice
o Comparison with prior studies
Failure to detect is major factor in missed lung
cancer
Advisable to compare with multiple prior
examinations
o Obtain CT for indeterminate opacities
Most often used strategy if old studies unavailable
o Obtain follow-up studies
Average doubling time of bronchogenic
carcinoma 100 days (40-400 days)
Standard is stability at follow-up at 2 years
Occasionally slower growth
o Avoidance of satisfaction of search error
Requires disipline to keep looking after initial
finding made
o Lower threshold for calling a lesion
Results in many more false positive results
 MISSED LUNG CANCER
Leads to many unnecessary work-ups and excess
expense
o Double reading
More time consuming
No guarantee of success, Mayo lung study had
many misses despite two independent readers
o Computer-aided detection (CAD)
Computer detects gray-level threshold, brightness,
lesion geometry
Approved as second reader for chest radiography
and CT: Supplements radiologist interpretation
CT CAD sensitivity for nodule detection is 71-84%
CT CAD typically detects nodules 4 mm or greater
CT CAD typically insensitive to ground glass
opacities
o Viewing protocols
26 Maximum intensity projection (MIP) makes
nodules more conspicuous from surrounding
blood vessels
Multiplanar reformations may increase
conspicuity
Cystic lung cancer
o 6% of lung cancer with wall 4 mm or less
o Occasionally air-crescent sign
I PATHOLOGY
General Features
General path comments
o Average doubling time of bronchogenic carcinoma
100 days (40-400 days)
o 7 doublings to grow from 3 mm to 1.5 cm
Epidemiology
o Missed lung cancer 6th most common malpractice
litigation in all specialties
o Missed lung cancer 2nd most common malpractice
litigation among radiologists (breast cancer #1)
o Nearly half of medicolegal actions result in
radiologist payments
o 90% of medicolegal cases chest radiographs, most of
the remainder CT

I DIFFERENTIAL DIAGNOSIS .1 I CLINICAL ISSUES

First Costochondral Junction Presentation
Appears as nodule inferior to junction Most common signs/symptoms: Asymptomatic but
Reconstructed images show connection to bone may have cough, chest pain, hemoptysis

Granuloma Demographics
Especially in epidemic areas Age: Typical age for lung cancer> 40 years old, HlV
No significant PET activity in old granulomas patients> 30 years old
Little enhancement with contrast 20 HU) Gender: More missed cancers in women

Hamartoma Natural History & Prognosis

PET negative Assuming constant growth: Typical bronchogenic
Popcorn calcification or fat tumor life span approximately 50 doublings

Round Pneumonia
Often ill-defined I DIAGNOSTIC CHECKLIST
Symptoms of infection (fever, chills)
Consider
Round Atelectasis Compare to prior films most important method to
Most often posterior lower lobes reduce errors
Supleural with pleural thickening Look beyond the obvious findings
Loss of volume Obtain follow-up studies
Comet-tailed sign of vessels extending into mass
Image Interpretation Pearls
Hematoma If lungs are hyperinflated, take an extra look around,
Trauma history especially in the upper lobes
Resolves over time
Arteriovenous Malformation
Feeding, draining vessels
I SElECTED REFERENCES
Enhances markedly with contrast 1. Rubin GD et al: Pulmonary nodules on multi-detector row
CT scans: performance comparison of radiologists and
CT Atypical Appearances of Lung Cancer computer-aided detection. Radiology. 234(1):274-83, 2005
Bronchioloalveolar cancer 2. KoJP et al: Lung nodule detection and characterization
o Minority manifest with airspace consolidation or with multislice CT. Radiol Clin North Am. 41(3):575-97, vi,
ground glass opacity 2003
3. Latief KHet al: Search for a primary lung neoplasm in
o Patients may present with copious sputum patients with brain metastasis: is the chest radiograph
production - bronchorrhea sufficient? AJRAmJ Roentgenol. 168(5):1339-44, 1997
Calcified lung cancer 4. White C5 et al: Primary carcinoma of the lung overlooked
o 19% have calcification at CT scan at CT: analysis of findings in 14 patients. Radiology.
o Usually large masses 199(1):109-15,1996
 MISSED LUNG CANCER
I IMAGE GALLERY
Typical
(Left) Frontal radiograph
shows a left upper lobe
nodule (arrow) that was not
identified prospectively
(Right) Axial CECT shows a
spiculated left upper lobe
nodule (arrow).

3
27

(Left) Frontal radiograph

shows a subtle left middle
lung (arrow) lesion that was
overlooked on initial
interpretation. (Right) Axial
CECT shows a lingular
nodule (arrow) that proved
to be lung cancer.

Typical
(Left) Axial CECT shows a
small lung lesion in the right
upper lobe that was not
recognized (arrow). (Right)
Axial CECT shows marked
enlargement of the lesion
(arrows) six months later.
Biopsy showed non-small
cell lung cancer.
 Non-Surgical
Normal Tubes and Catheters IV-4-2
Abnormal Tubes and Catheters IV-4-6
Cardiac Pacemakers IV-4-10
Pleurodesis IV-4-14

Surgical
Median Sternotomy IV-4-18
Thoracotomy IV-4-20
Esophageal Resection IV-4-24
Thoracoplasty and Apicolysis IV-4-26
Pneumonectomy IV-4-30
Lung Volume Reduction and Bullectomy IV-4-34
Lung Transplantation IV-4-38
Posttransplant Lymphoproliferative Dz IV-4-42
Lobar Torsion, Lung IV-4-46
Cardiac Volvulus IV-4-S0
 NORMAL TUBES AND CATHETERS

Graphic shows intubated patient with neck flexion the Frontal radiograph coned down view shows tip of
endO/facheal tube tip descends; with neck extension it endO/facheal tube (open arrow) in good position with
ascends. the head in neu/fal position (not shown). The tip is 5 em
4 from the carina (arrow).

2
Tip of tube 5-7 cm from carina
ITERMINOLOGY o Cervical flexion: ET tube may descend 2 cm
Abbreviations and Synonyms Tip of mandible overlies clavicles
Cardiopulmonary support and monitoring devices, Tip is now 3-5 cm from carina
lines, tubes o Cervical extension: ET tube may ascend 2 cm
Tip of mandible off film
Definitions Tip is now 7-9 cm from carina
Optimal positioning of these tubes, lines, catheters o Tube width
Ideally, at least 2/3 width of trachea
o Cuff
I IMAGING FINDINGS Should not bulge tracheal wall or narrow tube
lumen
General Features o Double lumen endotracheal tube
Best diagnostic clue: Normal and variant anatomy of Tips placed into each mainstem bronchus
accessed structure; recognition of incorrect course of Tracheostomy tubes: No mobility with cervical flexion
lines and tubes and extension
Location: Trachea, bronchi, pleura, veins, heart, aorta o Tip several cm above carina
Size: Variable o Tube width 1/2 to 2/3 width of trachea
Morphology: Variable o Cuff should not distend tracheal wall
Nasogastric or feeding tubes (NGT)
Radiographic Findings o NGT: Suction of fluid in supine position: Proper
Endotracheal tubes (Err) location fundus
o Carina is at T5 to T7 level o Suction of air in supine position: Proper location
o Err: Neutral head and neck position antrum

DDx: Permanent Lines, Tubes, Stents

Coronary Artery Stem Dual Lead Pacer

 NORMAL TUBES AND CATHETERS
Key Facts
Imaging Findings
Best diagnostic clue: Normal and variant anatomy of
accessed structure; recognition of incorrect course of
lines and tubes
ETT: Neutral head and neck position
Tip of tube 5-7 cm from carina
Cervical flexion: ET tube may descend 2 cm
Cervical extension: ET tube may ascend 2 cm
Tracheostomy tubes: No mobility with cervical
flexion and extension
NGT: Suction of fluid in supine position: Proper
location fundus
Suction of air in supine position: Proper location
antrum
Chest tubes: For pneumothorax in supine patient:
Optimal position, anterosuperior
Chest tubes
o 10-40 F in size, depending on the viscosity of the
material to be drained
o Straight, J or pigtail catheters
o Chest tubes: For pneumothorax in supine patient:
Optimal position, anterosuperior
o For hydrothorax in supine patient: Optimal
position, place posteroinferior
o Empyema, hemothorax
Must be drained early
Central venous lines (CVL)
o Access from subclavian, internal jugular, antecubital,
or femoral veins
o Peripherally inserted central catheter (PICC), smaller
catheters peripherally inserted at or slightly
proximal to the antecubital fossa
o 1-3 lumens
o CVL: Ideal position, distal superior vena cava or
upper portion right atrium
Swan-Ganz catheters, pulmonary artery catheter
o Access from subclavian, internal jugular, antecubital,
or femoral veins
o Swan-Ganz catheters: Ideal position, right or left
pulmonary artery
o Deflated balloon
o Measurement performed with balloon inflation at in
lower lobe (zone 3 of West)
Intra-aortic counterpulsation balloon ([ACPB)
o Access from common femoral artery
o Long balloon (28 cm) inflates during diastole,
deflates during systole
o IACPB: Ideal position, tip distal to left subclavian
artery
Surgically implanted catheters
o Reservoir in infraclavicular fossa, over the upper
abdomen, or over the lower ribs
o Catheter tip at distal superior vena cava
Transvenous pacemakers: Permanent or temporary
o Permanent: Pulse generator and lead wires with
electrodes for contact with the endocardium or
myocardium
Pulse generator: Infraclavicular fossa
For hydrothorax in supine patient: Optimal position,
place posteroinferior
CVL: Ideal position, distal superior vena cava or
upper portion right atrium
Swan-Ganz catheters: Ideal position, right or left
pulmonary artery
(ACPB: Ideal position, tip distal to left subclavian
artery
NECT: To plan and assess for adequate drainage of
complex pleural fluid/empyema or air collections
Best imaging tool: Radiography; CT rarely needed to
assess position of tube or catheter
Protocol advice: Radiography should always be done
after insertion of a tube or line to assure correct
position and identify complications
Single or dual leads 4
Ventricular pacer: Frontal radiograph, tip at apex
of right ventricle; lateral radiograph: Anterior 3
course, tip 3-4 mm deep to epicardial stripe
Atrial pacer: Frontal radiograph, tip directed
medially to right atrial appendage
Cardiac resynchronization therapy (CRT)
o Right or left ventricular pacing or left ventricular
pacing
o Lead placed in tributary of coronary sinus
o Option for implantable defibrillator
Automatic implantable cardioverter defibrillator (AICD
or ICD)
o Sensing and shocking electrodes, in right atrium and
right ventricle
Diaphragmatic pacing
o Radiofrequency receiver implanted in subcutaneous
tissue
o Electrode placed on phrenic nerve posterior to
clavicle
Polyurethane catheters are stiff for percutaneous
insertion, soften at body temperature
o Correctly placed catheter may normally migrate
distally with softening
CT Findings
NECT
o NECT: To plan and assess for adequate drainage of
complex pleural fluid/empyema or air collections
o Demonstrate relation between chest tube and fluid
collections
Fluoroscopic Findings
Fluoroscopic guidance for insertion of lines and tubes
Ultrasonographic Findings
Ultrasound guidance for insertion of lines and tubes
Imaging Recommendations
Best imaging tool: Radiography; CT rarely needed to
assess position of tube or catheter
 NORMAL TUBES AND CATHETERS
Protocol advice: Radiography should always be done
after insertion of a tube or line to assure correct
position and identify complications
I DIFFERENTIAL DIAGNOSIS
Pitfalls
External wires, tubes, clamps, syringes that lie on or
under the patient
Supporting lines and catheters that lack radio-opaque
markers
I PATHOLOGY
General Features
General path comments: Injury of adjacent structures
even with properly placed lines and tubes
Etiology
o Pulmonary, cardiovascular failure
4 o To titrate administration of fluids and medication
o Fluid or air collections compromising respiration or
4 cardiovascular function
Epidemiology
o Intensive care and post-operative patient:
Cardiopulmonary support devices
o Patients with arrhythmias: Pacemakers
Gross Pathologic & Surgical Features
Airway tubes: Mucosal ulceration, scarring, airway
colonization
Cardiovascular lines: Thrombus formation line tip or
occluding lumen
Microscopic Features
Line infection, cellulitis, septic emboli; tracheitis,
mucosal ulceration, fibrosis
IClINICAllSSUES
Presentation
Most common signs/symptoms
o Tracheal tubes
Intubation for patients with respiratory failure
Double lumen endotracheal tube: For differential
ventilation of the two lungs to accommodate
differences in lung compliance
o Tracheostomy: For patients requiring long term
intubation
o Nasogastric or feeding tubes: To drain stomach
contents in patients prone to aspirate or for feeding
o Chest tubes: For drainage of pneumothorax or
pleural fluid/empyema
For treatment of malignant effusion: Talc or
doxycycline administration
Non-draining chest tubes may be occluded or
poorly placed
o Concern for infection: Infected cardiovascular lines
removed and cultured
Systemic candidiasis in patients with central
venous lines and receiving broad spectrum
antibiotics
o Central venous catheters
To maintain optimal blood volume or long term
drug administration
Monitoring pressure or infusion of medication
and nutrition
o Swan-Ganz catheters .
To measure hemodynamic pressures and cardiac
output
Pulmonary capillary wedge pressure reflects left
atrial and left ventricular end-diastolic volume
o Intra-aortic counterpulsation balloon
To improve coronary artery perfusion and heart
function (after load reduction)
o Surgically implanted catheters
For long term venous access
Instillation of fluids, medications,
chemotherapeutic agents, parenteral nutritional
solutions, and blood products
Withdrawal of blood samples
o Transvenous pacemakers
For heart block or bradyarrhythmias
o CRT with implantable defibrillator
CRT improves ejection fraction, left ventricular
size, and mitral regurgitation
Prophylaxis for fatal ventricular tachyarrhythmias
Atrial synchronized ventricular pacing to optimize
atrioventricular timing
Biventricular pacing to promote ventricular
synchrony
o Automatic implantable cardioverter defibrillator
(AICD or ICD)
Patient monitoring and therapy in case of
ventricular tachycardia or fibrillation
o Diaphragmatic pacing
For patients with hypoventilation with intact
phrenic nerve and functioning diaphragm
Quadriplegia, hypoventilation - central nervous
system origin
Demographics
Age: Neonate to elderly
Gender: M = F
Natural History & Prognosis
Intubated patients with long term mechanical
ventilation requirement
o Tracheostomy tube usually placed 1-3 weeks after
endotracheal intubation
Fluoroscopy and sonography for line or tube insertion
o Safer, faster, and better than relying on anatomic
landmarks
Empyema: Poor or delayed drainage will result in
fibrothorax and require decortication
Cardiac pacemakers improve cardiac function, reduce
the severity of clinical symptoms, and reduce
mortality and morbidity
Treatment
None for properly placed lines and tubes
I SElECTED REFERENCES
1. Hunter TB et al. Medical devices of the chest.
Radiographies. 24(6):1725-46, 2004
 NORMAL TUBES AND CATHETERS

I IMAGE GALLERY

(Left) Frontal radiograph

shows left subclavian
approach for a central
venous line (arrow) with the
tip at the distal superior vena
cava in good position (open
arrow). (Right) Frontal
radiograph shows internal
jugular vein approach (open
arrow) for a right central
venous line with the tip at
the cavoatrial junction
(arrow).

4
5
(Left) Frontal radiograph
shows right chest tube
(arrows) in good position to
drain a right pneumothorax
(curved arrow). (Right)
Frontal radiograph shows left
chest tube (arrows) in good
position at the lower
hemithorax in a patient with
necrotizing pneumonia and
empyema.

Typical
(Left) Frontal radiograph
shows marker (open arrow)
for tip of aortic
counterpulsation balloon
device. Arrows outline aortic
arch calcification. The tip is
in good position distal to
origin of subclavian artery.
(Right) Frontal radiograph
shows Swan Canz catheter
(arrows) with the tip (open
arrow) in the main
pulmonary artery. The
feeding tube tip is in poor
position in the fundus of the
stomach (curved arrow).
 ABNORMAL TUBES AND CATHETERS

Tip of Swan Canz catheter is too far out in a pulmonary Frontal radiograph shows ETT tip at mid esophagus
artery sub-segmental branch. As the balloon is inflated (arrow). Note distended esophagus (curved arrow) and
there is injury of the vessel wall, pseudoaneurysm stomach with air (open arrow).
4 formation and pulmonary hemorrhage.

6
Bronchus intermedius intubation - atelectasis of
ITERMINOlOGY left tung and right upper lobe
Abbreviations and Synonyms ETT tip just beyond vocal cords: Vocal cord injury
Cardiopulmonary support and monitoring devices, Pharyngoesophageal intubation: Tube does not
lines, tubes correspond with tracheal air shadow, dilated
esophagus and stomach, aspiration, small volume
Definitions lungs
Sub-optimal positioning of these tubes, lines, catheters o Tracheolaryngeal injury
Perforation pyriform sinus: Pneumomediastinum,
subcutaneous emphysema, pneumothorax
IIMAGING FINDINGS Tracheolaryngeallaceration: Tube oriented to
right, pneumomediastinum, subcutaneous
General Features emphysema, cuff> 2.8 cm
Best diagnostic clue: Lines and tubes do not track with o Overinflated cuff: Cover tip of tube, pinch tube,
normal anatomy of the structure that is catheterized or deflect tip laterally
drained o Aspiration/pneumonia
Location: Trachea, bronchi, pleura, veins, heart, aorta Aspiration broken teeth, fillings, denture parts
during intubation; mucus plugging ETT
Radiographic Findings 5-10 ml of fluid may pool above ET cuff, cuff
Endotracheal tubes (ETT) deflation results in aspiration
o Malposition Suspect if normal air above cuff replaced with soft
Right mainstem bronchus intubation: Atelectasis tissue density
of left lung, hyperinflation right lung; sidehole Subsegmental atelectasis to overt
may ventilate left lung; right pneumothorax consolidation/pneumonia in dependent lung

DDx: Abnormal Permanent lines, Tubes, Stents

Pacer Lead Fracture Pacer Lead Thru RV Lead In Coronary Sinus

 ABNORMAL TUBES AND CATHETERS
-Key Facts
Imaging Findings
Right main stem bronchus intubation: Atelectasis of
left lung, hyperinflation right lung; sidehole may
ventilate left lung; right pneumothorax
Chest tube in the chest wall: Outer wall of chest tube
is not visible
Sidehole in chest wall may lead to massive
subcutaneous emphysema or empyema necessitans
CVL malposition: Infusion of fluid into mediastinum,
heart, pericardium, liver, pleura
CVL: Pneumothorax after placement
Swan Ganz catheter: Pulmonary infarction, from
wedged catheter with or without clot, with or
without inflated balloon tip
Torn catheter between clavicle and first rib "osseous
pinch"; embolization of catheter fragment
o Barotrauma: Alveoli are overdistended and rupture
from high peak pressures with mechanical
ventilation
Interstitial emphysema; air dissects along
bronchovascular connective tissue subpleural and
to mediastinum (Macklin effect)
Pneumomediastinum, pneumothorax
o Sinusitis with nasotracheal intubation
Tracheostomy
o Peri-operative: Hemorrhage, infection,
airleak/pneumomediastinum, pneumothorax, tube
malaligned with trachea
o Overinflated cuff: 1.S times tracheal width at level of
clavicles; cuff can cover tip of tube, pinch tube,
deflect tip laterally
Nasogastric or feeding tubes (NGT)
o Tip in bronchus, lung, or pleura; atelectasis if
occluding airway, pneumothorax if penetrate lung
o Feeding tubes: Tip in esophagus or gastric fundus,
aspiration/consolidation if fluid administered
Chest tubes
o Malposition
Inadequate drainage, air, fluid or empyema
Tube often in fissure, major or minor; track with
fissure; mayor may not function properly
Tip impinging on mediastinum may erode into
arteries, veins or esophagus
Chest tube in the chest wall: Outer wall of chest
tube is not visible
Penetrating lung resulting in bronchopleural
fistula
Sidehole in chest wall may lead to massive
subcutaneous emphysema or empyema
necessitans
Central venous lines (CVL)
o Malposition
Tip in right atrium, right ventricle, internal
jugular, upper extremity, hepatic, superior
intercostal, azygos, periscapular veins; subclavian
artery, aorta
Tip through vein wall, into pleura or mediastinum
Lines can change position over time; follow-up films
to check for change
Clinical Issues
Infection CVLs, usually staphylococcus, candidiasis
with broad spectrum antibiotics
Endotracheal or tracheostomy tubes (late sequelae)
Tracheostenosis: At stoma, cuff, tip or multiple foci,
at tip usually 1.S cm below stoma, circumferential,
1-4 cm long
Tracheomalacia: Overinflated cuff site; extra thoracic,
airway narrowing with inspiration; intrathoracic,
airway narrowing with expiration
Delayed drainage of hemothorax or empyema,
fibrothorax and decortication
Into right atrium, ventricle, pericardium, liver; 4
right ventricle: Arrhythmias
o CVL malposition: Infusion of fluid into 7
mediastinum, heart, pericardium, liver, pleura
o CVL: Pneumothorax after placement
o Mediastinal hematoma after placement
o Catheter breakage and embolization to superior or
inferior vena cava, right heart, pulmonary artery,
lung
o Aseptic or septic thrombus on catheter with
pulmonary embolization; fibrin sheath occlusion
o Thrombosis of vein
Directly related to duration of catheterization
Potential source for pulmonary emboli
o Air embolism, rare
Swan-Ganz catheters
o Swan Ganz catheter: Pulmonary infarction, from
wedged catheter with or without clot, with or
without inflated balloon tip
o Arrhythmias especially if tip in right ventricle
o Pulmonary artery pseudoaneurysm formation or
rupture due to overdistention of balloon in small
pulmonary artery
Pseudoaneurysm: Elliptical pulmonary nodule
long axis paralleling vasculature within 2 cm of
hila, usually right lung
o Pulmonary hemorrhage if aneurysm ruptures
Intra-aortic counterpulsation balloon (lACPB)
o Too high, may occlude brachiocephalic arteries,
cerebral embolus
o Too low, may occlude celiac, renal, superior
mesenteric arteries
o Aortic dissection, balloon may tear intima
o Ischemia of lower extremity on side of insertion
o Helium gas embolus from rupture of the balloon
Surgically implanted catheters
o Infection, septic emboli, thrombosis, aseptic emboli
o Torn catheter between clavicle and first rib "osseous
pinch"; embolization of catheter fragment
Transvenous pacemakers
o Malposition of leads: Unintended placement in
coronary sinus (atrioventricular groove)
 ABNORMAL TUBES AND CATHETERS
o Coronary sinus: Lead points to left humeral head on
frontal radiograph, posteriorly on lateral radiograph
I CLINICAL ISSUES
o Broken lead between clavicle and first rib "osseous Presentation
pinch" Most common signs/symptoms
o Rotation of pulse unit in the soft tissues by patient o Variable; dyspnea, wheezing, chest pain,
causing fracture or shortening of pacer lead palpitations, respiratory or cardiac failure, fever,
(twiddling sign) aspiration
o Myocardial perforation: Hemopericardium, rare o Malpositioned CVL or Swan Ganz: Incorrect pressure
CT Findings measurements
o Infection CVLs, usually staphylococcus, candidiasis
NECT
o CT to evaluate position of chest tube and with broad spectrum antibiotics
relationship to air, fluid or empyema collection o Seizures in those with air embolism
o To evaluate for tracheostenosis or malacia Other signs/symptoms
MPRs or 3D volume rendering to show trachea o With malpositioned ETTs, the higher the inspired
and bronchi during inspiration and expiration oxygen the more rapid development of atelectasis
100% inspired oxygen, immediate atelectasis with
Fluoroscopic Findings bronchial occlusion
Fluoroscopy to adjust malpositioned tubes o Fibrin sheath sign: Catheter may be flushed but not
Evaluation of trachea and larynx during inspiration aspirated
and expiration to show fixed stenosis or
Demographics
4 tracheomalacia
Age: Neonate to elderly
Contrast injection of central line may demonstrate
8 thrombus at the line tip or occlusion Gender: M = F

Imaging Recommendations Natural History & Prognosis

Endotracheal or tracheostomy tubes (late sequelae)
Best imaging tool
o Radiography should always be done after insertion o ETT: Laryngeal injury: Scarring posterior glottis,
or repositioning of a tube or line fusion posterior commissure, arytenoid injury,
o Lines can change position over time; follow-up films subglottic stenosis
to check for change o Fistulas to adjacent structures, artery, vein,
Protocol advice: Standard CT may be helpful; to esophagus
evaluate desired location, course and tip of line, tube, o Tracheostenosis: At stoma, cuff, tip or multiple foci,
catheter at tip usually 1.S cm below stoma, circumferential,
1-4 cm long
o Tracheomalacia: Overinflated cuff site;
I DIFFERENTIAL DIAGNOSIS extra thoracic, airway narrowing with inspiration;
intrathoracic, airway narrowing with expiration
External Superimposed Tubing, Lines, Delayed drainage of hemothorax or empyema,
fibrothorax and decortication
Devices
May be mistaken for intrathoracic monitoring and Treatment
support devices Tracheal stents or surgery for stenosis or malacia
Infected cardiovascular line, culture, treat with
antibiotics; line may not have to be removed, may
I PATHOLOGY clear with antibiotics
Fibrin sheath-infuse tissue plasminogen activator, if
General Features unsuccessful exchange catheter
Etiology Interventional snare retrieval for embolized catheter
o Malposition: Inexperience, patient body habitus, fragments
using anatomic landmarks for placement
o ET/tracheostomy tubes: Overinflated cuff, low
position and mobility of ETT tip I SELECTED REFERENCES
o Infection: Immunosuppressed, on broad spectrum
I. Hunter TB et al. Medical devices of the chest.
antibiotics
Radiographies. 24(6):1725-46, 2004
Epidemiology: Malposition: ETT, 20%; CVL, 33% 2. Tseng M, et al: Radioiogic placement of central venous
Gross Pathologic & Surgical Features catheters: Rates of success and immediate complications in
3412 cases. Can Assoc RadiolJ. 52 (6):379-84, 2001
Tracheal ulceration, stenosis, fistulization; venous 3. Gayer G, et al: CT diagnosis of mal positioned chest tubes.
thrombosis, thromboembolism, aseptic/septic BrJ Radiol. 73(871):786-90,2000
embolism
Microscopic Features
Culture of CVL tip may show Staphylococcus or
candida; systemic septic embolism
 ABNORMAL TUBES AND CATHETERS
I IMAGE GALLERY
Typical
(Left) Frontal radiograph
shows ETT tip at right
mainstem bronchus (white
arrow). Black arrow shows
carina. (Right) Right
posterior oblique
esophagram shows
tracheostomy tube (arrow).
Note tracheoesophageal
fistula (open arrow) and
barium aspiration at distal
trachea and main bronchi
(curved arrow).

4
9
(Left) Frontal radiograph
shows right chest tube
(arrow) tracking with major
fissure. (Right) Axial NECT in
the same patient shows right
chest tube (open arrow) at
major fissure. Large anterior
pneumothorax (arrow) not
drained.

Typical
(Left) Frontal radiograph
following CVL placement
attempt shows large superior
mediastinal hematoma (open
arrows). Note that trachea is
deviated to left (curved
arrows). (Right) Frontal
radiograph shows knotted
CVL tip (arrow). Catheter
was successfully removed
percutaneously.
 CARDIAC PACEMAKERS

Frontal radiograph shows biventricular pacemaker with Lateral radiograph shows biventricu/ar pacemaker with
leads in the right atrial appendage (curved arrow), right leads in the right atrial appendage (curved arrow), right
ventricle (arrow) and in a coronary vein on surface of ventricle (arrow) and in a coronary vein on surface of
left ventricle (open arrow). left ventricle (open arrow).

10
ITERMINOlOGY IIMAGING FINDINGS
Abbreviations and Synonyms General Features
Permanent cardiac pacing Best diagnostic clue: Position of leads within cardiac
Biventricular pacemaker chambers
o Cardiac resynchronization therapy (CRT) Location
Implantable cardioverter defibrillator (ICD) o Loose subcutaneous tissue anterior chest
Pacemaker may be positioned in left or right chest
Definitions wall
North American Society of of Pacing and Occasionally in abdominal wall for epicardial
Electrophysiology generic pacemaker code pacemakers
o First letter denotes chamber(s) paced o Lead location variable: Right atrium, right ventricle,
A = atrium; V = ventricle; D = dual (both atrium coronary vein via coronary sinus
and ventricle) Right atrium locations: Atrial appendage,
o Second letter describes which chambers sense sino-atrial node, atrio-ventricular node
electrical signals Right ventricular locations: Right ventricular apex,
A = Atrium; V = ventricle; D = dual right ventricular outflow tract
o Third letter denotes response to sensed events Left ventricular location variable in epicardial
I = inhibition; T = triggering; D = dual (inhibition location
and triggering)
Size
o Fourth letter denotes activation of rate response o Pacemaker units variable in size depending on
feature (R) function
o Leads 2-3 mm in thickness

DDx: Pacemaker Complications

Misplaced Lead
 CARDIAC PACEMAKERS

Key Facts
Imaging Findings Pneumothorax: 1.5% of procedures
Lead location variable: Right atrium, right ventricle, Dislodgement occurs in 2-3% usually within 24-48
coronary vein via coronary sinus hours of placement
Single chamber pacing Pathology
Single lead in right atrium near sinoatrial node, right
Sinus node dysfunction: Most common reason for
ventricular apex, or right ventricular outflow tract
pacemaker insertion
Dual chamber pacing
Epidemiology: Over 200,000 pacemakers placed
Leads typically positioned in right atrial appendage annually
and right ventricular apex
Biventricular pacemaker Clinical Issues
Dual chamber pacemaker distribution with third lead Most common signs/symptoms: Normally
passing through coronary sinus functioning pacemaker should not be associated with
Implantable cardioverter defibrillators symptoms
Leads are larger and have a coiled spring appearance Expected longevity of pacemaker, 5-10 years
as compared to pacemaker leads
Early complications occur in 4-5%

Morphology: Leads may exit from pacer clockwise or o Lead related complications: Perforation, 4
counterclockwise dislodgement, diaphragmatic stimulation,
malposition 11
Radiographic Findings
Perforation may result in cardiac tamponade
Single chamber pacing Dislodgement occurs in 2-3% usually within 24-48
o Single lead in right atrium near sinoatrial node, hours of placement
right ventricular apex, or right ventricular outflow Diaphragmatic stimulation due to inadvertent
tract stimulation of phrenic nerve
Dual chamber pacing Late complications occur in approximately 3%
o Leads typically positioned in right atrial appendage o Twiddler syndrome: Subconscious, inadvertent or
and right ventricular apex deliberate rotation of pacemaker in its subcutaneous
Biventricular pacemaker pocket
o Dual chamber pacemaker distribution with third Change in orientation of pacemaker unit in
lead passing through coronary sinus pocket
o Left ventricle lead situated on surface usually in Change in lead direction exiting pacemaker (e.g.
lateral or posterolateral cardiac vein clockwise to counterclockwise)
o Cardiac vein position definitively determined by Retraction of leads toward pacemaker unit
posterior position on lateral radiographs Increased number of wire loops around pacemaker
Epicardial pacemaker unit
o Pacemaker unit usually in abdominal wall with lead o Lead fracture: May be difficult to visualize if there is
on cardiac surface no displacement
o Usually situated over right ventricle Usually when pinched between clavicle & first rib
Implantable cardioverter defibrillators Suspect when lead position is not changed but
o Typically 2 electrodes, one SVC (defibrillator) and pacemaker does not capture
apex right ventricle (defibrillating and sensing)
o May be a component of biventricular pacemaker CT Findings
o Leads are larger and have a coiled spring appearance CECT
as compared to pacemaker leads o Useful for detecting chamber perforation
o Relief loop in the left subclavian region often Tip of lead projecting outside of right ventricular
constructed to help prevent lead migration myocardium
o Normally, there may be a lucency just distal to Exact location may be difficult due to beam
proximal electrode not to be confused with a hardening artifact
fracture Hemopericardium may be present;
Implantable epicardial defibrillators (anterior and hemoperitoneum uncommon
posterior cardiac patches) less common o Detection of venous thrombus
o Patches may have crenulated appearance due to Extensive venous collateral circulation in chest
local fibrosis or fluid collection beneath patch wall
o Sensing unit in chest or upper abdominal wall
Detection of radiographically evident complications MR Findings
Early complications occur in 4-5% MR relatively contraindicated in patients with
o Pneumothorax: 1.5% of procedures pacemaker
o Hemothorax: Due to trauma to subclavian artery o Absolute contraindication in pacemaker dependent
patients
 CARDIAC PACEMAKERS
a Can be performed safely in patients with demand
pacemakers, particularly exams remote from chest
(e.g., brain)
Pacemaker should be pretested outside MR suite
Cardiologist in attendance during procedure
Monitor during examination, communicate with
patient between sequences
Post test pacemaker outside MR suite
Significant change in pacing thresholds in
approximately 10%
Fluoroscopic Findings
Fluoroscopy may be useful to examine leads for
incomplete fracture or dislodgement
Ultrasonographic Findings
May be used to assess venous complications or fluid
around pacemaker unit
a Symptomatic venous thrombosis in 5%; risk
increases with multiple leads
4 Imaging Recommendations
Best imaging tool: Frontal and lateral chest
12 radiographs for initial evaluation and follow-up for
complications
I DIFFERENTIAL DIAGNOSIS
Other Implanted Pacing Devices in Chest
Wall
Vagal nerve stimulator: Leads extend into neck,
terminate in region of carotid artery
Deep brain stimulator: Leads continue cephalad
Retained leads from previously removed pacemaker
I PATHOLOGY
General Features
Etiology
a Indications for pacemaker placement
Sinus node dysfunction: Most common reason for
pacemaker insertion
Long term therapy for symptomatic bradycardia
Neurocardiogenic syncope
Hypertrophic obstructive cardiomyopathy
Congestive heart failure; cardiac
resynchronization therapy
Epidemiology: Over 200,000 pacemakers placed
annually
IClINICAllSSUES
Presentation
Most common signs/symptoms: Normally functioning
pacemaker should not be associated with symptoms
Other signs/symptoms
a Pacemaker syndrome: Constellation of symptoms
related to ventricular pacing
Congestive: Dyspnea, orthopnea, elevated neck
veins, hepatomegaly, pedal edema
Hypotensive: Near syncope or syncope at pacing
onset, decrease in systolic pressure> 20 mmHg
Nonspecific: Headache and fatigue
a Atrio-ventricular block in single chamber right atrial
pacing
a Sudden cardiac arrest due to conduction
disturbances related to malposition or malfunction
a Pacemaker pocket complications: Hematoma, pain,
infection
Demographics
Age: Variable depending on condition; majority over
SO years of age
Gender: More frequently placed in men
Natural History & Prognosis
Expected longevity of pacemaker, 5-10 years
In patients with bacteremia: Risk for infected
thrombus on leads and subsequent septic emboli to
lungs
Treatment
Replacement fractured wires
Perforation treated by withdrawing wire and
rescrewing into myocardium
Surgical replacement of pacemaker unit at battery
end-of-Iife
I DIAGNOSTIC CHECKLIST
Consider
Lead fracture and dislodgement when history of
pacemaker malfunction
Image Interpretation Pearls
Evaluation of pacemaker should be performed with all
other extraneous superficial leads removed
Carefully evaluate pacemaker unit to look for change
in orientation
Compare direction of leads exiting pacemaker unit to
prior radiographs and initial post placement
radiograph
I SELECTED REFERENCES
1. Brown DW et al: Epidemiolob'Y of pacemaker procedures
among Medicare enrollees in 1990, 1995, and 2000. Am J
Cardiol. 95(3):409-11, 2005
2. Lamas GA et al: Evidence base for pacemaker mode
selection: from physiology to randomized trials.
Circulation. 109(4):443-51,2004
3. Loewy J et al: Reconsideration of pacemakers and MR
imaging. Radiographies. 24(5):1257-67; discussion 1267-8,
2004
4. Martin ET et al: Magnetic resonance imaging and cardiac
pacemaker safety at 1.5-Tesla. J Am Coli Cardiol.
43(7):1315-24,2004
5. Trohman RG et al: Cardiac pacing: the state of the art.
Lancet. 364(9446):1701-19, 2004
6. Wiegand UK et al: Long-term complication rates in
ventricular, single lead VDD, and dual chamber pacing.
Pacing Clin Electrophysiol. 26( 10): 1961-9, 2003
7. Daly BD et al: Nonthoracotomy lead implantable
cardioverter defibrillators: Normal radiographic
appearance. AJR. 161:749-52, 1993
 CARDIAC PACEMAKERS
I IMAGE GALLERY
Typical
(Left) Frontal radiograph
shows single lead ICD with
typical coil spring
appearance of/ead (curved
arrows) and lead positioned
in right ventricular outflow
tract (arrow). (Right) Frontal
radiograph shows implanted
rectangular epicardial
patches (open arrows) with
crenulated margins over
surface of right and left
ventricle. Leads extend
inferiorly (arrow) to ICD
unit.

4
13
(Left) Frontal radiograph
shows dual lead pacemaker
following lead replacement.
Previous leads (open arrows)
are left in situ with
functioning leads in right
atrial appendage (curved
arrow) and right ventricular
apex (arrow). (Right) Axial
CECT shows expected
location of ventricular leads
of biventricular pacemaker
with lead positioned in right
ventricular apex (arrow) and
in coronary vein on surface
of left ventricle (open
arrow).

Variant
(Left) Frontal radiograph
shows bivenlricular
pacemaker leads via left
superior vena cava and
coronary sinus. Leads are at
AV node (curved arrow),
right ventricular apex
(arrow) and left ventricular
surface (open arrow). (Right)
Frontal radiograph shows
dual lead ICD in patient with
corrected transposition of
great arteries. Leads are in
morphologic right atrium
(curved arrow) and right
ventricle (arrow) in anatomic
position of left atrium and
ventricle.
 PLEURODESIS

Frontal radiograph shows chest tube in place and Axial CECT shows lobular circumferential pleural
diffuse pleural thickening with remaining posterior thickening with scattered focal calcification (curved
loculated fluid pocket medially (open arrows). arrow). An anterior pleural drain is also present (arrow).
4
14
Chemotherapy agents: Cisplatin, doxorubicin,
ITERMINOLOGY etoposide, fluorouracil, mitomycin-C
Abbreviations and Synonyms Tetracycline derivatives: Doxycycline,
Pleural sclerosis minocycline
Other agents: Powdered collagen, distilled water,
Definitions Betadine, interferon-beta
Mechanical or physical methods used to fuse the o Potential new agent with fewer side effects:
parietal and visceral pleura and prevent fluid or gas Transforming growth factor-beta
accumulation In rabbits, can induce fibrosis without
o Most frequently using talc inflammation
Hydrated magnesium silicate Also used for recurrent benign effusions
Inexpensive, can be stored o Related to congestive heart failure (CHF)
Generally shows highest success rate for o Related to transdiaphragmatic transfer of ascites
malignan t effusions of all agen ts o Related to systemic lupus erythematosis (SLE)
4-8% risk of respiratory failure after talc exposure Also useful for recurrent pneumothorax
Side effects may be related to smaller particle size o Most often related to chronic obstructive pulmonary
o Can be done with bleomycin disease (COPD)
More expensive o May also be spontaneous
Readily available, must be used immediately o May be seen in lymphangioleiomyomatosis
o Third most common agent is tetracycline o Catamenial pneumothorax: Due to thoracic
Not currently commercially available endometriosis
Inexpensive, can be stored Also used for chylothorax
o Physical abrasion can also be used o After coronary artery bypass graft (CABG)
o A wide variety of other agents have been tried o After esophagectomy

DDx: Other Calcified Lesions

Pleural Metastases Hematoma Tuberculosis

 PLEURODESIS
Key Facts
Terminology Diffuse pleural thickening
Mechanical or physical methods used to fuse the Usually unilateral
parietal and visceral pleura and prevent fluid or gas May have associated loculated pleural fluid
accumulation Best imaging tool: Chest CT
Chemotherapy agents: Cisplatin, doxorubicin, Top Differential Diagnoses
etoposide, fluorouracil, mitomycin-C
Pleural Metastasis
Tetracycline derivatives: Doxycycline, minocycline
Empyema
Other agents: Powdered collagen, distilled water, Pleural Tuberculosis
Betadine, interferon-beta Pleural or Extrapleural Hematoma
Also useful for recurrent pneumothorax Mediastinal Mass
Overall success ranges from 50-90% Calcified Asbestos Plaques
Main side effects are pain and fever
Higher fever may increase chance of success Pathology
Various agents used, all producing damage to
Imaging Findings
mesothelium and fibrosis
High attenuation pleural density
Calcification may not be visible on plain film

o Trauma-related o Calcification may not be visible on plain film 4

o After heart-lung transplant o Diffuse pleural thickening
Similar process can be used for recurrent symptomatic 15
May be smooth
pericardial effusion May be nodular
Overall success ranges from 50-90% o Costophrenic angle blunting
o Decreased in patients on steroids o Usually unilateral
o May be decreased by non steroidal May occasionally be done bilaterally if pleural
anti-inflammatories (NSAIDs) metastases are bilateral
o Requires host inflammatory response for fusion to
occur CT Findings
o Generally requires complete drainage of air or fluid High attenuation within pleural space
before instillation of agents Usually unilateral, unlike asbestos-related pleural
o Also requires lung to be expandable calcifications
o Contraindicated in trapped lung (non expandable) May have associated pleural thickening
Main side effects are pain and fever o Smooth
o Higher fever may increase chance of success o Nodular
o May indicate more robust inflammatory response May have associated loculated pleural fluid
o Also risk of re-expansion pulmonary edema if fluid Imaging Recommendations
is too rapidly drained
Best imaging tool: Chest CT
Protocol advice: IV contrast not needed
I IMAGING FINDINGS
General Features I DIFFERENTIAL DIAGNOSIS
Best diagnostic clue Pleural Metastasis
o High attenuation pleural density
May produce relatively smooth rind
Loculated pleural fluid pockets
Most often lobular
Diffuse pleural thickening May be solid, fluid or mixed attenuation
Location May be unilateral or bilateral
o Pleural
Most are not calcified
o Often basal o Except metastases from some mucinous tumors,
o May be medial or lateral bone tumors, cartilage tumors
Size: Variable
Morphology Empyema
o Peripheral Generally lens shaped or lobular
o Lentiform, lens-shaped Most often mixed fluid and soft tissue attenuation
o Variable central attenuation Gas is very rare except after instrumentation
May have central fluid Most are not calcified
May have peripheral calcification Generally unilateral
May be soft tissue density throughout
Pleural Tuberculosis
Radiographic Findings Thick pleural rind
Radiography Often calcified late
 PLEURODESIS
o Calcium is chunky and thick
Generally unilateral
IClINICAllSSUES
Pleural or Extrapleural Hematoma Presentation
Diffuse or localized pleural thickening Most common signs/symptoms: Symptomatic
Often mixed attenuation recurrent pleural fluid
Generally unilateral Other signs/symptoms
May have minimal calcification o Shortness of breath
o Chest wall pain
Mediastinal Mass o Fatigue
May mimic medial pleural mass or fluid
May be calcified, generally central rather than
Demographics
peripheral Age: Often over 40, for malignant causes
Gender: M = F
Chest Wall Mass
May mimic pleural mass
Natural History & Prognosis
May be calcified depending on origin Best results if fluid can be completely drained prior to
o Bony or cartilaginous tumors most often calcified procedure
o Generally diffuse or central calcifications o Also vital to be sure that collapsed lung can
re-expand to fill the space
Calcified Asbestos Plaques o If lung is trapped, or has been collapsed for
4

Focal, not diffuse
Generally bilateral
extended period, pleurodesis is likely to fail
More difficult to perform if pleural space already has
Often squared in shape adhesions
16
Most often located along diaphragm domes, adjacent May be repeated, but results are generally best after
to inner surface of ribs initial treatment
May be calcified or soft tissue attenuation
o In individual patient, some may be calcified and
some non-calcified I DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
I PATHOLOGY Diffuse mild pleural thickening with calcification
In planning for procedure, it is important to drain all
General Features fluid
Etiology o May require image guided drainage
o Used for treatment of recurrent pleural effusions o May require repeat CT scanning to determine
o Various agents used, all producing damage to location and loculation
mesothelium and fibrosis o May require surgical drainage
Talc
Visible as crystals under polarized light
Bleomycin I SElECTED REFERENCES
Tetracycline I. Bennett R et al: Management of malignant pleural
Epidemiology effusions. Curr Opin Pulm Med. 11(4):296-300,2005
o Most frequently used in cancer patients 2. Marrazzo A et al: Video-Thoracoscopic Surgical Pleurodesis
o May also be used in benign recurrent effusions, in the Management of Malignant Pleural Effusion: The
chylothorax Importance of an Early Intervention. J Pain Symptom
o May also be used in recurrent pneumothorax Manage. 30(1):75-79, 2005
3. Yildirim E et al: Rapid pleurodesis in symptomatic
Associated abnormalities
malignant pleural effusion. EurJ Cardiothorac Surg.
o Other signs of tumor, if done for malignant process 27(1):19-22,2005
Adenopathy 4. Haddad FJet al: Pleurodesis in patients with malignant
Lung metastases pleural effusions: talc slurry or bleomycin? Results of a
Pleural masses prospective randomized trial. World J Surg. 28(8):749-53;
o Other signs of COPD, if done for recurrent discussion 753-4,2004
pneumothorax 5. Ukale V et al: Inflammatory parameters after pleurodesis in
Bullae recurrent malignant pleural effusions and their predictive
Vascular attenuation value. Respir Med. 98(12):1166-72, 2004
6. West SD et al: Pleurodesis for malignant pleural effusIons:
Paraseptal emphysema current controversies and variations in practices. Curr Opin
Gross Pathologic & Surgical Features Pulm Med. 10(4):305-10,2004
7. Bondoc AYet al: Arterial desaturation syndrome following
Dense fibrosis in pleural space pleurodesis with talc slurry: incidence, clinical features,
Loculated fluid collections and outcome. Cancer Invest. 21(6):848-54,2003
Microscopic Features
Dense fibrosis
Talc
 PLEURODESIS
I IMAGE GAllERY
Other
(Left) Frontal radiograph
shows large left malignant
effusion in a patient with
non-Hodgkin lymphoma.
This patient is a good
candidate for pleurodesis as
CT shows no /oeulations.
(Right) Axial CECT shows
large left malignant effusion
with a chest tube in place,
marked compression of the
left lung and mediastinal
shift. Pleurodesis will require
initial complete drainage.

4
Typical 17
(Leh) Axial NECT shows
diffuse slightfy lobular right
pleural thickening (arrows)
without calcification after
pleurodesis. (Right) Axial
CECT shows diffuse right
pleural thickening extending
into the major fissure
(arrows) after right
pleurodesis.

Variant
(Left) Axial CECT shows
loculated fluid extending inlO
an azygos fissure (arrows)
after talc pleurodesis. (Right)
Axial CECT shows a
subpulmonic loculation of
fluid (arrows) after talc
pleurodesis.
 MEDIAN STERNOTOMY

Drawing shows normal sternotomy (left) and Frontal radiograph (coned down image) shows a
dehiscence (middle and right). Note displacement of midsternal stripe (arrows) measuring> 3 mm in patient
sternal wires (blue arrows) and midline sternal stripe with sternaldehiscence.
4 (red arrows), signs of dehiscence.

18 o Atelectasis in bases (90%) left> right

ITERMINOLOGY o Edema, usually mild
Definitions Mediastinal bleeding
Most common type of incision for cardiac operations o Initial recovery room film baseline mediastinal
(especially coronary artery bypass surgery) and width
anterior mediastinal masses o May normally slightly increase in width first 24
Extends from suprasternal notch to below xyphoid; hours
closed with 4-7 stainless steel wires, which normally o Progressive widening after 24 hours or rapid change
form a straight, vertical row in width in first 24 hours are concerning for
bleeding
Sternal dehiscence
o "Wandering wires" sign is moderately sensitive (>
IIMAGING FINDINGS 80%) and highly specific
General Features Refers to displacement or rotation of wires as a
Best diagnostic clue change from baseline radiograph
o Best CXR clue of dehiscence is "wandering wires" o "Midsternal stripe" (lucency> 3 mm) is a rare sign of
o Best CXR clue of bleeding is progressive mediastinal dehiscence
widening CT Findings
o Best CT clues of mediastinitis are localized
CTA: Can be used to separate acute aortic dissection
mediastinal fluid collection and mediastinal gas
from rebleeding
Radiographic Findings CECT
Immediate recovery room film, expected findings o CT is best modality for evaluating post-operative
o Position of tubes and catheters infections of sternum and mediastinum

DDx: Post-Operative Mediastinal Widening

Dehiscence Hematoma
 MEDIAN STERNOTOMY

Key Facts
Terminology Best CXR clue of bleeding is progressive mediastinal
Most common type of incision for cardiac operations widening
(especially coronary artery bypass surgery) and Best CT clues of mediastinitis are localized
anterior mediastinal masses mediastinal fluid collection and mediastinal gas
Extends from suprasternal notch to below xyphoid; CT is best modality for evaluating post-operative
closed with 4-7 stainless steel wires, which normally infections of sternum and mediastinum
form a straight, vertical row Pathology
Imaging Findings Overall mortality low (1%), but up to 50% with major
Best CXR clue of dehiscence is "wandering wires" complications

Sternum: Dehiscence (sternal separation);

osteomyelitis (irregularity, periosteal new bone,
I CLINICAL ISSUES
sclerosis); fluid collections Presentation
Mediastinitis: Mediastinal gas and localized fluid
Rebleeding
collections; high specificity after 14 days o General indications: > 1,500 ml blood loss, excessive
Other Modality Findings mediastinal drainage
Nuclear medicine findings
o Bone scintigraphy and gallium scans can help to
Dehiscence
o Asymptomatic or nonspecific chest pain; sternal
4
evaluate for osteomyelitis of the sternum "click" on physical exam
19
Imaging Recommendations Natural History & Prognosis
Best imaging tool: Contrast-enhanced CT Rebleeding occurs generally within first 24 hours
Dehiscence or mediastinitis most common 10-14 days
Treatment
I DIFFERENTIAL DIAGNOSIS Rebleeding requires reoperation
Aortic Dissection Drain fluid collections for mediastinitis
Surgical debridement for dehiscence
May also cause mediastinal widening in post-operative
period
Identification of luminal flap on CTA
I SELECTED REFERENCES
1. Boiselle PM et al: Wandering wires: frequency of sternal
I PATHOLOGY wire abnormalities in patients with sternal dehiscence. AJR
Am J Roentgenol. 173(3):777-80, 1999
General Features 2. Jolles H et al: Mediastinitis following median sternotomy:
CT findings. Radiology. 201(2):463-6, 1996
Epidemiology 3. Goodman LR et al: Complications of median sternotomy:
o Coronary artery bypass grafting most frequently computed tomographic evaluation. AJR Am J Roentgenol.
performed thoracic operation, valve replacement #2 141(2):225-30,1983
o Overall mortality low (1%), but up to 50% with
major complications

I IMAGE GALLERY

(Left) Axial NECT shows a localized retrosternal fluid collection fopen arrow) and mediastinal gas (arrow) in a patient with mediastinitis 15 days
after CABG. (Center) Frontal radiograph shows normal vertical alignment (arrows) of sternal wires on post-operative day 7. Slight offset to right
is due to slight rotation of patient. (Right) Frontal radiograph of same patient, center image obtained several days later shows rightward
displacement (arrows) of several wires, due to sternal dehiscence (I'wandering wires/l).
 THORACOTOMY

Frontal radiograph shows staple lines (arrows), sites 01 Axial CECT in a post-operative patient shows a large
wedge resections for metastatic adenocarcinoma. 4 em right effusion with floating regions of increased
mass right lower lobe (curved arrow) represents attenuation (arrows). Diagnosis: Hemothorax.
4 recurrence.

20 o Post-pneumonectomy: Contralateral mediastinal

!TERMINOLOGY shift, consider hemothorax, chylothorax, empyema,
Abbreviations and Synonyms Bp fistula, esophagopleural fistula, recurrent tumor
Pulmonary resection; post-operative (post-op); (late)
bronchopleural fistula (BP fistula) Radiographic Findings
Definitions Radiography
Pneumonectomy: Intrapleural, extra pleural, o Pneumonectomy
intrapericardial, sleeve pneumonectomy Post-ope Expanded contralateral lung, midline
Lobectomy, limited resection (sleeve lobectomy, trachea, air-filled post-pneumonectomy space
segmentectomy, nonanatomic parenchyma-sparing < 7 days: Mediastinum remains midline or shifts
resection), wedge resection toward surgery side
7 days post-pneumonectomy: Air-fluid level at
lower 1/2 to 2/3 of hemithorax
IIMAGING FINDINGS Complete filling post-pneumonectomy space with
fluid, 2-4 months
General Features Opaque post-pneumonectomy space, permanent
Best diagnostic clue mediastinum shift to surgery side
o Post-op rapidly enlarging pleural effusion may Right pneumonectomy: Heart shifts to right and
represent hemothorax, chylothorax or empyema posteriorly; left lung herniates anterior to heart
o Pleural fluid with air-fluid levels or air bubbles, Left pneumonectomy: Heart shifts to left; right
consider BP or esophagopleural fistula lung herniates anterior or posterior to heart
o Lobectomy

DDx: Pneumonectomy or lobectomy

Lung Collapse Lobar Collapse Malignant Effusion

 THORACOTOMY

Key Facts
Imaging Findings Top Differential Diagnoses
Post-op rapidly enlarging pleural effusion may Complete Lung or Lobar Atelectasis
represent hemothorax, chylothorax or empyema Radiation Therapy
Pleural fluid with air-fluid levels or air bubbles, Pleural Effusion
consider BP or esophagopleural fistula
Post-pneumonectomy: Contralateral mediastinal Pathology
shift, consider hemothorax, chylothorax, empyema, Bronchial stump breakdown: Due to surgical
BP fistula, esophagopleural fistula, recurrent tumor complication, ischemia, radiation or infection or
(late) recurrent tumor
7 days post-pneumonectomy: Air-fluid level at lower Clinical Issues
1/2 to 2/3 of hemithorax
Pneumonectomy: Mortality 6%; morbidity up to
Complete filling post-pneumonectomy space with
60%; lobectomy: Mortality 2%; morbidity up to 40%
fluid, 2-4 months
Opaque post-pneumonectomy space, permanent Diagnostic Checklist
mediastinum shift to surgery side
.
CECT to show recurrent tumor at the bronchial
stump, a common location

Ipsilateral mediastinal shift; returns to midline or a Complication, postpneumonectomy syndrome: 4

close to midline as remaining lobes on surgical
side hyperinflate
Hilum shifts toward lobectomy site; ipsilateral
diaphragmatic elevation
Clips at bronchus, staples along incomplete fissure
Reorientation of remaining lobes; with right upper
or lower lobectomy, right middle lobe rotates to
fill vacated space
Appearance resembles atelectasis of lobe that is
resected; juxta phrenic peak for upper lobe
resection
o Segmental or subsegmental resection
Staples and linear scar at surgery site
For solitary pulmonary nodule, shelled out nodule
may fill with blood, thus nodule may "reappear"
following surgery
a Pneumothorax
Air leak more common with smaller segmental or
wedge resections, requires several days of chest
tube drainage
o Air space pattern following thoracotomy
Edema: Typically diffuse with septal lines,
peribronchial cuffing and diffuse airspace
opacities, and small bilateral effusions
Atelectasis: Most commonly subsegmental in the
dependent lung, especially the medial basilar
segment left lower lobe
Pneumonia: Similar appearance to atelectasis or
edema, suspect with progressive worsening of
radiographic abnormalities
Hemorrhage: From surgical contusion at the
operative site, more common with wedge
resections
Adult respiratory distress syndrome (ARDS):
Peripheral diffuse airspace opacities similar pattern
to cardiac pulmonary edema
o Complication, lung herniation: Through surgical
incision in chest wall
Accentuated by expiration; progressive separation
of involved ribs
Most common following right pneumonectomy
Late complication due to marked hyperinflation 21
of left lung
Left mainstem bronchus compressed by
mediastinal vascular structures and spine
o Complication, cardiac herniation: Most common
following right pneumonectomy
Cardiac apex laterally or posteriorly directed
against right chest wall
o Complication, lobar torsion: Most common with
right upper lobectomy, torsion of right middle lobe
Large dense lobe, followed by volume loss, pleural
effusion
Hilum displaced opposite direction from expected
lobe resected
o Complication, post-operative space: Excessive fluid
or air in pleural space
Hydrothorax, from poorly positioned chest tube
or mal positioned intravenous catheter
Hemothorax, early accumulation due to arterial
bieeding
Chylothorax, late complication from thoracic duct
injury, seen when patient begins eating in the
post-operative period
Empyema, late in post-op period
Post-pneumonectomy BP fistula, early due to
inadequate surgical technique or infection of the
bronchial stump
Post-pneumonectomy BP fistula, late (months):
Recurrent malignancy
BP fistula: Failure to fill with pleural fluid, a drop
of> 2 cm of in previous location air-fluid level, or,
persistent pneumothorax despite functioning
chest tubes
CT Findings
CECT
o Pleural space
Pleural enhancement and air bubbles, common
and not specific for empyema
Hemothorax, acutely high attenuation fluid or
fluid-fluid level
 THORACOTOMY
Chylothorax: Measurement of attenuation of a Esophagopleural fistula: Due to surgical
pleural fluid not helpful complication, adenitis, empyema, recurrent tumor
a Air-leak a Torsion of a lobe or lung: Remaining lobe rotates on
Bronchial stump breakdown may be its bronchovascular pedicle, 180-degree torsion leads
demonstrated, more commonly suspected due to to ischemia, infarction, gangrene
clustered air bubbles around stump (or along Most common immediately post-operative
esophagus for esophageal-pleural fistula) a Cardiac herniation through a pericardial defect;
a Gossypiboma: Usually due to retained surgical following intrapericardial pneumonectomy, usually
sponge right side
Sharply defined, round mass with a Within 24 hours following pneumonectomy
high-attenuation central portion and an Epidemiology
enhancing wall a Persistent pneumothorax 10-20%; bronchopleural
Cystic masses with an infolded pattern fistula, 2-13%, more common on right
a Post-pneumonectomy empyema, 2-16%
Fluoroscopic Findings
Esophagram: Nonionic contrast useful to evaluate
esophagopleural fistula ICLINICAl ISSUES
Imaging Recommendations Presentation
Best imaging tool: Radiography, usually suffices for Most common signs/symptoms: Fever common, seen
post-op follow-up with atelectasis or infections
4 Protocol advice: CECT to evaluate post-op
bronchopulmonary and pleural complications Natural History & Prognosis
22 Esophagopleural fistula: Most within 6 weeks of
surgery
I DIFFERENTIAL DIAGNOSIS Delayed complications
a Post right pneumonectomy syndrome: Distal
Complete Lung or Lobar Atelectasis trachea and left main bronchus compressed between
No prior surgery, no hilar clips, CT shows atelectatic aorta and pulmonary artery
lobe with intact bronchus, pulmonary artery and vein a Post left pneumonectomy syndrome: Upper lobe
Radiation Therapy bronchus, bronchus intermedius, or right middle
lobe bronchi compressed between pulmonary artery
Outcome similar to surgery with irradiated lung
and spine
removed by cicatricial atelectasis and scarring
a Recurrent tumor may present as empyema, BP
No hilar clips or chest wall surgical changes
fistula
CT better demonstrates nonanatomic boundaries of
Pneumonectomy: Mortality 6%; morbidity up to 60%;
irradiated lung
lobectomy: Mortality 2%; morbidity up to 40%
Pleural Effusion Bronchopulmonary fistula: Mortality 30-70%, from
No ipsilateral mediastinal shift; ultrasound or CT to aspiration pneumonia and ARDS
show effusion
Treatment
Post-op space complications: Treated with prolonged
chest tube drainage and antibiotics
I PATHOLOGY a Hemothorax: Reoperation; delay in treatment may
General Features result in fibrothorax and require decortication
Etiology
a Pulmonary opacities result of surgical manipulation,
mechanical ventilation, narcotics, splinting, poor I DIAGNOSTIC CHECKLIST
cough reflex, aspiration
Image Interpretation Pearls
a Air-leak from incomplete fissures, suture line,
CECT to show recurrent tumor at the bronchial
bronchopleural fistula
stump, a common location
a Bronchial stump breakdown: Due to surgical
complication, ischemia, radiation or infection or
recurrent tumor
I SELECTED REFERENCES
a Empyema: Surgical contamination or due to
bronchopleural fistula 1. Kim EAet al: Radiographic and cr Findings in
Develops most commonly more than 1 week Complications Following Pulmonary Resection.
following surgery Radiographies 22(1):67-86, 2002
2. Bhalla M: Noncardiac thoracic surgical procedures.
a Hemothorax: From systemic, intercostals,
Definitions, indications, and postoperative radiology.
mediastinal vessel laceration Radial Clin North Am 34(1):137-55, ]996
Most commonly early complication in the 3. Seo)B et al: Neofissure after lobectomy of the right lung:
immediate post-operative period radiographic and cr findings. Radiology. 201(2):475-9,
1996
 THORACOTOMY

I IMAGE GALLERY
Typical
(Left) Frontal radiograph
postoperative after right
upper lobectomy shows
rightsided shift of the
trachea (curved arrow) and
a right chest tube (arrow).
(Right) Frontal radiograph in
same patient the following
day shows opacification of
the repositioned right middle
lobe (arrows). Bronchoscopy
excluded lobar torsion and
mucus plugging

4
Typical 23
(Left) Frontal radiograph
post-pneumonectomy shows
airfilled left hemithorax
(arrow). Note shift of heart
and mediastinum to the left
(open arrows). (Right)
Lateral radiograph in same
patient shows relroslernal
lucency (arrows) that
represents herniation rightor
lung anterior to heart.

Variant
(Left) Frontal radiograph in
the same patient 2 weeks
later shows airfluid level
(arrow). Shift of the heart to
the right (open arrow) and
air bubbles (curved arrows)
suggest empyema. (Right)
Axial CECT in the same
patient shows thickened
enhancing pleura, air
bubbles, airfluid level
(arrows) and concave
mediastinal pleura (curved
arrow). Diagnosis:
Post-pneumonectomy
empyema.
 ESOPHAGEAL RESECTION

Frontal radiograph shows appearance following right Esophagram shows an esophagogastrectomy and a
thoracotomy & esophagectomy. Mediastinum widened normal esophagogastric anastomosis (arrow).
(arrows) from gastric pull-up. Intrathoracic NG tube
4 crosses anastamosis (curved arrows).

24 o Benign and malignant lesions of the distal

ITERMINOlOGY esophagus, gastroesophageal junction, and gastric
Synonyms cardia
Esophagectomy Radical en bloc esophagectomy
o Potentially curable tumor in pre and intra-operative
Types of Esophagectomy staging
Transthoracic esophagectomy through a right Antireflux repairs
thoracotomy o Through a thoracotomy (Belsey Mark IV operation)
Transhiatal esophagectomy without thoracotomy o Through a laparotomy (Nissen, Hill, and Guarner
Transthoracic esophagectomy through a left procedures)
thoracotomy
Contraindications
Radical en bloc esophagectomy
Transthoracic esophagectomy through a right
thoracotomy
I PRE-PROCEDURE o Esophageal carcinomas located within 20 em of the
incisors
Indications o Patients with a previous right thoracotomy
Transthoracic esophagectomy through a right Transhiatal esophagectomy without thoracotomy
thoracotomy o Tracheobronchial or aortic involvement
o Carcinoma involving the upper two-thirds of the Peri-Operative Imaging
esophagus
Chest radiograph
o High grade dysplasia in Barrett esophagus
o Often helpful in detecting complications
o Destruction of the lower two thirds of the esophagus
Widened mediastinum
by caustic ingestion
Pneumomediastinum
o Complications of reflux esophagitis failed
Cervical or subcutaneous emphysema
Transhiatal esophagectomy without thoracotomy
Pleural effusion
o Curative or palliative resection of
Contrast studies using water soluble contrast agents
thoracic-cervicothoracic esophageal carcinoma
(Gastrografin)
o Removal of thoracic esophagus after pharyngectomy
o Exclude a leak into mediastinum
or pharyngolaryngectomy for esophageal carcinoma
o Esophageal stricture CT
o Intrathoracic anastomosis and metallic clips
o Neuromotor dysfunction (achalasia, spasm,
o Mediastinal fluid collections
scleroderma)
o Post-surgicallymphocele
o Recurrent gastroesophageal reflux
o Vascular injuries
o Perforation
o Caustic injury Follow-Up
Transthoracic esophagectomy through a left CTand MR
thoracotomy
 ESOPHAGEAL RESECTION

Key Facts
Terminology Recurrent laryngeal nerve injury
Transthoracic esophagectomy through a right Most common: Pneumothorax, pneumonia, pleural
thoracotomy effusion, and aspiration bronchiolitis
Transhiatal esophagectomy without thoracotomy Anastomotic leak
Transthoracic esophagectomy through a left Mediastinitis and sepsis
thoracotomy Tumor recurrence
Radical en bloc esophagectomy Tumor of the esophageal substitute (stomach or
colon)
Problems & Complications
Hemorrhage

o Tumor recurrence Ulceration of the esophageal substitute

Postvagotomy dumping
Other Complications
I PROBLEMS & COMPLICATIONS o Tumor recurrence
o Tumor of the esophageal substitute (stomach or
Complications colon)
Most feared complication(s)
o Intra-operative complications 4
Hemorrhage I SELECTED REFERENCES
Injury to the tracheobronchial tree 25
1. Meloni GB et aI: Postoperative radiologic evaluation of the
Recurrent laryngeal nerve injury esophagus. Eur) Radiol. 53(3):331-340, 2005
o Post-operative complications 2. Kantarci M et al: Comparison of CT and MRI for the
Most common: Pneumothorax, pneumonia, diagnosis recurrent esophageal carcinoma after operation.
pleural effusion, and aspiration bronchiolitis Dis Esophagus. 17: 32-37, 2004
Atelectasis 3. Kim 5H et al: Esophageal Resection: indications,
Adult respiratory distress syndrome techniques, and radiologic assessment. RadioGraphies.
Delayed hemorrhage 21:1119-1140,2001
Anastomotic leak 4. Tilanus HW et al: Esophagectomy with or without
thoracotomy: is there any difference? ) Thorac Cardiovasc
Mediastinitis and sepsis
Sorgo 105:898-903, 1993
Arrhythmia, myocardial infarction, pericardial 5. Page RD et al: Esophagogastrectomy via left
tamponade thoracophrenotomy. Ann Thorac Surg. 49:763-766, 1990
Delayed gastric emptying 6. Becker CD et al: CT evaluation of patients undergoing
Chylothorax transhiatal esophagectomy for cancer.) Comput Assist
Herniation of abdominal viscera through the Tomogr.IO:607 -611, 1986
hiatus 7. Agha FP et al: Colonic interposition: radiographic
o Functional complications evaluation. A)R Am) Roentgenol. 142:703-708, 1984
8. Skinner DB. En bloc resection for neoplasms of the
Anastomotic stricture
esophagus and cardia.) Thorac Cardiovasc Surg. 85:59-69,
Redundancy and impaired emptying 1983
Obstruction at the upper thoracic inlet or
diaphragmatic hiatus
Reflux esophagitis

[IMAGE GALLERY

(Left) Lateral chest view shows a normal colonic interposition in the anterior mediastinum. (Center) Barium study shows colonic interposition
with normal esophagocolic and cologastric anastomosis. (Right) Axial NECT shows a large tumor (arrows) developed in a previous gastric
interposition.
 THORACOPLASTY AND APICOLYSIS

Frontal radiograph shows right chest wall thoracoplasty Frontal radiograph shows right-sided lucite ball
of ribs 1-7 (curved arrows) and left-sided paraffin plombage (arrow) and obliteration of the right upper
plombage with incomplete rim calcification (arrows). lobe. Lucite balls surrounded by fluid and fill most of the
4 created extrapleural space.

26 Phrenoplasty: Division diaphragmatic-pericardial

I TERMINOlOGY attachments to allow hemidiaphragm to rise higher in
Abbreviations and Synonyms hemithorax and reduce pleural dead space
Pleural tent, extrapleural plombage, parietal
pleurolysis, oleothorax
I IMAGING FINDINGS
Definitions
Collapse therapy: Surgical procedures designed to
General Features
collapse upper lobe cavitary disease of tuberculosis, Best diagnostic clue: Chest wall deformity or
used in the mid-20th century before effective expansion of the extra pleural space with lucite balls or
antituberculous antibiotics paraffin wax
Thoracoplasty: Operative removal of the ribs to Location: Typically upper hemithorax due to the
approximate the chest wall to the underlying lung to predominant location of tuberculous cavities
effect collapse of the lung or obliteration of the pleural Size: Variable, typically span 6-7 rib interspaces
space Morphology: Lucite balls surrounded by air or fluid
Alexander procedure: Rib resection and deformation of Radiographic Findings
the chest wall
Radiography
Schede procedure: Expansion of the extrapleural space o Indications for collapse therapy
with tissue implants Cavitary tuberculosis, unresponsive to medical
Extrapleural apicolysis (pleural tent): Performed to
therapy
reduce apical pleural dead space following upper After 1950, number of operations decreased
lobectomy significantly with introduction of effective
antimycobacterial antibiotics

DDx: Chest Wall Mimics

Dormant Empyema Right Lung Atelectasis

 THORACOPLASTY AND APICOLYSIS

Key Facts
Terminology Mesothelioma
Collapse therapy: Surgical procedures designed to Extrapleural Apicolysis
collapse upper lobe cavitary disease of tuberculosis, Hydropneumothorax
used in the mid-20th century before effective Pancoast tumor
antituberculous antibiotics Pathology
Thoracoplasty: Operative removal of the ribs to
Oleothorax: Injected mineral and vegetable oils into
approximate the chest wall to the underlying lung to
extrapleural space
effect collapse of the lung or obliteration of the
pleural space Clinical Issues
Extrapleural apicolysis (pleural tent): Performed to Hemoptysis: Recurrent granulomatous disease or
reduce apical pleural dead space following upper plombage erosion into major vessel
lobectomy Collapse therapy curative in 75% with mycobacterial
tuberculosis
Top Differential Diagnoses
With reemergence of multidrug resistant tuberculosis,
Thoracoplasty or Extrapleural Plombage
collapse therapy again being used to treat cavitary
Dormant tuberculous empyema disease
Posttraumatic or postsurgical chest wall deformity

Treatment of post-operative bronchopleural fistula Mediastinal compression from large extrapleural 4

or empyema
o Thoracoplasty
Ribs segmentally (more than 2 fractures per rib)
broken and displaced into thoracic cavity
Resection of 6-7 ribs, periosteum preserved to
allow rib regeneration
Extent of resection determined by underlying
extent of cavitary disease
o Extrapleural plombage
Extrapleural space created and enlarged with
foreign bodies, typically either LuciteT" spheres or
paraffin wax
Size of mass determined by extent of cavitary
disease
Typically 15-30 lucite balls used
Lucite'" balls surrounded by air initially and
eventually space fills with fluid
o Phrenoplasty
Elevated hemidiaphragm on operative side
Normal diaphragmatic motion
Hemidiaphragm may develop abnormal contour
o Lungs
Successful treatment, underlying lung with
calcified granulomas and cicatricial atelectasis
from healed granulomatous disease
Adjacent pleural thickening along thoracoplasty
or extra pleural plombage
o Complications of thoracoplasty
Complications usually develop in peri-operative
period
Bronchopleural fistula
Prolonged air leak
Hemorrhage
Empyema
o Complications of extra pleural plombage (15%)
Complications develop either acutely or long term
Acute oil embolism from oleothorax
Local infection acute or chronic including
complications from bronchopleural or cutaneous
fistula
mass
Plombage migration 27
Erosion into major vessels or ribs
Lipoid pneumonia from oil aspiration through
bronchopleural fistula
Secondary malignant tumors: Sarcoma,
non-Hodgkin lymphoma, bronchogenic
carcinoma
Radiographic abnormalities often heralded by
change in size and shape of previous stable
plombage mass
Dispersion of lucite balls suggests expansion of
extrapleural space
Extrapleural apicolysis (pleural tent)
o Indications
After upper lobectomy, if remaining lobes unable
to fill the vacated space, surgeon will generally
perform a pleural tent
Parietal pleura at lung apex pulled off the chest
wall to create a larger extra pleural space and
reduce the intrapleural space
Fewer complications with extrapleural space than
residual pleural space
Less air leak and decreased hospitalization
o Appearance
Initial: Air-containing space inseparable from air
in the pleural space
Day 2: Apical air-fluid level as fluid fills the
extrapleural space
Over 30 days: Air completely resorbed, fluid soft
tissue thickening produces apical cap, will
gradually diminish in size over time
o Complications
Hemorrhage or infection
Suspect when there is progressive increase in size
of extra pleural space
CT Findings
Oleothorax has lipid density (-100 HU)
Imaging Recommendations
Best imaging tool
 THORACOPLASTY AND APICOLYSIS
o Chest radiography usually suffices to evaluate status o Pain from plombage uncommon
of surgery o Chronic chest wall pain common problem after
o CT useful as problem solving tool thoracoplasty
o Scoliosis convex to side of thoracoplasty
Other signs/symptoms
I DIFFERENTIAL DIAGNOSIS o Hemoptysis: Recurrent granulomatous disease or
plombage erosion into major vessel
Thoracoplasty or Extrapleural Plombage o Reduced chest wall mobility, susceptible to
Dormant tuberculous empyema pneumonia
o Represents partially healed tuberculous empyema o Rare cause of right to left shunt through remaining
o Focal pleural mass with variable thick-walled calcific lung on side of thoracoplasty
shell o Chronic hypoxia leading to pulmonary artery
o May eventually burrow into lung (bronchopleural hypertension
fistula) or out the chest wall (empyema necessitatis)
Posttraumatic or postsurgical chest wall deformity Demographics
o Deformity usually less extensive than thoracoplasty Age
o History of trauma or surgery o More common in elderly since procedure common
o Surgery usually for Stage IIIb bronchogenic in the 1940's and 1950's
carcinoma o Still used worldwide in younger individuals
Invariably done in conjunction with lobectomy
Natural History & Prognosis
Mesothelioma
4 o Rind of circumferential pleural thickening
Collapse therapy curative in 75% with mycobacterial
tuberculosis
o Chest wall deformity due to pleural restriction and
28 Extrapleural plombage foreign body, complications
loss of volume of the affected hemithorax
may arise decades later
o History of asbestos exposure
o Chest wall pain, weight loss, malaise Treatment
Atelectasis Thoracoplasty vs. extra pleural plombage
o Loss of rib interspaces common with lobar o Plombage
atelectasis Single operation could do both hemithoraces at
o Rarely chest wall may more severely deform with the same time
severe lung atelectasis No physical deformity as with thoracoplasty
o Chest wall usually abnormal from muscular Better in poor risk patients
dystrophy or metabolic bone disease Preserves lung function
Extrapleural Apicolysis Shorter hospital stay
Fewer post-operative complications
Hydropneumothorax
o Mimics pleural tent Surgical procedures were abandoned with introduction
of effective antimycobacterial drugs
o Loculated hydropneumothorax usually signifies
inadequate chest tube drainage With reemergence of multidrug resistant tuberculosis,
collapse therapy again being used to treat cavitary
o May lead to empyema
disease
Pancoast tumor
o Apical smooth pleural thickening May have to remove plombage material if
o Erosion of 1st rib complications arise
o Older smoker o May require decortication
o Pain, extension into brachial plexus, Horner
syndrome
I SELECTED REFERENCES
I. Tezel CS et al: Plombage thoracoplasty with Lucite balls.
I PATHOLOGY 2.
Ann Thorac Surg. 79(3): 1063, 2005
Brunelli A et al: Pleural tent after upper lobectomy: a
General Features randomized study of efficacy and duration of effect. Ann
Thorac Surg. 74(6):1958-62, 2002
Epidemiology
3. Uchida T et al: Developing bronchial fistulas as a late
o Prevalence drug resistant tuberculosis 10-15% to one complication of extra periosteal plombage. Jpn J Thorac
or more drugs Cardiovasc Surg. 47(5):214-7,1999
o M tuberculosis case rate 10 per 100,000 in western 4. Jouveshomme S et al: Preliminary results of collapse
world therapy with plombage for pulmonary disease caused by
Oleothorax: Injected mineral and vegetable oils into multidrug-resistant mycobacteria. Am J Respir Crit Care
extrapleural space Med. 157(5 Pt 1):1609-15, 1998
5. Ibarra-Perez C et al: More on thoracic neoplasms related to
Lucite sphere plombage. Chest. 107(2):581-2, 1995
ICLINICAl ISSUES
Presentation
Most common signs/symptoms
 THORACOPLASTY AND APICOLYSIS

I IMAGE GALLERY

Typical
(Left) Frontal radiograph
shows recent lucile
plombage. 8alls are
surrounded by air. 8alls lill
most of the extrapleural
space. (Right) Frontal
radiograph 8 years later.
8alls are now surrounded by
Iluid. The overall size of the
extrapleural space is the
same.

4
Other 29
(Left) Frontal radiograph
shows expansion of an
extrapleurallucite plombage
(curved arrows). 8alls are
separated by large quantity
ollfuid. Space was inlected.
Healed granulomas in the
lelt upper lobe (arrow).
(Right) Frontal radiograph
shows right-sided
thoracoplasty and lelt-sided
parallin plombage with rim
calcification. Oil has
ruptured and is partly
extruded inleriorly (arrows).

Typical
(Left) Anteroposterior
radiograph immediately
post-operative shows
postsurgical pleural tent. Air
is located in the eXlrapleural
space (arrows). Palient had
right upper lobe lobectomy.
(Right) Anteroposterior
radiograph 8 days later
shows an air-fluid level at the
right apex (arrows) in the
eXlrapleural space from
extrapleural apicolysis.
 PNEUMONECTOMY

Frontal radiograph 10 days following pneumonectomy. Frontal radiograph 2 months later Air-fluid level has
Pneumonectomy space approximately SO% filled with dropped. No change in mediasUnal shift.
fluid. Slight mediasUnal shift to the left. Air pockets Bronchopleural fistula.
(arrows) are normal.

o Evolution of pneumonectomy space

ITERMINOLOGY Fills with fluid and slowly shrinks in size, rate
Definitions varies
Standard pneumonectomy intrapleural Typically 1/2 to 2/3 of hemithorax fills with fluid
Extended and radical pneumonectomy extension of in 1 week
resection into parietal pleura, chest wall, or diaphragm Complete filling with fluid in 2-4 months
Pleuropneumonectomy extra pleural plane of Small quantity of air may persist indefinitely
dissection Bubble of air may be dispersed in fluid, does not
Postpneumonectomy syndrome: Rare delayed equate with empyema or fistula
complication of pneumonectomy where extreme Air-fluid level varies < 1 cm with respiration
mediastinal shift compresses the airway between the Up to 3.5 cm contralateral shift on expiration
spine and aorta or pulmonary artery o Final appearance
Degree of mediastinal shift depends on
compliance of contralateral lung
IIMAGING FINDINGS Volume loss pneumonectomy side
Elevation diaphragm, mediastinal shift,
Radiographic Findings approximation of ribs on pneumonectomy side
Normal appearance after pneumonectomy Variable sized soft-tissue opacity of residual
o Initial appearance pneumonectomy space
Mediastinum either midline or slight shift to Complications
pneumonectomy space o Expansion of pneumonectomy space with either air
Completely air-filled space or fluid
Chest tube mayor may not be present (used for Acute: Hemorrhage or bronchopleural fistula
bloody or infected pleural space) Subacute: Chylothorax, hemorrhage, empyema

DDx: Pneumonectomy Complications

Bronchopleural Fistula Pneumonectomy Syndrome Chylothorax

 PNEUMONECTOMY

Key Facts
Imaging Findings Degree of mediastinal shift depends on compliance of
Initial appearance contralateral lung
Mediastinum either midline or slight shift to Top Differential Diagnoses
pneumonectomy space Cardiac Volvulus
Completely air-filled space Postpneumonectomy Pulmonary Edema
Evolution of pneumonectomy space Bronchopleural Fistula
Fills with fluid and slowly shrinks in size, rate varies Pneumonia
Typically 1/2 to 2/3 of hemithorax fills with fluid in 1
Empyema
week Chylothorax
Complete filling with fluid in 2-4 months Esophagopleural Fistula
Small quantity of air may persist indefinitely Right to Left Shunt
Bubble of air may be dispersed in fluid; does not Postpneumonectomy Syndrome
equate with empyema or fistula
Air-fluid level varies < 1 cm with respiration Pathology
Up to 3.5 cm contralateral shift on expiration Fluid in pneumonectomy space accumulates from
Final appearance bleeding, lymphatics, and passive transudation

Late: Empyema, recurrent tumor

I DIFFERENTIAL DIAGNOSIS
4
Mimic Atelectasis of lung will shift the
31
mediastinum to the nonoperated side Cardiac Volvulus
o Drop in air-fluid level of pneumonectomy space Follows intrapericardial pneumonectomy, especially
Bronchopleural fistula on the right
Empyema necessitatis Develops in immediate (first 24 hours) post-operative
Diaphragmatic rent period
lncisional dehiscence Dramatic acute cardiovascular collapse
CT Findings Requires immediate surgery
Cardiac apex lies in the right costovertebral angle
CTA
o Stump thrombosis (dextrocardia)
In situ thrombus may form in residual pulmonary Postpneumonectomy Pulmonary Edema
artery stump (10%) Multifactorial: Hypervolemia, cardiac insufficiency
More likely to develop in longer stump Typically develops 24-48 hours after pneumonectomy
Thrombus is normal, probably does not need to be Diffuse interstitial or alveolar consolidation residual
treated lung
NECT Fatal unless treated
o Normal contour between pneumonectomy space
and mediastinum convex to the side of surgery Bronchopleural Fistula
Concave contour suggests increased fluid pressure Bronchial stump dehiscence: Incidence 5%
in the pneumonectomy space from empyema or o Early: From ischemia or faulty surgical technique
recurrent tumor o Late: From infection or recurrent tumor
o Long term pneumonectomy space Radiography
1/3 solid fibrothorax o Persistent pneumothorax
2/3rds have unilocular fluid-filled space with thick o Pneumonectomy space fails to fill with pleural fluid
fibrous edge or
o Expiration scans o Drop in air-fluid level of> 2 cm or
Useful when symptoms persist following surgical o Reappearance of air in previously opacified
relief of post pneumonectomy syndrome to test for pneumonectomy space
bronchomalacia o Consolidation of remaining lung due to drainage of
Airway will collapse with expiration, normal fluid from the pneumonectomy space
caliber at inspiration Decubitus radiographs of the non-operated side down
Normal airway diameters decrease 50% with contraindicated (possible aspiration into remaining
expiration lung)
Small fistulas may close spontaneously without
Imaging Recommendations treatment
Best imaging tool Overall mortality 15%
o Chest radiographs useful for post-operative
surveillance Pneumonia
o CT useful for further investigation or to investigate High fatality rate
confusing radiographic abnormalities Fever and leukocytosis
 PNEUMONECTOMY
Nonspecific focal or diffuse opacities in nonoperated o Counterclockwise rotation of the heart
lung o Marked herniation of the left lung into the right
hemithorax
Empyema o Distal trachea or left main bronchus compressed
Most typically develops in the first week between the aorta or pulmonary artery and vertebral
post-operatively, but may be delayed years body
o Delayed empyemas probably due to hematogenous o Extremely small pneumonectomy space
dissemination to persistent fluid collections After left pneumonectomy
Fever and leukocytosis, though delayed empyemas o Right aortic arch common
may have little symptoms o Distal trachea or right main bronchus compressed
Often associated with bronchopleural fistula or between aorta or pulmonary artery & vertebral body
esophagopleural fistula
Radiography similar to bronchopleural fistula
Chylothorax !PATHOlOGY
Direct injury to thoracic duct at time of surgery Gross Pathologic & Surgical Features
Chylous effusion doesn't accumulate until diet returns
Fluid in pneumonectomy space accumulates from
Develops in first post-operative week, especially after
bleeding, lymphatics, and passive transudation
resumption of diet
Expansion of pneumonectomy space with fluid
Pleural thoracentesis yields chyle
I CLINICAL ISSUES
Treatment: Restrict fat intake supplemented with
medium-chain triglycerides, may require surgical Natural History & Prognosis
32 ligation Mortality 6%; morbidity up to 60%
Esophagopleural Fistula o Complications including mortality 3x greater on the
Early due to direct injury at time of surgery or late right
from empyema or recurrent tumor o Major causes of death
Radiography similar to bronchopleural fistula Pneumonia
Esophagram with non ionic contrast or barium will be Respiratory failure
positive Pulmonary embolism
More common after right pneumonectomy Myocardial infarction
Bronchopleural fistula
Right to left Shunt Empyema
Extremely rare
Treatment
Inferior vena cava flow directed through atrial septal
defect due to anatomical alterations from surgery Aggressive treatment required for
Present with platypnea-orthodeoxia syndrome o Pulmonary edema
(dyspnea and hypoxia in upright position) o Atelectasis
Treated with atrial septal occluder o Post-operative pneumonia
Surgical treatment
Postpneumonectomy Syndrome o Bronchopleural fistula
Postpneumonectomy syndrome: Nearly always follows May require muscle flaps or other vascular pedicle
right pneumonectomy, following left to heal bronchus
pneumonectomy much less common o Long term drainage pneumonectomy space for
Postpneumonectomy syndrome usually occurs in 1st empyemas or broncho/esophageal pleural fistulas
year following pneumonectomy Eloesser flap or Clagett procedure
Dyspnea and recurrent pulmonary infections Clagett procedure: Open drainage of empyema
Pulmonary function tests with instillation of antibiotics
o Forced vital capacity larger than normal
o Diminished peak flow
Postpneumonectomy syndrome: More common in I SElECTED REFERENCES
young I. Crosbie PA et al: A rare complication of pneumonectomy:
o Young age capable of marked compensatory Diagnosis made by a literature search. Respir Med.
hyperinflation of remaining lung 99(9):] 198-200, 2005
Correction of pneumonectomy space 2. Kwek BH et al: Postpneumonectomy Pulmonary Artery
o Silastic or space occupying implant to reexpand the Stump Thrombosis: CT Features and Imaging Follow-up.
pneumonectomy space Radiology. 2005
3. Kim EA et al: Radiographic and CT Findings in
Malacia more common the longer the duration of
Complications Following Pulmonary Resection.
compressed airway Radiographies. 22(1):67-86, 2002
o Bronchial stents for bronchomalacia 4. Bhalla M: Noncardiac thoracic surgical procedures.
After right pneumonectomy Definitions, indications, and postoperative radiology.
o Marked rightward and posterior deviation of the Radial C1in North Am. 34(1):137-55, 1996
mediastinum 5. Spiro PW et al: Radiology of the chest after thoracic
surgery. Semin Roentgenol. 23(1):9-31,1988
 PNEUMONECTOMY
I IMAGE GALLERY
Typical
(Left) Frontal radiograph 1st
of a series of 6 shows
immediate normal
posl~operatjve
pneumonectomy
appearance. Mediastinum
midline. Space completely
air-filled. Films from the next
14 days folow. (Right)
Frontal radiograph 5 days
later. Mediastinum has
slightly shifted to the left.
Pneumonectomy space is
approximately 50% filled
with fluid.

4
Typical 33
(Left) Frontal radiograph 9
days following
pneumonectomy. Space is
approximately 90% filled
with fluid. Slight mediastinal
shift unchanged from prior.
Hyperinflated right lung.
(Right) Frontal radiograph 10
days following
pneumonectomy.
Mediastinal shift unchanged.
Small quantity subcutaneous
emphysema (arrow). No
change in fluid-filled space.

Variant
(Left) Frontal radiograph 73
days following
pneumonectomy.
Mediastinal shift,
subcutaneous emphysema,
and fluid-filled
pneumonectomy space
essentially unchanged.
(Right) Frontal radiograph 14
days following
pneumonectomy. Expansion
pneumonectomy space.
Trachea now midline
(arrow). Thoracentesis
diagnosis chylothorax.
 LUNG VOLUME REDUCTION AND BULLECTOMY

Frontal radiograph of patient referred for possible Axial NECT of same patient shows marked
bullectomy shows large bulla occupying much of left hyperlucency of both lungs from bilateral emphysema,
lung with a band of compressed, atelectatic lung in left less severe on right. LArge left bulla compresses normal
4 upper lobe (arrows). lung, causing band of atelectasis (arrows).

34
Patients with lower lobe emphysema
ITERMINOlOGY o Patients with high baseline exercise capacity have
Abbreviations little to gain
Lung volume reduction surgery (LVRS) o Advanced stage lung carcinoma is contraindication
Forced expiratory volume in 1 second (FEV,) to LVRS
Ventilation-perfusion (V/Q) SOlo of patients evaluated for LVRShave stage 1
lung carcinoma
Definitions These limited stage lung carcinomas can be
Apical bullectomy: Resection of lung bullae, which resected during LVRS
occur more frequently in lung apices
Giant bullectomy: Resection of a dominant lung bulla
Getting Started
that is causing dyspnea Things to Check
LVRS:Resection of portions of emphysematous lung o Before LVRS
not composed of a single bulla Confirm that patient is a nonsmoker
Arterial blood gases
Pulmonary function tests
I PRE-PROCEDURE 6 minute walk exercise test
Imaging before apical bullectomy
Indications o Chest radiography
Apical bullectomy: A ruptured bulla, typically in an Ruptured apical bulla typically presents with
apical location, causes a pneumothorax pneumothorax
Giant bullectomy: A bulla occupying> 30% of a lung Apical bulla often occult on chest radiography
causes dyspnea Imaging before giant bullectomy
LVRS:Heterogeneous emphysema with upper lobe o Chest radiography
predominance reduces exercise tolerance Radiographic indication for giant bullectomy is
dominant bulla compressing lung
Contraindications o Chest CT
To LVRS Shows overall size of bulla
o Smoking within last 6 months Confirms that emphysema is not diffuse
o Less than 70% or greater than 130% of ideal weight Demonstrates extent of compressed lung
o Pulmonary fibrosis Can reveal additional lung disease
o Bronchiectasis Imaging before LVRS
o Previous pleurodesis or thoracotomy o Chest radiography
o High-risk of death Chest radiography is good for initial screening
FEV, no more than 20% of predicted Lung compression & heterogeneity of emphysema
Homogeneous emphysema on CT predict functional outcome from LVRS
Carbon monoxide diffusing capacity no more o Conventional & high-resolution chest CT
than 20% of predicted
 LUNG VOLUME REDUCTION
Key Facts
Pre-procedure
Apical bullectomy: A ruptured bulla, typically in an
apical location, causes a pneumothorax
Giant bullectomy: A bulla occupying> 30% of a lung
causes dyspnea
LVRS:Heterogeneous emphysema with upper lobe
predominance reduces exercise tolerance
5% of patients evaluated for LVRShave stage 1 lung
carcinoma
Procedure
Peripheral emphysema is accessible for LVRS,but
central emphysema cannot be resected easily
Stapled wedge resection is standard procedure
Surgeon is careful to spare uninvolved lung during
bullectomy
Used to select patients with upper lobe
predominant, heterogeneous emphysema
Target areas of peripheral, surgically accessible
emphysema
Exclude patients with additional disqualifying
lung disease
Severity of emphysema can be quantified by 2- or
3-dimensional analysis or visual scoring
o Ventilation-perfusion imaging
V/Q imaging & CT have similar predictive value
for improvement in FEV, after LVRS
Visual assessment of V/Q scans & quantitative
V/Q scores correlate closely
Therefore, computer-derived scoring is probably
unnecessary
IPROCEDURE
Location
Best procedure approach
o Apical bullectomy & giant bullectomy
Video-assisted thoracoscopic approach is least
invasive
When necessary, a muscle-sparing thoracotomy
can be used
o LVRS
Although unilateral LVRSwas tried, results were
better with bilateral LVRS
Median sternotomy or video-assisted
thoracoscopic approaches can be used
Initial results using laser were not as favorable as
with staple bullectomy
Therefore, laser resection fell out of favor
Peripheral emphysema is accessible for LVRS,but
central emphysema cannot be resected easily
Procedure Steps
Apical bullectomy
o When bullae are small & resection is limited, laser
resection of apical bullae can be used
Giant bullectomy & LVRS
o Stapled wedge resection is standard procedure
AND BULLECTOMY
Goal of LVRSis wedge resection of 25-30% of total
lung volume
Number of candidates for LVRSmarkedly exceeds
lungs available for transplant
Improvement in elastic recoil has been proposed as a
main mechanism of improvement after LVRS
Post-procedure
Benefits of LVRScan be demonstrated up to 5 years
Problems & Complications
Difficulty weaning patients from ventilator
Air leak/persistent pneumothorax
Overwhelming sepsis is an important cause of death
after LVRS
LVRSpatients are often debilitated & susceptible to
pneumonia
o Surgeon is careful to spare uninvolved lung during 4
bullectomy
o Goal of LVRSis wedge resection of 25-30% of total 35
lung volume
Alternative Procedures/Therapies
Surgical
o Lung transplant
Number of candidates for LVRSmarkedly exceeds
lungs available for transplant
Transplant more expensive than LVRS
Substantial delay while waiting for transplant lung
LVRScan be performed as soon as patient finishes
rehabilitation
Other
o Supportive medical therapy
Smoking cessation
Pulmonary & exercise rehabilitation
Oxygen & nutritional supplementation
Medications, including bronchodilators & steroids
o Bronchoscopic lung volume reduction
Experimental procedure
Involves placing specially designed valves into
bronchi leading to regions of worst emphysema
Mechanisms of Action of LVRS
Improvement in elastic recoil has been proposed as a
main mechanism of improvement after LVRS
o Flaccid, nonfunctional emphysematous lung is
resected
o Resection allows small airways to remain open
longer during expiration
Improvement in diaphragmatic function
Improvement in respiratory muscle efficiency
Reduction in regions of V/Q mismatch
Morbidity
National emphysema treatment trial (LVRScompared
to medical treatment)
o Percent with improvement in health-related quality
of life at 24 months
All LVRSpatients (33%), all medically-treated
patients (9%)
 LUNG VOLUME REDUCTION
High-risk patients: LVRS(10%), medical (0%)
Other than high-risk patients: LVRS(37%),
medical (10%)
Predominantly upper lobe emphysema with low
exercise capacity: LVRS(48%), medical (10%)
Mortality
National emphysema treatment trial (LVRScompared
to medical treatment)
a Total mortality (number of deaths/person-year)
All LVRSpatients (0.11), all medically-treated
patients (0.11)
High-risk patients: LVRS (0.33), medical (0.18)
Other than high-risk patients: LVRS(0.09),
medical (0.10)
Predominantly upper lobe emphysema with low
exercise capacity: LVRS(0.07), medical (O.IS)
AND BULLECTOMY
Stapler sealed with bovine pericardium addressed
problem of prolonged air leak
Pleural tent is sometimes used to help reduce size
of pleural space
a Cardiac problems, particularly coronary artery
disease, are prevalent in LVRSpatients
Complications
Most feared complication(s): Overwhelming sepsis is
an important cause of death after LVRS
Other Complications
a After LVRS
Post-operative bleeding can require re-exploration
LVRSpatients are often debilitated & susceptible
to pneumonia
High incidence of pneumonia & prolonged chest
tube drainage increase chance of empyema

I POST-PROCEDURE I SElECTED REFERENCES

Expected Outcome I. Schipper PH et al: Outcomes after resection of giant
emphysematous bullae. Ann Thorac Surg. 78(3):976-82,
No recurrence of pneumothorax after apical 2004
36 bullectomy 2. Ciccone AM et al: Long-term outcome of bilateral lung
After giant bullectomy or LVRS volume reduction in 2S0 consecutive patients with
a Exercise tolerance increases as manifested by emphysema.) Thorac Cardiovasc Surg. 125(3):513-25,2003
improvement in 6 minute walk 3. Fishman A et al: A randomized trial comparing
a FEV, improves lung-volume-reduction surgery with medical therapy for
severe emphysema. N Engl) Med. 348(21):2059-73, 2003
Benefits of LVRScan be demonstrated up to 5 years
4. Greenberg)A et al: Giant bullous lung disease: evaluation,
Things To Do selection, techniques, and outcomes. Chest 5urg Clin N
Am. 13(4):631-49,2003
Post-operative care: Pleural drainage until 5. Meyers BFet al: Chronic obstructive pulmonary disease.
pneumothorax resolves 10: Bullectomy, lung volume reduction surgery, and
Continue optimal supportive medical management of transplantation for patients with chronic obstructive
emphysema pulmonary disease. Thorax. 58(7):634-8, 2003
6. Hunsaker ARet al: Lung volume reduction surgery for
Things To Avoid emphysema: correlation of CT and V/Q imaging with
Resumption of cigarette smoking physiologic mechanisms of improvement in lung function.
Radiology. 222(2):491-8, 2002
Findings and Reporting 7. National Emphysema Treatment Trial Research Group:
Location of central lines & tubes Patients at high risk of death after lung-volume-reduction
Presence or absence of pneumothorax, pneumonia or surgery. N Engl) Med. 345(15):1075-83, 2001
pleural collection 8. Austin )H: Pulmonary emphysema: imaging assessment of
lung volume reduction surgery. Radiology. 212(1):1-3,
Imaging 1999
Chest radiography 9. Maki DD et al: Advanced emphysema: preoperative chest
a Used to monitor critically ill patients in intensive radiographic findings as predictors of outcome following
lung volume reduction surgery. Radiology. 212(1):49-55,
care unit after surgery 1999
Chest CT 10. Thurnheer R et al: Role of lung perfusion scintigraphy in
a Evaluate pneumonia, empyema, sepsis or prolonged relation to chest computed tomography and pulmonary
pneumothorax function in the evaluation of candidates for lung volume
a For individual lungs after LVRS reduction surgery. Am) Respir Cril Care Med.
CT-derived mean lung capacity decreased 13% & 159(1):301-10,1999
residual volume decreased 20% 11. Becker MD et al: Lung volumes before and after lung
volume reduction surgery: quantitative CT analysis. Am)
Respir Crit Care Med. 157(5 PI 1):1593-9, 1998
12. Rozenshtein A et al: Incidental lung carcinoma detected at
I PROBLEMS & COMPLICATIONS CT in patients selected for lung volume reduction surgery
to treat severe pulmonary emphysema. Radiology.
Problems 207(2):487-90, 1998
LVRS
a Difficulty weaning patients from ventilator
a Air leak/persistent pneumothorax
Severely emphysematous lung is extremely friable
Friable lung is very difficult to suture, so air leaks
are common
 LUNG VOLUME REDUCTION AND BULLECTOMY
I IMAGE GALLERY

(Left) Axial NECT 0( patient

evaluated for lung volume
reduction surgery shows
severe, heterogeneous,
bilateral emphysema with
predominantly peripheral
involvement & central
sparing (arrows). (Right)
Coronal SPECT perfusion
scan in same patient shows
heterogeneous perfusion
defects related to
emphysema, worse in upper
lobes, with sparing of central
lung in lung bases (open
arrows).

4
(Left) Coronal NECT
37
reconstruction of same
patient before lung volume
reduction surgery shows
severe bilateral emphysema
with central sparing,
corresponding to normal
lung (open arrows). (Right)
Coronal NECT
reconstruction after bilateral
upper lobe wedge resection
of emphysematous lung
demonstrates substantial
reduction in lung volume
with elevation of both hila &
hemidiaphragms.

(Left) Frontal radiograph

after unilateral lung volume
reduction surgery shows
small right pneumothorax
(arrows) & post-operative
changes in right upper lobe
(open arrows). (Right) Axial
NECT after bilateral lung
volume reduction surgery
shows right pneumonia
(arrow) & pneumothorax
(open arrow). Wedge
resection in left upper lobe is
visible (curved arrows).
 LUNG TRANSPLANTATION

Sagittal NECT shows patient after single right lung Axial HRCT shows patient after right single lung
transplantation for emphysema. Note the hyperinflated transplantation for pulmonary fibrosis. Note the volume
emphysematous left native lung and the subsequent reduction of the fibrotic right native lung.
4 mediastinal and cardiac shirt.

38
o Severe psychiatric illness
!TERMINOlOGY o Active or recent cigarette smoking
Abbreviations o Severe malnutrition
Lung transplantation (LTX) o Poor rehabilitation potential
Single lung transplantation (SLTX) Relative
Double lung transplantation (DLTX) o Poorly controlled chronic medical conditions
Heart and lung transplantation (HLTX) o Requirement of > 20 mg prednisolone per day
o Mechanical ventilation (excluding noninvasive)
Synonyms o Extensive pleural disease
Lung transplantation o Airway colonization with pan-resistant bacteria
Definitions Organ Allocation - Issues
Replacement of diseased native lung or heart-and-lung Global shortage of organs due to geographically
by parts of lung, entire lung, both lungs, or variable juridical, ethical, and medical considerations
heart-and-Iung block from an allograft donor Allocations of lungs principally based on waiting time
without regard for severity of illness or medical
urgency
I PRE-PROCEDURE Only exception is a 90 days credit granted at the time
of listing to patients with idiopathic pulmonary
Indications fibrosis (disproportionally high mortality rate in this
Frequent group)
a Emphysema Lung is most fragile organ in potential donor and may
a Cystic fibrosis be damaged by excessive fluid overload, aspiration,
o Idiopathic pulmonary fibrosis and ventilator associated pneumonia in the intensive
o Primary pulmonary hypertension care setting
o Eisenmenger syndrome Less than 20% of donors have lungs suitable for
Less frequent harvest
o Sarcoidosis Lung can tolerate only brief period of ischemia,
o Lymphangioleiomyomatosis typically less than six hours
o Eosinophilic granuloma This limits geographic distribution of allografts and
o Drug or radiation induced pulmonary fibrosis precludes prospective HLA crossmatching
Contraindications Peri-Operative Imaging
Absolute Chest radiography
o Severe extra pulmonary organ dysfunction o Detect or exclude absolute and relative
o Acute critical illness contraindications
o Active or recent cancer o Choose side for single lung transplantation
o Active extra pulmonary infection o Detect infection and other concurrent disease
 LUNG TRANSPLANTATION

Key Facts
Terminology Post-procedure
Replacement of diseased native lung or 1 year survival: 70.7%
heart-and-Iung by parts of lung, entire lung, both 3 year survival: 54.8%
lungs, or heart-and-Iung block from an allograft 5 year survival: 42.6%
donor Median survival: 3.7 years
Pre-procedure Problems & Complications
Global shortage of organs due to geographically Reperfusion edema
variable juridical, ethical, and medical considerations Primary graft failure
Less than 20% of donors have lungs suitable for Airway complications
harvest Infection
Acute rejection
Procedure Chronic rejection
Heart-and-Iung transplantation Post-transplant lymphoproliferative disorders
Single lung transplantation Bronchogenic carcinoma
Double lung transplantation Recurrence of disease in transplanted lung
Transplantation of lobes from living donors

a Should be repeated every 3 months because of o Donors should be larger than recipients so that 4
usually long delay from peri-operative assessment to donors lobes fill each hemithorax
transplantation 39
CT
a Detect or exclude absolute and relative I POST-PROCEDURE
contraindications
a Screen for lung cancer Expected Outcome
a Detect primary graft failure Survival
a Detect complications such as infection, rejection, or o 1 year survival: 70.7%
neoplasm o 3 year survival: 54.8%
Ventilation-perfusion scintigraphy o 5 year survival: 42.6%
a Assess functionality of diseased lungs o Median survival: 3.7 years
a Select lung to be transplanted
FOG-PET
a Noninvasive evaluation of pulmonary nodules or I PROBLEMS- & COMPLICATIONS
hilar and mediastinal lymph nodes
Problems
Immunosuppression
I PROCEDURE o Initiated in the immediate peri-operative period
o Continued for the rest of recipients life
Surgical Techniques o Standard regimens: Cyclosporine, azathioprine,
Heart-and-lung transplantation mycophenolate mofetil, and prednisolone
a First procedure successfully implemented o Myriad side effects
a Still performed in Eisenmenger syndrome or left o Numerous interactions with other commonly
ventricular dysfunction prescribed medications
Single lung transplantation
Complications
a Most commonly employed technique
a Technical approach is relatively easy Most feared complication(s)
a One donor lung can be used for two recipients o Reperfusion edema
Double lung transplantation Occurs in nearly all transplanted lungs
a Sequential performance of two single lung Reimplantation response caused by capillary
transpiantations at one time permeability
a Cardiopulmonary bypass can be avoided by Severity is closely related to ischemic time
ventilating contralateral lung during each Radiographic findings are nonspecific and
implantation resemble those in patients with left ventricular
o Commonly performed in cystic fibrosis or failure, fluid overload, and acute rejection
bronchiectasis (removal of both infected native Up to 98% of patients show radiographic signs of
lungs) reperfusion edema immediately after
Transplantation of lobes from living donors transplantation
o Recently developed technique Radiologic findings are maximal within first 3
o Bilateral implantation of lower lobes from two days after transplantation and decrease thereafter
blood-groups compatible living donors o Primary graft failure
Occurs in approximately 15% of patients
 LUNG TRANSPLANTATION
Is a form of acute respiratory distress syndrome
Presumed to reflect ischemia-reperfusion injury
Surgical trauma and lymphatic disruption might
be other contributors
Severe hypoxemia and widespread opacities on
chest radiograph are key findings
HRCT: Combination of ground-glass opacities and
consolidations
Mortality rate: Up to 60%
o Airway complications
Occurs in less than 15% of patients
Complete dehiscence of bronchial anastomosis
requires immediate surgical correction
Partial bronchial dehiscence is managed
conservatively by evacuation of associated
pneumothorax and reduction of corticosteroids
Anastomotic stenosis is managed by stent
placement
o Infection
Substantially more frequent than in other solid
4 organ transplants
Most likely related to allograft exposure to
external environment
40
Pseudomonas aeruginosa, cytomegalovirus, and
aspergillus are the most common infectious agents
Can display both typical and atypical finding on
chest radiograph and CT
Early diagnosis is crucial for therapeutic success
o Acute rejection
Most transplant recipients have at least one
episode of acute rejection
HLA mismatching appears to be a risk factor
Incidence is greatest 100 days after
transplantation
Clinical manifestations are malaise, low grade
fewer, dyspnea, cough, impaired oxygenation,
leucocytosis
Radiograph and CT may show alveolar, nodular,
or interstitial opacities, and pleural effusions
Histologic proof by transbronchial biopsy is
mandatory
Treated by high dose intravenous prednisolone
o Chronic rejection
Major factor that limits long term survival of lung
transplant recipients
Found in up to 70% of patients who survive for 5
years
Histologically manifests as "bronchiolitis
obliterans" (BO), a fibroproliferative process that
targets the small airways, causing submucosal
fibrosis and luminal obliteration
Because BO is of heterogeneous distribution,
transbronchial biopsy yields low sensitivity
Chronic rejection therefore defined by functional
demonstration of air flow limitation and termed
"bronchiolitis obliterans syndrome" (BOS)
Chest radiograph often normal
HRCT shows, bronchial wall thickening,
peribronchial opacities, tree-in-bud pattern, and
air trapping
Expiratory CT sections are mandatory in patients
with suspected BOS
Air trapping may become pathologic before lung
function parameter characteristic for BOS
deteriorate
In the absence of effective treatment for BOS,
attention has focussed on preventive strategies
Prognosis of BOS is poor, with a mortality rate of
40% within 2 years after diagnosis
Other Complications
o Post-transplant Iymphoproliferative disorders
o Bronchogenic carcinoma
o Recurrence of disease in transplanted lung
o Right or left heart failure
o Pulmonary hypertension
o Iatrogenic renal and hepatic disease caused by
medication
CT Follow-Up after Transplantation
Modality of choice for imaging symptomatic patients
Role in asymptomatic patients not clearly defined
Many institutions perform CT follow-up every 12
months
Expiratory sections in these follow-up CT
examinations mandatory to detect air trapping caused
by BOS
CT might have the potential to detect BOS before
functional deterioration occurs
If confirmed in prospective trials, this could
implement CT into routine diagnostic algorithms after
transplantation
I SELECTED REFERENCES
1. Bankier AA et al: Air trapping in heart-lung transplant
recipients: variability of anatomic distribution and extent
at sequential expiratory thin-section CT. Radiology.
229(3):737-42,2003
2. Boehler A et al: Post-transplant bronchiolitis obliterans. Eur
Respir J. 22(6):1007-18, 2003
3. Collins Jet al: Bronchogenic carcinoma after lung
transplantation: frequency, clinical characteristics, and
imaging findings. Radiology. 224(1):131-8, 2002
4. Collins J: Imaging of the chest after lung transplantation. J
Thorac Imaging. 17(2):102-12,2002
5. Estenne M et al: Bronchiolitis obliterans after human lung
transplantation. Am J Respir Crit Care Med. 166(4):440-4,
2002
6. Bankier AA et al: Bronchiolitis obliterans syndrome in
hearl-lung transplant recipients: diagnosis with expiratory
CT. Radiology. 218(2):533-9, 2001
7. Collins Jet al: Frequency and CT findings of recurrent
disease after lung transplantation. Radiology. 219(2):503-9,
2001
8. Collins J et al: CT findings of pneumonia after lung
transplantation. AJR Am J Roentgenol. 175(3):811-8,2000
9. Arcasoy SM et al: Lung transplantation. N Engl J Med.
340(14):1081-91,1999
10. Collins J et al: Lung transplantation for
Iymphangioleiomyomatosis: role of imaging in the
assessment of complications related to the underlying
disease. Radiology. 210(2):325-32,1999
11. Leung AN et al: Bronchiolitis obliterans after lung
transplantation: detection using expiratory HRCT. Chest.
113(2):365-70, 1998
12. Worthy SA et al: Bronchiolitis obliterans after lung
transplantation: high-resolution CT findings in 15
patients. AJR Am J Roentgenol. 169(3):673-7,1997
 LUNG TRANSPLANTATION
I IMAGE GALLERY

(Left) Axial NECT shows

typical postsurgical
alterations after lung
transplantation. Left image:
Chest wall defect (arrows).
Right image: Irregularities at
the bronchial anastomosis
(arrow). (Right! Axial HRCT
shows lung transplant
recipient with acute allograFt
dysfunction 4 days after
transplantation. Dysfunction
manifests as combination of
ground-glass opacities (white
open arrows) and small
consolidations (black open
arrows). Note postsurgical
pneumomediastinum.
4
41
(Left) Axial HRCT in
inspiration shows findings
typical for chronic rejection:
Bronchial wall thickening
(arrow), peribronchial
opacities (white open
arrow), and tree-in-bud
(black open arrows). (Right!
Axial HRCT in expiration of
the same patient shows the
fourth classical finding of
chronic rejection: Air
trapping (arrows). Expiratory
sections are mandatory (or
CT of lung transplant
recipients.

(Left) Axial CECT shows lung

transplant recipient with
combined tuberculous
(arrows) and fungal (open
arrow) infection. TB shows
air space nodules, fungus
shows consolidation with
ground-glass halo. (Right!
Axial CECT shows lung
transplant recipient with
central adenocarcinoma. The
carcinoma occurred 5 years
after transplantation and
grew to the displayed size
within 6 months.
 POSTTRANSPLANT LYMPHOPROLIFERATIVE DZ

Axial CECT Shows mu/Uple slightly enlarged axillary Axial CECT Shows pasttransplant Iymphoproliferative
lymph nodes (arrows) in a patient with pasttransplant disease after lung transplantaUon. Focal consolidation
Iymphoproliferative disorder after liver transplantation. (arrows) and nodules (open arrow).
4
42
!TERMINOlOGY Radiographic Findings
Nodule(s) (50%)
Abbreviations and Synonyms o Solitary pulmonary nodule (50%)
Posttransplant lymphoproliferative disease (PTLD) Well-circumscribed
Posttransplant lymphoma 3 mm to 5 cm in size (average 2 cm)
Definitions Rarely cavitate
Posttransplant lymphoproliferative disorder of B cells Random distribution
related to Ebstein-Barr virus (EBV) infection Variable rate of growth, usually slow progression
o Multiple pulmonary nodules (50%)
Characteristics similar to solitary pulmonary
I IMAGING FINDINGS nodule
Consolidation
General Features o Multifocal consolidation (8%)
Best diagnostic clue: Combination of lung (nodules or o Usually subsegmental in size
consolidation) and hilar or mediastinal adenopathy o Bronchovascular location with air bronchograms
Location o While nodules and consolidation can coexist, more
o Lung parenchyma and mediastinum most common common that pattern is either primarily nodular or
o Also affects thymus, pericardium, esophagus, primarily consolidation
abdominal organs, tonsils, and lymph nodes Ground-glass opacities, centrilobular nodules, and
Size: Nodule(s) average 2 cm in size thin-walled cysts suggest lymphocytic interstitial
Morphology: Well-defined nodule(s), rarely cavitate pneumonia (UP)
o Also seen in immunosuppressed patients, especially
bone marrow transplantation

DDx: Posttransplant Lymphoproliferative Disorders

Cryptogenic Organizing Pneumonia Invasive Aspergillosis Adenocarcinoma

 POSTTRANSPLANT LYMPHOPROLIFERATIVE DZ

Key Facts
Terminology Cryptogenic Organizing Pneumonia (COP)
Posttransplant lymphoproliferative disorder of B cells Bronchogenic Carcinoma
related to Ebstein-Barr virus (EBV) infection Pathology
Imaging Findings Related to EBV infection, immunosuppression with
Nodule(s) (50%) cyclosporine allows unrestricted proliferation of EBV
Multifocal consolidation (8%) infected cells, may become monoclonal and
Hilar and mediastinal adenopathy (50%) malignant
May rarely have large mass (10%) that tends to Intrathoracic involvement in 70% of those with PTLD
envelop mediastinal vessels Clinical Issues
Combination of lung (nodules or consolidation) and Infectious mononucleosis-like syndrome (20%)
adenopathy highly suggestive of PTLD Oral symptoms common (tonsillitis, sinusitis, otitis
Thymic involvement rare but relatively specific for media)
PTLD Reduction of immunosuppression (especially decrease
Top Differential Diagnoses cyclosporine dosage)
Bacterial and Fungal Pneumonia May develop graft rejection with treatment of PTLD

Mediastinal
Imaging Recommendations
4
a Hilar and mediastinal adenopathy (50%)
Multiple primarily involving para tracheal, anterior Best imaging tool: CT useful to characterize both lung 43
mediastinal and aorto-pulmonary lymph nodes pathology and degree of mediastinal and hilar
Average 4 em in diameter adenopathy
May rarely have large mass (10%) that tends to Protocol advice: CECT neck, chest, abdomen, and
envelop mediastinal vessels pelvis: Widespread disease commonly involving
a Nodal enlargement may also involve bronchus multiple nodal and extra nodal sites
associated lymphoid tissue (BALT) and narrow
airways
Combination of lung (nodules or consolidation) and I DIFFERENTIAL DIAGNOSIS
adenopathy highly suggestive of PTLD
Bacterial and Fungal Pneumonia
Pleura
a Pleural effusions may accompany other Clinical symptoms of infection
intrathoracic disease, not seen as an isolated finding Commonly associated with pleural effusion
Resolution following treatment Nodules less well-defined and more common to be
a Slowly over a period of weeks cavitary than PTLD
a Rarely rapid over a few days Cryptogenic Organizing Pneumonia (COP)
CT Findings Often subpleural and basilar in distribution
Focal rounded areas of parenchymal consolidation
CECT
a Usually demonstrates more nodules or adenopathy Air bronchograms common
not apparent on chest radiographs Good response to steroids
a Nodules Bronchogenic Carcinoma
May have low density centers and occasionally Inhomogeneously enhancing soft tissue mass on CECT
demonstrate halo sign May be indistinguishable from PTLD
Usually located along peribronchovascular or Biopsy often required to establish diagnosis
subpleural areas
a Mediastinal adenopathy (50%) Diffuse Pulmonary Hemorrhage
Usually associated with either pulmonary nodules Often in the context of transbronchial biopsy
or consolidation Associated with hemoptysis and anemia
a Thymic involvement rare but relatively specific for Widespread parenchymal opacity, usually not nodular
PTLD
a Pericardial thickening or effusion (10%)
a Esophageal wall thickening I PATHOLOGY
Nuclear Medicine Findings General Features
PET: Especially useful to evaluate extranodal sites that General path comments
may be radiographically occult a BALTextends from nodal clusters in airway
bifurcations to lymphocyte clusters at proximity of
lymphatics in terminal bronchiole
 POSTTRANSPLANT LYMPHOPROLIFERATIVE DZ
o PTLD thought to be a stepwise progression from
benign lymphoid polyclonal hyperplasia to frank
I CLINICAL ISSUES
lymphoma Presentation
Early diagnosis important before disease evolves Most common signs/symptoms
into aggressive forms o Infectious mononucleosis-like syndrome (20%)
Etiology o Oral symptoms common (tonsillitis, sinusitis, otitis
o Related to EBV infection, immunosuppression with media)
cyclosporine allows unrestricted proliferation of EBV o Lymphadenopathy (new lumps and bumps)
infected cells, may become monoclonal and o Symptoms related to nodal (l/3rd) or extranodal
malignant (2/3rd's) sites
o EBV virus a herpes virus High index of suspicion in transplant patient with
Nearly 100% of population seropositive new symptoms
Causes clinical syndrome of infectious Early diagnosis important as Iymphoproliferative
mononucleosis in adults disease evolves from more benign to more
EBV seropositivity most important risk factor for malignant forms
development of PTLD Other signs/symptoms: May manifest as an incidental
Risk of developing PTLD in EBV positive donor finding on follow-up radiographic study
and EBV negative recipient 25-50%
Epidemiology Demographics
o Intrathoracic involvement in 70% of those with Age
4 PTLD
o Prevalence varies from 1-15% in transplantation
o Can occur at any age
Highest in children
44 Directly related to intensity of Gender: No gender predisposition
immunosuppression Ethnicity: Caucasian children more common
Intestinal transplants (20%)
Heart, lung, or liver transplants (10%) Natural History & Prognosis
Bone marrow transplantation or kidney Develops 1 month to several years following
transplants (1%) transplantation
Extranodal (2/3rd's) o Most common within first year after transplantation
o Head & neck: Waldeyer ring (nasopharyngeal, Lethal if untreated
oropharyngeal tonsils) o Mortality 20%
o Bowel wall thickening Poor prognostic factors
o Esophageal wall thickening o Early onset
o Splenomegaly o Infectious mononucleosis presentation
oliver: Focal low-attenuation masses (1-4 cm in o Multiple sites
diameter) or diffuse hepatic infiltration o CNS involvement
o Central nervous system (CNS): Focal intra axial o Monoclonal tumor
masses o T cell origin (90% are B cell origin)
Nodal (1/3rd) Treatment
o Lymphadenopathy in any lymph node group:
No pathognomonic radiographic features: Biopsy
Retroperitoneal, mesenteric, axillary
usually necessary to differentiate from infections
Gross Pathologic & Surgical Features o Fine needle aspiration, do not usually need core
Single organ or site (50%) specimens
o Brain (60%) Reduction of immunosuppression (especially decrease
o Individual abdominal organs (20%) cyclosporine dosage)
Multiple sites (50%) o May develop graft rejection with treatment of PTLD
o May have to be retransplanted
Microscopic Features Antiviral drugs controversial
Categories CNS involvement requires intrathecal therapy
o Plasmacytic hyperplasia Chemotherapy and radiation therapy for aggressive
Most common in oropharynx or lymph nodes disease
Usually polyclonal
o Polymorphic B-cell hyperplasia and lymphoma
Lymph nodes or extranodal sites
Usually monoclonal
I SELECTED REFERENCES
1. Gottschalk 5 et al: Post-transplant Iymphoproliferative
o Immunoblastic lymphoma or multiple myeloma disorders. Annu Rev Med. 56:29-44, 200S
Widespread disease
Monoclonal
 POSTTRANSPLANT LYMPHOPROLIFERATIVE DZ

I IMAGE GALLERY
Variant
(Left) Axial HRCT shows LIP
after bone marrow
transplantation. LIP manifests
with cysts (arrows) and
diffuse ground-glass opacities
in the entire lung. (Right)
Axial HRCT shows LIP in a
patient after renal
transplantation. In this
patient, cysts (arrows)
predominate over
ground-glass opacities.

4
Variant 45
(Left) Axial CECT shows
SALT as a late complication
of heart transplantation.
Neoplastic tissue (open
arrow) causes narrowing of
the left main bronchus.
(Right) Axial CECT shows
occlusive SALT in a
segmental bronchus (open
arrows).

(Left) Coronal CECT shows

large central mediastinal
mass engulfing vessels.
Differential includes
bronchogenic carcinoma
and frank lymphoma. (Right)
Axial CECT shows multiple
pulmonary nodules. The
majority of nodules have
ground-glass halos.
Differential includes PHD
and angioinvasive
aspergillosis.
 LOBAR TORSION, LUNG

Anteroposterior radiograph shows normal AnterofXJsterior radiograph within a few hours shows
post-operative appearance following right upper rapid opacification of the residual lung sharply
lobectomy. Single chest tube mid-medial hemithorax. demarcated by the reoriented major fissure (arrows).
4 Lungsnormal. Rightmiddle lobe torsion.

46 o Fissures
ITERMINOlOGY Neofissure (reoriented major fissure after right
Abbreviations and Synonyms upper lobe lobectomy) may extend below hilus
Torsion of the lung, volvulus of a lobe o Lobe
Affected lobe has appearance of atelectasis
Definitions "Collapsed lobe" will be in abnormal location
Rotation of the bronchovascular pedicle with resultant Affected lobe may lose volume initially due to
airway obstruction and venous compromise leading to bronchial obstruction and then increase in
ischemia, infarction, and gangrene volume due to venous obstruction
Rapid opacification of affected lobe
post-operatively
IIMAGING FINDINGS Affected lobe may change position on sequential
films
General Features Lobar air trapping rare; seen in infants
Best diagnostic clue: Rapid opacification of affected Right upper lobectomy with torsion of right
lobe middle lobe most common
Location: Right middle lobe torsion most common o Hilum
following right upper lobectomy Displaced in a direction inappropriate for the lobe
Size: Lobar that appears atelectatic
Morphology: Lobe initially loses volume and then Abnormal course of pulmonary vasculature (e.g.,
increases in size hilar vessels coursing laterally and sweeping
superiorly)
Radiographic Findings Bronchial cutoff or distortion
Radiography o Mediastinum

DDx: lobar Torsion

RUL Atelectasis Contusion Pneumonia

 LOBAR TORSION, LUNG

Key Facts
Terminology Pneumonia
Rotation of the bronchovascular pedicle with Infarction
resultant airway obstruction and venous compromise Hemorrhage
leading to ischemia, infarction, and gangrene Blunt Chest Trauma: Contusion or Fractured
Bronchus
Imaging Findings
Pathology
Best diagnostic clue: Rapid opacification of affected
lobe 70% follow right upper lobe lobectomy, 15% left
Location: Right middle lobe torsion most common upper lobe lobectomy
following right upper lobectomy Clinical Issues
Morphology: Lobe initially loses volume and then Rapid development shock in post-operative period
increases in size Diagnosis made median 10 days after operation
Neofissure (reoriented major fissure after right upper High mortality if unrecognized (10-20%)
lobe lobectomy) may extend below hilus "Spill-over": Rerotation of lobe may result in airway
Top Differential Diagnoses flooding with serosanguineous fluid
Lobar Atelectasis

May be shifted away from the affected lung

o Pleura
Development of pleural effusion suggests
infarction
Accumulation of pleural effusion may not occur
with indwelling chest tube drainage
CT Findings
CECT
o Hilum
Tapered obliteration of proximal pulmonary
artery and bronchus
Delayed filling of pulmonary artery with contrast
Pulmonary artery acutely kinked
o Lobe
Volume may be increased rather than decreased
Ground-glass attenuation to full consolidation
Bulging fissures with unusual orientation
Unexpected location of affected lung
Angiographic Findings
Conventional
o Abnormal course of pulmonary artery
o Slow filling of the affected vessels
o Fusiform tapering of the torsed vessel
Imaging Recommendations
Best imaging tool
o Portable chest radiography usually suffices to suggest
diagnosis, critical element: Awareness of signs of
torsion
o CECT may be useful in selected cases
I DIFFERENTIAL DIAGNOSIS
lobar Atelectasis
Most common misinterpretation for lobar torsion
Torsed right middle lobe mimics right upper lobe
atelectasis
Need to know surgical history
Atelectasis of lung common post-operatively due to
retained secretions and splinting
Aspiration
4
Opacification in dependent lung 47
May have identical radiographic findings
Pneumonia
Usually develops later in post-operative course
fever and elevated white blood cell count also occurs
with torsion
Infarction
Acute
o Partial anomalous upper lobe venous return to
systemic veins one of the more common
o Inadvertent ligation of normal or anomalous artery
or vein
Subacute
o Usually develops later in post-operative course
o Usually not lobar in size
o CT pulmonary angiography useful to exclude emboli
Hemorrhage
Post-operative hemorrhage usually occurs in
immediate post-operative period
May have hemoptysis
May have similar radiographic findings
Blunt Chest Trauma: Contusion or Fractured
Bronchus
Contusion
o Immediate complication from trauma
o Opacified lung stabilized over 24 hours and then
slowly resolves over 3-5 days
Fractured bronchus
o Persistent or progressive air leak into mediastinum
or pleura
o Immediate complication from trauma
o Enlarging pneumothorax or pneumomediastinum
should be investigated with bronchoscopy
o "fallen lung sign" opacified lung lying in unusual
location
 LOBAR TORSION,
I PATHOLOGY
General Features
Etiology
o Usually post-operative complication
Most common following right upper lobe
lobectomy with torsion of the right middle lobe
Other combinations: Left lower lobe torsion
following left upper lobectomy, right lower lobe
torsion following right upper lobe lobectomy
Rare complication following lung transplantation
o To eliminate surgical space vacated by the resected
RUL, right middle lobe must be mobilized by
Completing incomplete fissures
Dividing inferior pulmonary ligament
Mobile lobe now more likely to torse
o May occur with chest trauma
Most common seen in children who have been
run over by a car
May be related to large pneumothorax allowing
4 the lobes to twist on the hilum
o Associated with lobar lung disease
48 Pneumonia or collapsed lobe
Presumably isolated lobe is heavy and with the
right anatomic relationships (complete fissures)
the abnormal lobe may torse
o Common condition in large dogs (especially Afghan
hounds)
Associated with chylothorax
Epidemiology
00.1% complication of pulmonary resections
o 70% follow right upper lobe lobectomy, 15% left
upper lobe lobectomy
Gross Pathologic & Surgical Features
Degree of clockwise (most common) or
counterclockwise rotation generally 180 degrees
(90-360 degrees)
Venous obstruction
o Edema eventually leading to hemorrhagic infarction
I CLINICAL ISSUES
Presentation
Most common signs/symptoms
o Rapid development shock in post-operative period
o Sudden cessation of post-operative air leak
Other signs/symptoms
o Bloody pleural effusion
o Bronchoscopy
Torsed bronchus will be collapsed extrinsically
Bronchoscope may pass through obstructed
bronchus with expulsion of considerable fluid
Natural History & Prognosis
Diagnosis made median 10 days after operation
High mortality if unrecognized (10-20%)
Lobectomy
o Mortality 2%, morbidity up to 40%
Treatment
Prophylactic
o Anchoring lobes to each other after lobectomy
LUNG
o Routinely done about 50% of the time
Reoperation
o "Spill-over": Rerotation of lobe may result in airway
flooding with serosanguineous fluid
o If lung infarcted will require additional lobectomy
or pneumonectomy
Surgical principles following lobectomy
o Ventral lung more mobile than dorsal lung and
generally fills the lobectomy space
o Final appearance of the chest similar to that of lobar
collapse
I DIAGNOSTIC CHECKLIST
Consider
Lobar torsion for any opacity that resembles lobar
atelectasis
I SELECTED REFERENCES
1. Grazia TJ et al: Lobar torsion complicating bilateral lung
transplantation. J Heart Lung Transplant. 22(1):102-6,
2003
2. Kim EAet al: Radiographic and CT findings in
complications following pulmonary resection.
Radiographies. 22(1):67-86, 2002
3. Cable DG et al: Lobar torsion after pulmonary resection:
presentation and outcome. J Thorac Cardiovasc Surg.
122(6):1091-3,2001
4. Neath PJ et al: Lung lobe torsion in dogs: 22 cases
(1981-1999). J Am Vet Med Assoc. 217(7):1041-4, 2000
5. Nonami Yet al: Lobar torsion following pulmonary
lobectomy. A case report. J Cardiovasc Surg (Torino).
39(5):691-3,1998
6. Spizarny DL et al: Lung torsion: preoperative diagnosis
with angiography and computed tomography. J Thorac
Imaging. 13(1):42-4, 1998
7. Velmahos GC et al: Pulmonary torsion of the right upper
lobe after right middle lobectomy for a stab wound to the
chest. J Trauma. 44(5):920-2, 1998
8. Andresen R et al: Lung torsion--a rare postoperative
complication. A case report. Acta Radiol. 38(2):243-5, 1997
9. Bhalla M: Noncardiac thoracic surgical procedures.
Definitions, indications, and postoperative radiology.
Radiol Clin North Am. 34(1):137-55,1996
10. Schamaun M: Postoperative pulmonary torsion: report of a
case and survey of the literature including spontaneous
and posttraumatic torsion. Thorac Cardiovasc Surg.
42(2):116-21,1994
11. Wong PSet al: Pulmonary torsion: a questionnaire survey
and a survey of the literature. Ann Thorac Surg.
54(2):286-8, 1992
12. Munk PLet al: Torsion of the upper lobe of the iung after
surgery: findings on pulmonary angiography. AJRAmJ
Roentgenol. 157(3):471-2, 1991
13. Kucich VAet al: Left upper lobe torsion following lower
lobe resection. Early recognition of a rare complication.
Chest. 95(5):1146-7,1989
14. Larsson 5 et al: Torsion of a lung lobe: diagnosis and
treatment. Thorac Cardiovasc 5urg. 36(5):281-3, 1988
15. Felson B:Lung torsion: radiographic findings in nine cases.
Radiology. 162(3):631-8, 1987
16. Moser E5et al: Lung torsion: case report and literature
review. Radiology. 162(3):639-43, 1987
17. Weisbrod GL: Left upper lobe torsion following left
lingulectomy. Can Assoc Radiol J. 38(4):296-8, 1987
 LOBAR TORSION, LUNG

I IMAGE GALLERY
Typical
(Left) Anteroposterior
radiograph shows
post-operative appearance
following right upper
lobectomy. Two chest lUbes
right apex. Iff-defined right
hilum and slight widening
superior mediastinum
probably from supine
position. (Right)
Anteroposterior radiograph
shows what appears to be
right upper lobe atelectasis.
Fissure extends below the
hilus (arrows). Note the
subtle mediastinal shift with
straightening of the trachea
(curved arrow).
4
49
(Left) Anteroposterior
radiograph shows immediate
post~operative position
following reduction of torsed
right middle lobe. Lobe
fixated to lower lobe by
surgical staples (arrow).
(Right) Anteroposterior
radiograph shows normal
post-operative chest
following right upper
lobectomy. Two chest lUbes
right apex. Minimal
subcutaneous emphysema.
Slight mediastinal shift to the
right. Right hilum slightly
indistinct.

(Left) Anteroposterior
radiograph 2nd
post-operative day. Rapid
opacification of upper lobe.
Note that the fissure extends
below the hilum (arrow).
Also hilum is displaced
inferiorly rather than
superiorly (curved arrow).
(Right) Anteroposterior
radiograph same patient 2
hours later. Further
opacification of the right
upper lung. Right middle
lobe torsion requiring
re-operation and removal of
the lobe.
 CARDIAC VOLVULUS

Coronal graphic shows cardiac volvulus (open arrow) Anteroposterior radiograph shows cardiac volvulus with
through a pericardial defect (curved arrows) into the apex directed laterally (arrow). Superior vena cava
right hemithorax following a pneumonectomy. The SVC obstruction with widened mediastinum (white open
4 is twisted (white arrow) and occluded. arrows) and empty pericardium (black open arrows).

50
Location: Right more typical from surgery, left more
ITERMINOLOGY common with trauma or congenital defect
Abbreviations and Synonyms Morphology: Any size pericardial defect, typically 8-10
Cardiac herniation, cardiac strangulation, cardiac cm in size
prolapse, dislocated heart, cardiac torsion, cardiac Radiographic Findings
incarceration
Radiography
Definitions o Normal appearance after pneumonectomy
Cardiac herniation through a pericardial rent with Pneumonectomy space empty with mediastinum
sudden development shock or superior vena cava either midline or slightly shifted to the
(SYC) syndrome pneumonectomy space
Pericardial rent typically from surgery but maybe seen Pneumonectomy space fills with fluid: 1/2 to 2/3
from trauma (blunt or penetrating) or be congenital of hemithorax fills with fluid in 1 week
Complete filling with fluid in 2-4 months
Mediastinum stays midline or gradually shifts to
IIMAGING FINDINGS the pneumonectomy space as the opposite lung
compensatorily hyperinflates, mediastinum
General Features should never shift to the non-operated side
Best diagnostic clue o Imperative that right and left sides be properly
o 180 degree rotation of heart into pneumonectomy identified on the film
space (cardiac apex pointed in wrong direction) o Partial or impending herniation
o Impending herniation: Tight spherical bulge as heart Tight spherical cardiac bulge (like the top of a
herniates through pericardial defect snow cone) as heart begins to herniate through
the pericardial defect

DDx: Cardiac Volvulus

Normal Cardiac Contour Absent Left Pericardium Pericardia I Sarcoma

 CARDIAC VOLVULUS

Key Facts
Imaging Findings Pathology
180 degree rotation of heart into pneumonectomy Right-sided herniation tends to be complete
space (cardiac apex pointed in wrong direction) Left-sided herniation tends to be partial
Impending herniation: Tight spherical bulge as heart
herniates through pericardial defect
Clinical Issues
Empty pericardia I sac SVC syndrome (50%)
Sudden hypotension and shock (50%)
Top Differential Diagnoses Usually occurs in the immediate post-operative
Airway Obstruction period
Congenital Absence Left Pericardium Adhesions develop in 3 days that prevent herniation
Pericardial Mass later, rare reports of delayed herniation 6 or more
Pulmonary Embolus months later
Intrathoracic Hemorrhage Mortality 40%
Bronchial Stump Dehiscence
Diagnostic Checklist
Cardiac Tamponade
Must be aware of this condition, call the surgeon

Heart shifts toward the operative side Metastases or sarcomas most common 4
Seen with either right or left-sided herniation
Pulmonary Embolus 51
a Right-sided herniation
Usually unmistakable Rare in the immediate post-operative period, more
Organo-axial rotation with cardiac apex on the common 2nd post-operative week
wrong side of the chest Chest radiograph may be normal
From CT perspective, heart undergoes No change in the pneumonectomy space
counterclockwise rotation CT pulmonary angiogram usually diagnostic
Clockwise rotation of indwelling Swan-Ganz Intrathoracic Hemorrhage
catheter New or rapid mediastinal widening or rapid filling
Empty pericardial sac pneumonectomy space
a Left-sided herniation Normal cardiac contour unless obscured by
Less easily recognizable hemorrhage
Marked shift of the mediastinum to the left
Notch along the left heart border at the level of Bronchial Stump Dehiscence
the pulmonary artery Enlarging pneumonectomy space with mediastinal
Abnormal cardiac contour with bulging left heart shift to the non-operative lung
border
Cardiac Tamponade
CT Findings Pulsus paradoxus
CECT Cardiac size may be normal or increased in size
a Rarely performed due to acuteness of presentation No change in pneumonectomy space
a Same findings seen with chest radiography
Imaging Recommendations
Best imaging tool: Portable chest radiographs have to
I PATHOLOGY
suffice, diagnosis usually radiographic General Features
General path comments
a Right-sided herniation tends to be complete
I DIFFERENTIAL DIAGNOSIS Torsion of the atriocaval junction with right
ventricular outflow tract obstruction
Airway Obstruction a Left -sided herniation tends to be partial
Inability to exchange air Strangulation with myocardial damage
Relieved with immediate suction Genetics: Defect may be congenital, if so usually on
Atelectasis or collapse of the non-operated lung the left
Pneumonectomy space will get bigger Etiology
Congenital Absence Left Pericardium a Common clinical setting
Leftward displacement of the heart Pneumonectomy or lobectomy
Partial pericardial resection to remove tumor or
Lung interposed between heart and diaphragm
Air-filled aorticopulmonary window access the pulmonary artery
Failure to close defect
Pericardial Mass a Factors that increase the chance of herniation
Abnormal cardiac contour due to mass and effusion Vigorous chest tube suction
 CARDIAC VOLVULUS
Rolling patient to the operative side (defect now
dependent and heart can fall out of pericardium)
Severe coughing
o Traumatic pericardial rupture usually left-sided and
occurs along the course of the phrenic nerve
Gross Pathologic & Surgical Features
Advantages intrapericardial approach
o Easier access to major pulmonary vessels for ligation
o More extensive excision of tumor can be achieved
Generally takes three days for adhesions to form
between cut edge of pericardium and heart
Defect does not have to be large, 8-10 em typical
Staging, Grading or Classification Criteria
Heart injury trauma scale
o Grade 1
Blunt cardiac injury or blunt or penetrating
pericardial wound without cardiac injury
o Grade 2
Blunt cardiac injury with heart block or
4 penetrating myocardial wound not extending to
endocardium
52 o Grade 3
Blunt cardiac injury with sustained or multifocal
ventricular contractions or cardiac injury with
septal rupture, pulmonary or tricuspid valve
incompetence, papillary muscle dysfunction or
distal coronary arterial occlusion
Blunt pericardiallaceration with cardiac
herniation
Blunt cardiac injury with cardiac failure
o Grade 4
Blunt or penetrating cardiac injury with aortic
mitral valve incompetence
Grade 3 injury with cardiac failure
Aortic or mitral valve incompetence or cardiac
injury of right ventricle, right atrium, or left
atrium
o Grade 5
Proximal coronary arterial occlusion or left
ventricular perforation
Stellate wound with < 50% tissue loss of the right
ventricle or right or left atrium
o Grave 6
Blunt avulsion of the heart or penetration wound
producing> 50% tissue loss of a chamber
I CLINICAL ISSUES
Presentation
Most common signs/symptoms
o SVC syndrome (50%)
o Sudden hypotension and shock (50%)
Other signs/symptoms
o Apex beat in opposite chest
o Bizarre electrocardiographic pattern
Natural History & Prognosis
Usually occurs in the immediate post-operative period
Adhesions develop in 3 days that prevent herniation
later, rare reports of delayed herniation 6 or more
months later
Mortality 40%
Late sequel
o Post-pericardiotomy syndrome
2-4 weeks following surgery
Fever, chest pain
Usually self-limited pericardial effusion, maybe
treated with steroids
o Constrictive pericarditis
Late problem months to years later
Dyspnea, peripheral edema
Thickened pericardium (> 3.5 mm)
Paradoxical septal motion at MR or CT
Enlarged hepatic veins and vena cava at MR or CT
Treatment
Rapid diagnosis and treatment essential
o Surgical goal: Close the pericardial defect or enlarge
the pericardial defect to prevent strangulation of the
heart
Roll immediately to non-operative side
May have to re-operate in hallway or intensive care
unit
I DIAGNOSTIC CHECKLIST
Consider
Must be aware of this condition, call the surgeon
Image Interpretation Pearls
Heart shifting into the operative side worrisome for
impending herniation
I SELECTED REFERENCES
1. LeeJ et al: Blunt pericardial rupture with cardiac
herniation: unusual radiographic findings. J Trauma.
56(1):211,2004
2. Zandberg IT et al: Sudden cardiac herniation 6 months
after right pneumonectomy. Ann Thorac Surg.
78(3):1095-7,2004
3. Shimizu J et al: Cardiac herniation following
intrapericardial pneumonectomy with partial
pericardiectomy for advanced lung cancer. Ann Thorac
Cardiovasc Surg. 9(1):68-72, 2003
4. Trentin C et al: Cardiac herniation after pneumonectomy:
report of 2 cases. Radiol Med (Torino). 105(3):230-3, 2003
5. Kim EAet al: Radiographic and CT findings in
complications following pulmonary resection.
Radiographies. 22(1):67-86, 2002
6. Buniva Pet al: Cardiac herniation and torsion after partial
pericardiectomy during right pneumonectomy. Tex Heart
Inst J. 28(1):73, 2001
7. Collet e Silva FSet al: Cardiac herniation mimics cardiac
tamponade in blunt trauma. Must early resuscitative
thoracotomy be done? Int Surg. 86(1):72-5, 2001
8. Schir F et al: Blunt traumatic rupture of the pericardium
with cardiac herniation: two cases diagnosed using
computed tomography. Eur Radiol. 11(6):995-9, 2001
9. Carrillo EHet al: Cardiac herniation producing tamponade:
the critical role of early diagnosis. J Trauma. 43(1):19-23,
1997
10. Brady MBet al: Cardiac herniation and voivulus:
radiographic findings. Radiology. 161(3):657-8, 1986
II. Gurney JW et al: Impending cardiac herniation: the snow
cone sign. Radiology. 161(3):653-5, 1986
 CARDIAC VOLVULUS

I IMAGE GALLERY

(Left) Anteroposterior
radiograph shows abnormal
cardiac contour (arrows)
following right
pneumonectomy. (Right)
Anteroposterior radiograph
follow-up shows complete
volvulus. Superior vena cava
obstruction (arrow) and
empty pericardium (open
arrows). Note the abnormal
notch (curved arrow).

4
53
(Left) Anteroposterior
radiograph shows abnormal
bulge along right heart
border (arrows) from
impending herniation
following right
pneumonectomy. (Right)
Anteroposterior radiograph
shows complete volvulus,
widened mediastinum from
superior vena obstruction
(arrow), and abnormal notch
(curved arrow).

(Left) Anteroposterior
radiograph shows abnormal
cardiac bulge (arrows) into
the right pneumonectomy
space from impending
herniation. (Right)
Anteroposterior radiograph
shows complete volvulus.
Superior vena cava
obstruction and empty
pericardium.
Ventilation and Lung Volumes IV-5-2
Lung Perfusion IV-5-6
Gravitational Lung Gradients IV-5-10
Aspiration IV-5-14
 VENTILATION AND LUNG VOLUMES

Frontal radiograph shows marked hyperinflation Axial HRCT shows normal air-trapping in the most
Flattened diaphragms at level of 8th anterior ribs. commonly encountered areas in the superior segment
Diaphragmatic slips on the right (arrows). Vesselsare left lower lobe (arrows) and the tip of the lingula
sparse. Air trapping from diffuse emphysema. (curved arrows).

Average normal lobar lung volumes (adult male)

ITERMINOLOGY o Right upper lobe: 1,140 ml
Abbreviations and Synonyms o Left upper lobe: 1,160 ml
Air trapping o Right middle lobe: 670
5 Definitions
o Left lower lobe: 1,550 ml
o Right lower lobe: 2,000 ml
2 Airway branching
a Asymmetric dichotomous pattern (each parent
divides into 2 daughter branches of unequal size) IIMAGING FINDINGS
Approximately 17 orders (counting from
peripheral to central) Radiographic Findings
o Airway branches -55,000 Expiratory chest radiography
a Total airway volume: 175 ml o Usefulness: Detect air trapping and make
Airway types: Conducting and respiratory pneumothorax more conspicuous
a Conducting airways Pneumothorax principle: Volume of air in pleural
Trachea to terminal bronchiole, small volume, space will not change with respiration but
high turbulent airflow in the first 6 generations proportionally takes up more volume at
o Respiratory airways expiration and easier to detect - (however, not
Respiratory bronchioles, large volume, diminished shown to be useful in observational studies)
laminar airflow o Full expiration = residual volume (RV): 1,680 ml
Normal lung volumes in adult normal adult
o Total lung volume: 6720 ml o Hemidiaphragms lies at the level of the 6th rib
o Tidal volume: 500 ml o Heart more transverse
Dead space volume: ISO ml o May be misinterpreted as interstitial lung disease

DDx: Airways Disease Mimics

Compensatory Hyperinflation Mosaic Perfusion Chronic Embolism Vascular Tree-In-Bud

 VENTILATION AND LUNG VOLUMES

Key Facts
Imaging Findings Pathology
Full expiration = residual volume (RV): 1,680 ml Small airways < 2 mm constitute < 2S% of the total
normal adult airway resistance
Functional lung: -910 HU to -600 HU Disease affecting primarily small airways can be
Emphysematous lung: -911 HU to -1024 HU extensive yet not affect airway resistance or dynamic
Mosaic attenuation due to small airways disease pulmonary function
Normal lung increases homogeneously in CT
attenuation in expiration with a dorsal gradient in
Clinical Issues
dependent lung due to gravity Pulmonary function tests global test of all airways,
Lobular air trapping can be identified in 60% of which contribute unequally to function
normals (generally no more than 1 lobule per CT If FEV1 greater than 2L or 50% predicted: Major
thoracic complications rare
section)
Top Differential Diagnoses Diagnostic Checklist
Compensatory Hyperinflation Dust exposed workers seeking compensation may
Mosaic Pattern from Pulmonary Vascular Disease perform expiratory maneuver in hopes of getting a
false positive diagnosis of interstitial lung disease

Hyperinflation (finding subjective and poor o Provides physiologic information concerning

sensitivity) regional lung function
o Hemidiaphragm flattening, level of the 7th rib o Normal lung increases homogeneously in CT
anteriorly attenuation in expiration with a dorsal gradient in
o Enlarged retrosternal clear space (> 2.5 cm between dependent lung due to gravity
sternum and ascending aorta) o Mean increase in lung attenuation: -130
o Increased radiolucency of the lung
o Widened pulmonary vascular branching pattern
(nondependent) - -220 (dependent) HU
o Lobular air trapping can be identified in 60% of
5
with decrease in normal number of vessels normals (generally no more than 1 lobule per CT 3
o False positive section)
Young athletes with large total pulmonary More common in the superior segments of the
capacity lower lobes and the ventral aspects of the right
Poor film technique middle lobe and lingula
Radiographic lung volumes Imaging Recommendations
o Assumptions made about geometric shapes
Best imaging tool: Expiratory HRCT to demonstrate air
introduces error into volume calculations
o Less accurate than pulmonary function trapping
o Never attained clinical status
CT Findings I DIFFERENTIAL DIAGNOSIS
NECT
o Lung density Compensatory Hyperinflation
Functional lung: -910 HU to -600 HU Normal physiologic response from loss of lung volume
Emphysematous lung: -911 HU to -1024 HU Signs of lobar collapse
HRCT Mosaic Pattern from Pulmonary Vascular
o Signs of small airways disease
Centrilobular ground-glass nodules or Disease
ground-glass opacities Mosaic pattern usually less well demarcated than that
Tree-in-bud pattern: Small centrilobular nodules seen with airways disease
connected to multiple branching linear structures Central pulmonary artery enlargement from
o Mosaic attenuation due to small airways disease pulmonary arterial hypertension
Areas of mixed ground-glass opacities sharply
demarcated from hyperinflated lung
More marked with expiratory scanning I PATHOLOGY
Pulmonary vasculature in hyperinflated lung
attenuated and smaller (due to hypoxic
General Features
vasoconstriction) General path comments
o Terminal bronchioles: > 30,000 with a mean
Expiratory CT
o More sensitive than pulmonary function tests for diameter of 0.48 mm
small airways disease o Distance from smallest terminal bronchiole to the
Pulmonary function tests global test of all airways, interlobular septum, pulmonary vein, or pleura
may be normal with radiographic evidence of reiativeiy constant 2.5 mm
airways disease
 VENTILATION AND LUNG VOLUMES
Small airways < 2 mm constitute < 25% of the total
airway resistance
o Disease affecting primarily small airways can be
extensive yet not affect airway resistance or dynamic
pulmonary function
Pathophysiology
o Lobar obstruction
Collapse develops in ] 8 to 24 hours if breathing
room air
Collapse develops in less than 5 minutes if
breathing 100% oxygen
Nitrogen absorbed slowly, delays the development
of atelectasis
Collapse may not develop with obstruction as
ventilation may occur across the pores of Kohn
and canals of Lambert
o Branching pattern important in distribution of
particulate material suspended in the inhaled air
Turbulent airflow in the larger airways (]st 6
generations) directs particles> 5 u in diameter
against the ciliated airways where they can be
removed from the lung
Particles < 5 u in diameter can deposit in the
respiratory bronchioles within the centrilobular
portion of the lobule
Particles tend to follow the straightest pathway
through the lung which is to the lung periphery
5 Staging, Grading or Classification Criteria
Severity of airway obstruction (FEVI or FVC)
4 Normal inspiratory flow with reduction during
o Mild: 70-79% predicted
o Moderate: 60-69% predicted
" Moderately severe: 50-59% predicted
o Severe: 35-49% predicted
o Very severe: < 35% predicted
I CLINICAL ISSUES
Presentation
Pulmonary function tests global test of all airways,
which contribute unequally to function
Pulmonary function tests: Static lung volumes -
spirometry
o Vital capacity (VC): Maximum volume of air that
can be expired slowly after a full inspiratory effort
Decreased with restrictive lung disease and loss of
strength of respiratory muscles
o Total lung capacity: Total volume of air within the
chest after a maximum inspiration (normal 6,720 ml
adult)
Normal inspiratory chest radiograph
o Functional residual capacity (FRC): Volume of air in
the lungs at the end of a normal expiration (40% of
TLC)
Balance of the outward chest wall recoil forces
with the inward recoil force of the lung
Patients can normally hold their breath longest at
FRC
Increased with emphysema, decreased with
restrictive lung disease and kyphoscoliosis
o Inspiratory capacity = TLC - FRC
o Residual volume: Volume of air remaining in the
lungs at the end of a maximal expiration
Normal expiratory chest radiograph
Normally about 25% of TLC
o Expiratory reserve volume (ERV): FRC - RV
Decreased in obesity (RV normally preserved)
Pulmonary function tests: Dynamic lung volumes
o Forced vital capacity (FVC): Volume of air expired
with maximal force
o Forced expiratory volume in ] sec (FEVl): Volume of
air forcefully expired during the first second after a
full breath
Normally accounts for> 75% of the FVC
o Mean forced expiratory flow during the middle half
of the FVC (FEF2s-?s): Slope of the line that
intersects the spirographic tracing at 25% and 75%
of the FVC
More sensitive indicator of early airway
obstruction
Bronchodilator challenge: Provides information about
reversibility of obstructive process
o Significant response if increase in FVC or FEV1 of
]5-20%
Flow-volume loop: Continuous recording of flow and
volume during a forced inspiratory and expiratory VC
maneuver
o Loop shape reflects the status of the lung volumes
and airways throughout the respiratory cycle
o Variable intrathoracic obstruction
forced expiration (flattened curve)
o Variable extra thoracic obstruction
Reduction of inspiratory flow (flattened curve)
with preservation of expiratory flow
o Fixed upper airway obstruction
Fixed inspiratory and expiratory flow
Substernal goiter, endotracheal neoplasms,
postintubation stenosis
Treatment
Predicted post-operative function
o If FEV, greater than 2L or 50% predicted: Major
thoracic complications rare
o Split-function ventilation/perfusion scanning
o Postoperative FEV1 = measured FEV1 x uptake % in
non-operated lung
Value < 0.8L indicates serious pulmonary
disability and the likelihood of high peri operative
morbidity and mortality
I DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Dust exposed workers seeking compensation may
perform expiratory maneuver in hopes of getting a
false positive diagnosis of interstitial lung disease
I SElECTED REFERENCES
1. Arakawa H et al: Expiratory high-resolution CT:diagnostic
value in diffuse lung diseases. AJRAm J Roentgenol.
175(6):1537-43,2000
 VENTILATION AND LUNG VOLUMES

I IMAGE GAllERY

(Left) Axial HRCT shows

inhomogeneous lung
attenuation. Ground-glass
opacities have larger vessels
(arrow) than less attenuated
lung (open arrow).
Bronchiolitis obliterans from
chronic graft-vs-host disease.
(Right) Axial HRCT at full
expiration. Contrast between
attenuated areas more
conspicuous. Note the
change in thoracic shape
with expiration and the
overall decrease in lung
volume. Often normal lung
(ground-glass) will decrease
in volume.

Typical
(Left) Axial NECT shows
numerous small branching
structures (tree-in-bud)
(arrows) from infectious
bronchiolitis. These small
airways are too small and
their walls too thin to be
identified normally. fRight)
contact radiograph barium
filled airways. Normal
terminal (curved arrow) and
respiratory bronchioles
(arrow) leading to alveolar
sacs. Normally these airways
can not be identified due to
their size on either
radiographs or HRCT.

fLeft) Frontal radiograph at

full inspiration shows patchy
consolidation at right base
farrows). Subtle enlargement
right hilum (curved arrow).
Patient complained of
cough. (Right) Frontal
radiograph at full expiration
shows normal decrease in
left lung volume with no
appreciable change in right
hemithorax. Note movement
of hemidiaphragms. Trachea
is bowed into left hemithorax
(arrow). Bronchus
intermedius completely
obstructed by tumor. Air
trapping right lung.
 LUNG PERFUSION
Frontalangiography shows enlarged long thoracic artery
(arrow) with transpleural collaterals (curved arrow)
supplying the right upper lobe. Patient had chronic
thrombotic occlusion of the truncus anterior.
ITERMINOLOGY
Definitions
Arterial branching pattern
5 o Arteries accompany bronchi
Supernumerary branches common and increase
6 toward the lung periphery
Supernumerary arteries branch at right angles
from the parent artery (airways never have right
angle branches)
Pulmonary artery pressure affected by gravity
o Mean pulmonary artery pressure 15 mm Hg (-20 cm
H20)
Apex lung approximately 20 cm above the left
atrium, mean pressure just adequate to reach apex
of lung
o Mean pulmonary venous pressure 5 mm Hg
o Average pulmonary blood flow 2.0 mL/min-ml
tissue
Perfusion (Q): Gravity alterations
o Main pulmonary artery inclined towards the left
upper lung, the jet effect across the pulmonic valve
increases blood flow 10% above similar regions in
the right upper lobe
DDx: Perfusion Abnormalities
PA Stenosis Atrial Septal Defect
in pulmonary valve stenosis, lelt upper lobe (LUL)
arteries are larger than corresponding arteries in the right
upper lobe (arrows). let effect across the valve
accentuates blood flow in the LUL.
o Zonal blood flow
Blood flow determined by pulmonary arterial (pa),
pulmonary venous (pv), and intra-alveolar
pressure (pA)
Zone 1: pA > pa > pv no blood flow through
collapsed capillaries
Zone 2: pa > pA > pv blood flow determined by
gradient between pulmonary arterial and
pulmonary alveolar pressure
Zone 3: pa > pv > pA blood flow determined by
arterial venous pressure gradient
Perfusion (Q): Nongravity alterations
o Normal 4 fold increase in flow from the central to
the periphery of the lung in isogravitational plane
Possibly related to length of arteries but less well
understood than perfusion alterations due to
gravity
o Secondary pulmonary lobule: Similar gradient from
the core bronchovascular structures decreasing to
the periphery of the lobule
Hypoxic vasoconstriction: Regional regulatory
mechanism to match ventilation with perfusion
Collateral Supply Right Lung
 LUNG PERFUSION
Key Facts
Terminology
Pulmonary artery pressure affected by gravity
Apex lung approximately 20 cm above the left
atrium, mean pressure just adequate to reach apex of
lung
Main pulmonary artery inclined towards the left
upper lung, the jet effect across the pulmonic valve
increases blood flow 10% above similar regions in the
right upper lobe
Normal 4 fold increase in flow from the central to the
periphery of the lung in isogravitational plane
Imaging Findings
Normal artery slightly larger (1.2:1) than adjacent
bronchus, best identified with end-on vessels
I IMAGING FINDINGS
Radiographic Findings
Radiography
o Normal pulmonary vascularity
Lower lung zone pulmonary arteries are larger
than upper zone arteries in the upright lung due
to gravity
Normal artery slightly larger (1.2:1) than adjacent
bronchus, best identified with end-on vessels
Veins run separate from arteries and bronchi
Swan-Ganz catheter tip located in the upper lung
or ventral lung (determined by lateral radiograph)
may give pressure measurements reflecting
alveolar pressure - not the desired pulmonary
venous pressure
Zone 1 or 2 conditions more likely to exist with
hypotension and positive pressure ventilation
(especially with PEEP)
o Pulmonary venous hypertension
With elevation of pulmonary venous pressure,
upper lobe vessels become equal in size to lower
lobe vessels (very subjective finding)
Early sign of left ventricular failure but seen with
any cause of elevated venous pressure (e.g., mitral
stenosis)
o Pulmonary arterial hypertension
Enlarged central pulmonary arteries with
decreased caliber of peripheral arteries
Normal interlobar pulmonary artery < 1.5 cm in
width
o Shunt vascularity (left to right shunt)
Increase in size of pulmonary arteries both central
and peripheral (right interlobar diameter similar
to trachea)
Not seen until shunt exceeds 2.5:1 (pulmonary
artery to aortic flow)
o Decreased flow
Chronic decrease in flow to a lung results in
collateral vessel supply to the lung through
systemic arteries
Swan-Ganz catheter tip located in the upper lung or
ventral lung (determined by lateral radiograph) may
give pressure measurements reflecting alveolar
pressure - not the desired pulmonary venous pressure
Top Differential Diagnoses
Mosaic Pattern from Small Airways Disease
Left to Right Shunts: Atrial Septal Defect (ASD),
Ventricular Septal Defect (VSD), Patent Ductus
Arteriosus (PDA)
Hepatopulmonary Syndrome
Cyanotic Pseudofibrosis
Clinical Issues
Orthodeoxia: Hypoxemia worsens when sitting up
and improves when lying supine
Collaterals especially well developed at right lung
apex, presumably because of the stimulus of
normally decreased upper lung zone flow due to
gravity
Collateral vessels have more unusual coarse and
branching pattern than pulmonary arteries
CT Findings 5
CECT
o Provides qualitative and quantitative measures of 7
pulmonary perfusion by measuring CT density
during bolus of IV contrast
Uses ionizing radiation, good temporal and spatial
resolution, serial studies impractical due to
residual contrast
o Mean pulmonary artery diameters
Main pulmonary artery: 24 mm
Right lobar: 15 mm -left lobar; 13 mm
HRCT
o Mosaic attenuation due to arterial disease
Areas of mixed ground-glass opacities poorly
demarcated from less attenuated lung
No accentuation with expiratory scanning other
than increase in attenuation due to decrease in
aerated lung
Central pulmonary arteries usually enlarged from
pulmonary arterial hypertension
MR Findings
Provides qualitative and quantitative measures of
pulmonary perfusion
o No ionizing radiation, good temporal and spatial
resolution, serial studies practical with arterial spin
labeling sequences (no contrast)
Nuclear Medicine Findings
V/Q Scan
o Provides qualitative and quantitative measures of
pulmonary perfusion
Uses ionizing radiation, limited temporal and
spatial resolution, serial studies impractical
 LUNG PERFUSION
I DIFFERENTIAL DIAGNOSIS
Mosaic Pattern from Small Airways Disease
Mosaic pattern usually more geographic and with
better demarcation between attenuated and less
attenuated lung
Normal central pulmonary arteries
left to Right Shunts: Atrial Septal Defect
(ASD), Ventricular Septal Defect (VSD),
Patent Ductus Arteriosus (PDA)
Pulmonary shunt vascularity
Differentiation classically depends on size of left
atrium and ascending aorta (essentially where shunt
decompresses back into lung)
Normal sized left atrium: ASD
Enlarged left atrium: VSD - pDA
Enlarged ascending aorta: pDA
Hepatopulmonary Syndrome
Liver disease, usually cirrhosis, need not be severe
Widened alveolar-arterial oxygen gradient (Aa
gradient)
Intrapulmonary arteriovenous shunting
Chest radiograph: Bibasilar nodular interstitial
thickening
CT: Dilated peripheral vasculature, arteries 2x the
5 diameter of adjacent bronchi (normal < 1.2)
Decrease diffusion capacity on pulmonary function
8 tests
Diameter of peripheral pulmonary artery directly
correlated with degree of hypoxemia
Improves with liver transplantation
Perfusion scanning: Uptake arterial circulation - brain,
kidneys, spleen (nonspecific, seen with any
right-to-Ieft shunt)
Thought to be secondary to excess nitric oxide (NO), a
potent vasodilator
Cyanotic Pseudofjbrosis
Severe decrease in pulmonary blood flow usually
secondary to pulmonary artery stenosis
Upper lung zone opacities, right greater than left, from
combination of transpleural collaterals and infarcts
Must differentiate from tuberculosis which has a
higher incidence in patients with conditions that
result in pulmonary oligemia
I PATHOLOGY
General Features
Microspheres traditional method to study pulmonary
perfusion in animal models
o Requires tissue sampling, not applicable in humans
Pulmonary capillaries and veins account for 40% of
pulmonary vascular resistance
Gross Pathologic & Surgical Features
Media types: Elastic and muscular; artery nearly
equally divided between elastic and muscular
segments
o Elastic arteries comprise approximately the first 6
generations, the remaining muscular
o Muscular arteries extend to the respiratory
bronchioles
o Supernumerary arteries usually muscular
IClINICAllSSUES
Presentation
Most common signs/symptoms
o Symptoms that suggest abnormal perfusion
Orthodeoxia: Hypoxemia worsens when sitting up
and improves when lying supine
Platypnea: Dyspnea improves when lying supine
Swan-Ganz catheterization (flow-directed
balloon-tipped pulmonary artery catheter)
o Indications
Diagnosis of shock states, hemodynamic forms of
pulmonary edema, diagnosis of pulmonary
hypertension, management of hemodynamic
status
o Directly measures pressure in the right atrium, right
ventricle, pulmonary artery and pulmonary capillary
wedge pressure (pCWp)
o Cardiac output (CO) measured by thermodilution
technique: Normal 4-7 L/min
o Observed values used to calculate pulmonary (pVR)
or systemic vascular resistance (SVR)
Normal pVR 80-120 dynes/cm2
o Cardiac pressures
Central venous pressure and right atrial pressure
normally range from 0-5 mm Hg and varies with
respiration
Pulmonary artery systolic pressure ranges from
20-30 mm Hg and is equal to the right ventricular
systolic pressure
o pCWP
Reflects the left atrial pressure (and indirectly left
ventricular end-diastolic pressure)
Assumes a continuous column of blood across the
capillary bed and pulmonary veins
Zone 2 and 3 conditions do not reflect left atrial
pressure
Downstream conditions such as pulmonary vein
stenosis and mitral stenosis obviate assumption
o PEEP
Positive airway pressures are transmitted to the
vascular space
Amount determined by lung compliance
(L'.volume/L'.pressure)
Less pressure transmitted in stiff lungs (ARDS),
more pressure in less stiff lungs (emphysema)
Esophageal balloon used to estimate pleural
pressure
Corrected pCWP ; measured PCWP - esophageal
pressure
I SElECTED REFERENCES
1. Vonk-Noordegraaf A et al: Noninvasive assessment and
monitoring of the pulmonary circulation. Eur RespirJ.
25(4):758-66, 2005
 LUNG PERFUSION

IIMAGE GAllERY
Typical
(Leh) Axial H RCT 3 mm
MIP Hepatopulmonary
syndrome. The segmental
pulmonary arteries are twice
the size of the adjacent
bronchi (arrows). Patient's
hypoxemia worsened in the
upright position
(orthodeoxia). (Right) Axial
HRCT 3 mm MIP shows
marked peripheral dilatation
of the pulmonary arteries in
hepatopulmonary syndrome.
Liver disease does not need
to be severe. Abnormal
vessels may resolve following
liver transplantation.

Typical
(Leh) Axial HRCT at full
inspiration. Mosaic
attenuation with disparity in
vsse/size (arrows), which
are decreased in the low
attenuation lung. (Right)
5
Axiall-IRCT at full expiration.
Both the high and low
9
attenuation areas have
increased in density. Mosaic
perfusion due to chronic
thromboembolism.

(Left) Frontal radiograph

shows abnormal opacity at
the right apex (curved
arrow). Severe pulmonary
valve stenosis. Both
pulmonary arteries small
(arrows). Cyanotic
pseudofibrosis. Tuberculosis
must be excluded. Opacity
combination of co/laterals
and scarring. (RighI) Lateral
radiograph shows calcified
pulmonary valve (curved
arrow). The right and left
pulmonary arteries are small
in caliber (arrows). Paucity
of peripheral pulmonary
vessels.
 GRAVITATIONAL LUNG GRADIENTS
Frontal radiograph shows diffuse interstitia/lung disease
worse in the upper lung zones. Diagnosis was chronic
Farmer's lung.
ITERMINOLOGY
Definitions
Perfusion (Q) upright lung
5 o Normal 18 fold difference from the top to bottom of
the lung due to effect of gravity on low pressure
10 pulmonary arterial system
Main pulmonary artery inclined towards the left
upper lung, the jet effect across the pulmonic
valve increases blood flow 10% above similar
regions in the right upper lobe
a Zonal blood flow
Blood flow determined by pulmonary arterial (pa),
pulmonary venous (pv), and alveolar pressure (pA)
Zone 1: pA > pa > pv, capillaries collapsed, no
blood flow, does not occur in normal lung but in
hypotensive patient on mechanical ventilation
and PEEP may develop zone 1 conditions in the
non dependent lung
Zone 2: pa > pA > pv, blood flow not determined
by gradient between arterial and venous pressure
but gradient between arterial and alveolar pressure
and may produce erroneous clinical pressure
readings from a wedged Swan-Ganz catheter
DDx: Gravitational Zones
EG Upper Lung Zone IPF Peripheral Zone
Frontal radiograph shows ground-glass and fine relicular
opacilies predominanUy within the periphery of the
lower lung zones (arrows) in this patient with idiopathic
pulmonary fibrosis.
Zone 3: pa > pv > pA, blood flow determined by
arterial venous pressure gradient
a Pathophysiology
Determines the distribution of hydrostatic
(cardiogenic edema) and blood borne insults
(hematogenous metastases)
Ventilation (V) upright lung
a Normal 3 fold difference from the top to the bottom
of the lung due to effect of gravity on intrapleural
pressure, transpulmonary gradients and alveolar size
a Branching pattern of the airways important in
distribution of particulate material suspended in the
inhaled air
Turbulent airflow in the large upper airways (1st 6
generations) directs particles> SIJ in diameter
against the ciliated airways where they can be
removed from the lung
Particles < SIJ in diameter can deposit in the
respiratory bronchioles within the centrilobular
portion of the lobule
Particles tend to follow the straightest pathway
through the lung which is to the lung periphery
o Pathophysiology
Asbestosis Lower Zone
 GRAVITATIONAL LUNG GRADIENTS
Key Facts
Terminology
Main pulmonary artery inclined towards the left
upper lung, the jet effect across the pulmonic valve
increases blood flow 10% above similar regions in the
right upper lobe
Upper lobes relatively alkalotic: Environment also
shared by gastric wall and medulla of kidney
Imaging Findings
Best diagnostic clue: Zonal distribution of disease as
important as patterns in the differential diagnosis of
diffuse lung disease
Normal physiologic gradients create zones or regions
of the lung which differ in terms of blood flow,
ventilation, lymphatic function, stress, and
concentrations of inhaled gases
Determines the distribution of inhaled insults
which acutely affect the lung
Ventilation to perfusion ratio (V/QJ upright lung
o Normal ratio decreases from the top (3.3) to the
bottom of the lung (0.6)
o Pathophysiology
Determines the concentration of inhaled gas
o Alveolar oxygen concentration upright lung
Apex lung: 132 mmHg and base lung: 89 mmHg
pH upright lung
o Normal pH decreases from apex lung (7.51) to base
(7.39)
o Upper lobes relatively alkalotic: Environment also
shared by gastric wall and medulla of kidney
o Pathophysiology
Affects the distribution of calcium which is less
soluble in alkalotic environment
Intrapleural pressure upright lung
o Normal pleural pressure increases down the lung
from apex (-10 cm water) to base (-2.5 cm water)
o Large transpulmonary pressure in upper lung zones
expand alveoli which are 4x larger at the top than at
the bottom
50% of total change in alveolar volume occurs
over the top 4 cm of the lung
Mi.iller maneuver (inspire against a closed glottis)
can generate pressures down to -80 mmHg
Valsalva maneuver (forced expiration against a
closed glottis) can generate pressures> 100 mmHg
o Pathophysiology
Determines the distribution of stress, (blebs, bullae
and cavities all more common in upper lung zone)
Lymphatic flow upright lung
o Flow 10-50 ml!hr
o Linearly increase down the lung due to gravitational
increase in pulmonary arterial pressure
o Aided by kinetics of respiratory motion
Diaphragmatic motion and excursion of anterior
and lateral chest wall
o Regions with slowest lymphatic flow
Concept that the lung is a map, with zones not
defined by anatomy but by regional differences in
physiology
Lung reacts to the internal environment to which it is
exposed
Morphology: In contrast to organs such as the brain
or kidney, the lung has no anatomic demarcation
such as the gray-white matter differentiation in the
brain or cortex-medulla organization in the kidney
Truly uniform distribution of disease pathologically
will be more apparent in the lower lung zones due to
summation across the greater thickness of the lower
lung zones
Truly uniform distribution of disease radiologically
may actually be more profuse in the upper lung zones
pathologically due to less summation across the less
thick upper lung zones
Upper lobes, especially the right due to slightly
higher pulmonary blood flow in the left upper
lung
Dorsal lung due to relatively decreased respiratory
motion posteriorly
o Pathophysiology
Determines the distribution of disease due to
chronic clearance of particulate material
5
11
IIMAGING FINDINGS
General Features
Best diagnostic clue: Zonal distribution of disease as
important as patterns in the differential diagnosis of
diffuse lung disease
Location
o Normal physiologic gradients create zones or regions
of the lung which differ in terms of blood flow,
ventilation, lymphatic function, stress, and
concentrations of inhaled gases
o Concept that the lung is a map, with zones not
defined by anatomy but by regional differences in
physiology
o Lung reacts to the internal environment to which it
is exposed
Morphology: In contrast to organs such as the brain or
kidney, the lung has no anatomic demarcation such as
the gray-white matter differentiation in the brain or
cortex-medulla organization in the kidney
Radiographic Findings
Radiography
o Distribution of disease usually readily apparent from
the frontal radiograph
Lung much thicker at the base than at the apex
Truly uniform distribution of disease
pathologically will be more apparent in the lower
lung zones due to summation across the greater
thickness of the lower lung zones
 GRAVITATIONAL LUNG GRADIENTS
Truly uniform distribution of disease o Tall lung at risk for
radiologically may actually be more profuse in the Spontaneous pneumothorax
upper lung zones pathologically due to less Silicosis
summation across the less thick upper lung zones o Short lung at risk for
o Disease process may be predominantly upper, lower, Asbestosis
or peripheral lung zone
Gross Pathologic & Surgical Features
CT Findings Regional physiology
HRCT o Upper lung zones
o Distribution of disease in cross-section readily Low blood flow
apparent Underventilated
Disease process may be predominantly peripheral, High oxygen concentration
dorsal, ventral, or central within the lung Low carbon dioxide concentration
o Vertical distribution apparent on reformation in the Alveoli expanded
coronal plane High stress and strain
Large transpulmonary pressure
Nuclear Medicine Findings o Lower lung zones
Standard tool with which physiologic processes in the High blood flow
lung studied Overventilated
Imaging Recommendations Low oxygen concentration
High carbon dioxide concentration
Best imaging tool
o Chest radiography to map the vertical distribution Alveoli small
Low stress and strain
of disease
Small trans pulmonary pressure
o CT or HRCT to map the horizontal or cross-sectional
distribution of disease
I CLINICAL ISSUES
5 I DIFFERENTIAL DIAGNOSIS Presentation
Upper Zone Lung Disease Most common signs/symptoms
12 o Platypnea-orthodeoxia
Sarcoidosis
Upright dyspnea and hypoxemia relieved with
Langerhans cell granulomatosis
supine positioning
Silicosis and Coal-worker's pneumoconiosis
o Differential diagnosis
Post-primary Tuberculosis
Right to left shunts
Chronic eosinophilic pneumonia
Hepatopulmonary syndrome
Emphysema, centrilobular
Arteriovenous malformations
Smoke inhalation
o Trepopnea
Neurogenic pulmonary edema
Dyspnea in decubitus position
Metastatic pulmonary calcification
o Differential diagnosis
Ankylosing spondylitis
Unilateral lung or pleural disease
Cystic fibrosis
Patients in congestive heart failure prefer the right
Lower Zone Lung Disease lateral decubitus position
Asbestosis
Lymphangitic metastases
Hematogenous metastases I DIAGNOSTIC CHECKLIST
Scleroderma
Idiopathic pulmonary fibrosis
Image Interpretation Pearls
Alpha-l antiprotease deficiency Distribution of disease important finding in
differential diagnosis
Peripheral Zone Lung Disease
Idiopathic pulmonary fibrosis (IPF)
Asbestosis I SELECTED REFERENCES
Hematogenous metastases 1. Murray JF: Bill Dock and the location of pulmonary
Chronic eosinophilic pneumonia tuberculosis: how bed rest might have helped
Scleroderma consumption. Am J Respir Crit Care Med. 168(9):1029-33,
2003
2. Gurney JW: Cross-sectional physiology of the lung.
I PATHOLOGY Radiology. 178(1):1-10, 1991
3. Gurney JW et al: Upper lobe lung disease: physiologic
General Features correlates. Review. Radiology. ] 67(2):359-66, 1988
Genetics
o Tall lung has more regional disparity than short
lung, little studied in diffuse lung disease
 GRAVITATIONAL LUNG GRADIENTS

I IMAGE GALLERY
Typical
(Left) Frontal radiograph
shows perihilar and upper
lung zone progressive
massive fibrosis from silicosis
due to poor clearance of
lymphatic particles from the
upper lung zones due to
normal gravitational
physiology. (Right) Axial
H ReT shows centriacinar
and subpleural nodules
aggregating together in the
dorsal aspect of the lung.
Lymph flow is decreased
dorsally compared to the
anterior lung.

Typical
(Leh) Frontal radiograph
shows diffuse reticular
interstitial disease and
honeycombing more severe
in the bases. (Right) Axial
HRCT shows peripheral
5
subpleural honeycombing
from IPF.Note summation 13
effect. Chest radiograph
appearance of distribution is
uniform across the lung
when actually the disease is
predominantly peripheral.

Typical
(Left) Frontal radiograph
shows diffuse fine reticular
interstitial thickening slightly
more profuse through the
lower lung zones. Note the
dilated esophagus (arrows).
(Right) Axial HRCT shows
intralobular septal thickening
and volume loss primarily in
lower lobes in this patient
with scleroderma. Dilated
esophagus (arrow).
 ASPIRATION
Anteroposterior radiograph shows bilateral ill-defined
air-space consolidations in the righl upper and lefllower
lobes. Differential: pneumonia (baclerial, viral), edema,
or hemorrhage.
!TERMINOlOGY
Abbreviations and Synonyms
Aspiration pneumonia: Pulmonary infection caused by
5 aspiration of colonized oropharyngeal secretions
Aspiration pneumonitis: Acute lung injury caused by
14 the aspiration of materials inherently toxic to the
lungs (gastric acid, milk, mineral oil, and volatile
hydrocarbons)
Definitions
Intake of a variety of solid or liquid materials into the
airways and lungs
Predisposing factors: Alcoholism, loss of
consciousness, structural abnormalities of the pharynx
and esophagus, neuromuscular disorders, and
deglutition abnormalities
Different aspiration syndromes
o Foreign bodies: Most common endobronchial mass
in children; food particles (vegetables) and broken
fragments of teeth (elderly); main and lobar
bronchi, commonest location
DDx: Diffuse Bilateral Opacities
PCP Pneumonia Alveolar Proteinosis
Axial CECT in same patient shows left lower lobe
homogeneous consolidation due to aspirated secretions
and atelectasis. Air bronchogram and opacified vessels
("CT angiogram" sign).
o Near Drowning: Pulmonary edema after acute
aspiration of massive amounts of fresh or salt water;
pneumonia may occur depending on the water
composition
o Mendelson syndrome: Aspiration of sterile gastric
contents during labor and delivery; can be severe
and fatal
o Exogenous lipoid pneumonia: Repeated aspiration
or inhalation of mineral oil or a related substance; in
children, cod liver oil and milk; in adults, oily nose
drops
o Lentil aspiration pneumonia: Granulomatous
pneumonitis caused by aspiration of leguminous
material such as lentils, beans, and peas
o Hydrocarbon pneumonia: Accidental poisoning in
young children; hydrocarbon-containing fluids
(petroleum) in fire eaters (fire-eaters pneumonia)
IIMAGING FINDINGS
General Features
Best diagnostic clue
o Gravity-dependent opacities
Hemorrhage
 ASPIRATION

Key Facts
Terminology In infants, lateral decubitus radiographs to investigate
Aspiration pneumonia: Pulmonary infection caused air trapping
by aspiration of colonized oropharyngeal secretions Top Differential Diagnoses
Aspiration pneumonitis: Acute lung injury caused by Bronchogenic carcinoma
the aspiration of materials inherently toxic to the Alveolar proteinosis
lungs (gastric acid, milk, mineral oil, and volatile
Bronchioloalveolar cell carcinoma
hydrocarbons)
Predisposing factors: Alcoholism, loss of Clinical Issues
consciousness, structural abnormalities of the Cough, wheezing, cyanosis, and tachypnea
pharynx and esophagus, neuromuscular disorders, Abrupt onset: After meat aspiration, mimics
and deglutition abnormalities myocardial infarction ("cafe coronary syndrome")
May resemble asthma
Imaging Findings
Gravity-dependent opacities Diagnostic Checklist
Radiopaque material within the airways (foreign Radiopaque opacity within the airways specific
body) finding
Atelectasis, segmental or lobar

o Radiopaque material within the airways (foreign o Near drowning: "Sand bronchogram" (radiopaque) if
body) sand is aspirated along with water
o Nodules and pneumatoceles after aspiration of
Radiographic Findings liquid hydrocarbon
Radiography o Associated complications: Necrotizing pneumonia,
o Unilateral or bilateral air-space consolidation in abscess, ARDS, and pulmonary embolism
dependent distribution
Recumbent patients: Superior segments of the
Scintigraphy with radio-labeled food
o Useful to document aspiration
5
lower lobes and posterior segments of the upper
lobes Imaging Recommendations 15
Upright patients: Basal segments of the lower In infants, lateral decubitus radiographs to investigate
lobes air trapping
o Diffuse perihilar consolidation o Dependent lung is less inflated (gravity)
o Consolidation with cavitation o Air-trapping: Dependent lung remains inflated and
Necrotizing pneumonia hyperlucent
Abscess Scintigraphy with radio-labeled food to document
o Focal mass aspiration
o Atelectasis, segmental or lobar
o Intrabronchial radiopaque material
Obstructive hyperinflation and air-trapping on I DIFFERENTIAL DIAGNOSIS
expiratory radiograph; common in children
Contralateral mediastinal shift Multiple Areas of Consolidation
Obstructive pneumonitis Diffuse bilateral opacities: Pulmonary edema;
Bronchiectasis: When long-standing retention of hemorrhage; diffuse alveolar damage
intra bronchial material Multifocal (patchy) airspace opacities: Organizing
pneumonia; eosinophilic pneumonia; sarcoid;
CT Findings tuberculosis; vasculitis
CTA: Can be used to evaluate for complications:
Abscess, empyema, and pulmonary embolism Atelectasis
NECT Bronchogenic carcinoma
o Air-space consolidation, solitary or multiple; Bronchial carcinoid
gravitational distribution Endobronchial metastases
o Atelectasis segmental or lobar Broncholithiasis
o Intra bronchial foreign bodies; CT valuable in
Crazy-Paving Pattern
selected cases (radiolucent material)
o Granulomatous pneumonitis (lentil aspiration Alveolar proteinosis
Pneumocystis jiroveci pneumonia
pneumonia)
Bronchioloalveolar cell carcinoma
Centrilobular ill-defined nodules
Linear and branching structures ("tree-in-bud") Pulmonary hemorrhage
Miliary nodules (foreign body reaction ARDS
granulomas) Focal Mass
o Lipoid pneumonia: Low attenuation (fat density) Bronchogenic carcinoma
focal consolidation and "crazy-paving" pattern
 ASPIRATION
!PATHOlOGY
General Features
Etiology: Variable; depends on the nature and amount
of aspirated material
Epidemiology
o 300,000 to 600,000 cases per year in the United
States
o Five to 15% of cases of community acquired
pneumonia
o Foreign bodies most common in healthy infants and
small children
Associated abnormalities
o Hiatal hernia; Zenker diverticulum; gastroesophageal
reflux
o Adults often have underlying conditions (e.g.,
neurologic disorders, alcoholism, esophageal
disorders, on mechanical ventilation,
tracheoesophageal fistula)
Gross Pathologic & Surgical Features
Food particles or teeth may be noted within the
airways
Edema and acute inflammation of airway (acutely)
Air-space edema, hemorrhage, organizing
bronchopneumonia, bacterial abscesses (acutely):
Bronchiectasis and fibrosis (chronically)
5 Image Interpretation
Microscopic Features
16 Airway
o Incorporation of foreign material in granulation
tissue
o Intraluminal granulation tissue, bronchostenosis, or
bronchiectasis
Lung
o Food particles, neutrophils, mononuclear cells and
giant cells, < 48 hours
o Pulmonary edema, hyaline membrane formation,
and alveolar hemorrhage (Mendelson syndrome)
o Alveolar lipid-laden macrophages, interstitial
accumulation of lipid material, variable amount of
fibrosis
o Pneumonia often due to aerobic, anaerobic, or
actinomycosis infection
o In chronic disease, features include recurrent
pneumonia, well-organized granulomas, obliterative
bronchiolitis, bronchiectasis, and fibrosis
o Cellular bronchiolitis and scattered interstitial
epithelioid granuloma with or without central
necrosis in response to durable cellulose
I CLINICAL ISSUES
Presentation
Most common signs/symptoms
o Variable; depends on the amount and type of
aspirated material
o Acute aspiration
Cough, wheezing, cyanosis, and tachypnea
Abrupt onset: After meat aspiration, mimics
myocardial infarction ("cafe coronary syndrome")
o Chronic or recurrent aspiration
May resemble asthma
Insidious onset with recurrent pneumonias;
usually basilar; may be multifocal (different
locations)
Other signs/symptoms: Occasionally hemoptysis
Natural History & Prognosis
up to 50% death rate for patients who develop ARDS
from Mendelson syndrome
Treatment
Prevention
o Medications to reduce gastric pH
o Elevation of head of bed
o Gastric suction with nasogastric tube
Post-aspiration
o Antibiotics for pneumonia
o Bronchoscopy to remove foreign bodies (i.e.,
peanuts, beans, teeth, etc.)
Surgery for gastroesophageal reflux for those failing
medical therapy
I DIAGNOSTIC CHECKLIST
Consider
High index of clinical suspicion
Pearls
Gravity dependent opacities should think aspiration
Radiopaque opacity within the airways specific finding
I SElECTED REFERENCES
1. Lee, KHet al: Squalene aspiration pneumonia in children:
radiographic and CT findings as the first clue to diagnosis.
Pediatr Radiol. 35: 619-623, 2005
2. Baron, SEet al: Radiological and clinical findings in acute
and chronic exogenous lipoid pneumonia.) Thorac
Imaging. IB: 217-224, 2003
3. Gimenez, A et al: Thoracic complications of esophageal
disorders. Radiographies. 22: S247-258, 2002
4. Marik PE:Aspiration pneumonitis and aspiration
pneumonia. N Engl) Med. 344:665-71,2001
5. Franquet T et al: Aspiration diseases: Findings, pitfalls, and
differential diagnosis. Radiographies. 20:673-85, 2000
6. Franquet T et al: Fire eater pneumonia:radiographic and CT
findings.] Comput AssistTomogr. 24:448-450,2000
7. Kavanagh PVet al: Thoracic foreign bodies in adults.
Clinical Radiology. 54:353-360,1999
8. Marom EMet al: The many faces of pulmonary aspiration.
A)R.I72:121-128,1999
9. Marom EMet al: Lentil aspiration pneumonia:
radiographic and CT findings.) Com put AssistTomogr.
22:598-600, 1998
10. Matuse T et al: Importance of diffuse aspiration
bronchiolitis caused by chronic occult aspiration in the
elderly. Chest. 110:1289-1293, 1996
11. Fox KAet al. Aspiration pneumonia following surgically
placed feeding tubes. Am) Surg. 170:564-566, 1995
12. DePaso W): Aspiration pneumonia. Clin Chest Med.
12:269-284,1991
13. Hunter TB et al: Fresh-water near-drowning: radiological
aspects. Radioiogy. 112:51-56, 1974
 ASPIRATION

I IMAGE GALLERY
Typical
(Left) Anteroposterior
radiograph shows extensive
symmetrical bilateral
consolidation after massive
gastric aspiration. (Right)
Anteroposterior radiograph
(magnified view) shows a
tooth in the main left
bronchus (arrow).

Typical
(Left) Axial HRCT in a fire
eater shows bilateral cysts
(pneumatoce/es), patchy
areas of ground-glass
attenuation, and nodular
opacities in the lower lobes.
5
(Courtesy O. Gomez-Santos,
MO). (Right) Anteroposterior
17
radiograph shows a
radiopaque foreign body
within the left main bronchus
(arrow).

Typical
(Left) Anteroposterior
radiograph (close-up view)
shows bilateral esophageal
speech devices (arrows)
within both main bronchi in
this 60 year old man after
radical laryngectomy (Right)
Axial NECT in the same
patient confirms the
presence of intra bronchial
foreign bodies. Left lower
lobe is partially collapsed.
Bilateral pleural effusions.
Metastases, Chest IV-6-2
Drug Reaction, Intrathoracic IV-6-6
Radiation-Induced Lung Disease IV-6-10
Immunosuppressed (Not AIDS) IV-6-14
AIDS IV-6-18
Sickle Cell Disease IV-6-22
 METASTASES, CHEST

Graphic shows typical variable-sized pulmonary Frontal radiograph shows multiple lung and pleural
metastatic nodules. Most are distributed in lower lungs metastases larrows) from melanoma. The
and near pleural surfaces. preponderance 01 blood-borne metastases are located
in the periphery of the lower lung.

Sharply-defined, variable-sized round pulmonary

I TERMINOlOGY nodules
Definitions Ill-defined margins seen in hemorrhagic
Cancer in areas of body distant from primary site metastases (commonly: Choriocarcinoma, renal
Lung most common site of metastases: 50% at autopsy cell carcinoma, melanoma)
Routes for spread: Hematogenous, lymphangitic, Preferentially distributed to lower lobes due to
gravitational forces and higher blood flow
bronchogenic
80% located within 2 em of pleura
Cavitation common with squamous or sarcoma
cell types
I IMAGING FINDINGS Miliary pattern seen with medullary carcinoma
6 General Features thyroid, melanoma, renal cell, and ovarian
carcinoma
Best diagnostic clue: Variable sized sharply defined
2 multiple pulmonary nodules Bone-forming tumors may calcify (osteogenic
Location sarcoma, chondrosarcoma, thyroid) and may be
o Hematogenous metastases in bases and lung confused with benign granulomas
periphery Occasionally associated with spontaneous
o Lymphangitic metastases focal, commonly will spare pneumothorax, especially from sarcoma cell type
an entire lobe(s) Solitary metastasis: Renal cell, colon, breast,
sarcomas, melanoma
Radiographic Findings o Lymphangitic pattern
Radiography Due to lymphangitic spread
o Vascular pattern Asymmetric nodular interstitial thickening, often
Due to hematogenous spread will spare a lobe(s)

DDx: Variable Patterns

Renal Thrombus Hemorrhagic Renal Carcinoma Sarcoma Tree-In-Bud

 METASTASES, CHEST
Key Facts
Terminology Occasionally associated with spontaneous
pneumothorax, especially from sarcoma cell type
Lung most common site of metastases: 50% at
Solitary metastasis: Renal cell, colon, breast,
autopsy
sarcomas, melanoma
Routes for spread: Hematogenous, lymphangitic,
bronchogenic Pathology
Imaging Findings Mechanical anatomic model: Metastases are filtered
Best diagnostic clue: Variable sized sharply defined out in the first draining organ, commonly the lung
multiple pulmonary nodules Environmental model: Metastases preferentially find
Ill-defined margins seen in hemorrhagic metastases target sites due to favorable molecular or cellular
(commonly: Choriocarcinoma, renal cell carcinoma, environments, known as the "seed and soil"
melanoma) hypothesis
Miliary pattern seen with medullary carcinoma Clinical Issues
thyroid, melanoma, renal cell, and ovarian carcinoma Germ cell metastases may evolve into benign
Bone-forming tumors may calcify (osteogenic teratomas which may then grow
sarcoma, chondrosarcoma, thyroid) and may be
confused with benign granulomas

Nodular interstitial thickening o Hematogenous nodules often have feeding artery

May be associated with small pleural effusions or ("cherry stem" appearance)
hilar or mediastinal adenopathy o Lymphangitic metastases characterized by
o Endobronchial pattern centrilobular peribronchial thickening (most
Due to bronchogenic seeding within the airways common) or beaded peripheral interlobular septa
or hematogenous dissemination to airway wall o Halo sign for hemorrhagic metastases (solid central
Atelectasis of lobe or lung or segment nodule surrounded by ground-glass opacity)
Post-obstructive pneumonia can be segmental,
lobar, or entire lung Imaging Recommendations
Seen with bronchioloalveolar cell carcinoma, basal Best imaging tool: CT most sensitive examination and
cell carcinomas of head and neck, breast, renal better characterizes pattern and extent of disease
and sarcomas
o Pleural pattern
I DIFFERENTIAL DIAGNOSIS
Due to lymphangitic or hematogenous spread
Pleural effusion, may be massive, free-flowing or
6
Multiple Pulmonary Nodules
loculated 3
Arteriovenous Malformations (AVMs)
Discrete masses in pleura
o Feeding arteries and draining veins
o Consolidative pattern
o "Cherry" stem arteries smaller than arteries entering
Due to hematogenous spread
AVMs
Mimic pneumonia, peripheral consolidation with
air-bronchograms Granulomas
o Often contain benign patterns of calcification
Lipidic growth similar to bronchioloalveolar cell
o Associated with calcifications in liver and spleen
carcinoma
o Bone forming primary tumor metastases maybe
o Pulmonary embolus pattern
confused for granulomas
Due to hematogenous spread
Wegener granulomatosis
Beaded enlarged vessels
o Usually cavitary
Mass conforming to vascular shape
o May be associated with subglottic stenosis
Pulmonary infarcts
o Mediastinal spread: Mediastinal or hilar mass Endobronchial Mass
Due to hematogenous or lymphangitic spread Bronchogenic carcinoma
Common with genitourinary tumors (prostate, o Associated with regional lymphadenopathy
renal, ovarian, testicular, transitional cell), head o More common than endobronchial metastasis
and neck cancers, melanoma, breast o Smoking history
CT Findings Broncholith
CECT o Calcified endobronchial mass
o Mirror chest radiographic findings Foreign body
o Most common endobronchial mass in children
o Most metastases develop within outer 1/3 of the
lung Interstitial lung Disease
o Beaded enlarged vessels for intravascular growth Idiopathic pulmonary fibrosis
o Intravascular tumor conforms to shape of o Septa smooth not beaded, honeycombing and
pulmonary arteries volume loss more common
HRCT
 METASTASES, CHEST
o Basilar and peripheral distribution
Scleroderma or other collagen vascular disease
o Septa smooth, not beaded
o Associated bone changes (rheumatoid) or esophageal
dilatation (scleroderma)
Asbestosis
o Associated with calcified pleural plaques
Chronic Consolidation
Bronchoalveolar cell carcinoma
o Septa usually not involved
o Lobular ground-glass pattern in multiple lobes
coalescing to frank consolidation
Chronic organizing pneumonia (BOOP)
o Subpleural basilar distribution
Pulmonary alveolar proteinosis
o Geographic distribution of ground-glass opacities
containing prominent intralobular thickening (crazy
paving pattern)
o Bronchogenic pattern typical tumors
bronchioloalveolar cell carcinoma (consolidation),
basal cell carcinoma head and neck (endobronchial)
o Mediastinal spread typical tumors nasopharyngeal,
genitourinary (renal, prostate, testicular, transitional
cell, ovarian), breast, melanoma
Associated abnormalities: Skeletal metastases, lytic or
sclerotic common depending on extent and type of
tumor
Gross Pathologic & Surgical Features
Lipidic growth
o No architectural distortion
o Tumor uses the lung as a scaffolding to grow
o Typical of bronchioloalveolar cell carcinoma
Hilic growth
o Architectural distortion
o Tumor expands and displaces surrounding lung
o Typical of hematogenous metastases

Mediastinal Mass Microscopic Features

Bronchogenic carcinoma Typical of primary tumor
o Large solitary mass, may obstruct airways and
Staging, Grading or Classification Criteria
vascular structures
Generally regarded as stage IV for most tumor staging
Lymphoma
o Multiple discrete nodes involving multiple lymph
node groups
ICLINICAL ISSUES
Pulmonary Embolus
Pulmonary embolus Presentation
o Transient, acute symptoms Most common signs/symptoms: Variable, depends on
o Will not distend the vessel pattern of spread, may be asymptomatic
Pulmonary artery sarcoma Demographics
o Common location main pulmonary artery
Age: Any age but more common in adult
o Solitary, not multiple
6 Natural History & Prognosis
Generally poor but depends on treatments available
4 I PATHOLOGY for the primary tumor type
Germ cell metastases may evolve into benign
General Features teratomas which may then grow
General path comments: Pathology reflects metastatic
route Treatment
Etiology Depends on histology of primary tumors, generally
o Metastatic models palliative radiation or chemotherapy
Mechanical anatomic model: Metastases are If lung only site, consider resection, especially if
filtered out in the first draining organ, commonly interval from primary resection to metastases> 1
the lung month
Environmental model: Metastases preferentially o Resection for osteosarcomas, solitary metastases, and
find target sites due to favorable molecular or slow growing tumors
cellular environments, known as the "seed and Percutaneous ablation promising palliative therapy
soil" hypothesis
Epidemiology
o Vascular pattern typical of carcinomas (lung, breast, I DIAGNOSTIC CHECKLIST
gastrointestinal tract tumors) and sarcomas
o Lymphangitic pattern typically of adenocarcinomas Consider
o Pleural pattern typically adenocarcinomas especially Metastatic disease in the differential of radiographic
lung and breast abnormalities in patients with history of malignancy
o Consolidative pattern typical tumor
adenocarcinoma of GI tract or lymphoma
o Pulmonary embolus pattern typical tumors I SELECTED REFERENCES
hepatoma, breast, renal cell carcinoma, 1. Sea JB et al: Atypical pulmonary metastases: Spectrum of
choriocarcinoma, angiosarcoma radiologic findings. Radiographies 21:403-17, 2001
 METASTASES, CHEST
I IMAGE GALLERY

(Left) Frontal radiograph

shows diffuse interstitial
thickening right lung and a
small pleural effusion from
Iymphangitic metastases.
Unilateral differential
includes: Pneumonia,
edema, aspiration, and
radiation fibrosis. fRight)
Axial HRCT in same patient
shows irregular septal
beading (arrow) from
Iymphangitic tumor.
Prominent cenlrilobular core
structures a/50 present.

(Left) Frontal radiograph

shows bibasilar nodules
(arrows) which were difficult
to find on the lateral film
typical of pleural lesions.
(Right) Axial CECT in same
patient shows moderate
sized effusion and multiple
discreet pleural nodules
(arrows) from metastatic
renal cell carcinoma to the
pleura.
6
5

(Left) Frontal radiograph

shows typical hematogenous
metastases. Multiple variable
sized sharply defined
pulmonary nodules. Pleural
metastasis (arrows), only one
edge visible, axis of tumor
parallels chest wall. (Right)
Axial CECT shows variable
sized nodules along blood
vessels most located along
lung edge or pleura. One
nodule has feeding vessel
(arrow).
 DRUG REACTION, INTRATHORACIC

Frontal radiograph shows right pleural effusion (arrow), Axial NEeT shows a right pleural effusion (arrow) and
sternotomy for coronary artery bypass graft surgery, and high density at the dependent atelectatic right lower
cardiomegaly. He was receiving amiooarone for lobe (curved arrow). Diagnosis: Amiodarone toxicity.
arrhythmias.

Patterns
ITERMINOlOGY o Diffuse alveolar damage (DAD): Identical to ARDS
Abbreviations and Synonyms from other causes
Diffuse vague/dense airspace disease with or
Hypersensitivity pneumonitis, systemic lupus
without interstitial opacities
erythematosis (SLE) syndrome
May lead to irreversible pulmonary fibrosis
Definitions Pleural effusions uncommon
Drug-induced disease of the thorax, approximately 40 o Hypersensitivity reaction: Within hours, days or
commonly used drugs months of starting drug; opacities may be fleeting,
("eosinophilic pneumonia-like")
Interstitial and/or alveolar opacities, patchy
6 I IMAGING FINDINGS peripheral airspace opacities, basilar
reticulonodular interstitial opacities
6 General Features May develop effusions
Best diagnostic clue: High index of suspicion that o Pulmonary edema: Indistinguishable from
pulmonary findings may be drug related; diagnosis by noncardiogenic or cardiogenic edema, diffuse mixed
exclusion of other etiologies interstitial/alveolar pattern
Location: Variable o Hemorrhage: Patchy or diffuse alveolar opacities
Size: Variable o SLE syndrome: Lupus-like pleuro-pericardial
Morphology: Variable, often nonspecific effusions (common), basal interstitial opacities
(uncommon)
Radiographic Findings o Pleural/mediastinal fibrosis: Pleural thickening,
Radiography: May be first indicator of pulmonary abnormal mediastinal contour, airway constriction
toxicity o Air leak: Pneumothorax/pneumomediastinum

DDx: Mixed Interstitial And Vague Airspace Opacities

Viral Pneumonia Pulmonary Edema Idiopathic Pulmonary Fibrosis

 DRUG REACTION, INTRATHORACIC

Key Facts
Terminology High CT density lung, pleura, liver, spleen:
Drug-induced disease of the thorax, approximately 40 Pathognomonic of amiodarone toxicity: Drug
commonly used drugs contains 37% iodine by weight

Imaging Findings Top Differential Diagnoses

Patterns
Diffuse alveolar damage (DAD): Identical to ARDS
from other causes
Hypersensitivity reaction: Within hours, days or
months of starting drug; opacities may be fleeting,
("eosinophilic pneumonia-like")
Pulmonary edema: Indistinguishable from
noncardiogenic or cardiogenic edema, diffuse mixed
interstitial/alveolar pattern
Hemorrhage: Patchy or diffuse alveolar opacities
SLE syndrome: Lupus-like pleuro-pericardial effusions
(common), basal interstitial opacities (uncommon)
Pneumonia: Viral pneumonia, pneumocystis,
mycoplasma, aspiration
Pulmonary edema, hemorrhage, diffuse alveolar
damage
Diagnostic Checklist
Nearly any focal or diffuse pulmonary process could
potentially be secondary to drug use
Differentiation requires investigation of drug history
and an individual's drug's pattern of pulmonary
injury

o Vasculitis: Patchy interstitial and or airspace

opacities, sub segmental, peripheral distribution, Imaging Recommendations
cavitation in areas of infarction Best imaging tool: HRCT best for detection and
Granulomatous vasculitis: Diffuse basilar characterization of ground-glass/interstitial lung
interstitial opacities disease
o Granulomas: Conglomerate basilar chronic masses Protocol advice: Thin slice CT, 1-3 mm supine and
(lipoid pneumonia) prone with inspiration/ expiration at 10-15 mm
o Thromboembolism: Identical to thromboembolism intervals
from other causes
Oil embolism: Miliary pattern, 24 to 48 hours post
lym ph angiography I DIFFERENTIAL DIAGNOSIS
o Pulmonary artery hypertension: Prominant main
Diffuse Airspace Disease
and central pulmonary arteries, right or left heart
Pneumonia: Viral pneumonia, pneumocystis,
failure from incompetent valves
o Lymphadenopathy: Hilar, mediastinal, cervical mycoplasma, aspiration 6
Idiopathic pulmonary fibrosis (IPF), collagen vascular
o Pulmonary calcification: Extremely fine, high 7
disease
density deposits, upper lobe predominance
Pulmonary edema, hemorrhage, diffuse alveolar
o Airways disease: Hyper-reactive airways and
hyperinflation from asthma; hyperinflation with damage
Multifocal airspace opacities: Eosinophilic pneumonia,
bronchiolitis obliterans
sarcoid, tuberculosis
CT Findings
NECT
o May detect pulmonary involvement, I PATHOLOGY
lymphadenopathy, pleuro-pericardial disease before
it is evident with radiography General Features
o High CT density lung, pleura, liver, spleen: General path comments
Pathognomonic of amiodarone toxicity: Drug o Direct lung toxicity: Damaged alveolar
contains 37% iodine by weight macrophages, neutrophils, and lymphocytes with
o Lipoid pneumonia: Low attenuation (fat density) release of cytokines and humoral factors that may
focal consolidation or mixed diffuse ground-glass result in fibrosis
and reticular opacities o Hypersensitivity reaction: Type I (immediate) and
o Mediastinal fibrosis: Soft tissue density encasing and type III (immune complex) response, eosinophilic
sometimes obliterating arteries, veins, bronchi (rare) infiltration
HRCT: Best characterize ground-glass, interstitial o Vasculitis: Type III and IV (cell-mediated), usually
(reticular nodular), alveolar opacities and their systemic, involving skin, kidneys, liver, seen with
distribution sulfonamides
Etiology
Nuclear Medicine Findings o DAD: Bleomycin, nitrosureas, busulfan,
Bone Scan: May be positive in the lungs with cyclophosphamide, methotrexate, mitomycin,
pulmonary metastatic calcification amiodarone, penicillamine, gold, oxygen
 DRUG REACTION, INTRATHORACIC
o Hypersensitivity reaction: Cromolyn sodium,
erythromycin, nitrofurantoin, isoniazid, penicillin,
IClINICAL ISSUES
sulfonamides, bleomycin, methotrexate, Presentation
procarbazine, penicillamine Most common signs/symptoms: Presentation: Varied,
o Pulmonary edema: Aspirin, codeine, nitrofurantoin, dyspnea, cough, fever, eosinophilia; drug toxicity
hydrochloroziazide, interleukin-2, heroin, cocaine, often overlooked as a cause
methadone, colchicine, epinephrine, contrast Onset: variable from immediate to years after drug
media, tricyclic antidepressants, mitomycin initiation
o Hemorrhage: Anticoagulants, estrogens, o Bleomycin, < 3 months; cytosine arabinoside < 21
penicillamine, quinidine days; busulfan, months to years; methotrexate,
o SLE syndrome: Procainamide, hydralazine, isoniazid, within weeks; cyclophosphamide, < 6 months to
phenytoin, nitrofurantoin, penicillin, sulfonamides, years; nitrosureas; nitrofurantoin 1 day to 1 month
digitalis, propranolol, thiazides, penicillamine, or> 6 months; hydrochlorothiazide < hours;
levodopa tricyclic antidepressants < hours; salicylates < hours;
Pleural effusions (without SLE syndrome): methysergide, 6 months to years; bromocriptine,
Methotrexate, procarbazine, nitrofurantoin, months to years; interleukin-2 usually < 8 days;
bromocriptine, methysergide, interleukin-2, amiodarone, approximately 6 months; hydantoin, 1
amiodarone week to 30 years
o Pleura/mediastinal fibrosis: Methysergide, With some cytotoxic drugs (nitrosureas) pre-existing
ergotamine, ergonovine pulmonary disease, smoking, irradiation, combination
o Pneumothorax/pneumomediastinum: Nitrosureas, chemotherapy increases risk for toxicity
cocaine Mitomycin: Asthma, microangiopathy hemolytic
o Vasculitis: Cromolyn sodium, sulfonamides, anemia (renal failure, thrombocytopenia,
penicillin, phenytoin, quinidine, hydralazine, noncardiogenic edema)
busulfan, thiouracil Penicillamine: Acute glomerulonephritis, pulmonary
o Granulomas: Methotrexate, nitrofurantoin, mineral hemorrhage, identical to Goodpastures
oils, talc
o Thromboembolism: Seen with oral contraceptives; Demographics
oil embolism: Seen with lymphangiography Age: Neonate to elderly
o Pulmonary artery hypertension: Talc, fenfluramine; Gender: M = F
damaged heart valves: Fenfluramine
o Hilar/mediastinallymphadenopathy: Methotrexate, Natural History & Prognosis
hydantoin Recovery after discontinuance of drug, variable
o Pulmonary calcification: Vitamin D, calcium Methotrexate: Reinstitution of treatment without
6 therapy recurrent toxicity
Mortality from respiratory failure
o Airways disease: Hyper-reactive airways: Propranolol,
8 neostigmine, aspirin o Mortality from toxicity: Nitrofurantoin 10%;
Bronchiolitis obliterans: Penicillamine, amiodarone, 20%; mitomycin (hemolytic-uremic
sulfasalazine, gold syndrome), > 90%
o Drug-induced phospholipidosis: Amiodarone Malignant transformation: Hydantoin
o Lipoid pneumonia: Aspiration of mineral oil lymphadenopathy may evolve into lymphoma, either
Hodgkin or non-Hodgkin
Gross Pathologic & Surgical Features
Most lung biopsies are not pathognomonic: Useful to Treatment
exclude other diseases and document pattern of lung Withdraw drug, corticosteroids
injury
Microscopic Features I DIAGNOSTIC CHECKLIST
Any diffuse interstitial pneumonia: DAD, usual
interstitial pneumonia, desquamative interstitial Consider
pneumonia Nearly any focal or diffuse pulmonary process could
Nonspecific interstitial pneumonia: Mononuclear potentially be secondary to drug use
cells, mild fibrosis, reactive type II pneumocytes,
uniform pattern (amiodarone, methotrexate, Image Interpretation Pearls
carmustine) Differentiation requires investigation of drug history
Cryptogenic organizing pneumonia: Immature and an individual's drug's pattern of pulmonary injury
fibroblasts plugging respiratory bronchioles, alveolar
ducts (bleomycin, cyclophosphamide, methotrexate)
Eosinophilic pneumonia: Eosinophils, lymphocytes, I SELECTED REFERENCES
plasma cells infiltrating alveolar septa (penicillamine, 1. Erasmus JJ et al: High-resolution CT of drug-induced lung
sulfasalazine, nitrofurantoin, aspirin) disease. Radiol Clin North Am. 40(1):61-72, 2002
2. Rossi SE et al: Pulmonary drug toxicity: Radiologic and
pathologic manifestations. Radiographs 20:1245-59, 2000
 DRUG REACTION, INTRATHORACIC

IIMAGE GALLERY

(Left) Frontal radiograph in a

woman on methotrexate for
rheumatoid arthritis shows
bilateral vague diffuse
opacities (arrows). (Right)
Axial HRCT shows diffuse
reticular/ground glass
opacities (arrow) and small
efFusions (curved arrows).
Methotrexate was stopped
and opacities cleared on
steroids. Hypersensitivity
pneumonitis.

Typical
(Left) Axial CECT shows
subpleural curvilinear bands
(arrows) and septal lines
(curved arrow).
Bronchoscopy showed
pulmonary eosinophilia.
Acute (orm of nitrofurantoin
lung toxicity. (Right) Axial
H RCT in a diabetic on
ciprofloxacin for 7 B months
shows small nodules (curved
arrows), micronodu/es (open
arrow) and septa/lines 6
(arrows). Open lung biopsy:
Interstitial pneumonia/COP. 9

Typical
(Left) Axial CECT shows
pericardia I effusion (arrow)
and tamponade; compressed
right atrium (curved arrow),
in a patient on minoxidil
treatment for hypertension.
Small effusions (open
arrows). SLE syndrome.
(Right) Frontal radiograph in
a 38 year old patient shows
diffuse airspace opacities
(arrows) indicating
pulmonary edema.
Toxicology screen showed
aspirin overdose. Patient
expired 9 days later.
 RADIATION-INDUCED LUNG DISEASE

Frontal radiograph shows bilaleral paramediaslinal Axial CECT in same palienl shows bilaleral
pulmonary fibrosis 9 months afler irradialion for parenthesis-shaped interfaces of densely consolidaled
mediastinal metastases from non-small cell carcinoma of pulmonary fibrosis (arrows). These curved interfaces
lung. indicale fully evolved radialion changes.

o Usually within radiation portals

!TERMINOLOGY o Uncommonly, RT to lung can induce BOOr
Abbreviations and Synonyms Migrating, generally peripheral, outside the
radiation portal
Radiation-induced lung disease (RILD)
Size: Extent of radiation changes varies with size of
Radiation therapy (RT)
Bronchiolitis obliterans organizing pneumonia (BOOP) radiation portals
Morphology
Definitions o Acute radiation pneumonitis: Diffuse alveolar
Radiation pneumonitis = radiation-induced damage
inflammation of lung, usually from radiation therapy o Chronic radiation changes: Fibrosis
(RT)
Radiographic Findings
6 Radiation fibrosis
usually from RT
= radiation-induced scarring of lung,
Radiography
o Typical timeline (rule of 4's)
10
4 weeks to deliver therapy of 40 Gy
4 weeks after end of therapy is earliest radiation
IIMAGING FINDINGS pneumonitis: Indistinct vessel margins in
General Features irradiated lung
Best diagnostic clue 4 months after end of therapy is peak radiation
o Pulmonary consolidation with sharply defined pneumonitis: Consolidation involving irradiated
linear or curvilinear interface lung
o RILD does not conform to an anatomic structure, 12 (4 x 3) months after end of therapy: After peak
but is confined to radiation portals pneumonitis, consolidation gradually clears &
evolves into scar (cicatricial atelectasis)
Location

DDx: lung Disease Associated With lung Cancer

. ,-/

Lobar Atelectasis Lymphangitic Spread

 RADIATION-INDUCED
Key Facts
Imaging Findings
Pulmonary consolidation with sharply defined linear
or curvilinear interface
RILD does not conform to an anatomic structure, but
is confined to radiation portals
PET can be very useful for detecting recurrent tumor
within regions of radiation fibrosis
Radiation pneumonitis can show increased glucose
metabolism in first few months after RT
Top Differential Diagnoses
Cardiac Pulmonary Edema
Bacterial Pneumonia
Recurrent Tumor
Lymphangitic Tumor
Drug Reaction
Scarring produces progressive volume loss in
irradiated lung
12-18 months following therapy: Cicatricial
atelectasis stabilizes
Thereafter, changes within radiation portal likely
related to recurrent tumor or infection
Timeline sequence accelerated 1 week for each
extra 10 Gy of therapy
o Typical patterns
Neck radiation: Apical consolidation with sharp
inferior margins
Paramediastinal radiation: Medial consolidation
with linear or parenthesis-shaped margins
Mantle radiation: V-shaped consolidation over
clavicles and along mediastinum, sharp margins
Tangential breast radiation: Obliquely oriented
margin of consolidation along anterior chest wall
Focal radiation: Varied, related to radiation portals
tailored to treat specific locations, such as a lung
mass
CT Findings
Patterns of radiation changes after conventional
radiation therapy
o Homogeneous, ground-glass opacities,
corresponding to minimal radiation pneumonitis
o Patchy consolidation within the radiation field
o Discrete consolidation within the radiation field, but
not totally involving it
o Solid consolidation totally involving the radiation
field, corresponding to fibrosis
Patterns of radiation fibrosis after 3-dimensional
conformal RT
o Modified conventional fibrosis less extensive than
usual with RT
o Mass-like fibrosis
o Scar-like fibrosis
MR Findings
Acute phase (1-3 months after irradiation)
o Enhancement of irradiated lung with
gadolinium-based contrast material is most
pronounced
LUNG DISEASE
Pathology
RT for carcinoma or lymphoma is most common
cause for RILD
Radiation pneumonitis - damage to type II
pneumocytes & endothelial cells, hyaline membranes
Clinical Issues
Gender: BOOP syndrome after RT occurs mostly in
women who have received RT for breast cancer
Diagnostic Checklist
Know stage of patient's carcinoma or lymphoma
If standard treatment for that stage of malignancy is
RT, pulmonary findings should be compared to
radiation portals
If ectatic bronchi within radiation fibrosis fill in with
soft tissue on follow-up CT, suspect recurrent tumor
Later phase (4 months after irradiation)
o First pass shows decreased enhancement, but
contrast material accumulates with redistribution
Nuclear Medicine Findings
V/Q Scan
o Radiation therapy causes a ventilation/perfusion
mismatch with nonsegmental distribution
conforming to radiation portal
o SPECT perfusion scans are good for assessing
regional effects of lung injury from RT
PET
o PET can be very useful for detecting recurrent tumor
within regions of radiation fibrosis
o Radiation pneumonitis can show increased glucose 6
metabolism in first few months after RT
11
Imaging Recommendations
Best imaging tool: High-resolution chest CT
Protocol advice: RILD is visible on conventional chest
CT protocols
I DIFFERENTIAL DIAGNOSIS
Cardiac Pulmonary Edema
Symmetrical bilateral interstitial thickening, parahilar
ground glass opacities and/or consolidation, usually
with cardiomegaly
Bacterial Pneumonia
Segmental or lobar consolidation, fever, increased
white blood cell count
Recurrent Tumor
Development of mass +/- associated lymph node
enlargement or other metastases
lymphangitic Tumor
Smooth or nodular thickening of interlobular septa
and peribronchovascular interstitium
Often associated with lymph node enlargement
More frequently unilateral
 RADIATION-INDUCED
Drug Reaction
Fibrosis, ground glass opacities or consolidation
Often caused by chemotherapy
I PATHOLOGY
General Features
Etiology
o RT for carcinoma or lymphoma is most common
cause for RILD
Total dose, fractionation, dose rate, type of RT &
volume of lung irradiated affect development of
RlLD
o RILD that occurs outside radiation portal is probably
a lymphocyte-mediated immune reaction, resulting
in BOOP
Epidemiology: Acute radiation pneumonitis develops
in 5-15% of patients who get RT
Associated abnormalities
o BOOP syndrome after RT for breast cancer
Seen in 2.4% of women treated with surgery and
RT for breast cancer, 2-7 months after RT
o Pleural thickening or effusion, pericardial effusion,
cardiomyopathy, calcified lymph nodes & thymic
cysts
o Blebs can develop in lung near regions of radiation
fibrosis
These blebs can rupture, causing pneumothorax
Gross Pathologic & Surgical Features
Radiation fibrosis - airless, firm lung with extensive
scar formation
6 Microscopic Features
Radiation pneumonitis - damage to type II
12 pneumocytes & endothelial cells, hyaline membranes
ICLINICAllSSUES
Presentation
Most common signs/symptoms
o Many patients are asymptomatic
o Cough, fever, shortness of breath
Other signs/symptoms
o Elevated polymorphonucleocyte count
o Elevated sedimentation rate
Clinical Profile
o Doses < 20 Gy rarely cause radiation pneumonitis
o Doses> 40 Gy usually cause radiation pneumonitis
Demographics
Age: RT is used mostly to treat carcinoma and
lymphoma, which occur more often in middle-aged
older people
Gender: BOOP syndrome after RT occurs mostly in
women who have received RT for breast cancer
Natural History & Prognosis
Acute: Capillary damage, edema, hyaline membranes
& mononuclear cell infiltration
Subacute: Increased mononuclear cells & fibroblasts
LUNG DISEASE
Chronic: Capillary sclerosis, alveolar & interstitial
fibrosis
Patients with pre-existing pulmonary fibrosis are at
greater risk for developing RILD
Treatment
Concomitant treatment with some chemotherapeutic
agents raises risk of RILD
Subsequent treatment with chemotherapy can cause
recall radiation pneumonitis
Steroids are standard treatment for radiation
pneumonitis and BOOP
Sudden withdrawal of steroids puts patients at risk for
recurrent radiation pneumonitis
I DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Know stage of patient's carcinoma or lymphoma
If standard treatment for that stage of malignancy is
RT, pulmonary findings should be compared to
radiation portals
If ectatic bronchi within radiation fibrosis fill in with
soft tissue on follow-up CT, suspect recurrent tumor
I SElECTED REFERENCES
1. Choi YW et ai: Effects of radiation therapy on the lung:
radioiogic appearances and differential diagnosis.
Radiographies. 24(4):985-97; discussion 998, 2004
2. Miwa 5 et al: The incidence and clinical characteristics of
bronchiolitis obliterans organizing pneumonia syndrome
after radiation therapy for breast cancer. Sarcoidosis Vase
Diffuse Lung Dis. 21 (3):212-8, 2004
3. Koenig TR et al: Radiation injury of the lung after
three-dimensional conformal radiation therapy. AJR Am J
Roentgenol. 178(6):1383-8, 2002
4. Ogasawara N et al: Perfusion characteristics of
radiation-injured lung on Gd-DTPA-enhanced dynamic
magnetic resonance imaging. Invest Radiol. 37(8):448-57,
2002
5. Chin BB et al: Nonsegmental ventilation-perfusion
scintigraphy mismatch after radiation therapy. Clin Nuel
Med. 24(1):54-6, 1999
6. Libshitz HI et al: Filling in of radiation therapy-induced
bronchiectatic change: a reliable sign of locally recurrent
lung cancer. Radiology. 210(1):25-7, 1999
7. Prakash U8: Radiation-induced injury in the
"nonirradiated" lung. Eur Respir J. 13(4):715-7, 1999
8. Frank A et al: Decision logic for retreatment of
asymptomatic lung cancer recurrence based on positron
emission tomography findings. Int J Radiat Oneal Bioi
Phys. 32(5):1495-512,1995
9. Roberts CM et al: Radiation pneumonitis: a possible
lymphocyte-mediated hypersensitivity reaction. Ann
Intern Med. 118(9):696-700, 1993
or 10. Pezner RD et al: Spontaneous pneumothorax in patients
irradiated for Hodgkin's disease and other malignant
lymphomas. IntJ Radiat Oncol Bioi Phys. 18(1):193-8,
1990
11. Libshitz HI et al: Radiation-induced pulmonary change: CT
findings. J Comput Assist Tomogr. 8(1):15-9,1984
 RADIATION-INDUCED LUNG DISEASE
I IMAGE GALLERY

(Left) Axial CECT shows

obliquely oriented interface
of fibrosis (arrows) in a
patient treated with
tangential radiation therapy
(or breast cancer. Oblique
orientation makes diagnosis
difficult on chest
radiography. Patient had le(t
lower lobectomy. (Right)
Axial CECT shows a sharply
marginated interface of
consolidation related to
radiation changes (arrows) 6
months after radiation
I
\ therapy (or squamous cell
carcinoma of lung.

Typical
(Left) Axial CECT shows
biapical central ground-glass
opacities with sharply
marginated interfaces
(arrows) related to neck
radiation therapy 15 months
ago (or squamous cell
carcinoma o( tongue. (Right)
Axial CECT in same patienl
14 months later shows
development o( dense
biapical (ibrosis. Margins o(
radiation changes (arrows) 6
remain sharp, but right
margin is curved, related to 13
Fibrosis.

Other
(Left) Axial CECT shows
sharply marginated
consolidation (open arrows)
characteristic or
bilateral
paramediastinal radiation
changes.
Pneumomediastinum
outlines thymus (arrows).
(Righi) Axial CECT o( same
patient shows pneumothorax
(curved arrow), a
complication of radiation
therapy, and
pneumomediastinum
(arrows). Open arrows
define margins of radiation
changes.
 IMMUNOSUPPRESSED (NOT AIDS)

Anteroposterior radiograph shows diffuse interstitial Frontal radiograph shows small bilateral pleural effusions
thickening in immunocompromised patient. and diffuse intersUtial thickening in
Bronchoalveolar lavage demonstrated diffuse alveolar immunocompromised febrile padent. Numerous Kerfey
hemorrhage. B lines from pulmonary edema.

Pulmonary emboli
I TERMINOlOGY Pulmonary hemorrhage
Definitions o 33% of pulmonary complications have multiple
A host at increased risk for life-threatening infection as etiologies
a consequence of congenital or acquired abnormality o Statistics don't substitute for sampling
of the immune system Radiographic Findings
Immune status determines the severity and Radiography
susceptibility to infection o Sensitivity for disease probably> 90%
o Accuracy of highly confident diagnosis 50% (toss
up)
I IMAGING FINDINGS
6 o Radiologic diagnostic
1/3rd
interpretations correct in only
General Features
14 Utility of chest radiography
Best diagnostic clue
o Detection prior to onset symptoms
o Lung main focus of complications in
o Evolution of pulmonary abnormalities
immunocompromised host
o Detection of complications
Infections account for 75% of complications in
o Monitor progress and therapy
immunosuppressed
a Diagnosis (rare)
o 25% non-infectious
Most findings nonspecific, common patterns include
Cardiogenic and noncardiogenic pulmonary
a Focal consolidation
edema
o Multiple pulmonary nodules
Drug toxicity
o Diffuse consolidation or interstitial lung disease
Extension of underlying disease (graft vs. host
o Pleural effusion
disease, malignancy, rejection)

DDx: Immunocompromised Patterns

Focal Opacity Multiple Nodules Diffuse Opacities

 IMMUNOSUPPRESSED (NOT AIDS)

Key Facts
Terminology Pathology
A host at increased risk for life-threatening infection Even with sampling, a precise cause not identified in
as a consequence of congenital or acquired 20%
abnormality of the immune system
Clinical Issues
Imaging Findings Often nonspecific findings, fever may not be due to
Lung main focus of complications in infection
immunocompromised host Fever seen with drug toxicity, hemorrhage, infections
Infections account for 75% of complications in outside the lung, transfusion reaction, radiation
immunosuppressed pneumonitis and occasionally pulmonary emboli
25% non-infectious Prognosis directly related to early detection
Decreased inflammatory response results in minimal Establishing a cause by an invasive procedure may
radiographic abnormalities not improve outcome by more than 20%
CT will detect pneumonia an average of 5 days before Finding a cause may even be elusive at autopsy
chest radiograph abnormalities
Diagnostic Checklist
Statistics don't substitute for sampling

Patterns
o Focal consolidation CT Findings
And pleural effusions associated with pulmonary NECT
infarcts and pneumonia o Feedi ng vessel sign
Rapid evolution 24 hours) suggests bacterial Septic emboli
pneumonia, pulmonary emboli, cardiogenic or Metastases
noncardiogenic pulmonary edema or hemorrhage o Necrotic mediastinal or hilar lymph nodes
Transient consolidation may be seen immediately Tuberculosis
following bronchoalveolar lavage (BAL), which is o Halo sign pulmonary nodule
commonly used to investigate lower respiratory Invasive aspergillosis
tract Candidiasis
Subacute or chronic evolution (days to weeks) Cytomegalovirus (CMV)
suggests fungal, nocardial, or mycobacterial Hemorrhagic metastases
o Bronchiectasis
infection
o Multiple pulmonary nodules Chronic infection with encapsulated bacteria 6
< 10 mm in diameter, often viral etiology Imaging Recommendations 15
> 3 cm in diameter, often invasive aspergillosis or
Best imaging tool
metastases o Chest radiography usually sufficient for clinical
Rapid evolution 24 hours) suggests bacterial practice
pneumonia or pulmonary edema
o CT more sensitive and will detect 20% more
Subacute or chronic evolution (days to weeks)
pneumonias
suggests metastases, fungal or mycobacterial
o CT will detect pneumonia an average of 5 days
pneumonia
before chest radiograph abnormalities
o Diffuse consolidation or interstitial lung disease
Rapid evolution 24 hours) suggests pulmonary
edema
Subacute or chronic evolution (days to weeks)
I DIFFERENTIAL DIAGNOSIS
suggests viral or pneumocystis jiroveci pneumonia Focal Consolidation, Consider
(PCP) or drug toxicity Bacterial pneumonia
o Pleural effusions Fungal pneumonia
Bilateral usually cardiogenic Pulmonary infarct
Large (over 1/2 of hemithorax) usually metastases Radiation therapy
Kerley B lines Hemorrhage
o Much more common with pulmonary edema Lymphoproliferative disorder
o Edema common due to
High volumes of fluid for chemotherapy Multiple Nodular Pattern, Consider
Chemotherapy or radiation damage to heart Fungal, especially Coccidioidomycosis, Cryptococcus,
Transfusion reactions, anemia and Aspergillosis
Neutropenia Nocardiosis
o Decreased inflammatory response results in minimal Mycobacterial species
radiographic abnormalities Septic emboli from indwelling catheters
Metastases
 IMMUNOSUPPRESSED (NOT AIDS)
Bleomycin drug toxicity Prone to infections with intracellular organisms:
Bronchiolitis obliterans organizing pneumonia (BOOP) Mycobacteria, Nocardia species, Legionelia species,
Post-transplant lymphoproliferative disorder (PTLD) Fungal infections (Cryptococcus, Histoplasmosis,
coccidioidomycosis) and viral infections (CMV,
Cavitary Nodules, Consider Herpes species, varicella-zoster, and Ebstein-Barr)
Staphylococcus and PCP
Tuberculosis o Splenectomy
Nocardia Prone to infections with encapsulated organisms
Septic emboli o Corticosteroid therapy
Fungi, especially invasive aspergillosis Prone to Staphylococcus aureus, legionella species,
Metastases, especially from squamous or sarcoma cell mycobacteria, nocardia species, pseudomonas
types aeruginosa, fungal infections (especially Candida
Diffuse Interstitial or Consolidative Pattern, species) viruses (especially CMV and
varicella-zoster) and PCP
Consider
pCP Gross Pathologic & Surgical Features
Viral, especially CMV or varicella-zoster Even with sampling, a precise cause not identified in
Edema, cardiogenic and noncardiogenic 20%
Drug reaction
Hemorrhage
Lymphangitic tumor IClINICAllSSUES
Nonspecific interstitial pneumonitis
Lymphocytic interstitial pneumonia Presentation
Most common signs/symptoms
Pleural Effusion, Consider o Often nonspecific findings, fever may not be due to
Cardiogenic pulmonary edema infection
Bacterial pneumonia o Febrile pneumonitis syndrome
Pulmonary infarction Fever seen with drug toxicity, hemorrhage,
Graft vs. host disease infections outside the lung, transfusion reaction,
radiation pneumonitis and occasionally
pulmonary emboli
I PATHOLOGY Demographics
General Features Age: Any age
General path comments
6 o Type of immunosuppression
Natural History & Prognosis
Defect in phagocytosis Prognosis directly related to early detection
16 Depends on underlying condition and response to
B-cell (antibody) and complement deficiency
T-cell (cell-mediated) therapy
Splenectomy Establishing a cause by an invasive procedure may not
Corticosteroid therapy improve outcome by more than 20%
Etiology Finding a cause may even be elusive at autopsy
o Phagocytic defect Diffuse disease mortality rate SO%
Seen with bone marrow suppression, Treatment
chemotherapy, leukemia, bone marrow transplant Empiric therapy with antibiotics often used in
and chronic granulomatous disease immunosuppressed, if no response more aggressive
Prone to infections with aerobic gram-negative sampling used
organisms, candida species, and aspergillus species Empiric diuresis often tried to exclude edema
o B-cell disorder: Antibody defect or complement
deficiency
Primary (x-linked agammaglobulinemia or I DIAGNOSTIC CHECKLIST
immunoglobulin deficiency)
Secondary (multiple myeloma, Waldenstrom, Image Interpretation Pearls
chronic lymphocytic leukemia) Statistics don't substitute for sampling
Prone to infections with encapsulated bacteria
(Streptococcus pneumoniae, Staphylococcus
aureus, Hemophilus influenzae, and Pseudomonas I SElECTED REFERENCES
aeruginosa)
1. Gosselin MV: Diffuse lung disease in the
Chronic infection may lead to bronchiectasis
immunocompromised non-HIV patient. Semin
o T-cell disorder: Cell-mediated defect Roentgenol. 37(1):37-53, 2002
Primary (DiGeorge or Nezelof syndrome) 2. Oh YW et al: Pulmonary infections in
Secondary (AIDS, lymphoma, leukemia, aging, immunocompromised hosts: the importance of correlating
steroid thera py) the conventional radiologic appearance with the clinical
setting. Radiology. 217(3):647-56, 2000
 IMMUNOSUPPRESSED (NOT AIDS)

I IMAGE GALLERY

(Left) Frontal radiograph

shows focal lung opacity in
the medial right base in
febrile immunocompromised
lymphoma patient.
Differential includes
infection, hemorrhage, or
recurrent disease. (Right)
Axial CECT shows focal
consolidation in the right
lower lobe. Transbronchial
biopsy demonstrated
recurrent lymphoma.

Typical
(Left) Anteroposterior
radiograph shows multiple
small nodular opacities
(arrow) predominantly
within the right lung in
febrile patient. Differential:
Pneumonia, septic emboli, or
metastases. (Right) Axial
CECT shows more nodules
than apparent on
radiograph. One contains air
bronchogram. 8M
demonstrated 6
histoplasmosis.
17

(Left) Anteroposterior
radiograph shows diffuse
miliary interstitial disease in
Febrile immunocompromised
patient. Differential:
Pneumonia (viral, bacteria!),
edema, hemorrhage, or drug
toxicity. (Right) Axial HRCT
shows innumerable miliary
nodules in random pattern
and a few scattered ground
glass opacities.
Transbronchial biopsy
demonstrated tuberculosis.
 AIDS

Graphic shows Pneumocystis carini; pneumonia. Axial NEeT shows cysts of varying sizes with adjacent
Diffuse ground-glass opacities with multiple thin walled ground-glass opacities.
pneumatoceles. There is a predilection for the
pneumatoceles to occur in the upper lobes.

ITERMINOlOGY IIMAGING FINDINGS

Abbreviations and Synonyms General Features
Human immunodeficiency virus (HIV) Best diagnostic clue
Acquired immune deficiency syndrome (AIDS) o Best clue for PCP is a bilateral, symmetrical,
CD4 count = helper T cell level, widely accepted "ground-glass" pattern
measure of immunocompromise o Best clue for bacterial pneumonia is focal
Highly active antiretroviral therapy (HAART) consolidation
Pneumocystis carinii pneumonia (PCP), also referred o Best clue for KS is a bronchovascular distribution of
to as Pneumocystis jiroveci pneumonia pulmonary opacities
Kaposi sarcoma (KS)
6 Definitions
Radiographic
Radiography
Findings

18 Infectious disease caused by HIV infection that causes o May be normal in PCP or mycobacterial infections
failure of the immune system o Solitary pulmonary nodule (SPN)
HIV virus infects CD4 T-lymphocytes Lung cancer, KS, atypical infection
CD4 < 200 cells/mm3 is an AIDS-defining event and o Multiple pulmonary nodules
places patient at risk for opportunistic infections and Fungal infection, nocardia, mycobacteria,
some neoplasms cytomegalovirus (CMV), lymphoma, KS,
metastases
o Cavitating pulmonary nodules
Septic emboli, nocardia, mycobacteria, fungal
infection, metastases
o Cysts

DDx: Lung Nodules In AIDS

Lymphoma Kaposi Sarcoma CMV Pneumonia

 AIDS

Terminology
CD4 count = helper T cell level, widely accepted
measure of immunocompromise
HlV virus infects CD4 T-lymphocytes
CD4 < 200 cells/mm3 is an AIDS-defining event and
places patient at risk for opportunistic infections and
some neoplasms
Imaging Findings
Best clue for PCP is a bilateral, symmetrical,
"ground-glass" pattern
Best clue for bacterial pneumonia is focal
consolidation
Best clue for KS is a bronchovascular distribution
pulmonary opacities
Bacterial pneumonia: Most common respiratory
infection
Key Facts
PCP: Decreased prevalence but still most common
opportunistic infection
Paradoxical worsening may occur during treatment
for TB in the setting of "immune reconstitution" from
HAART
Chest radiograph: 1st line study for detection and
follow-up
CT: 2nd line study for problems unresolved by
radiographs or to detect occult disease (e.g., PCP)
when chest radiograph is negative
Clinical Issues
Marked reduction in morbidity and mortality with
of HAART in western nations; limited access to HAART
on global basis

PCP, lymphocyte interstitial pneumonia (UP) Pleural fluid

o Pleural effusion Lymphadenopathy
Kaposi sarcoma, lymphoma, mycobacterial, o Kaposi sarcoma
bacterial or fungal infection Thickening of bronchovascular bundles, which
o Lymphadenopathy may progress to "flame-shaped" coalescent areas of
Infection (mycobacterial or fungal, bacillary consolidation radiating outward from hila
angiomatosis), KS, lymphoma, lung cancer Poorly defined nodules
o Focal consolidation Reticular and nodular opacities with basilar
Bacterial pneumonia, TB, lymphoma predominance
Specific entities Lymphadenopathy (50%)
o Bacterial pneumonia: Most common respiratory Atelectasis, lobar (5%), due to endobronchial
infection Kaposi
50% focal consolidation (segmental or lobar) o Lymphoma (high grade B cell non-Hodgkin)
50% other patterns: Diffuse disease, nodules,
cavities
Nodules (40%), well-defined, may rapidly enlarge
or cavitate, air bronchograms common
6
Cavitary nodules characteristic of septic emboli Consolidation (30%) 19
o PCP: Decreased prevalence but still most common Reticular opacities (20%)
opportunistic infection Pleural effusions (50%)
Chest radiograph normal in up to 40% cases Lymphadenopathy (20%)
Bilateral, perihilar or diffuse symmetrical o Lung cancer
distribution of finely granular, reticular, or Lung nodule or mass
ground-glass opacities Lymphadenopathy and pleural effusion common
Cysts (30%), predisposed to spontaneous Advanced stage of disease common at time of
pneumothorax presentation
Lymphadenopathy or pleural effusion rare (should Non-infectious, non-neoplastic conditions
suggest alternate diagnosis) o Lymphocytic interstitial pneumonia (LIP)
o Tuberculosis 30-40% pediatric AIDS patients, less common in
Pattern depends upon CD4 count adults
> 200: Post-primary pattern (poorly defined upper Chest radiograph: Miliary pattern; CT miliary
lobe opacities, nodules, cavities) nodules in peribronchovascular distribution,
< 200: Primary pattern (consolidation, ground glass, cysts
lymphadenopathy) o Nonspecific interstitial pneumonia (NSlP)
HlV associated with higher rate of miliary pattern, Can mimic PCP, but usually occurs at higher CD4
lymphadenopathy, bronchogenic spread, and count (> 200)
extrapulmonary disease o Bronchiectasis
Paradoxical worsening may occur during Increased prevalence in HlV-positive individuals
treatment for TB in the setting of "immune Usually a sequelae of prior/recurrent infections
reconstitution" from HAART o Bronchiolitis obliterans
o Cryptococcus (most common fungal infection) May complicate infections such as PCP
Reticular or reticular and nodular pattern o Emphysema
Nodules, with or without cavitation HIV-positive smokers have increased susceptibility
Consolidation to emphysema
 AIDS
Premature bullae common in intravenous drug a Patients with respiratory infections typically present
abusers with cough and fever
Cardiovascular complications Abrupt onset symptoms and duration < 1 week
a Pulmonary arterial hypertension typical of bacterial pneumonia
a Cardiomyopathy Gradual onset symptoms and duration> 1 week
a Premature atherosclerosis typical of PCP
a Patients with neoplastic conditions typically present
CT Findings with weight loss and systemic symptoms
CT is more sensitive and specific than chest a An abnormal radiograph in the absence of
radiography, used for selected indications respiratory symptoms is concerning for TB or
a Assess for radiographically occult infection (PCP) nontuberculous mycobacterial infection
a Further characterize nonspecific radiographic a Dyspnea and cough are common but nonspecific
findings respiratory symptoms that may occur in a variety of
a Plan or guide biopsy procedures pulmonary conditions
a Staging of AIDS-related neoplasms Other signs/symptoms: KS may present with
Imaging Recommendations hemoptysis from endobronchial lesions; skin lesions
are present in majority of patients with thoracic
Chest radiograph: 1st line study for detection and
involvement
follow-up
CT: 2nd line study for problems unresolved by Natural History & Prognosis
radiographs or to detect occult disease (e.g., PCP) With malignancies, generally poor
when chest radiograph is negative Marked reduction in morbidity and mortality with
HAART in western nations; limited access to HAART
on global basis
I DIFFERENTIAL DIAGNOSIS
Treatment
Lymphadenopathy Prophylactic treatment for PCP
Diffusely enhancing: KS or bacillary angiomatosis a Trimethoprim-sulfamethoxazole (Bactrim):
(infection that mimics KS) Common
Peripherally enhancing with necrotic centers: TB, a Aerosolized pentamidine: Rarely used
nontuberculous mycobacterial or fungal infection Antibiotics for specific infections
Soft tissue density: Lymphoma, lung cancer, Radiation, chemotherapy for malignancies; surgery for
generalized HlV lymphadenopathy early stage lung cancer
Retroviral therapy
Lung Nodules
6 Centrilobular distribution, < 1 em: Usually infectious
a HAART = highly active antiretroviral therapy
Random distribution, > 1 em: Usually neoplastic
20 Peribronchovascular distribution: KS I DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
I PATHOLOGY Important to employ an integrated approach to
interpretation that combines radiographic pattern
General Features recognition with knowledge of clinical presentation,
General path comments CD4 count, risk factor for HlV, and current drug
a HlV infection depletes helper T cells (CD4) leading therapy (e.g., PCP prophylaxis, HAART)
to immunosuppression
a Normal CD4 count 800-1000 cells/mm3, HlV
depletes 50 cells/year (prodromal period I SELECTED REFERENCES
approximately 10 years)
Epidemiology 1. Morris MI et al: Pulmonary infections in the
immunocompromised host. Cardiopulmonary Imaging
a Spread through close contact with bodily fluids
Categorical Course Syllabus. American Roentgen Ray
a At risk: Multiple sexual partners, homosexual Society, 85-] 03, 2005
contact, IV drug abuse, hemophiliacs 2. Brecher CW et al: CT and radiography of bacterial
a Global epidemic respiratory infections in AIDS patients. A]R Am]
Roentgenol. ]80(5):]203-9,2003
Microscopic Features 3. Boiselle PM et ai: Update on lung disease in AIDS. Seminars
Infections require sputum or tissue sampling, silver in Roentgenology. 37:54-71, 2002
stain for PCP 4. Saurborn DP et al: The imaging spectrum of pulmonary
tuberculosis in AIDS.] Thorac Imaging. ] 7(]):28-33, 2002
S. Edinburgh K] et al: Multiple pulmonary nodules in AIDS:
I CLINICAL ISSUES usefulness of CT in distinguishing among potential causes.
Radiology. 2]4(2):427-32, 2000
6. Kang EY et al: Detection and differential diagnosis of
Presentation
pulmonary infections and tumors in patients with AIDS:
Most common signs/symptoms Value of chest radiography versus CT. A]R. 166:]5-9, 1996
 AIDS
I IMAGE GALLERY
Typical
(Left) Frontal radiograph
shows focal consolidation in
RUL (arrow) due to bacterial
pneumonia. (Right) Lateral
radiograph shows segmental
distribution of consolidation
in posterior segment RUL
(arrows).

(Left) Frontal radiograph

shows solitary mass in RUL
with irregular margins and
pleural tag, highly suggestive
of primary lung cancer.
(Right) Axial NEeT shows
spiculated margins of RUL
mass, which proved to
represent an
adenocarcinoma.

6
21

aeft)hon~/~d0g~ph
shows diffuse mediastinal
widening suggestive of
lymphadenopathy. (Right)
Coronal CECT shows diffuse
bilateral lymph node
enlargement, which proved
to be due to lymphoma.
 SICKLE CELL DISEASE

Axial CECT in a 25 year old woman with sickle cell Lateral radiograph shows H-shaped vertebra with
disease shows a right paraspinal oval 50ft tissue mass step-off deformities of the superior and inferior endplates
(arrow) that represents extramedullary hematopoiesis. (Reynold sign) (arrows), characteristic skeletal changes
of sickle cell disease.

I TERMI NOLOGY Radiographic Findings

Recurring episodes acute chest syndrome
Abbreviations and Synonyms
o Lung: Initially may have normal radiograph
Acute chest syndrome, sickle cell chronic lung disease o Nonspecific opacities focal or diffuse
Definitions Interstitial thickening or consolidation
Sickle cell disease due to abnormal hemoglobin which Location: Basilar peripheral lower lung zone
deforms when deoxygenated Focal disease: Lobar, multilobar; segmental,
Acute chest syndrome, common and recurrent, due to subsegmental
pneumonia or infarction Interstitial thickening may be cardiac or
Sickle cell chronic lung disease, the result of recurrent noncardiac
6 bouts of acute chest syndrome Pulmonary venous hypertension
Pleural effusions
22 Heart
IIMAGING FINDINGS o Cardiomegaly, common
Mediastinum
General Features o Posterior mediastinal mass, paraspinal or paracostal
Best diagnostic clue: Expanded ribs and H-shaped masses (extramedullary hematopoiesis)
vertebra, absent spleen o Unilateral or bilateral, smooth, sharply marginated
Location: Lower lobe predominance with acute chest o Initial rapid growth, then stable
syndrome, pulmonary edema Subdiaphragmatic: Small spleen, may be calcified
Size: Variable extent of pulmonary opacification (autosplenectomy)
Morphology: Cardiomegaly and pulmonary airspace Skeletal changes
and/or interstitial opacities o Pathognomonic H-shaped vertebrae (10%)

DDx: Pulmonary Airspace Opacities

ARDS Pneumonia Hemorrhage

 SICKLE CELL DISEASE

Key Facts
Terminology Mosaic pattern of perfusion due to microvascular
Sickle cell disease due to abnormal hemoglobin occlusion
which deforms when deoxygenated Protocol advice: Serial radiography to evaluate
Acute chest syndrome, common and recurrent, due response to therapy for acute chest syndrome
to pneumonia or infarction Top Differential Diagnoses
Sickle cell chronic lung disease, the result of recurrent Pneumonia, Pulmonary Edema, Pulmonary
bouts of acute chest syndrome Embolism, Hemorrhage
Imaging Findings Pathology
Best diagnostic clue: Expanded ribs and H-shaped Pneumonias due to encapsulated organisms such as:
vertebra, absent spleen Streptococcus pneumoniae, Hemophilus influenzae,
Location: Lower lobe predominance with acute chest most common
syndrome, pulmonary edema
Posterior mediastinal mass, paras pinal or paracostal Clinical Issues
masses (extramedullary hematopoiesis) Pulmonary artery hypertension: Cor pulmonale, 33%
CECT: High osmolarity contrast contraindicated as it High output failure
may enhance sickling

o Step-off deformity superior and inferior endplates Protocol advice: Serial radiography to evaluate
(Reynold sign) response to therapy for acute chest syndrome
o Bone sclerosis
o Ribs: Expansion of medullary space, periosteal
reaction I DIFFERENTIAL DIAGNOSIS
CT Findings Pneumonia, Pulmonary Edema, Pulmonary
CECT: High osmolarity contrast contraindicated as it Embolism, Hemorrhage
may enhance sickling Sickle cell lung disease indistinguishable from other
HRCT causes of lung disease
o Mosaic pattern of perfusion due to microvascular
occlusion H-Shaped Vertebrae
o Geographic areas of hypoperfusion Gaucher disease
Areas of decreased attenuation
Fewer and attenuated pulmonary arteries and
o Spleen not small (may be enlarged)
Paroxysmal nocturnal hemoglobinuria
6
veins o Spleen normal, no lung findings 23
o Geographic areas of hyperperfusion Alcoholics: No marrow expansion, spleen normal
Areas of ground glass opacity
Redistribution of flow to lung with less
microvascular disease I PATHOLOGY
o Diffuse ground glass opacities
Hemorrhagic edema caused by reperfusion of General Features
ischemic lung General path comments: Red blood cells sickle when
o Airspace consolidation deoxygenated
Pneumonia, pulmonary infarction or fat Genetics
embolism o Autosomal recessive inheritance
o Valine substitution for glutamic acid in hemoglobin
Nuclear Medicine Findings (Hb S)
V/Q Scan o Homozygous (Hb 55); sickle cell trait (Hb SA)
o Lung perfusion study, heterogeneous perfusion due (normal hemoglobin - Hb A)
to microvascular disease o Heterozygous traits, sickle cell-thalassemia
o Resembles findings of pulmonary embolism o Hemoglobin S has some protection from malaria
Bone Scan Etiology
o Bone scan: To show bone infarction o Change in chemical composition of protein
o Bone scan and Indium-1l1labeled leukocyte hemoglobin
imaging: To show osteomyelitis o Substitution of glutamic acid by valine in both beta
Tc-99m Sulfur Colloid: Uptake in masses due to chains of hemoglobin molecule (hemoglobin SS)
extramedullary hematopoiesis o Red blood cells sickle and elongate with
Imaging Recommendations deoxygenation and dehydration
o Decreased pliability of red blood cells while
Best imaging tool: Radiography usually suffices to
traversing the microcirculation
detect complications
 SICKLE CELL DISEASE
o Widespread capillary obstruction by sickled cells and
accompanying in situ thrombosis
o Etiology pathogenesis of acute chest syndrome
Multifactorial, exact cause rarely determined
Pneumonia and/or infarctions from thrombosis or
fat embolus
Upper lobe consolidation more likely pneumonia
because oxygen tension highest in upper lung
zones due to high V/Q ratio
o Etiology of pulmonary artery hypertension
Microvascular occlusion, chronic hypoxia,
repeated injury to lungs with scarring
o Etiology of skeletal abnormalities
Microvascular occlusion and infarction at
end plates (H-shaped vertebra)
Bone infarcts (sclerosis)
Extramedullary hematopoiesis with marrow
expansion (rib expansion)
Epidemiology
o 0.15% African-American population are
homozygous
8% have sickle cell trait; sickle cell trait seems to
have protective effect against malaria
o Acute chest syndrome, up to 40%
Children 100 times more susceptible to
pneumonia than other children; recurrence rate,
30%
Fat embolism in 13-75% of patients
o Sickle cell chronic lung disease, approximately 4%
Gross Pathologic & Surgical Features
Capillary obstruction by sickled cells leads to ischemia
and infarction
Multiorgan involvement, especially thoracic
6 Microscopic Features
Autosplenectomy: Impaired immunity due to
24
functional asplenia
o Pneumonias due to encapsulated organisms such as:
Streptococcus pneumoniae, Hemophilus influenzae,
most common
Staphylococcus aureus, Chlamydia pneumoniae,
Salmonella, respiratory syncytial virus,
Mycoplasma pneumonia
Fat embolism (bone infarction): Lipid-laden
macrophages in the bronchoalveolar lavage fluid
IClINICAL ISSUES
Presentation
Most common signs/symptoms
o Acute chest syndrome: New radiographic opacity
with fever, cough, chest pain, dyspnea, productive
cough, wheezing, hemoptysis
Hypoxemia, leukocytosis
Second leading cause of hospitalization
Recurring in up to 80% of patients
Other signs/symptoms
o Hyper-reactive airway disease, 40% of children
o Nocturnal oxyhemoglobin desaturation, obstructive
sleep apnea
Acute chest syndrome: Difficult to distinguish
infectious from noninfectious etiology
o Pneumonia that does not resolve with treatment,
consider tuberculosis
o Fat embolism: Pulmonary findings preceded by bone
pain
o Difficult to distinguish in situ thrombosis from
pulmonary embolism
Sickle cell chronic lung disease
o Interstitial restrictive lung disease and pulmonary
artery hypertension
Left ventricular dysfunction: Due to combination of
o High output failure from anemia, especially when
hemoglobin is < 7 gm/dl
o Intravascular volume overload, treatment related
o Renal failure (microinfarction of kidney)
Demographics
Age: Inherited, present at birth
Gender: M = F
Ethnicity: Most prevalent in African-Americans
Natural History & Prognosis
Sickle cell chronic lung disease: 4%: Acute chest
syndrome sequelae
o Fibrosis indicated by, fine reticular interstitial
opacities; linear opacities or bands
o Interlobular septal thickening, peripheral
wedge-shaped opacities, architectural distortion
o Traction bronchiectasis, pleural tags
Pulmonary artery hypertension: Cor pulmonale, 33%
o Enlargement of central pulmonary arteries, pruning
of peripheral vessels
o Cardiomegaly with right ventricular configuration;
right ventricular hypertrophy
o Late in natural history of sickle cell disease
High output failure
o Cardiomegaly with right and left heart chamber
enlargement; pulmonary edema
o Pulmonary venous hypertension, widened vascular
pedicle
Median survival, male: 42 years; females: 48 years
> 20% have fatal pulmonary complications; 25% due
to acute chest syndrome
o Thromboembolism, 25% of autopsies
Treatment
Exchange transfusions, supportive therapy with
oxygen, cautious hydration
Antibiotics for presumed pneumonia, analgesics for
bone pain and splinting
Bronchodilators for hyper-reactive airway disease
Hydroxyurea: Increases hemoglobin F, decreasing
hemoglobin S
Pneumococcal vaccination
I SELECTED REFERENCES
1. Siddiqui AKet al: Pulmonary manifestations of sickle cell
disease. Postgrad Med). 79(933):384-90, 2003
2. Hansell OM: Small-vessel diseases of the lung:
CT-pathologic correlates. Radiology. 22S(3):639-53, 2002
3. Leong CS et al: Thoracic manifestations of sickle cell
disease.) Thocac Imaging. 13:128-34,1998
 SICKLE CELL DISEASE

IIMAGE GALLERY

(Left) Frontal radiograph

shows bilateral lower lobe
airspace opacities (arrows)
in a 29 year old man with
dyspnea, fever, leukocytosis.
Sputum and blood cultures
were negative. Acute chest
syndrome. (Right) Frontal
radiograph shows
cardiomegaly and diffuse
bilateral pulmonary opacities
(arrows) in a sickle cell
disease patient intubated for
respiratory failure. Autopsy
diagnosis: Fat embolism.

Typical
(Left) Frontal radiograph
shows marked cardiomegaly
and prominent main
pulmonary artery (arrow)
indicating pulmonary arterial
hypertension in a patient
with sickle cell disease.
(Right) Axial H ReT in same
patient shows a mosaic
pattern of perfusion. Note
attenuated vessels in areas of
decreased attenuation
(arrows) and dilated vessels 6
(curved arrow) and ground
glass in hyperperfused lung. 25

Typical
(Left) Nuclear perfusion
study in same palient shows
multiple segmental perfusion
defects (arrows). Ventilalion
study was normal. (Right)
Pulmonary angiogram in
same patient excludes
pulmonary embolism and
shows decreased vascularity

and perfusion
microvascular
(arrow)
occlusion,
due to

etiology for pulmonary artery

hypertension (open arrow).
 1

PART V
PaUems

Introduction and Overview [ill

Patterns rn
 INTRODUCTION TO PATTERNS
o
2

Frontal raalDfP{1h shcrNs perihi/ar batwing Radiograph 01 OIIerlapping saeerls sIICN>'the Moire
consolidation due to uremia. tJifff!renUa/ includes eIIfrt Screens are lines (arrcNVS)however when
pulmonary edema. aNeoIar pI'Ofeioosis and superimposed they produce nodules (open arrcNVS)and
Pneumocyslis infection. honeycomb patrem.

o Coalescence of opacities
ITERMINOLOGY o AIr bronchogram
Synonyms Anatomic Relationships
Airspace and alveolar often used interchangeably Chest radiograph a summation image of lung and lung
Definitions pathology
Method used to categorize diffuse lung disease (OLD) o In terms of histologic correlation, chest radiograph
and develop a differential diagnosis is 22,000 slides thick

IIMAGING ANATOMY IANATOMY-BASED IMAGING ISSUES I

General Anatomic Considerations
Originally 2 categories: Alveolar and interstitial
o Expanded to nodular and ground-glass opacities
o Interstitial often subcategorized into reticular, and
reticuionoduiar patterns
Even though histologic terms used as patterns, one Is
better off viewing the terms as describing radiographic
patterns rather than truly reflecting anatomic
pathology
o Wide range of variability of use of terms used to
describe patterns, thus interobserver agreement poor
Normal lung thicker at the base than at the apex
o Truly uniform distribution of disease pathologically
will be more apparent in the lower lung zones due
to summation across the greater thickness of the
lower lung zones
o Truly uniform distribution of disease radiologically
may actually be more profuse in the upper lung
zones pathologically due to less summation across
the less thick upper lung zones
Critical Anatomic Structures
Interstitium: Radiographic criteria
o Kerley A or B lines
o Peribronchial cuffing
o Honeycombing
Alveolar: Radiographic criteria
o Distribution
Lobar or segmental
"Batwing" central perihilar
o Poor margination
Key Concepts or Questions
In addition to pattern, what other factors are
important In evaluation of diffuse lung disease?
o Distribution of disease
o Chronicity: Acute, subacute or chronic
o Lung voiumes: Small, normal, or large
o Associated findings: Pleura, hilum or mediastinum,
cardiac, pulmonary vasculature
Is the distribution or the pattern more helpful in
differential diagnosis?
o Distribution of disease: Upper lung zone, lower lung
zone, central, or peripheral more helpful than
pattern in differential diagnosis
What do you do with a mixed pattern?
o Choices: Choose the predominant pattern or the less
severe pattern
Predominant choice based on assumption that
this process most likely reflects the underlying
disease
Less severe choice based on assumption that
predominant pattern may represent coalescence of
less severe pattern
Imaging Approaches
Chest radiography, useful In detection and initial
characterization of OLD
o Correct diagnosis in 40-80% of cases
HRCTprocedure of choice to evaluate OLD
o Confident interpretations Increased over that of
chest radiography
 INTRODUCTION TO PATTERNS

Differential Diagnosis o
0 0 Granulomas (hypersensitivity pneumonitis, 3
Interstitial Lung Disease (90 Yo all Cases) sarcoidosis)
o Sarcoidosis 0 Histiocytosis (Langerhans granulomatosis)
o Histiocytosis (Langerhans granulomatosis) 0 Interstitial pneumonia (rheumatoid lung,
o Idiopathic pulmonary fibrosis dermatomyositis)
o Tumor (lymphangitic) 0 Pneumoconiosis (asbestosis)
o Failure (chronic edema) 0 Scleroderma and rheumatoid arthritis
o Aspiration
o Collagen vascular disease Interstitial Lung Disease and Hyperinflation
o Environmental dusts (organic and inorganic) o Lymphangiomyomatosis
o Drug reaction o Neurofibromatosis
o Sarcoidosis
Honeycomb Lung o Emphysema
o Drug reaction (especially bleomycin and o Histiocytosis (Langerhans granuiomatosis)
methotrexate)
o Idiopathic pulmonary fibrosis

o For example, pulmonary edema both interstitial and

Imaging Protocols alveolar
International Labor Office (ILO)Classification of the Summation
pneumoconiosis o Moire pattern: Superimposition of a repetitive
o Uses pattern analysis to codify the radiographic pattern may result in an image different from that of
abnormalities of pneumoconiosis the individual components
o Classification based on comparing the worker's film o Exampie: linear pattern may resuit in moire pattern
with a set of standard radiographs composed of lines and nodules
Reader determines the underlying opadty Subtraction
(pattern) and quantitates the profusion of o Superimposition of nodules may be subtracted from
opadties on a 12-point scaie theillm
o Small opacities based upon size and shape (round or Shown experimentally
irregular) Shown pathologically where number of nodules
Round opacities characterized as p (up to 1.5 mm (silica) more than shown by radiography
diameter), q (1.5-3 mm) and r (3-10 mm) Modifying factors
Irregular opacities characterized as s (up to 1.5 o Emphysema
mm diameter), t (1.5-3 mm) and u (3-10 mm) Alters the distribution and pattern of the
o Classification has acceptable interobserver underlying lung disease
variability For example, edema or pneumonia usually do not
o Nationallnstltute of Occupational Safety and Health affect the destroyed lung
(NIOSH)administers and certifies physicians in the
use of the 1W system for classifying radiographs for
the presence of pneumoconiosis I PATHOLOGIC ISSUES
A "B"Iader has taken and passed a standardized
examlnatipn designed to test knowledge and General Pathologic Considerations
perceptual abHity in classifying radiographs One-to-one correlation with pathologic slides poor
according to the ILO system
o Application to non-pneumoconiosis diffuse lung
disease has not met with cllnlcal acceptance
However, an atlas of patterns would be useful to
I PATHOLOGY-BASED IMAGING
compare to examination in question, similar to ISSUES
what is done in the ILO classification Key Concepts or Questions
Imaging PittjUs What is the aIrspace?
Nodules may be either interstitial or alveolar o Structures distal to the respiratory bronchioles,
Ground-glass opacities may be et interstitial or mostly alveoli
alveolar o By far the largest volume in the lung
interstitial disease may 6D the atWeOIarspace What is the interstitium?
o Air bronchograms may occur with interstitial lung o Connective tissue skeleton of the lung that extends
disease along the aIrways and blood vesseis (axial interstitial
Examples: Sarcoidosis, lymphoma compartment) and the peripheral subpleural
Alveolar disease may also involve the interstitium connective tissue that makes up the Interlobular
septa (peripheral interstltla1 compartment)
INTRODUCTION TO PATTERNS

PnetpnocysdC:jItoyed pneumonia
Lower Lung-ZOne Predominant Interstitial
Disease
Idiopathic pulmonary fibrosis
~ reactions
Aspiration
Bloncb1ectl111S
CryplItlIf'aI('OJ'W'tft7.ll\gpneumonia
Asbestosis
Rheumatoid arthritis
Scleroderma
Nodular Pattern 9 mm diameter)
Metastases
Malignant tumors: BronchioloaIveoJar cell carcinoma,
lymphoma, Kaposi sarcoma
Granulomas: Slin.uldosIs, bypenensltivlty
pneumonitis, LalJ8erbans granulolQtosIs, fungi,
tuI:IercuIesis
Pneumoconiosis: Silicosis &: CWP
1theamatokt artIuitIs
Laryngeal papUlomatosls
. .Amy1oklosls
Alveolar mlcrolitbiasIs
Miliary Pattern 1.5 mm diameter)
.. ~
SaK;oidosis
J.ansetbans pabulOJRlttOSlS
liypenenslttvltpneumonttis
'DIkosis
"AIVeoIar~
Jntentltlall.un&Disease and Pleural Effusion
""' Pu1Iilonary edema
Lymp~ II,letast.1Ses
"L~~osis
~ Iupuurytbematosus
~Utbdtis
DNg reacttoas
 INTRODUCTION TO PATTERNS

IIMAGE GALLERY . ~r
5
Acute Lung Consolidation V-I-2
Chronic Lung Consolidation V-I-6
Upper Lung Zone Predominant Disease V-I-IO
Basilar Peripheral Lung Zone Disease V-I-I4
Focal Increased Density Lung Disease V-I-I8
Focal Decreased Density Lung Disease V-I-22
Multiple Pulmonary Nodules V-I-26
Cystic Lung Disease V-I-30
HRCT: Bronchocentric Pattern V-I-34
HRCT: Lymphatic Pattern V-I-38
HRCT: Ground-Glass Opacities V-I-42
HRCT: Mosaic Pattern of Lung Attenuation V-I-46
HRCT: Tree-In-Bud Pattern V-I-SO
Anterior Mediastinal Mass V-I-54
Middle Mediastinal Mass V-I-58
Posterior Mediastinal Mass V-I-62
 ACUTE LUNG CONSOLIDATION
1
2

Frontal radiograph from a patient with acute onset fever, Lateral radiograph shows typical appearance of a left
cough, dyspnea, and chest pain shows dense opacity in upper lobar pneumonia secondary to pneumococcal
left upper lobe, in this case lobar pneumonia from infection.
Pneumococcal pneumonia.

o Silhouette sign (Felson)

ITERMINOlOGY An intrathoracic opacity, if in anatomic contact
Abbreviations and Synonyms with a border of heart or aorta, will obscure that
Airspace disease border
An intrathoracic lesion not anatomically
Definitions contiguous with a border or a normal structure
Pathologic term: Disease process (fluid or cells) that will not obliterate that border
replaces the normal air-spaces in the lung Location
o Results in a homogeneous opacity characterized by o Central "bat-wing" distribution
little or no volume loss, effacement of pulmonary Classically cardiogenic pulmonary edema, also
vessels and if the airways remain air-filled: Air pulmonary alveolar proteinosis and Pneumocystis
bronchograms jiroveci pneumonia
CT: Consolidation refers to a region of dense lung o Peripheral "reverse bat-wing" distribution
opacity that completely obscures vessels, unlike Classically chronic eosinophilic pneumonia
ground-glass opacities Size: Secondary pulmonary lobule to entire lobes and
lung
Morphology
I IMAGING FINDINGS o Acinar pattern
Cluster of rounded opacities in the lung, each
General Features measuring 4-8 mm in diameter
Best diagnostic clue Aggregation of acinar shadows produces an
o Air bronchograms extended inhomogeneous opacity
Branching lucencies representing aerated airways
surrounded by consolidated lung parenchyma

DDx: Chronic Consolidation

Bronchoalveo/ar Carcinoma Chronic Eosinophilic Pneumonia Sarcoidosis

 ACUTE LUNG CONSOLIDATION
Key Facts
Terminolo
. gy
PathologIC term:
D'
Isease process
(fl 'd
Ul
II ) th t
or ce s
..
replaces the normal au-spaces m thehlun g . db'
Results in a homogeneous opacity c aractenze y pneumonia
little or no volume loss, effacement of pulmonary
'f h ... fll d' A'
vessels an d I t e aHways remam aH- Ie. u Area of consolidation tends to fade away: No matter
bronchogr~ms .,
CT: ConsolidatIOn refers to a regIOn of dense .Iung
opacity that completely obscures vessels, unlike
..
ground-glass opaCities
Imaging Findings
Air bronchograms
Sil?ouette sign (Felson)
Acmar pattern
o Consolidation: Cloud-like opacity (cumulus or
altocumulus) with indistinct non-anatomic borders,
homogeneous or inhomogeneous
Radiographic Findings
Time course
o Acute and or rapid evolution (hours to days):
Edema, pneumonia, hemorrhage, aspiration, acute
eosinophilic pneumonia
Pneumonia
o Origin in air-space quickly spreads throughout the
lung through pores of Kohn
Will eventually result in lobar involvement
Lobar patterns typical with bacterial pneumonia
o Spread down the bronchi delimited by segmental
anatomy
Aspiration pneumonia typically bilateral,
perihilar, gravitational dependent regions of lung
o Resolution
Area of consolidation tends to fade away: No
matter what the size or shape of the consolidated
lung, resolution tends to occur throughout the
abnormal lung simultaneously
o Resolution of community acquired pneumonia
50% complete by 2 weeks, 66% complete by 4
weeks, 75% complete by 6 weeks
o Resolution depends on virulence of organism and
host resistance
Nosocomial organisms (staphylococcus
pneumonia, enteric gram-negative organisms)
generally complete resolution 6 months
Resolution of radiographic opacities usually lags
behind improvement of clinical symptoms
Clinically, 20% of patients have delayed
resolution, accounts for 10% of all bronchoscopies
o Factors associated with delayed resolution
Age> 50 or smoking history
Chronic illness (chronic obstructive pulmonary
disease, alcoholism, diabetes mellitus, renal
failure)
Bacterial pathogen (especially nosocomial
pneumonia) or multilobar involvement
1
Consolidation: Cloud-like opacity (cumulus or 3
a
altocumulus) with indistinct non-anatomic borders,
h . h ogeneo s
omogeneous or m om u
Acute and or rapid evolution (hours to days): Edema,
hemorrhage, aspiration, acute
. h'I" .
eosmop I ICpneumoma
what the size or shape of the consolidated lung,
resolution tends to occur throughout the abnormal
I' It I
ung slmu aneous y
A' .
nglOgram sign
CT fluid bronchogram
Top Differential Diagnoses
Atelectasis
Chronic Lung Consolidation
Lung cancer (accounts for 10% of delayed
resolution)
o Recommended follow-up for resolution of
pneumonia
Anytime for atypical clinical features or persistent
symptoms
Age> 40 or smoker, 7 & 12 weeks after initiation
of treatment
Pulmonary edema
o Defined as the abnormal accumulation of fluid in
the extravascular compartments of the lung
Formation governed by Starling equation: Balance
of hydrostatic and protein osmotic pressure
between the intravascular and surrounding
interstitial space
Excess fluid first accumulates into interstitial space
(holds 6x the normal interstitial volume)
Once extravascular space has increased
approximately 50%, alveolar flooding occurs
o Distribution: Gravity dependent & central
"bat-wing" distribution
o Gravity dependent: Bases in upright position, dorsal
lung in supine position
Gravitational shift test: Edema will shift in 2-3
hours with change in position
o Bat-wing edema: Acute central, nongravitational
distribution of alveolar edema (seen in < 10%)
Pathophysiology: Hydraulic conductivity or
lymphatic clearance
Hydraulic conductivity: Mucopolysaccharides fill
perivascular space and inhibit flow of fluid, with
excess extravascular water, this extracellular
matrix allows water to flow centrally
Lymphatic clearance: Cortex undergoes greater
respiratory excursion than the central lung, aiding
lymphatic clearance of extracellular water
o Extensive emphysema results in asymmetric edema
in less affected regions
o Resolution
With return to normal of abnormal hemodynamic
pressures edema should resolve in 12-36 hours
Pulmonary hemorrhage
 ACUTE LUNG CONSOLIDATION
1 o Defined as the abnormal accumulation of blood in
the extravascular compartments of the lung
4 Multifactorial etiology: Diffuse often due to
immunologic injury to the capillary endothelium
o Distribution: Dependent on etiology, often similar
to pulmonary edema
Diffuse pulmonary consolidation not gravitational
dependent
Pulmonary infarction: Wedge - peripheral
consolidation (Hampton hump)
o Resolution
Macrophage clearance: Engulf blood products and
removed by lymphatic system
Diffuse consolidation evolves over 2-3 days into
reticular interstitial pattern
Initially hemorrhage clears completely, with
repeated episodes eventually develop residual
interstitial lung disease (hemosiderosis)
Focal infarcts tend to melt away (like an ice cube):
With loss of vascular supply, resolution starts at
the edges and proceeds towards the center of the
infarct
CT Findings
Angiogram sign
o Enhancing pulmonary vessels in a homogeneous
low-attenuating lung consolidation
Due to mucin filled air-spaces
Consolidated lung should have less attenuation
then chest wall musculature
o 1st reported as sign of bronchoalveolar cell
carcinoma
o Other entities: Pneumonia, pulmonary edema,
lipoid pneumonia, pulmonary hemorrhage,
obstructive pneumonitis due to central bronchial
obstruction, lymphoma, and gastrointestinal
carcinoma metastases
CT fluid bronchogram
o Fluid-filled bronchi in consolidated (or atelectatic)
lung suggests partial or total bronchial obstruction
Margins of consolidation can show ground-glass
opacity, implies that process spreads out into
surrounding air-spaces through pores of Kohn
Imaging Recommendations
Best imaging tool
o Chest radiographs usually suffice for detection,
characterization, and monitoring follow-up
o CT frequently obtained when patient's clinical
condition not as expected
o CT very helpful in characterizing associated
abnormalities: Pleural space, adenopathy, lung
abscess, or underlying conditions such as
emphysema
I DIFFERENTIAL DIAGNOSIS
Atelectasis
Occurs when air in the air-spaces is resorbed:
Resorptive, cicatricial, adhesive, passive
Volume less is key distinguishing feature
o Primary sign: Displacement of fissures
o Secondary signs: Hilar displacement, elevation
hemidiaphragm, mediastinal shift, compensatory
hyperinflation
Complete lobar collapse suggests central airway
obstruction
o Due to foreign body, airway stenosis, but most often
occurs due to carcinoma
Chronic lung Consolidation
Bronchoalveolar cell carcinoma
Cryptogenic organizing pneumonia [bronchiolitis
obliterans organizing pneumonia (BOOP)]
Lipoid pneumonia or chronic aspiration
o Gravitationally dependent, fat density at CT for
lipoid pneumonia
Chronic eosinophilic pneumonia
o Reverse bat-wing upper lobe predominant
Lymphoma or pseudolymphoma
o Bronchovascular distribution, usually recurrence of
known disease
Sarcoidosis (especially alveolar form)
o Large masses, typically have hilar and mediastinal
adenopathy
Alveolar proteinosis
Amyloidosis
I PATHOLOGY
Gross Pathologic & Surgical Features
Communication between alveolar spaces (pores of
Kohn) allows disease to spread throughout a lobe, only
delimited by pleura barrier
Pleural fissures usually incomplete centrally, allows
spread between lobes
Microscopic Features
Alveolar walls remain intact: Lung usually returns to
normal when acute consolidative processes resolve
ICLINICAL ISSUES
Presentation
Most common signs/symptoms
o Symptoms depend on underlying process
o Clinically, most common cause of consolidation,
causing egophony ("E" to "A" changes) at
auscultation, is pneumonia
Other signs/symptoms
o Sputum analysis
Non-protein rich fluid in cardiogenic pulmonary
edema (occasionally hemorrhagic)
Inflammatory cells and organisms in pneumonia
Blood and hemosiderin laden macrophages in
hemorrhage
Bronchorrhea in bronchioloalveolar carcinoma
(rare)
I SELECTED REFERENCES
1. Kjeldsberg KMet al: Radiographic approach to multifocal
consolidation. Semin Ultrasound CT MR. 23(4):288-301,
2002
 ACUTE LUNG CONSOLIDATION

I IMAGE GALLERY 1
5

(Left) Frontal radiograph

from a patient with a history
of coronary artery disease
and acute onset dyspnea
shows typical perihilar
butterfly pattern pulmonary
edema from acute left heart
decompensation. (Right)
Frontal radiograph obtained
after an attempt at suicide by
hanging shows bilateral
upper lung zone perihilar
opacities resulting (rom
negative pressure pulmonary
edema.

(Left! Coronal oblique CECT

shows pulmonary
hemorrhage into the lingula
after a penetrating injury to
left lung. Note exquisite
demonstration of blood
filling clusters of airspaces
(arrows), abutting the major
fissure. (Right! Axial CECT
shows relatively
hypoattenuating right middle
lobe pneumonia (arrow)
from fluid filled alveoli
juxtaposed against airless
and higher attenuating right
lower lobe atelectasis
(curved arrow).

Typical
(Left) Axial CECT shows
pulmonary hemorrhage in
right middle lobe due to
blunt trauma and pulmonary
laceration (arrow) and
subsequent bleeding into
surrounding alveoli. (Right!
Axial CECT shows right
upper lobe pneumonia
superimposed upon
centri/obular pulmonary
emphysema (arrow). Note
less than typical
consolidation pattern
secondary to focal areas of
alveolar destruction.
 CHRONIC LUNG CONSOLIDATION
1
6

Frontal radiograph shows local consolidation in the right Frontal radiograph shows multiple large airspace masses
lower lobe (arrows). Consolidation progressively (arrows) that contained air-bronchograms. Films were
worsened over 2 months. Differential is chronic unchanged over 1 month period. Differential is chronic
consolidation. Bronchia/oalvea/ar cell carcinoma. consolidation. Alveolar sarcoidosis.

o Silhouette sign (Felson)

ITERMINOlOGY An intrathoracic opacity, if in anatomic contact
Abbreviations and Synonyms with a border of heart or aorta, will obscure that
Airspace disease border
An intrathoracic lesion not anatomically
Definitions contiguous with a border or a normal structure
Pathologic term: Disease process (fluid or cells) that will not obliterate that border
replaces the normal airspaces in the lung Location
o Results in a homogeneous opacity characterized by o Central "bat-wing" distribution
little or no volume loss, effacement of pulmonary Classically cardiogenic pulmonary edema, also
vessels and if the airways remain air-filled: Air pulmonary alveolar proteinosis and Pneumocystis
bronchograms jiroveci pneumonia
CT: Consolidation refers to a region of dense lung o Peripheral "reverse bat-wing" distribution
opacity that completely obscures vessels, unlike Classically chronic eosinophilic pneumonia
ground-glass opacities Size: Secondary pulmonary lobule to entire lobes and
lung
Morphology
IIMAGING FINDINGS o Acinar pattern
Cluster of rounded opacities in the lung, each
General Features measuring 4-8 mm in diameter
Best diagnostic clue Aggregation of acinar shadows produces an
o Air bronchograms extended inhomogeneous opacity
Branching lucencies representing aerated airways
surrounded by consolidated lung parenchyma

DDx: Acute Consolidation

Pulmonary Edema Pulmonary Hemorrhage Acute Eosinophilic Pneumonia

 CHRONIC LUNG CONSOLIDATION

Key Facts
1
Terminology Sarcoid, alveolar 7
Migratory nonsegmental pulmonary consolidation
Pathologic term: Disease process (fluid or cells) that
replaces the normal airspaces in the lung Drug toxicity, amiodarone

Imaging Findings Top Differential Diagnoses

Time course: Focal or diffuse consolidation persisting Atelectasis
more than 30 days Acute Lung Consolidation
Time limitation arbitrary, in contrast to acute Pathology
consolidation that evolves rapidly (minutes to days) Lepidic growth: Cells use the normal lung
Cryptogenic organizing pneumonia architecture to grow
Bronchoalveolar cell carcinoma
Alveolar proteinosis Diagnostic Checklist
Aspiration Cardiogenic pulmonary edema can be very chronic,
Lipoid pneumonia before invasive diagnosis a trial of diuretics is
Lymphoma warranted to exclude pulmonary edema
Pseudolymphoma Chronic consolidation (arbitrary> 30 days duration)
Chronic eosinophilic pneumonia limited differential diagnosis

o Consolidation: Cloud-like opacity (cumulus or Chronic eosinophilic pneumonia

altocumulus) with indistinct non-anatomic borders,
homogeneous or inhomogeneous
Radiographic Findings
Time course: Focal or diffuse consolidation persisting
more than 30 days
o Time limitation arbitrary, in contrast to acute
consolidation that evolves rapidly (minutes to days)
Cryptogenic organizing pneumonia
o Synonym: Bronchiolitis obliterans organizing
pneumonia (BOOP)
o Etiology: Idiopathic, infection, drugs, transplants,
toxic fume inhalation
o Patterns: Chronic airspace consolidation (70%)
peripheral and slightly more common lower lobes
Diffuse interstitial pattern: (15%)
Focal consolidation
Multiple large masses
o Unilateral 5%
o Tends to wax and wane
Bronchoalveolar cell carcinoma
o Focal or diffuse consolidation most common pattern
o Progressively worsens
Alveolar proteinosis
o Central pulmonary consolidation
o No pleural effusions, Kerley B lines
o Does not respond to diuretics
Aspiration
o Bilateral (or unilateral) foci of consolidation in
dependent lung (lung bases in erect position, dorsal
lung in supine position)
o Evidence of esophageal reflux or swallowing disorder
Lipoid pneumonia
o Findings similar to aspiration
o History of mineral oil use or oily nose drops
Lymphoma
o Multiple foci of consolidation, often nodular or
mass-like
o More common in the central aspect of the lung
Pseudolymphoma
o Focal consolidation, often mass-like starting at the
hilum, progressing towards the lung periphery
o Peripheral consolidation primarily in upper lung
zones
o May wax and wane
o Normal heart size: No pleural effusions or
adenopathy
o Resolution
Inner edge of peripheral consolidation last to
resolve, may leave lines paralleling the chest wall
Rapid resolution with steroids
o Recurrence: Same place, same size, same shape
Sarcoid, alveolar
o Multiple large airspace opacities often worse in the
upper lung zones
o Usually associated with symmetric hilar adenopathy
o High incidence of spontaneous pneumothorax
Churg-Strauss syndrome
o Migratory nonsegmental pulmonary consolidation
o Pleural effusions (1/3)
o May have adenopathy
o History of asthma
Drug toxicity, amiodarone
o Single or multiple foci of consolidated lung
o History of cardiac disease, may have pacemakers or
implanted defibrillators
CT Findings
Angiogram sign
o Enhancing pulmonary vessels coursing in
homogeneous low-attenuation lung consolidation
Due to mucin filled air-spaces
Consolidated lung should have less attenuation
than chest wall musculature
1st described as sign of bronchoalveolar cell
carcinoma
Other entities: Pneumonia, pulmonary edema,
lipoid pneumonia, pulmonary hemorrhage,
obstructive pneumonitis due to central bronchial
obstruction, lymphoma, and gastrointestinal
carcinoma metastases
o CT fluid bronchogram
Fluid-filled bronchi in consolidated (or atelectatic)
lung suggests partial or total bronchial obstruction
 CHRONIC LUNG CONSOLIDATION
1 Specific diseases
o Alveolar proteinosis
8 "Crazy-paving" pattern: Geographic ground-glass
opacities containing polygonal lines
o Chronic eosinophilic pneumonia
Reverse "Bat-wing" distribution primarily I PATHOLOGY
involving the upper lung zones
Resolves from the outside in, inside edge last to General Features
resolve: May see linear or band-like opacities
paralleling chest wall
o Lymphoma
Bronchovascular distribution often more central
in the lung
May have adenopathy
o Lipoid pneumonia
Consolidated lung is of low attenuation (fat
density) due to aspirated material
o Amiodarone drug toxicity
History of cardiac disease, often with pacemakers
or implanted defibrillators
Consolidated lung of high density due to iodine
molecules in amiodarone
Liver also accumulates the drug and is of high
density
Imaging Recommendations
Best imaging tool
o Chest radiographs usually suffice for detection,
characterization and monitoring follow-up
o CT useful as problem solving tool
I DIFFERENTIAL DIAGNOSIS
Atelectasis
Occurs when air in the airspaces is resorbed:
Resorptive, cicatricial, adhesive, passive mechanisms
Volume loss is key distinguishing feature Consider
o Primary sign: Displacement of fissures
o Secondary signs: Hilar displacement, elevation
hemidiaphragm, mediastinal shift, compensatory
hyperinflation
Complete lobar collapse suggests central airway Image Interpretation
obstruction
o Due to foreign body, airway stenosis, but most often
due to bronchogenic carcinoma
Acute Lung Consolidation
Pulmonary edema, cardiogenic and noncardiogenic
o Cardiogenic pulmonary edema can be chronic if
untreated
Pneumonia
o Some pneumonias, especially fungal, may be very
slow to resolve
Pulmonary hemorrhage 3.
o Recurrent hemorrhage can mimic chronic
consolidation
o Each episode of hemorrhage will evolve over 3-5
days
o Usually have iron deficient anemia
Acute eosinophilic pneumonia
o Migratory nonsegmental pulmonary consolidation
o Evolves over days, may wax and wane
Aspiration
o Recurrent consolidation, often in the same location,
often admixed with reticulonodular opacities,
bronchiectasis, and cicatricial fibrosis
Lepidic growth: Cells use the normal lung architecture
to grow
o Lung architecture not destroyed
o Seen with bronchoalveolar cell carcinoma,
lymphoma, and occasionally gastrointestinal
carcinoma metastases
Gross Pathologic & Surgical Features
Communication between alveolar spaces (pores of
Kohn) allows disease to spread throughout a lobe, only
delimited by pleura barrier
Pleural fissures usually incomplete centrally, allows
spread between lobes
Microscopic Features
Alveolar walls remain intact: Lung usually returns to
normal when consolidative process resolves
o Aspiration and lipoid pneumonia may eventually
result in cicatricial fibrosis and bronchiectasis
I CLINICAL ISSUES
Presentation
Most common signs/symptoms: Symptoms depend on
underlying process
I DIAGNOSTIC CHECKLIST
Cardiogenic pulmonary edema can be very chronic,
before invasive diagnosis a trial of diuretics is
warranted to exclude pulmonary edema
Pearls
Chronic consolidation (arbitrary> 30 days duration)
limited differential diagnosis
I SELECTED REFERENCES
1. Mochimaru H et al: Clinicopathological differences
between acute and chronic eosinophilic pneumonia.
Respirology. 10(1):76-85,2005
2. Baron SE et al: Radiological and clinical findings in acute
and chronic exogenous lipoid pneumonia. J Thorac
Imaging. 18(4):217-24, 2003
Jayakrishnan B et al: Chronic lobar consolidation. Int J
Clin Pract. 57(2):112-4, 2003
4. Kjeldsberg KM et al: Radiographic approach to multifocal
consolidation. Semin Ultrasound CT MR. 23(4):288-301,
2002
5. Arakawa H et al: Bronchiolitis obliterans with organizing
pneumonia versus chronic eosinophilic pneumonia:
high-resolution CT findings in 81 patients. AJR Am J
Roentgenol. 176(4):1053-8,2001
6. Bosanko CM et al: Lobar primary pulmonary lymphoma:
CT findings. J Com put Assist Tomogr. ] 5(4):679-82, 1991
 CHRONIC LUNG CONSOLIDATION

I IMAGE GALLERY 1
9

(Left) Frontal radiograph

shows diffuse pulmonary
consolidation. Earlier
radiographs over the
previous month were
essentially unchanged (not
shown). (Right) Axial NECT
shows diffuse consolidation
in the dependent dorsal
lung. Consolidated lung is of
low attenuation (-100 1-1U)
from lipoid pneumonia.

Typical
(Left) Frontal radiograph
shows central consolidation
(I'bat-wing") (arrows) which
was chronic (not shown).
Patient was asymptomatic.
Pulmonary alveolar
proteinosis. (Right) Frontal
radiograph shows multiple
nonsegmentaf areas of
consolidation in the lung
periphery (arrows). Findings
were chronic. Chronic
eosinophilic pneumonia.

(Left) Frontal radiograph

shows large central
homogeneous opacities.
These areas of focal
consolidation had gradually
enlarged over several years.
Oifferential is chronic
consolidation. (Right) Axial
NECT Shows
air-bronchograms (arrows) in
consolidated lung. No
adenopathy or pleural
effusions. Biopsy proven
pseudolymphoma.
 UPPER LUNG ZONE PREDOMINANT DISEASE
1
10

Coronal NECT shows nodules and cYS15(arrows) more Frontal radiograph shows diffuse nodules and
profuse in the upper lobes from Langerhans ce/l ground-glass opacities more profuse in the periphery of
granulomatosis. !he upper lobes (arrows) due to sarcoidosis.

o Consider the lung as a map, with zones not defined

I TERMINOlOGY by anatomy but by regional differences produced by
Definitions physiology
Predominant radiographic abnormality, either airspace Pattern and distribution of disease not a random
or interstitial space, found in the upper lung zones process but the end result of the interaction
between a pathologic process with its
environment
Soil and seed concept: Seeds (pathologic process)
IIMAGING FINDINGS finds certain soils (physiologic regions) more
General Features conducive to growth or propagation
Best diagnostic clue Radiographic Findings
o Diffuse lung disease worse in the upper lung zones
Radiography
o Distribution versus pattern
o Distribution of disease usually readily apparent from
In differential diagnosis, distribution more helpful
the frontal radiograph
than the pattern of abnormality
Normally, the lung much thicker at the base than
Agreement between observers about location
at the apex
higher than agreement about the pattern
Truly uniform distribution of pathology will be
Location more apparent in the lower lung zones due to
o Normal physiologic gradients create zones or regions
summation across the greater thickness of the
of the lung which differ in terms of blood flow,
lower lobes
ventilation, lymphatic function, stress, and
concentration of inhaled gases

DDx: Lower Lobe Peripheral

Idiopathic Pulmonary Fibrosis Asbestosis Scleroderma

 UPPER LUNG ZONE PREDOMINANT DISEASE

Key Facts 1
Terminology Sarcoidosis 11
Predominant radiographic abnormality, either Langerhans Cell Granulomatosis
airspace or interstitial space, found in the upper lung Silicosis & Coal Worker Pneumoconiosis
zones Centrilobular Emphysema
Chronic Eosinophilic Pneumonia
Imaging Findings Chronic Hypersensitivity Pneumonitis
In differential diagnosis, distribution more helpful Cystic Fibrosis
than the pattern of abnormality Allergic Bronchopulmonary Aspergillosis
Consider the lung as a map, with zones not defined Neurogenic Pulmonary Edema
by anatomy but by regional differences produced by Smoke Inhalation
physiology Metastatic Pulmonary Calcification
Pattern and distribution of disease not a random Ankylosing Spondylitis
process but the end result of the interaction between
a pathologic process with its environment
Diagnostic Checklist
Distribution of disease important finding in
Top Differential Diagnoses differential diagnosis
Post-Primary Tuberculosis

Uniform radiographic distribution may actually be

more profuse in the upper lung zones Silicosis & Coal Worker Pneumoconiosis
pathologically due to less summation across the Long term exposure to occupational dusts
less thick upper lobes Simple (nodular interstitial thickening) may progress
to pulmonary massive fibrosis (PMF)
CT Findings Nodules follow lymphatics in the lung, more profuse
HRCT: Vertical distribution apparent by comparing in the dorsal lung
severity between upper lobe sections and lower lobe Probable pathophysiology: Lymphatic clearance
sections or by reformatting in the coronal plane
Centrilobular Emphysema
Imaging Recommendations Sequelae of long term smoking
Best imaging tool Precursor may be respiratory bronchiolitis (also more
o Chest radiography sufficient to determine profuse in upper lung zones)
distribution of disease Probable pathophysiology: Lymphatic clearance and
o HRCT useful to further characterize underlying ventilation/perfusion ratio (v/QJ
pattern
Chronic Eosinophilic Pneumonia
Predominant peripheral lung ("photographic negative"
I DIFFERENTIAL DIAGNOSIS of pulmonary edema)
Rapid response to corticosteroid therapy
Post-Primary Tuberculosis Probable pathophysiology: Unknown
Apical posterior segment of the upper lobes (dorsal
Chronic Hypersensitivity Pneumonitis
lung)
Cavitary disease along with patchy areas of History of antigen exposure
consolidation and bronchial wall thickening Upper lobe distribution especially common in Farmer's
lung
Probable pathophysiology: Lymphatic clearance and
Midlung predominance in many other antigen
oxygen concentration
exposures (Bird Breeder's lung)
Sarcoidosis Probable pathophysiology: Lymphatic clearance
Chronic granulomatous process of unknown etiology
Cystic Fibrosis
Multiorgan disease
Bronchovascular distribution primarily on Autosomal recessive gene disorder which produces
thick viscous secretions
cross-sectional imaging
Bronchiectasis more severe in upper lobes, especially
Probable pathophysiology: Lymphatic clearance
the right upper lobe
Langerhans Cell Granulomatosis Probable pathophysiology: Ventilation
Granulomas contain Langerhans cell (which process
Allergic Bronchopulmonary Aspergillosis
antigens)
Seen almost exclusively in young smokers Often asthmatic history, abnormal hypersensitivity
Probably an allergic reaction to constituent of cigarette reaction to Aspergillus organisms
puff Central bronchiectasis often with peripheral sparing
Nodules evolving into bizarre shaped cysts Also seen in patients with cystic fibrosis
Probable pathophysiology: Lymphatic clearance Probable pathophysiology: Ventilation
 UPPER LUNG ZONE PREDOMINANT
1 Neurogenic Pulmonary Edema
Any central nervous system (CNS) insult that acutely
12
raises intracranial pressure
Edema due to both hydrostatic and capillary leak
Develops within minutes to hours of CNS insult
Probable pathophysiology: Perfusion
Smoke Inhalation
Burning wood or plastic products create volatile
compounds that produce a chemical pneumonitis
High ventilation/perfusion ratio in upper lung zones
concentrates inhaled gases
Probable pathophysiology: V/Q ratio
Metastatic Pulmonary Calcification
Deposition of calcium in otherwise normal tissue
Seen with hypercalcemic states, especially renal failure
High pH in upper lung zones, calcium less soluble in
alkaline environment
Probable pathophysiology: V/Q ratio
Ankylosing Spondylitis
Fibrocystic change in upper lobes
Seen in < 2% of patients with ankylosing spondylitis
Probable pathophysiology: Unknown, may be due to
distribution of stress and strain in the lung
I PATHOLOGY
General Features
General path comments
o Standard pathologic investigation of whole lungs is
the Gough-Wentworth technique
Thin (1 mm) section through formalin inflated
lung
Sagittal section through the left lung standard
Etiology
o Nearly all physiologic processes such as ventilation
and perfusion are gravity dependent
Diseases showing a preponderance in the upper
lung zones seem paradoxical
o Regional physiology (upright position)
Diminished blood flow and ventilation
High oxygen concentration and low carbon
dioxide concentration
Alveoli expanded, high stress and strain on
connective tissue
o Pathologic process can be acute (neurogenic
pulmonary edema) or chronic (silicosis)
o For particulate diseases such as silicosis,
concentration of particles reflects the distribution of
disease
o Normally particles removed by lymphatics
Lymphatic function dependent on pulmonary
artery pressure and respiratory motion
Normal linear gradient of pulmonary artery
pressure highest in the dependent lung
Respiratory motion dependent on diaphragmatic
excursion and chest wall motion
Least respiratory motion in dorsal upper lung
zones
DISEASE
Even though the majority of particles are acutely
deposited in the lower lung zones (in the erect
position), lymphatic function removes those
particles
Chronically particles tend to accumulate in the
dorsal aspect of the upper lung zones
Due to inclination of the main pulmonary artery
to the left, left upper subject to a jet effect across
the pulmonary valve
Left upper lobe normally has about 10% more
perfusion than the right upper lobe
End result the right upper lobe retains more
particles than the left upper lobe, thus
radiographic abnormalities due to the chronic
retention of particles tend to be more severe in the
right upper lobe than the left upper lobe
Tall lung has more regional disparity than short lung,
little studied in diffuse lung disease
o Tall lung at risk for
Spontaneous pneumothorax (para septal
emphysema)
Silicosis
o Short lung at risk for
Asbestosis
Gross Pathologic & Surgical Features
Surgical specimens: Pathologist cannot determine
where a specimen originated by gross inspection
o Collaboration with radiology useful to determine
whether lung sample is representative of the
pathologic process in the lung
IClINICAllSSUES
Presentation
Pulmonary function tests
o Global test of entire system
o Zones or regions of the lung contribute unequally to
overall function
o Upper lung zones contribute less to overall function
Pathologic processes tend to be more severe before
producing functional abnormalities
Best results for lung volume reduction surgery in
those with upper lobe predominant disease
o Lower lung zones contribute more to overall
function
Pathologic processes usually less severe before
producing functional abnormalities
I DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Distribution of disease important finding in
differential diagnosis
I SElECTED REFERENCES
1. Gurney JW: Cross-sectional physiology of the lung.
Radiology. 178(1):1-10, 1991
2. Gurney JW et al: Upper lobe lung disease: physiologic
correlates. Review.Radiology. 167(2):359-66, 1988
 UPPER LUNG ZONE PREDOMINANT DISEASE
I IMAGE GALLERY 1
13

(Left) Frontal radiograph

shows diffuse interstitia/lung
disease worse in the upper
lung zones. Long term farmer
with farmer's lung. (Right)
Frontal radiograph shows
perihilar and upper lung
zone progressive massive
fibrosis (arrows) from
silicosis.

(Left) Frontal radiograph

shows diffuse nodularity
(arrows) more profuse in the
upper lung zones. Mild
cardiomegaly and bipolar
pacemaker. Long history of
renal dialysis. Metastatic
pulmonary calcification.
(Right) Frontal radiograph
shows biapical
bronchiectasis in the upper
lobes from cystic fibrosis.
Pathologic changes are
worse in the right upper
lobe.

(Left) Anteroposterior
radiograph shows dense
diffuse consolidation worse
in the upper lung zones from
smoke inhalation. (Right)
Anteroposterior radiograph
shows dense consolidation in
the upper lung zones
following acute eNS insult.
Neurogenic pulmonary
edema.
 BASILAR PERIPHERAL LUNG ZONE DISEASE
1
14

Coronal CECT shows peripheral basilar distribution of Axial CECT shows peripheral subpleural reticular
asbestosis (arrows). Calcified diaphragmatic plaques interstitial thickening and traction bronchiectasis from
(open arrows). chronic inlersliljal lung disease in patient with
rheumatoid arthritis.

o Consider the lung as a map, with zones not defined

tTERMINOlOGY by anatomy but by regional differences produced by
Definitions physiology
Predominant radiographic abnormality, either airspace Soil and seed concept: Seeds (pathologic process)
or interstitial space, found in the periphery of lower finds certain soils (physiologic regions) more
lobes conducive to growth or propagation
Lung reacts to the internal environment to which
it is exposed
IIMAGING FINDINGS Morphology: In contrast to organs such as the brain or
kidney, the lung has no anatomic demarcation such as
General Features the gray-white matter differentiation in the brain or
Best diagnostic clue the cortex-medulla organization in the kidney
o Diffuse lung disease worse in the periphery of the
Radiographic Findings
lower lung
Radiography
o Distribution versus pattern
o Distribution of disease usually readily apparent from
In differential diagnosis, distribution more helpful
the frontal radiograph
than the pattern of abnormality
Normal, the lung much thicker at the base than at
Agreement between observers about location
the apex
higher than agreement about the pattern
Truly uniform distribution of pathology will be
Location
more apparent in the lower lung zones due to
o Normal physiologic gradients create zones or regions
summation across the greater thickness of the
of the lung which differ in terms of blood flow,
lower lobes
ventilation, lymphatic function, stress, and
concentrations of inhaled gases

DDx: Upper lobe Peripheral

Silicosis Chronic Eosinophilic Pneumonia Sarcoidosis

 BASILAR PERIPHERAL LUNG ZONE DISEASE
Key Facts
Terminology
Predominant radiographic abnormality, either
airspace or interstitial space, found in the periphery
of lower lobes
Imaging Findings
In differential diagnosis, distribution more helpful
than the pattern of abnormality
Consider the lung as a map, with zones not defined
by anatomy but by regional differences produced by
physiology
Lung reacts to the internal environment to which it is
exposed
Top Differential Diagnoses
Idiopathic Pulmonary Fibrosis
Asbestosis
Uniform radiographic distribution may actually be
more profuse in the upper lung zones
pathologically due to less summation across the
less thick upper lobes
o Peripheral process refers to the periphery of each
lobe
Thus, peripheral process will also be found in the
"center" of the hemithorax as pathology follows
the outlines of the fissures
Peripheral process may also appear to be relatively
uniform across the frontal radiograph due to
summation of the peripheral pathology across the
lung
CT Findings
HRCT
o Distribution of disease in axial plane useful to
determine peripheral distribution
Vertical distribution apparent by comparing
severity between upper lobe sections and lower
lobe sections or by reformatting in the coronal
plane
Imaging Recommendations
Best imaging tool
o Chest radiography sufficient to determine
distribution of disease
o HRCT useful to further characterize underlying
pattern
I DIFFERENTIAL DIAGNOSIS
Idiopathic Pulmonary Fibrosis
Usual interstitial pneumonitis (UlP) pattern in lung
biopsy
Subpleural honeycombing usually striking
Traction bronchiectasis necessary for diagnosis
Asbestosis
Asbestos bodies at microscopic section
Pleural plaques common (80%)
Occupational history important
1
Scleroderma 15
Rheumatoid Arthritis
Cryptogenic Organizing Pneumonia (COP)
Bleomycin Drug Toxicity
Hematogenous Metastases
Adult Respiratory Distress Syndrome (ARDS)
Sarcoidosis
Silicosis or coal worker pneumoconiosis
Chronic eosinophilic pneumonia
"Negative" of "bat-wing" cardiogenic edema
Paraseptal emphysema
Clinical Issues
Upper lung zones contribute less to overall function
Peripheral lung beyond the reach of bronchoscopic
biopsy
Scleroderma
UIP pattern or more commonly nonspecific interstitial
pneumonia (NSIP) pattern in lung biopsy
Associated finding of dilated esophagus
Rheumatoid Arthritis
UIP pattern or more commonly NSIP pattern in lung
biopsy
Associated findings of erosive arthritis
Cryptogenic Organizing Pneumonia (COP)
Multifactorial etiology: Idiopathic, reaction to toxic
inhaled gases
Alveolar loosely organized granulation tissue
extending into bronchioles
Patchy areas of consolidation, other findings includes
nodules and reticular pattern
Bleomycin Drug Toxicity
cOP pattern at lung biopsy
Often nodular, mimicking hematogenous metastases
Hematogenous Metastases
80% within 2 cm of a pleural surface
Multiple sharply defined variably sized pulmonary
nodules
Adult Respiratory Distress Syndrome (ARDS)
Diffuse alveolar damage (DAD) histologic pattern
Ground-glass opacities to dense consolidation
accentuated in dependent lung
Etiology: Numerous medical and surgical conditions
Clinical features: Low arterial oxygenation despite
high levels of inspired oxygen concentration
Upper Lobe Peripheral Distribution
Sarcoidosis
o May have adenopathy (usually regresses as lung
disease worsens)
o Perilymphatic pattern, more typically has a
bronchovascular distribution
Silicosis or coal worker pneumoconiosis
 BASILAR PERIPHERAL
1 a Adenopathy common (sometimes with egg-shell
calcification)
16 a Perilymphatic nodules more profuse in the dorsal
aspect of the right upper lobe
a Nodules may aggregate together into progressive
massive fibrosis (PMF)
a Occupational history important
Chronic eosinophilic pneumonia
a Peripheral consolidation
a "Negative" of "bat-wing" cardiogenic edema
a Rapid resolution with steroid therapy
Paraseptal emphysema
a More common in tall individuals
a Sometimes associated with intravenous (IV) drug
abuse
I PATHOLOGY
General Features
General path comments
a Standard pathologic investigation of whole lungs is
the Gough-Wentworth technique
Thin (1 mm) sections through formalin inflated
lung
Sagittal section through the left lung standard
Etiology
a For particulate diseases such as asbestosis:
Concentration of particles reflects the distribution of
disease
a Normally particles removed by lymphatics
Lymphatic function dependent on pulmonary
artery pressure and respiratory motion
Normal linear gradient of pulmonary artery
pressure highest in the dependent lung
Respiratory motion dependent on diaphragmatic
excursion and chest wall motion
Least respiratory motion in the dorsal upper lung
zones
Even though the majority of particles are acutely
deposited in the lower lung zones (in the erect
position), lymphatic function removes those
particles
Chronically particles tend to accumulate in the
dorsal aspect of the upper lung zones
Due to inclination of the main pulmonary artery
to the left, left upper lobe subject to a jet effect
across the pulmonary valve
Left upper lobe normally has about 10% more
perfusion than the right upper lobe
End result: Right upper lobe retains more particles
than the left upper lobe, thus radiographic
abnormalities due to the chronic retention of
particles tend to more severe in the right upper
lobe than the left upper lobe
a Long fibrous particles (asbestos) are not removed by
lymphatics because macrophages are unable to
engulf and remove the particles
Hence, the pathology of asbestosis tends to reflect
the initial deposition of particles in the periphery
of the lower lung
a Small round particles (silica) are engulfed by
macrophages and removed by lymphatics
LUNG ZONE DISEASE
Hence the pneumoconiosis due to small round
particles (coal and silica) reflects the eventual
accumulation of retained particles in the dorsal
aspect of the upper lobes
Tall lung has more regional disparity than short lung,
little studied in diffuse lung disease
a Tall lung at risk for
Spontaneous pneumothorax (paraseptal
emphysema)
Silicosis
a Short lung at risk for
Asbestosis
Gross Pathologic & Surgical Features
Surgical specimens: Pathologist cannot determine
where a specimen originated by gross inspection
a Collaboration with radiology useful to determine
whether lung sample is representative of the
pathologic process in the lung
I CLINICAL ISSUES
Presentation
Pulmonary function tests
a Global test of entire system
a Zones or regions of the lung contribute unequally to
overall function
a Upper lung zones contribute less to overall function
Pathologic processes tend to be more severe before
producing functional abnormalities
Best results for lung volume reduction surgery in
those with upper lobe predominant disease
a Lower lung zones contribute more to overall
function
Pathologic processes usually less severe before
producing functional abnormalities
Treatment
Surgical biopsy
a Peripheral lung beyond the reach of bronchoscopic
biopsy
a Thorascopic biopsy usually required
Biopsy should be directed to ground-glass
opacities, consolidation, nodules
I DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Distribution of disease important finding in
differential diagnosis
I SElECTED REFERENCES
1. Murray JF: Bill Dock and the location of pulmonary
tuberculosis: how bed rest might have helped
consumption. Am J Respir Crit Care Med. 168(9): 1029-33,
2003
2. Gurney JW: Cross-sectional physiology of the lung.
Radiology. 178(1):1-10, 1991
3. Gurney JW et al: Upper lobe lung disease: physiologic
correlates. Review. Radiology. 167(2):359-66, 1988
 BASILAR PERIPHERAL LUNG ZONE DISEASE
I IMAGE GALLERY 1
17
Typical
(Left) Frontal radiograph
shows peripheral basilar
interstitia/lung disease in
asbestosis. Calcified pleural
plaque (arrow). (Right) Axial
H RCT shows reticular
interstWaf thickening and
traction bronchiectasis from
asbestosis. Interstitial disease
is predominantly peripheral
(arrows) in asbestosis.

Typical
(Left) Frontal radiograph
shows diffuse interstitial lung
disease. Basilar distribution.
Peripheral predominance not
evident from the chest
radiograph. (Right) Axial
NECT shows fine reticular
pattern and ground-glass
opacities in the periphery of
the lower lobes. Dilated
esophagus (arrow).
Nonspecific interstitial
pneumonia in scferoderma.

Typical
(Left) Axial NECT shows
multiple sharply defined
variable sized pulmonary
nodules from hematogenous
metastases from breast
carcinoma. 80% of
metastases occur within 2
em of a pleural surface.
(Right) Axial NECT shows
mullifocaf areas of
consolidation and
ground-glass opacities in the
periphery of the right middle
lobe, lingula, and right lower
lobe (arrows). Idiopathic
cryptogenic organizing
pneumonia.
 FOCAL INCREASED DENSITY LUNG DISEASE
1
18

Axial CECT shows focal increased density manifesting as Axial CECT shows focal increased density manifesting as
pulmonary nodule in bronchogenic carcinoma granuloma (arrows). Borders of lesion are welf-defined.
(arrows). Spiculated borders of nodule are highly Bronchiectasis (open arrows) suggest chronic local
suggestive or malignancy. inflammatory process.

Morphology
ITERMINOlOGY o Shape
Abbreviations and Synonyms Round or oval
Lobulated
Mass
Amorphous
Nodule
o Borders
Consolidation
Well-defined
Definitions Ill-defined
Area with markedly increased density Spiculated
Partly or entirely surrounded by lung parenchyma of o Matrix
normal or decreased density Homogeneous
Always localized, never diffuse Inhomogeneous
Either solitary or multiple Calcified
To be differentiated against o Surrounding
o Subtle increased density ("ground-glass opacities") Direct contact to vessels
o Countless diffuse lesions ("miliary pattern") Direct contact to bronchi
o Segmental or lobar lesions (atelectasis, pneumonia) Direct interphase with pleura, chest wall, or
mediastinum
o Associated abnormalities
I IMAGING FINDINGS Pleural effusion or thickening
Hilar or mediastinal adenopathy
General Features
Location: Occurs in any anatomic location of the lung Imaging Recommendations
Size: Occupies entire range of possible sizes Best imaging tool: CT

DDx: Focal Increased Density

Ground-Glass Miliary Pattern Lobar Disease

 FOCAL INCREASED DENSITY LUNG DISEASE
Key Facts
1
Terminology Collagen-Vascular 19
Area with markedly increased density Inflammatory
Partly or entirely surrounded by lung parenchyma of Benign Neoplasms
normal or decreased density Malignant Neoplasms
Always localized, never diffuse Miscellaneous and Mimics
Either solitary or multiple Clinical Issues
Imaging Findings Depends upon disorder
Best imaging tool: CT History and presentation important for diagnosis
Perform native scans to detect calcifications Diagnostic Checklist
Perform post-contrast scans to assess enhancement Direct contact to vessels
pattern and vascularity Direct contact to bronchi
Top Differential Diagnoses Lobulated borders
Congenital Calcification
Inhalational Spiculation
Vascular Growth

Protocol advice Nocardiosis

o Verify that lesion is truly pulmonary, versus Mycoplasmosis
Pleural o Viral
Mediastinal Measles
Pericardial Varicella-Zoster
Neurogenic o Parasites
o Perform native scans to detect calcifications Hydatid cyst
o Perform post-contrast scans to assess enhancement Paragonimiasis
pattern and vascularity Abscess
Bronchocele
Bronchiectasis, mucous-filled
I DIFFERENTIAL DIAGNOSIS Fungus ball
Fibroxanthoma ("inflammatory pseudotumor")
Congenital Histiocytoma
Pulmonary sequestration Plasma cell granuloma
Bronchogenic cyst Sclerosing hemangioma
Foregut cyst Lipoid granuloma ("paraffinoma")
Organizing pneumonia
Inhalational
Conglomerate mass (silicosis) Benign Neoplasms
Mucoid impaction (allergic aspergillosis) Originating from lung parenchyma
o Hamartoma
Vascular
o Adenoma
Hematoma Originating from fat
Hemangioma o Lipoma
Arteriovenous malformation Originating from fibrous tissue
Infarction o Fibroma
Vasculitic nodule Originating from muscle
Pulmonary vein varix o Leiomyoma
Septic emboli Originating from neural tissue
Collagen-Vascular o Neurofibroma
Rheumatoid nodules o Paraganglioma
Wegener granulomatosis o Schwannoma
Wegener variants Originating from lymphoid tissue
o Lymph node, intrapulmonary
Inflam matory Deposits in lung parenchyma
Granuloma o Calcium
o Fungal o Amyloid
Histoplasmosis o Endometrioma
Coccidiomycosis o Splenosis
Cryptococcosis o Hematopoiesis, extramedullary
o Bacterial
Tuberculosis
 FOCAL INCREASED DENSITY LUNG DISEASE
1 Malignant Neoplasms I DIAGNOSTIC CHECKLIST
20
Primary disease Image Interpretation Pearls
o Bronchogenic carcinoma
o Lymphoma Direct contact to vessels
o Suggests vascular or hematogenous origin
o Sarcoma
o Arteriovenous malformation, septic emboli,
o Plasmocytoma
hematogenous spread of metastases
o Carcinoid
Direct contact to bronchi
Secondary disease: Metastases
o Breast o Suggests bronchial or bronchiolar origin
o Bronchogenic infection, bronchogenic carcinoma,
o Colon
infectious bronchiolitis
o Prostate
o Ovary Lobulated borders
o Suggests organizing lesion
o Testes
o Suggests that lesion has multiple cell types growing
o Kidney
with different speed
o Thyroid
o Seen in both benign and malignant lesions
Miscellaneous and Mimics Calcification
Rounded atelectasis o The more complete a lesion is calcified, the more
Interlobar pleural effusion likely the lesion is benign
Pleural mass o Homogeneous central calcification: Granuloma is
o Fibroma likely
o Mesothelioma o Inhomogeneous, diffuse, or amorphous
Mediastinal mass calcification: Can also occur in malignant lesions
o Cyst Spiculation
o Tumor o Suggests aggressive growth
Chest wall structures o Is very common in bronchogenic carcinoma
o Nipple o Bronchogenic carcinoma must be excluded
o Skin tumor Growth
Artifacts o Slow or absent growth
o Buttons Granuloma, hamartoma, inflammatory
o ECG-Electrodes pseudotumor, low grade adenocarcinoma,
rounded atelectasis
o Rapid growth
I CLINICAL ISSUES Bronchogenic carcinoma, organizing pneumonia,
bronchial carcinoid, infarction, Wegener
Presentation granulomatosis, metastases from seminoma,
Most common signs/symptoms choriocarcinoma, osteosarcoma
o Depends upon disorder o Decrease in size over time: High likelihood for
o History and presentation important for diagnosis benign lesion (exception: Cavitary malignant lesions
Age may temporarily collapse)
Previous disease o Lesions with "doubling time" :s 4 weeks and", 16
Systemic disorder months have low likelihood for malignancy
Professional exposure o Caveat: "Doubling time" does not refer to diameter
Environmental exposure of lesions, but to their volume
Smoking history o If a lesion doubles in diameter, its volume increases
Immunologic status by 2"
Extrathoracic malignancy Example: If a lesion increases in diameter from 4
Cough mm to 5 mm, its volume has doubled!
Fever
Sputum production
Hemoptysis I SELECTED REFERENCES
Weight loss I. RyuJH et al: Cystic and cavitary lung diseases: focal and
diffuse. Mayo Clin Proc. 78(6):744-52,2003
Demographics Oikonomou A et al: Organizing pneumonia: the many
2.
Age: Any age morphological faces. Eur Radiol. 12(6):1486-96,2002
Gender: No gender predilection 3. Shady Ket al: CT of focal pulmonary masses in childhood.
Radiographies. 12(3):505-14, 1992
Natural History & Prognosis 4. Naidich DP et al: Radiographic evaluation of focal lung
Depends upon disorder disease. Clin Chest Med. 12(1):77-95, 1991
5. Templeton PAet al: High-resolution computed
Treatment tomography of focal lung disease. Semin Roentgenol.
Depends upon disorder 26(2):]43-50, ]99]
6. Woodring JH: Unusual radiographic manifestations of lung
cancer. Radiol Clin North Am. 28(3):599-618, ] 990
 FOCAL INCREASED DENSITY LUNG DISEASE
I IMAGE GALLERY 1
21

(Left) Coronal CECT shows

pulmonary sequestration at
base of right lung (arrows).
Lesion contains fat and
calcified foci. Lesion has
interphase with lung
parenchyma, mediastinum
and spine. (Right) Coronal
CECTshowsrounded
atelectasis (open arrow).
Lesion has interface with
lung parenchyma and
thickened pleura. Lesion
shows characteristic
comet-tail-like bands
(arrows).

(Left) Coronal CECT shows

oval-shaped focal increased
density (arrow) with vascular
contact (curved arrow)
corresponding to an
arteriovenous malformation
of the right lower lobe.
(Right) Coronal CECT shows
focal increased density
(arrow) corresponding to a
"Hampton hump" after
pulmonary embolism. Lesion
has well-defined interface
with pleura and ill-defined
interface with lung.

(Left) Axial CECT shows

focal increased densities
corresponding to
cryptogenic organizing
pneumonia (arrows).
Densities are peripheral,
ill-defined and show
occasional air
bronchograms. (Right) Axial
CECT shows focal increased
densities (arrows)
corresponding to metastases
from seminoma. Lesions are
completely surrounded by
lung parenchyma and have
well-defined borders.
 FOCAL DECREASED DENSITY LUNG DISEASE

22

Axial HRCT shows focal decreased density from a cavity Axial H RCT shows focal decreased densities due to
following aspergillus infection (arrow). Note centrilobular emphysema (arrows). Lesions are
ground-glass halo surrounding cavity (curved arrows). surrounded by normal lung parenchyma. Cough
induced subcutaneous emphysema (open arrows).

Protocol advice
I TERMI NOlOGY o Volumetric CT performed to assess profusion of
Definitions lesions
Area with markedly decreased attenuation o HRCT performed to assess subtle details and relation
Partly or entirely surrounded by lung parenchyma of to constituents of secondary pulmonary lobule
normal or increased attenuation o Expiratory HRCT for assessment of potential
Always localized, never diffuse air-trapping
Either solitary or multiple
To be differentiated against
a Extrapulmonary but intrathoracic air accumulations I DIFFERENTIALDIAGNOSIS
(pneumothorax)
Miscellaneous and Artifacts
o Air in pathological bronchi (bronchiectasis)
Overpenetrated chest film
o Air in solid or semisolid structures (abscess)
Rotation of patient
Chest Wall Defect
IIMAGING FINDINGS Asthenic body habitus
General Features Mastectomy
Poland syndrome (absent pectoralis muscle)
Location: Any lobe, although cavities are more
common in the upper lobes Cystic Lesions
Size: Occupies entire range of possible sizes Congenital
o Congenital lobar emphysema
Imaging Recommendations
o Cystic adenomatoid malformation
Best imaging tool: CT o Bronchial atresia

DDx: Focal Decreased Density Lung Disease

Pneumothorax Abscess
 FOCAL DECREASED DENSITY LUNG DISEASE

Key Facts
1
Terminology Top Differential Diagnoses 23
Area with markedly decreased attenuation Chest Wall Defect
Partly or entirely surrounded by lung parenchyma of Cystic Lesions
normal or increased attenuation Emphysematous Lesions
Always localized, never diffuse Cavities
Either solitary or multiple Airways
Imaging Findings Diagnostic Checklist
Best imaging tool: CT Focal decreased density surrounded by normal lung
Volumetric CT performed to assess profusion of parenchyma
lesions Focal decreased density surrounded by lung
HRCT performed to assess subtle details and relation parenchyma of increased density
to constituents of secondary pulmonary lobule Focal decreased density surrounded by pathological
Expiratory HRCT for assessment of potential non-pulmonary parenchyma ("cystic" or "cavitary"
air-trapping disease)

o Bronchogenic cyst Lymphoma

o Sequestration Melanoma
Acquired Osteosarcoma
o Pseudocyst Vascular
o Pneumatocele o Infarction
o Hydatid disease Thromboembolic (rare)
o Bleb Septic
o Bulla o Wegener granulomatosis
o Rheumatoid arthritis
Emphysematous lesions lnhalational
Centrilobular emphysema o Conglomerate mass in silicosis
Pan lobular emphysema
Paraseptal emphysema Vascular
Interstitial emphysema Decreased blood flow caused by
o Pulmonary embolism (Westermark sign)
Cavities o Chronic thromboembolic pulmonary hypertension
Infectious (mosaic perfusion)
o Bacterial o Pulmonary artery hypoplasia
Staphylococcus
Klebsiella Cardiac
Pseudomonas Decreased blood flow caused by
Anaerobes o Right-to-left shunt
Streptococcus, beta-hemolytic o Eisenmenger physiology of left-to-right shunt
Escherichia coli
Airways
Gram-negative organisms
o Aspiration pneumonia Increased pulmonary air content caused by airway
obstruction or compression
Anaerobes
Mixed gram-negative organisms o Bronchial compression
o Granulomatous infection Hilar or mediastinal mass
Tuberculosis Cardiomegaly
o Endobronchial obstruction
Sarcoidosis
Fungal: Nocardiosis, Coccidiomycosis, Foreign body
Histoplasmosis, Blastomycosis, Mucormycosis, Bronchogenic carcinoma
Sporotrichosis, Aspergillosis, Cryptococcosis Bronchial carcinoid
o Parasitic infection o Small airway obstruction
Hydatid disease Infectious pan bronchiolitis
Swyer-James (or Macleod) syndrome
Neoplastic
o Primary tumors o Small airway constriction
Squamous cell carcinoma (up to 30,1, cavitate) Bronchiolitis obliterans (air-trapping)
Bronchoalveolar carcinomas (up to 18% cavitate)
o Metastases
Squamous cell metastases
Adenocarcinoma
 FOCAL DECREASED DENSITY LUNG DISEASE
1 I PATHOLOGY Best seen in gravity-dependent zones of the lung
Best seen on expiratory HRCT sections
24 General Features o Air-trapping, pathologic
Etiology Involves multiple secondary pulmonary lobules
o Bronchial occlusion Not age dependent
Example: Air-trapping following bronchiolitis Indirect sign for more central small airways
obliterans obstruction
o Vascular occlusion Best seen in gravity-dependent zones of the lung
Example: Pulmonary embolism Best seen on expiratory HRCT sections
o Remodeling of lung architecture o Decreased perfusion
Example: Inflammatory and neoplastic disorders Indicative of more central pulmonary arterial
o Disruption of elastic fibre network obstruction
Example: Emphysema Agenesis, atresia, or stenosis
o Unknown and multifactorial mechanisms Compression or infiltration by neoplasm
Example: Langerhans cell histiocytosis Embolic obstruction (pulmonary embolism,
"Westermark sign")
o Centrilobular emphysema
ICLINICAL ISSUES Focal decreased density surrounded by lung
parenchyma of increased density
Presentation o Increased perfusion
Most common signs/symptoms Localized pulmonary edema (e.g., mitral
o Depend upon disorder regurgitation)
o History and presentation important for diagnosis Reactive hyperperfusion (e.g., chronic
Age thromboembolic pulmonary hypertension)
History of previous disease Perivascular abnormalities (e.g., plexiform
Travel history arteriopathy)
Drugs or medical treatment Focal decreased density surrounded by pathological
Systemic disorder non-pulmonary parenchyma ("cystic" or "cavitary"
Immunological status disease)
Smoking history o Lung laceration
Cough o Adenomatoid malformation
Fever o Sequestration
Sputum production o Honeycombing
Hemoptysis o Tracheobronchial papillomatosis
Laboratory test results o Rheumatoid nodules
o Evolution of lesions o Pneumoconiosis
Acute or subacute: Evolve over days or weeks o Sarcoidosis
Suggest infectious or progressive inflammatory o Wegener disease
disorders, or embolic and cardiovascular disorders o Langerhans cell histiocytosis
Chronic: Evolve over months or years o Infarction
Suggests neoplastic or long-standing inflammatory o Pneumatocele
or fibrotic disorders o Pneumocystis jiroveci pneumonia
o Tuberculosis
Demographics o Carcinoma
Age: Any age o Lymphoma
Gender: No gender predilection o Kaposi sarcoma
o Metastases
Natural History & Prognosis
o Inflammatory pseudotumor
Depends upon disorder
Treatment
Depends upon disorder I SELECTED REFERENCES
1. Ryu)H et al: Cystic and cavitary lung diseases: focal and
diffuse. Mayo Clin Proe. 78(6):744-52, 2003
I DIAGNOSTIC CHECKLIST 2. Muller NL et al: Chronic obstructive pulmonary disease. 4:
imaging the lungs in patients with chronic obstructive
Consider pulmonary disease. Thorax. 57(11):982-5, 2002
3. Worthy 5A et al: Mosaic attenuation pattern on
Focal decreased density surrounded by normal lung
thin-section CT scans of the lung: differentiation among
parenchyma infiltrative lung, airway, and vascular diseases as a cause.
o Air-trapping, physiologic Radiology. 205(2):465-70, 1997
Restricted to single secondary pulmonary lobule 4. Teel G5 et al: Imaging of small airways disease.
Increases with age Radiographies. 16(1):27-41, 1996
Depends from smoking habits (more smoking> 5. Thurlbeck WM et al: Emphysema: definition, imaging, and
more air-trapping) quantification. A)R Am) Roentgenol. 163(5): 1017-25, 1994
 FOCAL DECREASED DENSITY LUNG DISEASE
I IMAGE GALLERY 1
25

(Left) Axial HRCT in

expiration shows focal
decreased density
corresponding to air-trapping
(arrows) in bronchiolitis
obliterans. Normal lung
(open arrows) increases in
density during expiration.
(Right) Axial CECT in
inspiration shows focal
decreased density in chronic
thromboembolic disease
(arrows). Note paucity of
vascular structures as
compared to norma/lung
(open arrows).

(Left) Axial CECT shows

focal decreased density
caused by a cavity in a
patient with tuberculosis
(arrows). Note irregular
borders of remodeled lung
parenchyma constituting the
cavity wall. (Right) Axial
CECT shows multiple focal
decreased densities caused
by post-infectious
pneumatoceles in an
intravenous drug abuser
(arrows). Note the size and
density variation of lesions.

Typical
(Left) Axial CECT shows
solitary focal decreased
density caused by Candida
infection (arrow) before
(left) and after (right)
treatment. Decreased density
disappears but ground-glass
persists. (Right) Axial CECT
shows large focal decreased
density caused by excavating
Wegener disease (arrows).
Note irregular borders of
lesion and air-fluid level
within cavity.
 MULTIPLE PULMONARY NODULES
1
26

Axial CECT shows well-circumscribed, bilateral lung Axial CECT shows a fine pattern of small, rounded
nodules (arrows) of varying size from c%n carcinoma. upper lobe nodules in a sandblaster who developed
Many nodules abut pleural surface, a typical location for silicosis. Oval mass in left upper lobe (arrow) represents
hematogenous metastases. conglomerate fibrosis.

o Lower lobe predominance: Asbestosis,

I TERMINOlOGY hematogenous metastases
Definitions o Bronchocentric: Sarcoid, Kaposi sarcoma
Size
Nodule: Round opacity, at least moderately
well-marginated & s 3 cm in maximum diameter o Maximum diameter s 3 cm
o Many conditions that cause lung nodules also cause
lung masses> 3 cm in diameter
o Metastatic lung nodules: Commonly variable in size
I IMAGING FINDINGS Morphology
General Features o Miliary: Tuberculosis, histoplasmosis, silicosis,
Best diagnostic clue coal-worker pneumoconiosis, sarcoidosis, metastases
o Acute angle sign (thyroid & melanoma), varicella pneumonia
When a lung nodule contacts pleura, surrounding o Cavitating: Septic emboli, metastases (squamous,
lung usually forms an acute angle sarcoma), infection (staphylococcus, fungal,
o Tapered margin sign tuberculosis), Wegener granulomatosis, rheumatoid
When lesion arises from pleura, chest wall or o Calcified: Tuberculosis, histoplasmosis, metastases
mediastinum, surrounding lung usually forms an Calcified metastases: Osteosarcoma,
obtuse angle chondrosarcoma, papillary thyroid, breast, ovary,
o Lung nodule: Usually either completely outlined by mucinous adenocarcinoma
lung or surrounding lung forms an acute angle o Ill-defined: Hemorrhagic metastases (before or after
Location treatment), Wegener granulomatosis, infection
o Upper lobe predominance: Silicosis, coal-worker o Clustered small nodules suggest benign etiology
pneumoconiosis, sarcoid Do not confuse with primary malignancy &
satellite nodules

DDx: Multiple lung Nodules

Rib Fractures Pleural Metastases Neurofibromatosis

 MULTIPLE PULMONARY NODULES

Key Facts
1
Imaging Findings Pneumoconioses 27
Lung nodule: Usually either completely outlined by Autoimmune Disease
lung or surrounding lung forms an acute angle Congenital
Clustered small nodules suggest benign etiology Pseudonodules
Stability of lung nodules for two years is a very strong Pathology
indicator of benignity Etiology: Metastases: Most common cause of multiple
Sharper borders of lung nodules usually distinguish lung nodules
them from foci of consolidation
CT helpful for detection of lung nodules < 1 em & Clinical Issues
differential diagnosis of complex disease In many cases cause for lung nodules is apparent
Contrast material may be unnecessary for CT, if only from clinical data & treatment can be instituted
indication is detection or follow-up of lung nodules In some cases patient's risk profile is low & nodules
are followed by radiography or CT
Top Differential Diagnoses Lung nodules can be biopsied via fluoroscopy, CT,
Malignancy bronchoscopy, thoracoscopy or open-lung biopsy
Infection
Sarcoid

Growth rate
o Rapid evolution favors benign diagnoses such as
I DIFFERENTIAL DIAGNOSIS
infection, septic emboli or vasculitis Malignancy
o Slow, steady growth favors hematogenous Hematogenous metastases
metastases a Melanoma, sarcoma, renal, thyroid, gastrointestinal,
o Improvement or variable changes favor infection, breast, testis, ovary
vasculitis or treated hematogenous metastases Diagnosis often evident in overall clinical &
o Longstanding stability favors infectious radiographic context
granulomatous disease, sarcoid & pneumoconiosis If diagnosis remains unclear, biopsy necessary
o Stability of lung nodules for two years is a very Bronchioloalveolar carcinoma
strong indicator of benignity a Often multifocal, slow-growing, can have air
Radiographic Findings bronchograms
Sharper borders of lung nodules usually distinguish Lymphoma
a Infiltrating, ill-defined, can have air bronchograms
them from foci of consolidation
Air bronchograms: Unusual in lung nodules, but often Posttransplant Iymphoproliferative disease
a Clinical context of immune suppression after
present in consolidation
transplant
CT Findings a Diagnosis complicated by possible infection in
Uniform, central or target calcification: Nearly always immune-suppressed patient
benign
Infection
a Calcified metastases: Rare exception
Fat in nodules indicates benignity (hamartomas) Septic emboli
a Blood or central line culture, cardiac ultrasound for
Nuclear Medicine Findings valvular vegetations
FOG-PET Fungal disease: Histoplasmosis, aspergillosis,
a Limited for evaluating nodules < 8 mm coccidioidomycosis, cryptococcosis
a Bronchioloalveolar carcinoma can be false negative a Antibody titer, blood or sputum culture, bronchial
a Marked uptake in lung nodules: Highly suspicious washings, open lung biopsy
for malignancy Tuberculosis: Typical & atypical
a Sputum smear & culture, bronchial washings
Imaging Recommendations
Best imaging tool Granulomatous Disease
a Chest radiography good for initial detection & Sarcoid
follow-up of most lung nodules", 1 em a Biopsy of involved organs, including endobronchial
a CT helpful for detection of lung nodules < 1 em & biopsy
differential diagnosis of complex disease
Pneumoconioses
Protocol advice
a Contrast material may be unnecessary for CT, if only Silicosis, coal-worker pneumoconiosis, asbestosis
a Work history, pleural plaques suggest asbestosis
indication is detection or follow-up of lung nodules
a Surrounding lung provides sufficient contrast for Autoimmune Disease
visualizing lung nodules Rheumatoid nodules
a History of rheumatoid arthritis, males> females
 MULTIPLE PULMONARY NODULES
1 Wegener granulomatosis
o Sinus & renal disease, nodules often cavitate
28
Congenital
Arteriovenous malformations
o Digital subtraction angiography or CT to confirm
vascular nodules
Multiple pulmonary hamartomas
oCT: Popcorn calcifications or fat
Pseudonodules
Skin lesions: Moles, neurofibromatosis
o Differentiate by physical exam, radiopaque skin
marker, Mach effect
Bone lesions: Rib fractures, metastases, bone islands
o Use low kVp bone radiographs with various
projections to confirm
Pleural plaques or pleural metastases
o Oblique radiographs or CT to confirm
I PATHOLOGY
General Features
Etiology: Metastases: Most common cause of multiple
lung nodules
Associated abnormalities
o Obstructive atelectasis
o Spread of tumor or infection into adjacent tissues
o Cavitating nodules: Pneumothorax
Microscopic Features
Metastases - focal collection of malignant cells remote
from primary tumor
Bronchioloalveolar cell carcinoma: Well-differentiated
adenocarcinoma, lepidic growth
Infection: Abscess
Sarcoidosis: Noncaseating granulomas, must exclude
other possible causes such as infection
Pneumoconioses
o Coal-worker pneumoconiosis: Coal macule
o Silicosis: Silicotic nodule
o Asbestosis: Asbestos body
Rheumatoid nodules: Necrobiotic lung nodule
Arteriovenous malformation: Focal shunting of
arterioles directly into venules
Hamartoma: Poorly organized cartilage, myxomatous
tissue, fat & epithelial elements
I CLINICAL ISSUES
Presentation
Other signs/symptoms
o Metastases: Dyspnea, hemoptysis, fever
o Infection: Fever, dyspnea, hemoptysis, elevated
white blood cell count
o Sarcoidosis: Dyspnea, cough, chest pain
o Pneumoconioses: Dyspnea, cough, chest pain
o Multiple arteriovenous malformations: Transient
ischemic attack, brain abscess, cyanosis
o Multiple pulmonary hamartomas: Endobronchial
hamartomas can cause obstruction
Clinical Profile
o Lemierre syndrome: Septic emboli, pharyngitis,
septic thrombosis of internal jugular vein
o Caplan syndrome: Necrobiotic nodules, rheumatoid
arthritis, coal or silica dust exposure
o Osler-Weber-Rendu syndrome: Multiple
arteriovenous malformations, telangiectasias,
epistaxis, autosomal dominant
o Carney syndrome: Lung chondromas, gastric
epithelioid leiomyosarcoma & extra-adrenal
paraganglioma
Management of multiple lung nodules
o Extremely complex problem involving multiple
criteria, including
Age, history of malignancy, history of smoking,
constitutional symptoms, imaging findings &
laboratory tests
o Relevant imaging findings include
Number, size, morphology & rate of growth of
lung nodules
o Constellation of clinical data determines patient's
risk profile
Management options
o In many cases cause for lung nodules is apparent
from clinical data & treatment can be instituted
o In some cases patient's risk profile is low & nodules
are followed by radiography or CT
o If nodules are ~ 8 mm, PET imaging can be used to
determine likelihood of malignancy
Options for pathological analysis
o If pathological analysis is necessary, tissue is
obtained from most accessible location
Lung nodules may not be most accessible
suspicious lesions
o Lung nodules can be biopsied via fluoroscopy, CT,
bronchoscopy, thoracoscopy or open-lung biopsy
o Biopsy material can be sent for gram stain, culture or
histologic analysis, as appropriate
Treatment
Hematogenous metastases: Chemotherapy
Selected cases of metastatic disease: Nodule resection
Septic emboli; infections with bacteria, fungi or
tuberculosis: Antibiotics
Pneumoconiosis: Removal from exposure, supportive
measures
Arteriovenous malformations: Embolization
I SELECTED REFERENCES
1. MacMahon H et al: Guidelines for management of small
pulmonary nodules detected on CT scans: a statement
from the Fleischner Society. Radiology. 237(2):395-400,
2005
2. Austin)H et al: Glossary of terms for CT of the lungs:
recommendations of the Nomenclature Committee of the
Fleischner Society. Radiology. 200(2):327-31, 1996
3. Lillington GA et al: Evaluation and management of solitary
and multiple pulmonary nodules. Clin Chest Med.
14(1):111-9,1993
4. Viggiano RW et al: Evaluation and management of solitary
and multiple pulmonary nodules. Clin Chest Med.
13(1):83-95,1992
 MULTIPLE PULMONARY NODULES

I IMAGE GALLERY 1
29

(Left) Coronal OSA after

injection in right pulmonary
artery shows multiple highly
vascular lung nodules
(arrows) and embolization
coils (curved arrows) in a
woman with
Osler-Weber-Rendu
syndrome. (Right) Frontal
radiograph shows multiple,
bilaterallung nodules in a
patient with Wegener
granulomatosis. Severa/lung
nodules have thick-walled
cavities (arrows).

Typical
aeft)Ax~/CECTshows
irregular, ill-defined, fine
nodularity (arrows) along
bronchovascular bundles in
patient with sarcoidosis.
(Right) Axial CECT
demonstrates a fine, diffuse,
miliary pattern of lung
nodules with a greater
profusion in dependent lung.
Muscle abscess culture was
positive for Mycobacterium
tuberculosis.

Variant
(Left) Frontal radiograph
magnification view shows
numerous, sma/J, calcified
lung nodules (arrows), likely
from old granulomatous
disease. Calcified hilar lymph
nodes (open arrows) are
visible. (Right) Axial CECT
shows several centrilobular
nodules (arrows) in
periphery of right lung in
woman with Mycobacterium
avium-intracellulare. Middle
lobe bronchiectasis (open
arrows) is also present.
 CYSTIC LUNG DISEASE

30

Axial HRCT shows bronchiectasis in the right lung. Axial CECT shows posttraumatic patient with lung
Border of cystic structures (arrows) is constituted by laceration (arrows). Borders of cystic structure is
bronchial tissue. constituted by lung parenchyma. Note pathological air
collections in pleura and chest wall.

o Congenital
I TERMI NOLOGY Bronchogenic cyst
Abbreviations and Synonyms Adenomatoid malformation
Descriptors for lesions can have overlapping meanings o Infections
and are often used interchangeably Coccidiomycosis
Pneumocystis jiroveci
Definitions Hydatid disease
Solitary or multiple foci of decreased or absent lung o Traumatic cysts
density with definable walls of various thickness Cavitary (wall thickness> 4 mm)
Are commonly encountered lesions on chest o Neoplastic
radiographs and CT Bronchogenic carcinoma
Differential diagnosis is broad because many different Metastasis
processes of congenital and acquired origin can cause Lymphoma
these abnormalities o Infections
Bacteria: Staphylococcus aureus, gram-negative
bacteria, Pneumococcus, Mycobacteria,
I DIFFERENTIAL DIAGNOSIS Melioidosis, Anaerobes, Actinomycosis,
Nocardiosis
Focal and Multifocal Cystic and Cavitary Fungi: Histoplasmosis, Coccidiomycosis,
Disease Blastomycosis, Aspergillosis, Mucormycosis,
Cystic (wall thickness < 4 mm) Cryptococcosis, Pneumocystis jiroveci,
o Blebs Sporotrichosis
o Bullae Parasites: Hydatid disease, Paragonimiasis,
o Pneumatoceles Amebiasis

DDx: Cystic Lung Disease

Wegener Granulomatosis Squamous Cell Carcinoma

 CYSTIC LUNG DISEASE

Key Facts
1
Terminology Langerhans cell histiocytosis 31
Solitary or multiple foci of decreased or absent lung Honeycomb lung
density with definable walls of various thickness Sarcoidosis, advanced
Are commonly encountered lesions on chest Pathology
radiographs and CT Vascular occlusion with ischemic necrosis
Differential diagnosis is broad because many different Dilatation of bronchi
processes of congenital and acquired origin can cause Disruption of elastic fiber network
these abnormalities Remodeling of lung architecture
Top Differential Diagnoses Unknown and multifactorial mechanisms
Congenital Clinical Issues
Infections Clinical context is crucial for accurate diagnosis
Neoplastic
Immunologic Diagnostic Checklist
Thromboembolism Complement HRCT by volumetric CT to assess
Progressive massive fibrosis (Pneumoconiosis) profusion of disease
Pulmonary Iymphangioleiomyomatosis

o Immunologic o Infarction
Wegener granulomatosis o Pneumatocele
Rheumatoid nodule o Pneumocystis jiroveci pneumonia
o Thromboembolism Granulation tissue
o Septic embolism o Granuloma
o Progressive massive fibrosis (Pneumoconiosis) o Abscess
o Bronchiectasis, localized o Tuberculosis
o Congenital o Fungal
Sequestration o Parasitic
Adenomatoid malformation Malignant tissue
o Carcinoma
Diffuse Cystic and Cavitary Disease o Lymphoma
Pulmonary lymphangioleiomyomatosis o Kaposi Sarcoma
Langerhans cell histiocytosis o Metastasis
Honeycomb lung Other tissues
o Idiopathic pulmonary fibrosis o Inflammatory pseudotumor
o Connective tissue disease o Postinterventional
o Asbestosis o Postsurgical
o Chronic hypersensitivity pneumonitis
Sarcoidosis, advanced
Bronchiectasis, diffuse I PATHOLOGY
Metastasis
General Features
Classification According to Parenchyma that
Etiology
Delimits "Cystic" or "Cavitary" lesions o Vascular occlusion with ischemic necrosis
Lung tissue (normal and abnormal) Example: Emboli with infarction
o Bulla o Dilatation of bronchi
o Traumatic lung cyst Example: Bronchiectasis
o Emphysema o Disruption of elastic fiber network
o Adenomatoid malformation Example: Emphysema
Bronchial tissue (normal and abnormal) o Remodeling of lung architecture
o Bronchiectasis Example: Idiopathic pulmonary fibrosis,
o Sequestration honeycombing
o Honeycombing o Unknown and multifactorial mechanisms
o Tracheobronchial papillomatosis Example: Langerhans cell histiocytosis,
Inflammatory or fibrotic tissue tracheobronchial papillomatosis
o Rheumatoid nodule
o Polyarteritis nodosa Gross Pathologic & Surgical Features
o Pneumoconiosis Parameters to consider
o Sarcoidosis o Solitary or multiple
o Wegener granuloma o Location
o Langerhans cell histiocytosis Central: More common in fibrotic and neoplastic
lesions
 CYSTIC LUNG DISEASE
1 Peripheral:
and metastatic
More common
lesions
in embolic, infectious, o Moderate in neoplastic
o Mild to absent in other lesions
lesions

32 o Thickness of cavity wall Irregularity of border

Thin wall: Common in congenital and benign o Marked irregularity in inflammatory and
lesions granulomatous lesions
Thick walls: Common in inflammatory, fibrotic, o Moderate irregularity in neoplastic lesions
granulomatous, and neoplastic disease o Mild to absent irregularity in other lesions
o Morphology of cavity wall Thickness of wall
Smooth and regular in congenital and benign o Extensive thickness in inflammatory and
lesions granulomatous lesions
Irregular and ill-defined in infectious, o Moderate thickness in neoplastic lesions
granulomatous, and malignant lesions o Mild thickness in other lesions
o Inner contour of cavity
Usually nodular or irregular in malignant lesions
Usually shaggy in inflammatory, granulomatous, I SELECTED REFERENCES
and fibrotic lesions 1. Abbott GF et al: From the archives of the AFlP: pulmonary
Usually smooth in other lesions Langerhans cell histiocytosis. Radiographics. 24(3):821-41,
o Nature of content 2004
Fluid: Common in congenital, inflammatory, and 2. Castaner E et al: Radiologic approach to the diagnosis of
parasitic lesions infectious pulmonary diseases in patients infected with the
Solid: Common in parasitic and neoplastic lesions human immunodeficiency virus. Eur J Radiol.
51(2):114-29,2004
3. Ryu JH et al: Cystic and cavitary lung diseases: focal and
diffuse. Mayo Clin Proc. 78(6):744-52, 2003
IClINICAllSSUES 4. Sundar KM et al: Pulmonary Langerhans cell histiocytosis:
emerging concepts in pathobiology, radiology, and clinical
Presentation evolution of disease. Chest. 123(5):1673-83,2003
Most common signs/symptoms 5. Vourtsi A et al: CT appearance of solitary and multiple
o Clinical context is crucial for accurate diagnosis cystic and cavitary lung lesions. Eur Radiol. 11(4):612-22,
o Parameters to consider are 2001
6. Pedrosa I et al: Hydatid disease: radiologic and pathologic
Age
features and complications. Radiographics. 20(3):795-817,
Gender
2000
Smoking history
7. Crans CA Jr et al: Imaging features of Pneumocystis carinii
Immune status pneumonia. Crit Rev Diagn Imaging. 40(4):251-84, 1999
Underlying pulmonary or systemic diseases 8. Washington Let al: Mycobacterial infection in
Drugs or medical treatment immunocompromised patients. J Thorac Imaging.
Environmental exposure 13(4):271-81,1998
Recent trauma 9. Gallant JE et al: Cavitary pulmonary lesions in patients
Travel history infected with human immunodeficiency virus. Clin Infect
Laboratory test results Dis. 22(4):671-82,1996
10. Ip M et al: Pulmonary melioidosis. Chest. 108(5):1420-4,
o Tempo of the disease process
1995
Acute and subacute processes that evolve over 11. McAdams HP et al: Radiologic manifestations of
days and weeks suggest infectious, progressive pulmonary tuberculosis. Radiol Clin North Am.
inflammatory disorders, cardiovascular, embolic, 33(4):655-78,1995
or traumatic causes 12. Kuhlman JE et al: Abnormal air-filled spaces in the lung.
Chronic processes suggest neoplastic Radiographics. 13(1):47-75, 1993
long-standing inflammatory or fibrotic disorders, 13. Grum CM et al: Chest radiographic findings in cystic
and congenital lesions fibrosis. Semin Respir Infect. 7(3):193-209, 1992
14. Buckner CB et al: Radiologic manifestations of adult
tuberculosis. J Thorac Imaging. 5(2):28-37, 1990
15. Davies SF: Histoplasmosis: update 1989. Semin Respir
I DIAGNOSTIC CHECKLIST Infect. 5(2):93-104,1990
16. Goodman PC: Pulmonary tuberculosis in patients with
Consider acquired immunodeficiency syndrome.] Thorac Imaging.
Complement HRCT by volumetric CT to assess 5(2):38-45, 1990
profusion of disease 17. Woodring]H et al: Pulmonary disease caused by
Rule of thumb that might be helpful in differential nontuberculous mycobacteria.] Thorac Imaging.
diagnosis of many cystic and cavitary lesions relies on 5(2):64-76,1990
18. Pennza PT: Aspiration pneumonia, necrotizing pneumonia,
consideration of
and lung abscess. Emerg Med Clin North Am. 7(2):279-307,
o Perilesional reaction
1989
o Irregularity of border 19. Weisbrod GL: Pulmonary angiitis and granulomatosis: a
o Thickness of wall review. Can Assoc Radiol j. 40(3):127-34, 1989
Perilesional reaction 20. Gross BH et al: CT of solitary cavitary infiltrates. 5emin
o Strongest in inflammatory and granulomatous Roentgenol. 19(3):236-42, 1984
lesions
 CYSTIC LUNG DISEASE
I IMAGE GALLERY 1
33

(Left) Axial CECT shows

intralobar lung sequestration
in right lower lobe (open
arrows). Cystic component
of lesion (arrow) is
constituted by
undifferentiated bronchial
epithelium. (Right) Axial
CECT shows cavitated
pulmonary inFarction
(arrows) after recurrent
pulmonary embolism.
Borders of cystic lesion are
constituted by fibrotic tissue.

(Left) Axial H RCT shows

necrotizing pneumonia
caused by Staphylococcus
aureus. Borders of cystic
lesion (arrows) are
constituted by inflammatory
tissue. (Right) Axial CECT
shows tuberculous cavity in
the right upper lobe. Borders
of cavitation (arrows) are
constituted by
granulomatous tissue. Note
numerous satellite lesions.

Typical
(Left) Axial HRCT shows
excavated mass in
sarcoidosis. Borders of lesion
(arrows) are constituted by
fibrous tissue. (Right) Axial
CECT shows excavated mass
in sificosis. Borders of lesion
(arrows) are constituted by
fibrous tissue.
 HRCT: BRONCHOCENTRIC PATTERN
1
34

Graphic shows structure of SPL. Core bronchovascular Axial HRCT shows multiple small centrilobular nodules
bundle (arrows). Centrilobular regions (open arrows). (open arrows) and ground-glass opacities with relaUve
Bronchocentric signs include mosaic perfusion, sparing of the lung periphery. Hypersensitivity
centrilobular nodules, holes, and cysts. pneumonitis.

o Small holes or cysts due to destruction or

I TERMI NOLOGY
Definitions
Location
Predominant radiographic abnormality found along
the distribution of small airways in the secondary
pulmonary lobule (SPL)
SPL: Smallest unit of lung structure marginated by
interlobular septa
I IMAGING FINDINGS
Morphology
General Features
Best diagnostic clue
o Centrilobular micronodules 5 mm in diameter)
within the secondary pulmonary lobule
Peripheral subpleural lung spared (if involved -+
lymphatic pattern)
o Tree-in-bud opacities: Small tubular or branching
"V" or "Y"opacities within the SPL
o Mosaic perfusion: Geographic sharply defined
regions of increased and decreased lung attenuation
obstruction of small bronchioles or cavitation of
nodules
o Acinus includes all structures distal to end terminal
bronchiole (normal adult lung has approximately
30,000 terminal bronchioles)
o SPL composed of 5-15 acini
o Distance from smallest terminal bronchiole (acinus)
to the interlobular septa, pulmonary vein, or pleura
relatively constant 2.5 mm
o Terminal bronchioles supply secondary pulmonary
lobule
Airways branch in asymmetric dichotomous
pattern (parent branch into 2 daughter branches
of unequal diameter and length)
9-14 generations from 15 mm diameter trachea to
1 mm terminal bronchiole
HRCT able to visualize normal airways with
diameters> 2 mm in diameter (larger terminal
bronchioles)

DDx: Bronchocentric Signs

Mosaic Perfusion Centrilobular Nodules & Cysts

 HRCT: BRONCHOCENTRIC
Key Facts
Terminology
Predominant radiographic abnormality found along
the distribution of small airways in the secondary
pulmonary lobule (SPL)
Imaging Findings
Centrilobular micronodules 5 mm in diameter)
within the secondary pulmonary lobule
Peripheral subpleural lung spared (if involved ...
lymphatic pattern)
Tree-in-bud opacities: Small tubular or branching "V"
or "Y"opacities within the SPL
Mosaic perfusion: Geographic sharply defined regions
of increased and decreased lung attenuation
Top Differential Diagnoses
Infectious Bronchiolitis
Radiographic Findings
Difficult to recognize: Nonspecific nodular or reticular
pattern due to summation of small nodules
Air-trapping difficult to recognize unless severe
o Lung density is 90% air, slight increases difficult to
recognize above normal
Sensitivity for some disease processes very poor
o < 10% for hypersensitivity pneumonitis
o < 10% for respiratory bronchiolitis
CT Findings
HRCT
o Bronchovascular bundle
Thickening normal paper thin bronchioles
Visualization of airways within 2 cm of the pleura
(normally below the limits of resolution of CT)
o Centrilobular nodules
Centrilobular nodules without subpleural or
peripheral lymphatic involvement
o Tree-in-bud
"Y"or "V" shaped branching tubular opacities
within the SPL
o Mosaic perfusion or attenuation
Sharply marginated regions of increased and
decreased lung attenuation in a variable
distribution
Vessels in hypoattenuated region smaller than
corresponding vessels in higher attenuated regions
Lung attenuation accentuated in expiration
o Small holes or cysts
Holes with no walls: Destruction of respiratory
bronchioles due to emphysema
Holes with thin walls: Pneumatoceles or cavitated
nodules
Imaging Recommendations
Best imaging tool
o HRCT at full inspiration and expiration
Lobular air-trapping can be identified in 60% of
normals (rule of thumb: No more than 1
hyperinflated 10buie/CT section)
PATTERN
1
Tuberculosis 35
Hypersensitivity Pneumonitis
Langerhans Cell Granulomatosis
Respiratory Bronchiolitis
Coal Worker's Pneumoconiosis
Bronchiolitis Obliterans
Centrilobular Emphysema
Aspiration
Laryngeal Papillomatosis
Clinical Issues
Depending on the pathologic response: Inhalational
diseases may start as a bronchocentric pattern (and
remain so) or evolve into a lymphatic pattern as
particulate material removed from the lung
o HRCT slab with maximum intensity projection
(MIP) or minimum intensity projection (minIP)
useful for micro nodular disease
I DIFFERENTIAL DIAGNOSIS
Infectious Bronchiolitis
Many types of acute lung infections, typically viral
and mycoplasma
Histologic lesion: Inflammatory cells thickening small
bronchioles
Tuberculosis
Mycobacterium tuberculosis: Tree-in-bud pattern
common with endobronchial spread
Mycobacterium avium complex (MAC): Centrilobular
nodules scattered throughout the lung associated with
ventral bronchiectasis in the right middle lobe and or
lingula
Hypersensitivity Pneumonitis
Acute, sub-acute or chronic allergic reaction in the
small airways
Diffuse ground-glass opacities or centrilobular nodules
associated with air-trapping in SPLs
Check for history of exposures
Histologic lesion: Loosely formed granulomas in the
small airways
Langerhans Cell Granulomatosis
Smoking related disease (probably allergic reaction to
component of cigarette smoke)
Centrilobular nodules evolve into thin-walled cysts
and then aggregate into bizarre shapes
More profuse and severe in upper lung zones, typically
spares costophrenic angles
Histologic lesion: Granulomas contain Langerhans cell
(normal antigen processing cell found in the lung)
Respiratory Bronchiolitis
Universal histologic pattern in smokers (also seen in
others living or working in dusty environments)
Faint centrilobular nodules in upper lung zones
 HRCT: BRONCHOCENTRIC
1 Radiographic abnormalities tend to be subtle unless
smoking history severe
36 May be precursor to centrilobular emphysema
Histologic lesion: Pigmented macrophages clustered
around 2nd order respiratory bronchioles
Coal Worker's Pneumoconiosis
Occupation history (chronic dust exposure)
Centrilobular nodules more profuse in upper lung
zones, especially the right upper lung
Histologic lesion: Coal macule centered in 2nd order
respiratory bronchiole
Bronchiolitis Obliterans
Nonspecific reaction from insults to the small airways:
Idiopathic, postinfectious, in halation aI injury,
connective tissue disease, transplant rejection, drug
toxicity
Predominant finding air-trapping at HRCT
Histologic lesion: Concentric luminal narrowing of the
membranous and respiratory bronchioles secondary to
peri bronchiolar inflammation and fibrosis without
intraluminal granulation tissue and polyps
Centrilobular Emphysema
Sequelae of long term smoking (both dose and time
related)
Punched out holes within the SPL more profuse and
severe in the upper lung zones
Precursor may be respiratory bronchiolitis
Histologic lesion: Enlargement and destruction of
respiratory bronchioles
Aspiration
Predisposing factors: Alcoholism, loss of
consciousness, structural abnormalities of the pharynx
and esophagus, neuromuscular disorders
Gravity-dependent opacities
o Recumbent position: Superior segments of the lower
lobes and posterior segments of the upper lobes
o Upright position: Basal segments of the lower lobes
Lentil aspiration pneumonia: Granulomatous
pneumonitis caused by aspiration of leguminous
material such as lentils, beans, and peas
o Centrilobular nodules or tubular branching
structures ("tree-in-bud")
Laryngeal Papillomatosis
Laryngeal nodules due to human papilloma virus
Bronchogenic spread down the airways forming
centrilobular nodules and cysts along the airways
Predominant dorsal distribution (gravity seeding)
I PATHOLOGY
General Features
Etiology
o For inhaled particulate disease, concentration of
particles reflects the distribution of disease
Small round particles are engulfed by
macro phages and removed by lymphatics
Inhalational diseases may begin as bronchocentric
pattern and evolve into lymphatic pattern
PATTERN
Physiology of particulate inhalation
o Small airways (physiologically defined as those < 2
mm in diameter) account for 25% of total airway
resistance
Diseases primarily affecting the small airways may
be severe before impairing pulmonary function
o Particles < 5 I.lin aerodynamic diameter escape
turbulent airflow of the central airways to reach the
SPL
o Particles settle out due to gravity predilectively in
the 2nd generation respiratory bronchioles
Gross Pathologic & Surgical Features
Secondary pulmonary lobule
o Core structures: Terminal bronchioles, arteries,
lymphatics
o Peripheral structures: Septa, veins, and lymphatics
Bronchovascular bundle (core structures)
o Arteries paired with bronchi (pulmonary veins run
in periphery of the lobule)
o Terminal bronchioles supply SPL
Bronchioles lack cartilage but contain cilia and
smooth muscle
I CLINICAL ISSUES
Presentation
Most common signs/symptoms: Nonspecific: Dyspnea,
cough +/- response to bronchodilators, wheezing
Other signs/symptoms: Extent of air-trapping on
expiratory CT correlates best with physiologic
impairment, however, diseases of the small airways
may be extensive and relatively severe before overall
lung function impaired
Natural History & Prognosis
Depending on the pathologic response: Inhalational
diseases may start as a bronchocentric pattern (and
remain so) or evolve into a lymphatic pattern as
particulate material removed from the lung
I DIAGNOSTIC CHECKLIST
Consider
Bronchocentric pattern can sometimes be Simulated
by angiocentric pathology (which is rare)
Image Interpretation Pearls
Centrilobular nodules in bronchocentric pattern
differentiated from lymphatic pattern by sparing of
the subpleural lung and fissures
I SELECTED REFERENCES
1. Gruden JF et al: Multinodular disease: anatomic
localization at thin-section CT--multireader evaiuation of a
simple algorithm. Radiology. 210(3):711-20, 1999
2. Colby TV et al: Anatomic distribution and histopathologic
patterns in diffuse lung disease: correlation with HRCT.J
Thorac Imaging. 11(1):1-26, 1996
 HRCT: BRONCHOCENTRIC PATTERN

I IMAGE GALLERY 1
37

(Left) Axial HRCT shows a

few small branching
opacities (arrows) that are
otherwise difficult to
characterize. (Right) Axial
HRCT shows 5 mm MIP
better demonstrates
uee-in-bud opacities
(arrows) and centrilobu/ar
nodules (open arrows) in
this patient with atypical
mycobacterial infection.

(Left) Axial HRCT shows

clusters of centrilobular
nodules (arrows). Periphery
of lung is spared except for
the largest nodules which
have grown to the edge of
the lung (open arrows).
Langerhans histiocytosis.
(Right) Axial HRCT shows
clusters of cysts in the
centri/obular portion of the
lung. Lung periphery is
spared. Langerhans
histiocytosis.

(Left) Axial H RCT shows

faint ground-glass nodules
(arrows) and branching
opacities (open arrows) in
smoker with respiratory
bronchiolitis. Asbestos
pleural plaque (curved
arrow). (Right) Axial NECT
shows well-defined holes
(arrows) without walls from
centrilobu/ar emphysema.
Some evidence suggests that
respiratory bronchiolitis is
precursor to centri/obu/ar
emphysema.
 HRCT: LYMPHATIC PATTERN

38

Axial graphic shows several features of the lymphaUc Axial HRCT shows lymphatic pattern in Iymphangitic
pattern. Note bronchovascular thickening (arrows). carcinomatosis. Bronchovascu/ar core structures are
Peripheral septa outline the polygonal shape of the SPls thickened (open arrows), septa are irregularly thickened
(open arrows). and in some areas beaded (curved arrow).

Peripheral network along pulmonary veins,

I TERMINOlOGY interlobular septa, and pleura
Definitions Alveoli devoid of lymphatics
Predominant radiographic abnormality found along Morphology
the distribution of lymphatics in the secondary o Central lymphatic network
pulmonary lobule (SPL) Abnormal bronchovascular bundle: Smooth or
SPL: Smallest unit of lung structure marginated by nodular thickening
interlobular septa Clusters of centrilobular nodules
o Peripheral lymphatic network
Subpleural nodules or pseudoplaques (chains of
IIMAGING FINDINGS nodules)
Septal thickening, smooth or nodular
General Features
Radiographic Findings
Best diagnostic clue
Difficult to recognize: Nonspecific reticular pattern
o Centrilobular micronodules 5 mm in diameter)
due to summation of pathology in lymphatic or
and subpleural micronodules within the secondary
perilymphatic tissue
pulmonary lobule
o Upper lung zone distribution
o Septal thickening and thickening of the core
Seen in those disorders due to the chronic
bronchovascular structures
retention of inhaled particulate matter
Location
o Kerley B lines: Peripheral lines 2 cm long and less
o Lymphatics in SPL form two networks
than 1 mm thick) perpendicular to the pleural
Central network arranged along the arteries and
surface
airways down to the respiratory bronchioles (RB)
(1st generation)

DDx: Smooth Septal Thickening

Lymphangiomatosis
 HRCT: LYMPHATIC PATTERN
Key Facts
Terminology
Predominant radiographic abnormality found along
the distribution of lymphatics in the secondary
pulmonary lobule (SPL)
Imaging Findings
Centrilobular micronodules 5 mm in diameter)
and subpleural micronodules within the secondary
pulmonary lobule
Septal thickening and thickening of the core
bronchovascular structures
Top Differential Diagnoses
Sarcoidosis
Lymphangitic Carcinomatosis
Cardiogenic Pulmonary Edema
Pulmonary Veno-Occlusive Disease
Thickened interlobular septa due to lymphatic
dilatation, venous distension, interstitial fluid or
tissue
Primarily seen in pulmonary edema due to venous
hypertension or lymphangitic spread of tumor
CT Findings
HRCT
o Bronchovascular core
Thickening normal paper thin bronchioles
Visualization of airways within 2 cm of the pleura
(normally below the limits of resolution of CT)
o Centrilobular nodules
Common with inhalation diseases and sarcoid
Centrilobular nodules alone (without subpleural
or peripheral lymphatic involvement) suggests a
disease process that is either bronchocentric or
angiocentric
o Septal thickening
Forms arcades or polygonal lines
May be smooth as in pulmonary edema
Or irregular thickened or beaded as in
lymphangitic tumor
Imaging Recommendations
Best imaging tool: HRCT slab with maximum intensity
projection (MIP) or minimum intensity projection
(minIP) useful for micronodular disease
I DIFFERENTIAL DIAGNOSIS
Sarcoidosis
Symmetric hilar and para tracheal adenopathy
o Adenopathy typically regresses as lung disease
worsens
More profuse in upper lung zones, especially the right
upper lung
Tends to follow bronchovascular distribution
o Often focal in the lung, cutting a "swath" from the
hilum to the lung periphery
1
Pneumoconiosis (Silica or Coal) 39
Respiratory Bronchiolitis & Anthracosis
Lymphocytic Interstitial Pneumonia
Diffuse Pulmonary Lymphangiomatosis
Erdheim Chester Disease
Pathology
For inhaled particulate diseases, concentration of
particles reflects the distribution of disease
Estimated normal human lymphatic flow 20 ml!hr
Diagnostic Checklist
Involvement of both the core structures and the
periphery of the SPL (subpleural or fissures)
characteristic of lymphatic pattern
Lymphangitic Carcinomatosis
History of malignancy, usually an adenocarcinoma
May have pleural effusions or adenopathy
May preferentially involve the bronchovascular
bundle (most common), the peripheral septa or both
Septal thickening usually irregularly or beaded
o Tends to be focal in the lung, sparing lobes or lungs
Cardiogenic Pulmonary Edema
Enlarged heart
Septal thickening smooth and uniform
Dependent ground-glass opacities from pulmonary
edema and pleural effusions common
Pulmonary Veno-Occlusive Disease
Enlarged central pulmonary arteries from pulmonary
arterial hypertension
Smooth septal thickening
Pneumoconiosis (Silica or Coal)
Adenopathy common (classically with egg-shell
calcification)
More profuse in upper lung zones, especially the right
upper lung
Nodules may aggregate together into progressive
massive fibrosis (PM F)
Occupational history important (chronic dust
exposure over many years)
Respiratory Bronchiolitis & Anthracosis
Universal histologic pattern in smokers (but seen in
others living in dusty environments)
Faint centrilobular nodules and occasional subpleural
nodules primarily in dorsal aspect upper lung zones
Radiographic abnormalities tend to be subtle unless
smoking history severe (high pack-years of unfiltered
cigarettes)
Lymphocytic Interstitial Pneumonia
Chronic antigenic stimulus elicits an
lymphoproliferative response: Idiopathic, autoimmune
(Sjogren), viral infection, immunodeficiency states
2-4 mm centrilobular and subpleural nodules
 HRCT: LYMPHATIC PATTERN
1 80% have associated thin-walled
80% have septal thickening
cysts
40
Amyloidosis
Extracellular protein deposition either primary or
secondary (chronic inflammatory conditions)
Forms: Tracheobronchial, pulmonary nodular,
adenopathy, diffuse septal in that order
Diffuse septal form has lymphatic pattern:
Centrilobular nodules, septal thickening, thickening
bronchovascular bundles
Amyloid deposition in perilymphatic tissue
Diffuse Pulmonary Lymphangiomatosis
Congenital lymphatic disorder characterized by
proliferation and dilatation of lymphatic channels
Smooth diffuse interlobular septal thickening, diffuse
effacement of mediastinal fat
o Basilar predominance
Pleural or pericardial effusions, usually chylous (50%)
Mediastinal adenopathy, mild
Erdheim Chester Disease
Non-Langerhans cell histiocytosis of unknown
etiology
Symmetric sclerotic bone lesions
Perirenal infiltration or aortic soft tissue encasement
Septal thickening smooth
I PATHOLOGY
General Features
Etiology
o For inhaled particulate diseases, concentration of
particles reflects the distribution of disease
Small round particles are engulfed by
macrophages and removed by lymphatics
Pathology from small round particles reflects the
eventual accumulation of retained particles in the
dorsal aspect of the upper lobes
Physiology of particulate inhalation
o Particles < 5 ~ in diameter reach the secondary
pulmonary lobule and settle out in the 2nd
generation respiratory bronchioles
o For insoluble particles, macrophages engulf the
particles and migrate either to the ciliated airways or
the lymphatics
o Particulate clearance from the acinus slow with a
half-time clearance of approximately 30 days
Lymphatic physiology
o Estimated normal human lymphatic flow 20 ml/hr
May increase 10 fold in patients with chronic
congestive heart failure
o Gravitational gradient: Lymphatic flow linearly
increases down the lung due to gravitational
increases in pulmonary arterial pressure
o Kinetic motion: Passive lymphatic flow due to
extraneous motion, primarily diaphragmatic
excursion and motion of chest wall
Increased flow in bases due to diaphragmatic
excursion
Increased flow anteriorly (due to pump handle
chest wall motion) and laterally (due to bucket
handle chest wall motion)
o Regions with slowest lymphatic flow
Upper lung zones, especially the right due to
slightly higher pulmonary blood flow in the left
upper lung (due to jet effect of blood flow across
the pulmonic valve)
Dorsal lung due to relatively increased respiratory
of motion anteriorly and laterally
o Pathologic correlation: Distribution of particles
For most particulate material, upper lobe contains
1.25x the concentration in the lower lobe
Analysis by lobe blurs the true magnitude of
distribution of particles, which is not dependent
on lobar anatomy but on gravitational gradients
Gross Pathologic & Surgical Features
Secondary pulmonary lobule
o Core structures: Terminal bronchioles, arteries,
lymphatics
o Peripheral structures: Septa, veins, and lymphatics
Bronchovascular bundle (core structures)
o Arteries paired with bronchi (pulmonary veins run
in periphery of the lobule)
o Terminal bronchioles supply SPL
Bronchioles lack cartilage but contain cilia and
smooth muscle
Acinus
o All structures distal to end terminal bronchiole
(normal adult lung has - 30,000 terminal
bronchioles)
o Includes RB, alveolar ducts, and alveolar sacs
o Lymphatics begin at 1st generation of RB
o Normal: 2-5 generations of respiratory bronchioles
o Round or elliptical shape - 8 mm in diameter
o Distance from smallest terminal bronchiole to the
interlobular septa, pulmonary vein, or pleura
relatively constant 2.5 mm
o Most pulmonary capillaries are within 1 mm of the
nearest lymphatic
I CLINICAL ISSUES
Presentation
Most common signs/symptoms: None related to
lymphatic pattern
I DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Involvement of both the core structures and the
periphery of the SPL (subpleural or fissures)
characteristic of lymphatic pattern
I SELECTED REFERENCES
1. Colby TV et ale Anatomic distribution and histopathologic
patterns in diffuse lung disease: correlation with HRCT. J
Thorac Imaging. 11 (1):1-26, 1996
 HRCT: LYMPHATIC PATTERN
IIMAGE GAllERY 1
41

(Left) Sagittal gross

pathology, section shows
nodular septal thickening
(arrows) and thickened core
structures (open arrows) in
Iymphangitic carcinomatosis.
(Right) Sagittal gross
pathology shows diffuse
smooth septal thickening
(arrows) from pulmonary
edema. Septa outline the
secondary pulmonary
lobules producing a quilt
patchwork appearance.

Typical
(Left) Axial HRCT shows
cluster of centrilobular
nodules (arrow) and several
subpleural nodules (or
pseudoplaques) (open
arrows) in patient with
silicosis. (Right)
Anteroposterior radiograph
shows numerous Kerley B
lines (arrows) in the right
lower lobe in patient with
acute pulmonary edema.

(Left) Axial HRCT shows

diffuse smooth septal
thickening (arrows) and
thickened major fissure
(curved arrow). Faint
centrilobu/ar nodules and
thickened core structures
also present (open arrows).
Erdheim Chester disease.
(Right) Axial HRCT shows
diffuse smooth septal
thickening farrows),
ground-glass opacities, and
focal consolidation (open
arrows) in patient with
diffuse septal form of
amyloidosis.
 FOCAL DECREASED DENSITY LUNG DISEASE

22

Axial HRCT shows focal decreased density from a cavity Axial H RCT shows focal decreased densities due to
following aspergillus infection (arrow). Note centrilobular emphysema (arrows). Lesions are
ground-glass halo surrounding cavity (curved arrows). surrounded by normal lung parenchyma. Cough
induced subcutaneous emphysema (open arrows).

Protocol advice
I TERMI NOlOGY o Volumetric CT performed to assess profusion of
Definitions lesions
Area with markedly decreased attenuation o HRCT performed to assess subtle details and relation
Partly or entirely surrounded by lung parenchyma of to constituents of secondary pulmonary lobule
normal or increased attenuation o Expiratory HRCT for assessment of potential
Always localized, never diffuse air-trapping
Either solitary or multiple
To be differentiated against
a Extrapulmonary but intrathoracic air accumulations I DIFFERENTIALDIAGNOSIS
(pneumothorax)
Miscellaneous and Artifacts
o Air in pathological bronchi (bronchiectasis)
Overpenetrated chest film
o Air in solid or semisolid structures (abscess)
Rotation of patient
Chest Wall Defect
IIMAGING FINDINGS Asthenic body habitus
General Features Mastectomy
Poland syndrome (absent pectoralis muscle)
Location: Any lobe, although cavities are more
common in the upper lobes Cystic Lesions
Size: Occupies entire range of possible sizes Congenital
o Congenital lobar emphysema
Imaging Recommendations
o Cystic adenomatoid malformation
Best imaging tool: CT o Bronchial atresia

DDx: Focal Decreased Density Lung Disease

Pneumothorax Abscess
 HRCT: GROUND-GLASS OPACITIES

I IMAGE GAllERY 1
45
Typical
(Left) Axial CECT shows
patchy eeo in the right
middle lobe, underneath
displaced rib fracture, typical
for focal pulmonary
hemorrhage and contusion
(arrow). (Right) Axial CECT
shows dense consolidation
within the left upper lobe
and surrounding eeofrom
this patient with
bronchioloalveolar
carcinoma.

(Left) Axial H RCT at end

exhalation shows 3 different
lung densities: Air-trapping
(curved arrow), norma/lung
(open arrow), and eeo
(arrow) in this patient with
hypersensitivity pneumonitis.
(Right) Axial NECT shows
patchy bilateral upper lobe
eeo from this patient with
AIDS and pneumocystic
pneumonia. Also note
several associated cysts
(arrows).

(Left) Axial HRCT shows

normal
gravitational-dependent
eeo in the posterior
peripheral righllower lobe
from microatelectasis
(arrows). This cleared
completely in the prone
position. (Right) Axial HRCT
shows peripheral basUar
honeycombing (open
arrows) in this patient with
idiopathic pulmonary
fibrosis. ceo in this case
(arrows) likely represents
micro{ibrosis beyond
resolution of scanner.
 HRCT: GROUND-GLASS
Incidental finding of normal gravitationally
dependent density from microatelectasis
44 Any type of pulmonary edema
Any type of pneumonia, but more typical for viral
or Pneumocystis pneumonia (PCP), depending on
underlying immune status
Bronchioloalveolar carcinoma and atypical
adenomatoid hyperplasia
Respiratory bronchiolitis and other
smoking-related interstitial lung diseases
o Less common etiologies include
Pulmonary hemorrhage syndromes
Systemic vasculitides
Sarcoidosis
Leukemic infiltration into lung
Any type of alveolar or interstitial filling process
beyond the resolution of the CT scanners such as
many of the idiopathic interstitial pneumonias
Drug toxicity, reflecting underlying interstitial
lung disease: Amiodarone, cyclophosphamide,
bleomycin, and carmustine
o Diffuse GGO consider pulmonary edema or
infection
o Peribronchial ground-glass nodules typical for
respiratory bronchiolitis (upper lobes) or
hypersensitivity pneumonitis (diffuse)
o Patchy upper lobe GGO consider sarcoidosis or
Pneumocystis pneumonia
o GGO halo around focal consolidation or nodules
Hemorrhage around an infection, such as invasive
pulmonary aspergillosis
Other fungal infections, viral infections, post-lung
biopsy hemorrhage
Associated abnormalities
o Commonly associated with other HRCT patterns
such as mosaic perfusion and crazy-paving
o Can be superimposed on any other lung disease or
process
o This can lead to confusing patterns, such as the
coexistence of GGO and air-trapping in patients
with hypersensitivity pneumonitis
Microscopic Features
Any alveolar or interstitial process that increases the
attenuation of the X-ray beam
Cellular infiltrates (tumor cells, macrophages,
neutrophils, etc.), alveolar fluid (blood, edema),
hyaline membranes, fibroblasts, etc.
Hyperemia
I CLINICAL ISSUES
Presentation
Most common signs/symptoms
o Depends upon etiology, any inhalational exposures,
underlying immune status
o Dyspnea, cough, hemoptysis
o Can be asymptomatic
Other signs/symptoms
o Duration of symptoms: Acute symptoms more likely
infection; chronic symptoms a broader range of
diagnostic possibilities
OPACITIES
o Immune status: More commonly true abnormality
in immunocompromised host
o Associated co-morbidities: E.g., achalasia and
interstitial pneumonia, pulmonary-renal syndromes,
hepatopulmonary syndromes, etc.
o Drug toxicity as diagnosis of exclusion
o Cigarette smoking history: Upper lung
peribronchiolar ground-glass nodules, pathologically
represent respiratory bronchiolitis or respiratory
bronchiolitis-interstitial lung disease
Demographics
Age: Any age
Natural History & Prognosis
Depends upon etiology
In patients with infections, GGO can clear quickly
with appropriate treatment
In patients with AIDS, upper lung GGO can progress
quickly to cystic destruction
Treatment
Depends upon etiology
In patients with usual interstitial pneumonia, steroid
therapy has not been shown to be effective, because
the GGO is just as likely to be fibrosis beyond the
resolution of the scanner as it is to represent
inflammation
I DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
If GGO seems slice dependent, e.g., on one slice but
not the next adjacent slice, be sure patient cooperated
with appropriate breath-holding
If paired inspiratory and expiratory scans shows the
combination of GGO, normal lung attenuation, and
areas of air-trapping, consider hypersensitivity
pneumonitis
o Peri bronchiolar inflammation causes the GGO, but
resulting small airways obstruction leads to
air-trapping
I SElECTED REFERENCES
1. Lee YRet al: CT halo sign: the spectrum of pulmonary
diseases. BrJ Radiol. 78(933):862-5, 2005
2. Lynch DAet al: Idiopathic interstitial pneumonias: CT
features. Radiology. 236(1):10-21, 2005
3. Miller WTJr et al: Isolated diffuse ground-glass opacity in
thoracic CT:causes and clinical presentations. AJRAmJ
Roentgenol. 184(2):613-22,2005
4. Castaner E et al: Radiologic approach to the diagnosis of
infectious pulmonary diseases in patients infected with the
human immunodeficiency virus. Eur J Radiol.
51(2):114-29,2004
5. Wittram C: The idiopathic interstitial pneumonias. CUff
Prohl Diagn Radiol. 33(5):189-99, 2004
6. Rossi SEet al: "Crazy-paving" pattern at thin-section CT of
the lungs: radiologic-pathologic overview. Radiographies.
23(6):1509-19, 2003
7. Nowers Ket al: Approach to ground-glass opacification of
the lung. Semin Ultrasound CT MR. 23(4):302-23, 2002
8. Collins J et al: Ground-glass opacity at CT:the ABCs.AJR
AmJ Roentgenol. 169(2):355-67, 1997
 HRCT: GROUND-GLASS OPACITIES

I IMAGE GAllERY 1
45
Typical
(Left) Axial CECT shows
patchy eeo in the right
middle lobe, underneath
displaced rib fracture, typical
for focal pulmonary
hemorrhage and contusion
(arrow). (Right) Axial CECT
shows dense consolidation
within the left upper lobe
and surrounding eeofrom
this patient with
bronchioloalveolar
carcinoma.

(Left) Axial H RCT at end

exhalation shows 3 different
lung densities: Air-trapping
(curved arrow), norma/lung
(open arrow), and eeo
(arrow) in this patient with
hypersensitivity pneumonitis.
(Right) Axial NECT shows
patchy bilateral upper lobe
eeo from this patient with
AIDS and pneumocystic
pneumonia. Also note
several associated cysts
(arrows).

(Left) Axial HRCT shows

normal
gravitational-dependent
eeo in the posterior
peripheral righllower lobe
from microatelectasis
(arrows). This cleared
completely in the prone
position. (Right) Axial HRCT
shows peripheral basUar
honeycombing (open
arrows) in this patient with
idiopathic pulmonary
fibrosis. ceo in this case
(arrows) likely represents
micro{ibrosis beyond
resolution of scanner.
 HRCT: MOSAIC PATTERN OF LUNG ATTENUATION
1
46
Axial graphic shows mosaic pattem of lung allenuation
with two different areas of sharply marginated lung
parenchyma. Note paucity of vascular markings in
lucent regions (arrows).
ITERMINOlOGY
Abbreviations and Synonyms
Mosaic attenuation
Mosaic perfusion
Definitions
Sharply marginated regions of increased and decreased
lung attenuation, in a mosaic pattern, with a variable
distribution depending upon the anatomic level of
involvement
Multiple different etiologies, but broadly divided into
2 main categories
By far, the most common cause of a mosaic pattern of
lung attenuation is obstructive small airways disease,
with air-trapping
a Small airways disease can be fixed or reactive
o Reactive (reversible) airways disease: Asthma
o Fixed (irreversible) small airways disease:
Obliterative bronchiolitis (constrictive bronchiolitis)
Important, but much less common etiology is chronic
small vessel pulmonary vascular obstruction
o Primary pulmonary artery hypertension
DDx: Mosaic Pattern
Hypersensitivity Pneumonitis Alpha-! Antiprotease Deficiency
Axial HRCT obtained at full inspiraUon shows a mosaic
pattern due to air-trapping secondary to constrictive
obliterative bronchiolitis. Note associated bronchiectasis
(arrows) as further evidence of airway injury.
o Secondary pulmonary artery hypertension: Chronic
thromboembolic disease
Can be substantial overlap in appearances; not always
possible to distinguish between these entities
IIMAGING FINDINGS
General Features
Best diagnostic clue
o For small airways diseases: Air-trapping
Indirect sign of obstructive small airways disease
Mosaic pattern of attenuation can be evident on
inspiratory CT, when severe
Milder small airways disease may be normal on
inspiration
Mosaic pattern indicative of air-trapping is
brought out or accentuated on expiratory CT
o It is patchy air-trapping that creates mosaic pattern
Diffuse air-trapping much more difficult to
recognize
o Bronchial dilatation or frank bronchiectasis, as a
sign of larger airway injury, commonly, but not
exclusively, seen with smaller airways injury
Pneumocystis Pneumonia
 HRCT: MOSAIC PATTERN OF LUNG ATTENUATION
Key Facts
Terminology
Sharply marginated regions of increased and
decreased lung attenuation, in a mosaic pattern, with
a variable distribution depending upon the anatomic
level of involvement
Imaging Findings
For small airways diseases: Air-trapping
Best imaging tool: HRCT or thin-section scanning
Paired inspiratory/expiratory HRCT scanning in
supine position when evaluating mosaic pattern
Top Differential Diagnoses
Infectious Pneumonia
Hypersensitivity Pneumonitis
Inflammatory (Cellular) Bronchiolitis
Panlobular Pulmonary Emphysema
o Decreased caliber of pulmonary arteries in lucent
regions can be seen with airways disease and
pulmonary vascular disease
o It is extent of air-trapping that is important
Mild air-trapping can be demonstrated as an
incidental finding in cigarette smokers, elderly,
and even in otherwise healthy young individuals
o Patients with pulmonary vascular disease may
exhibit secondary air-trapping
Location
o Typically subsegmental or smaller
o Multifocal and patchy
o Dependent upon site of injury to airway or vascular
tree
Size
o Can involve sublobular structures (acini), lobules,
segments, lobes, or even a whole lung
o Example of whole lung involvement is Swyer-James
syndrome with unilateral hyperlucent lung
Morphology: Sharply defined margins depending
upon level of injury to airway or artery
CT Findings
Small airways diseases
o When suggested on inspiratory HRCT, expiratory
HRCT scans confirm air-trapping
o Expiratory HRCT scans increase the likelihood of
identifying air-trapping when not apparent on
inspiratory scans
Pulmonary vascular diseases
o Geographic regions of increased attenuation
represent hyperperfused normal lung: Show dilated
vessels
o Decreased size and number of vascular markings in
lucent lung relative to more opaque lung
o Also demonstrates air-trapping, typically without
direct evidence of airways disease such as
bronchiectasis
o Central pulmonary artery enlargement a more
recognizable feature
Imaging Recommendations
Best imaging tool: HRCT or thin-section scanning
1
Pathology 47
Constrictive bronchiolitis
Concentric luminal narrowing of membranous and
respiratory bronchioles secondary to submucosal and
peri bronchiolar inflammation and fibrosis
Pulmonary arterial hypertension
Intimal cellular proliferation and medial smooth
muscle hypertrophy, mainly in walls of muscular
arteries
Clinical Issues
Small airways disease
Dyspnea, cough, +/- response to bronchodilators,
wheezing, no fever
Pulmonary vascular disease
Exertional dyspnea, no cough, no response to
bronchodilators, no wheezing, no fever
Protocol advice
o Paired inspiratory/expiratory HRCT scanning in
supine position when evaluating mosaic pattern
Little or no increase in attenuation, or decrease in
volume of lucent lung on expiratory CT
Decreased size and number of vascular markings
in lucent lung relative to more opaque lung
o Lateral decubitus CT scanning can be used as an
adjunct in patients incapable of following breathing
instructions to elucidate air-trapping
Gravitationally dependent lung will demonstrate
air-trapping
I DIFFERENTIAL DIAGNOSIS
Infectious Pneumonia
Many types of acute lung infections, typically viral
and mycoplasma
Distinct clinical scenario
Hypersensitivity Pneumonitis
Acute, sub-acute or chronic allergic reaction in the
small airways
Can cause diffuse or patchy regions of both
air-trapping and ground-glass attenuation
Inflammatory (Cellular) Bronchiolitis
Acute inflammatory, typically infectious disease of the
bronchiole wall
Results in tree-in-bud pattern, ground-glass
attenuation, frank pneumonia
Air-trapping an uncommon secondary feature
Panlobular Pulmonary Emphysema
Alpha-! antitrypsin deficiency, intravenous abuse of
methylphenidate
Distinguishing features: More diffuse low attenuation
parenchymal destruction, vascular distortion, basilar
lung predominance
 HRCT: MOSAIC PATTERN OF LUNG ATTENUATION
1 I PATHOLOGY
48 General Features
Genetics: Primary arterial hypertension can be familial
Etiology
o Constrictive bronchiolitis
Cryptogenic, post infectious (viral), toxic fume
inhalation, bone marrow and lung transplantation
rejection, rheumatoid arthritis, inflammatory
bowel disease, and drug toxicity (penicillamine
therapy)
Occurs in up to 50% of patients with lung
transplants
Classic toxic inhalational etiology is Silo-filler's
lung
10% of patients receiving allogeneic bone marrow
transplants graft-vs.-host disease (GVHD)
Not to be confused with cryptogenic-organizing
pneumonia (bronchiolitis obliterans-organizing
pneumonia)
o Pulmonary artery hypertension
Mean pulmonary artery pressure greater than 25
mm Hg at rest or greater than 30 mm Hg during
exercise
Primary is considered idiopathic
Secondary: Chronic interstitial lung disease;
congenital cardiac left-to-right shunt;
thromboembolic disease (including tumor,
parasitic such as Schistosomiasis, and talc; chronic
alveolar hypoxia
Epidemiology: Cryptogenic bronchiolitis obliterans is
an uncommon entity most common in older women
Gross Pathologic & Surgical Features
Pulmonary artery hypertension
o Central arterial thrombosis, premature
atherosclerosis, aneurysmal dissection of pulmonary
arteries, and hypertrophy and dilatation of right
heart
Microscopic Features
Constrictive bronchiolitis
o Concentric luminal narrowing of membranous and
respiratory bronchioles secondary to submucosal
and peri bronchiolar inflammation and fibrosis
o No intraluminal granulation tissue or polyps, as seen
with cryptogenic organizing pneumonia
Pulmonary arterial hypertension
o Intimal cellular proliferation and medial smooth
muscle hypertrophy, mainly in walls of muscular
arteries
o Necrotizing arteritis and plexiform lesions are
additional histologic features found exclusively in
primary pulmonary hypertension and congenital
cardiac shunt
o Plexiform lesions are hallmark of chronic,
irreversible disease, representing focal disruption of
the internal elastic lamina of a muscular pulmonary
artery by a "glomeruloid" plexus of endothelial
channels
I CLINICAL ISSUES
Presentation
Most common signs/symptoms
o Small airways disease
Dyspnea, cough, +/- response to bronchodilators,
wheezing, no fever
o Pulmonary vascular disease
Exertional dyspnea, no cough, no response to
bronchodilators, no wheezing, no fever
Other signs/symptoms
o Extent of air-trapping on expiratory CT correlates
best with physiologic impairment
o Bronchiolitis obliterans syndrome (BaS)
Lung transplant patients, based on reduction in
the forced expiratory flow volume in one second
(FEV1) to less than 80% of post-transplant
baseline
Other causes such as infection, anastomotic
stenosis, or disease recurrence must be excluded
CT findings in BaS include bronchial dilation,
bronchial wall thickening, and air-trapping
Treatment
Constrictive bronchiolitis can progress to the need for
lung transplant
Chronic pulmonary artery thromboembolic disease
can necessitate thrombectomy
I DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Paired inspiratory/expiratory thin-section CT to
increase detection and confidence in diagnosis of
air-trapping as secondary sign of small airways disease
I SELECTED REFERENCES
1. Pipavath SJ et al: Radiologic and pathologic features of
bronchiolitis. AJR Am J Roentgenol. 185(2):354-63,2005
2. Arakawa H et al: Chronic pulmonary thromboembolism.
Air trapping on computed tomography and correlation
with pulmonary function tests. J Com put Assist Tomogr.
27(5):735-42,2003
3. Tanaka N et al: Air trapping at CT: high prevalence in
asymptomatic subjects with normal pulmonary function.
Radiology. 227(3):776-85, 2003
4. Arakawa H et al: Expiratory high-resolution CT: diagnostic
value in diffuse lung diseases. AJR Am J Roentgenol.
175(6):1537-43,2000
5. Sherrick AD et al: Mosaic pattern of lung attenuation on
CT scans: frequency among patients with pulmonary artery
hypertension of different causes. AJR Am J Roentgenol.
169(1):79-82,1997
6. Worthy SA et al: Mosaic attenuation pattern on
thin-section CT scans of the lung: differentiation among
infiltrative lung, airway, and vascular diseases as a cause.
Radiology. 205(2):465-70, 1997
7. King MA et al: Chronic pulmonary thromboembolism:
detection of regional hypoperfusion with CT. Radiology.
191(2):359-63,1994
 HRCT: MOSAIC PATTERN OF LUNG ATTENUATION
I IMAGE GALLERY
Variant
(Left) Paired inspiratory and
expiratory CT scans from
patient with asthma show
normal parenchyma at
inspiration and patchy
air-trapping at exhalation
only (arrows). (Right) Axial
HRCT shows extensive lower
lobe bronchiectasis (arrows)
associated with diffuse low
attenuation parenchyma
representing difficult to
recognize diffuse
air-trapping.

Typical
(Left) Axial HRCT from a
patient with chronic
pulmonary thromboembolic
disease shows mosaic
perfusion pattern. Note
paucity of vascular markings
in lucent lung. No evidence
of larger airway disease.
(Right) Axial HRCT from
another patient with chronic
pulmonary thromboembolic
disease shows similar mosaic
perfusion pattern. Note
paucity of vascular markings
in lucent lung, and enlarged
central vasculature.

(Left) Axial HRCT shows

air-trapping associated with
extensive bronchiectasis
(arrows), yielding a mosaic
pattern of lung attenuation.
Note sharp, geographic
margination of abnormal
lucent and normal denser
lung. (Right) Coronal
reformatted CT from the
caudal aspect of lungs shows
lobular air-trapping (arrows)
as an incidental finding in an
otherwise normal adult
patient.
 HRCT: TREE-IN-BUD PATTERN'
1
50

Graphic shows appearance of the TlB pattern, as would Transverse NECT shows a TlB pattern in the right lower
typically be seen on a transverse CT scan. Note the lobe (arrow), along with nonspecific patchy opacities in
clusters of nodules and branching tubular structures other lobes, secondary to aspiration and resulUng
having an airway distribution. bronchiolitis.

Nonspecific finding evident in many different disease

ITERMINOLOGY processes that can cause small airway inflammation,
Abbreviations and Synonyms primarily infectious, but including immunologic
disorders, congenital diseases, and idiopathic causes
Tree-in-bud (TIE)
a Infectious etiologies include: Bacteria, viruses,
Definitions parasites, and fungus
Direct sign of bronchiolitis Less frequent finding in fungal and viral
a Tree-in-bud pattern infections
a Indirect signs include mosaic attenuation and Aspiration of a variety of materials, including gastric
air-trapping contents, water or blood, associated with a variable
Descriptive term for fairly sharply circumscribed inflammatory response may result in TIB
nodules or tubular branching soft tissue opacities
within or near secondary pulmonary lobule and acini
Tree-in-bud pathologic correlates I IMAGING FINDINGS
a Dilated bronchioles filled with mucus, pus,
granulation tissue, or fluid General Features
a Bronchiolar impaction, typically inflammatory Best diagnostic clue
a Bronchiolitis a Associated upper lobe cavitary destruction when
a Peri bronchiolar inflammation and fibrosis with seen in patients with M. tuberculosis
bronchiolectasis, with filling of dilated bronchioles a In patients of Asian ethnicity, diffuse peripheral TIB
with secretions suggest diffuse panbronchiolitis
a Very rarely seen as tumor emboli within small a Esophageal abnormalities in chronic aspiration
vessels: Thrombotic microangiopathy of pulmonary
tumors

DDx: Tree-In-Bud

Atrial Sarcoma Metastases Miliary Tuberculosis Sarcoidosis

 HReT: TREE-IN-BUD PATTERN

Key Facts
Terminology
Descriptive term for fairly sharply circumscribed
nodules or tubular branching soft tissue opacities
within or near secondary pulmonary lobule and acini
Very rarely seen as tumor emboli within small vessels:
Thrombotic microangiopathy of pulmonary tumors
Imaging Findings
Within 3-5 mm of pleural surface
TIB opacities tend to occur in patchy distribution in
lung periphery
Best imaging tool: High-resolution CT
Top Differential Diagnoses
Angiocentric Hematogenous Metastases
Hematogenous Infection
Lymphangiocentric or Peribronchovascular Nodules
Hypersensitivity Pneumonitis
Respiratory Bronchiolitis and Respiratory
Bronchiolitis-Associated Interstitial Lung Disease
Clinical Issues
In proper clinical setting, suspect active,
endobronchial spread of M. tuberculosis
Age: Elderly immunocompetent women with middle
lobe and lingular bronchiectasis and TIB: Suspect
non-tuberculous mycobacterial infection
Diagnostic Checklist
First and foremost, TIB pattern is nonspecific, but
usually represents some type of bronchiolitis nit is
NOT pathognomic for endobronchial tuberculosis

o Central bronchiectasis and "finger-in-glove" sign in

cystic fibrosis, ciliary dysmotility syndromes, or Hematogenous Infection
allergic bronchopulmonary aspergillosis Miliary tuberculosis or fungal infection
o Kartagener syndrome: Situs inversus, sinusitis, Lymphangiocentric or Peribronchovascular
bronchiectasis
o Associated air-trapping helpful in distinguishing Nodules
airway disease from vascular TIB Irregular nodular opacities in and around
Location bronchovascular bundles
o Can appear as multiple clusters of nodules Lymphatic distribution includes subpleural regions
surrounding the central portion of secondary and interlobular septa
pulmonary lobule Sarcoidosis is typical
o Within 3-5 mm of pleural surface
Hypersensitivity Pneumonitis
o TIB opacities tend to occur in patchy distribution in
lung periphery Ill-defined hazy ground-glass density nodules with a
more diffuse distribution, centered around the
Often gravitationally dependent distribution,
secondary pulmonary lobule
when result from endobronchial spread from a
Check for history of exposures
larger area of infection or from aspiration
etiologies Classic exposure in bird fanciers
o Can be diffuse (e.g., diffuse panbronchiolitis) Respiratory Bronchiolitis and Respiratory
Size: Clusters of 2-4 mm nodules or branching tubular
soft tissue opacities
Bronchiolitis-Associated Interstitial Lung
Morphology Disease
o Typical "Y"or "V" shaped Cigarette smokers
Like a "tree-in-bud" Predominantly in upper lungs
o Similar to the "finger-in-glove" concept of impacted
bronchiectatic airways, but on a much smaller scale Lymphangitic Tumor
Beaded or irregular thickening of interlobular septa
Imaging Recommendations Thickening of bronchovascular bundles
Best imaging tool: High-resolution CT Known malignancy, typically an adeoncarcinoma

I DIFFERENTIAL DIAGNOSIS I PATHOLOGY

Angiocentric Hematogenous Metastases General Features
Vascular "tree-in-bud" Etiology
Does not have tubular or branching opacities like o Any type of bronchial disease, typically infectious
airway-centric disease or microangiopathic metastases Classically mycobacterial
Small vessels run into or emanate from nodules Associated abnormalities
Nodules may have ground-glass halo from hemorrhage o In patients with M. tuberculosis, upper lobe cavitary
Common with metastases from colorectal, lung, destruction
breast, thyroid, renal cell carcinomas, melanomas, and o Airway stenosis causing air-trapping
seminomas
 HRCT: TREE-IN-BUD PATTERN
1 Gross Pathologic & Surgical Features
52 Nodules distributed in centrilobular regions
Dilatation of bronchioles but to lesser degree than
classic bronchiectasis
Microscopic Features
Chronic inflammatory lesions distributed in
centrilobular regions, typically respiratory bronchiole
Infectious bronchiolitis shows epithelial necrosis and
inflammation of bronchiolar walls and intraluminal
exudates
Bronchioles impacted with inflammatory debris
Thickened airway walls
Bronchiolar intraluminal granulation tissue
Proliferation of bronchus-associated lymphoid tissue
frequent
Tuberculosis: Solid caseous material filling or
surrounding terminal or respiratory bronchioles or
alveolar duct
IClINICAL ISSUES
Presentation
Most common signs/symptoms
o Subacute or chronic cough
o Acute respiratory illness
Other signs/symptoms
o In proper clinical setting, suspect active,
endobronchial spread of M. tuberculosis
TlB in up to 3/4 of such patients
TIB a spilling of infectious material from cavitary
destruction elsewhere
Such patients are potential public health hazard
Consider patient isolation until contagion issues
are solved
o Aspiration TlB pattern
Unconscious, swallowing disorders, esophageal
malignancy, tracheoesophageal fistula
o Follicular bronchiolitis and lymphocytic interstitial
pneumonia TIB pattern
Associated with immunologic conditions such as
rheumatoid arthritis and Sjogren syndrome
Demographics
Age: Elderly immunocompetent women with middle
lobe and Iingular bronchiectasis and TIB: Suspect
non-tuberculous mycobacterial infection
Gender: In older women, non-tuberculous
mycobacterial infections has been coined "lady
Windermere" syndrome
Natural History & Prognosis
Depends on underlying etiology
Clears to normal, in most cases of acute infectious
bronchiolitis
Can result in permanent bronchiolectasis, especially
with mycobacterial infections
Treatment
For infectious bronchiolitis: Appropriate antibiotics
For tuberculosis: Appropriate antibiotic regimens
For diffuse panbronchiolitis: Erythromycin
I DIAGNOSTIC CHECKLIST
Consider
First and foremost, TlB pattern is nonspecific, but
usually represents some type of bronchiolitis -- it is
NOT pathognomic for endobronchial tuberculosis
Immune status of patient
o Immunosuppressed patients can have any number
of pathogens cause TlB pattern
Chronicity
o Chronic symptoms suggests an indolent infection
such as an atypical mycobacterial infection, or
immunologic disease
o Acute symptoms suggest acute infectious
bronchiolitis such as mycoplasma infection
Co-morbidities such as Sjogren syndrome or known
primary malignancy
I SELECTED REFERENCES
1. Heo IN et al: Pulmonary tuberculosis: another disease
showing clusters of small nodules. AJRAmJ Roentgenol.
184(2):639-42, 2005
2. Pipavath 5J et al: Radiologic and pathoiogic features of
bronchiolitis. AJRAm J Roentgenol. 185(2):354-63, 2005
3. Rossi5Eet al: Tree-in-bud pattern at thin-section CT of the
lungs: radiologic-pathologic overview. Radiographies.
25(3):789-801,2005
4. Jeong VJet al: Nontuberculous mycobacterial pulmonary
infection in immunocompetent patients: comparison of
thin-section CT and histopathologic findings. Radiology.
231(3):880-6,2004
5. Franquet T et al: Disorders of the small airways:
high-resolution computed tomographic features. 5emin
Respir Crit Care Med. 24(4):437-44, 2003
6. Franquet T et ai: Infectious pulmonary nodules in
immunocompromised patients: usefulness of computed
tomography in predicting their etiology. J Comput Assist
Tomogr. 27(4):461-8, 2003
7. Eisenhuber E:The tree-in-bud sign. Radiology.
222(3):771-2,2002
8. Franquet T et al: Thrombotic microangiopathy of
pulmonary tumors: a vascular cause of tree-in-bud pattern
on CT. AJRAmJ Roentgenol. 179(4):897-9,2002
9. Waitches GM et al: High-resolution CT of peripheral
airways diseases. Radiol Clin North Am. 40(1):21-9, 2002
10. Collins): CT signs and patterns of lung disease. Radiol Clin
North Am. 39(6):1115-35, 2001
11. Hansell DM: 5mall airways diseases: detection and insights
with computed tomography. Eur RespirJ. 17(6):1294-313,
2001
12. Tack D et al: Tree-in-bud pattern in neoplastic pulmonary
emboli. AJRAm J Roentgenol. 176(6):1421-2,2001
13. LeeJV et al: Pulmonary tuberculosis: CT and pathoiogic
correlation. J Comput AssistTomogr. 24(5):691-8, 2000
14. Collins Jet al: CT patterns of bronchiolar disease: what is
"tree-in-bud"? AJRAmJ Roentgenol. 171(2):365-70, 1998
15. Hong 5H et al: High resolution CT findings of miliary
tubercuiosis. J Comput AssistTomogr. 22(2):220-4, 1998
16. Aquino 5Let al: Tree-in-bud pattern: frequency and
significance on thin section CT.J Comput AssistTomogr.
20(4):594-9, 1996
17. ImJG et al: CT-pathology correlation of pulmonary
tuberculosis. Crit Rev Diagn Imaging. 36(3):227-85,1995
18. 1mJG et al: Pulmonary tuberculosis: CT findings--early
active disease and sequential change with antituberculous
therapy. Radiology. 186(3):653-60, 1993
 HReT: TREE-IN-BUD PATTERN

I IMAGE GALLERY 1
53

(Left) Transverse NECT

shows diffuse Tl8 pattern
(arrows) from this patient
with infectious bronchiolitis.
Note the many clusters Df
nodules, each having a
centrilobular distribution.
(Right) Transverse HRCT
shows innumerable
ill-defined centrilobular hazy
opaciNes (arrows) from a
smoker with respiratory
bronchiolitis, as a differential
dx case to contrast with
Dther cases Df Tl8 pattern.

(Left) Transverse NECT

shows cavitary destruction
and air-fluid level in right
lung from tuberculDsis, and
endDbrDnchial spread intD
left lung (arrDw), with Tl8
pattern, suggesting active
infectiDn. (Right) Transverse
NEeT shows extensive
microangiopathic tumor
embDIi throughout the right
lung (arrows) from a primary
atrial sarcoma, causing a
rare, nDn-airway-related, Tl8
pattern.

Typical
(Left) Transverse NECT
shDws patchy, bilateral Tl8
pattern in a
gravity-dependent
distribution (open arrows),
in case D( eSDphageal
cancer, prestenolic dilation
(arrow), and resulting
chronic aspiratiDn. (Right)
Transverse NECT shDws
diffuse Tl8 pattern from this
patient with mycoplasma
infection and resulting
infectious bronchiolitis
(arrDws).
 ANTERIOR MEDIASTINAL MASS
1
54

Sagittal graphic shows various components of the Lateral radiograph shows anterior mediastinal mass from
mediastinum. Anterior mediastinum includes region in Hodgkin lymphoma (arrows) filling in retrostema/ clear
red farrow). Middle mediastinum is colored gold and space and obscuring the anterior border of the
posterior mediastinum is colored blue. ascending aorta.

o Filling in of retrosternal clear space on lateral view

ITERMINOLOGY o Cervicothoracic sign: Loss of interface of mass with
Definitions lung above clavicular heads when anterior
Anatomy of anterior mediastinum o Thyroid lesion usually contiguous with gland
o Extends from thoracic inlet to diaphragm Location
o Bounded anteriorly by sternum o Lymph nodes: Variable in location
o Bounded posteriorly by ascending aorta, Cardiophrenic nodes: Posterior to xiphoid and 7th
brachiocephalic vessels, superior vena cava and coastal cartilage, anterior to pericardium
anterior pericardium Internal mammary nodes: Medial intercostal
Felson considers anterior border of trachea and spaces adjacent to internal mammary artery and
posterior pericardium as posterior border vein
o Lateral borders defined by mediastinal pleura o Thymus: Anterior-superior mediastinum extends
o No fascial planes separating from middle from above manubrium to fourth costal cartilage
mediastinum Involutes over time; seen in less than 20% of
o Contents of normal anterior mediastinum: Fat, subjects over 50 by CT
thymus, lymph nodes, internal mammary vessels o Anterior mediastinal thyroid lesions usually descend
May contain thyroid, parathyroid or germ cell to left of trachea
rests Size
o Normal lymph nodes should be less than 1 em in
short-axis
I IMAGING FINDINGS o Thymus: In adults width less than 2 em; thickness
less than 0.8 em
General Features Morphology
Best diagnostic clue

DDx: Anterior Mediastinal Mass Mimics

Lung Cancer Right Ventricular Hypertrophy Sternal Mass (Plasmacytoma)

 ANTERIOR MEDIASTINAL MASS

Key Facts
1
Terminology Top Differential Diagnoses 55
Anatomy of anterior mediastinum Primary Lung Neoplasm
Extends from thoracic inlet to diaphragm Cardiovascular Lesions
Bounded anteriorly by sternum Lesions Arising in Sternum
Bounded posteriorly by ascending aorta,
brachiocephalic vessels, superior vena cava and
Clinical Issues
anterior pericardium Benign lesions often incidental finding; occasional
Contents of normal anterior mediastinum: Fat, symptoms due to mass effect
thymus, lymph nodes, internal mammary vessels Aggressive lesions usually symptomatic due to
invasion or mass effect
Imaging Findings
Filling in of retrosternal clear space on lateral view
Diagnostic Checklist
Well-defined mass with invisible medial margin on Always consider vascular lesions in differential for
frontal radiograph mediastinal masses
Best imaging tool: CT to delineate size, location, Assess for connection to thyroid gland in superior
morphology and content anterior mediastinal mass

o Normal lymph node morphology: Oblong or Hematogenous metastases to liver and lung
triangular with fatty hilum o Thymolipoma
o Normal thymus: Usually bilobed draped over Variable amounts of fat; soft, pliable lesion
ascending aorta May change position on subsequent scans
Lymphoma
Radiographic Findings o Non-Hodgkin lymphoma
Radiography Rarely isolated to anterior mediastinum;
o Anterior junction line: Approximation of right and associated with other mediastinal and
left lungs anteriorly extra-thoracic adenopathy
Separated by four layers of pleura and variable Calcification absent prior to treatment
amount of fat o Hodgkin lymphoma
o Anterior pleural reflection: Superior interface of lung Nodal aggregation, may be limited to anterior
with anterior mediastinal structures mediastinum
On left should be medial to transverse aortic arch Cervical adenopathy with contiguous nodal
o General findings of anterior mediastinal mass spread
Well-defined mass with invisible medial margin Thymic enlargement
on frontal radiograph Lesions of germ cell origin
Mass in retrosternal clear space with obscuration o Mature teratoma
of ascending aorta on lateral exam Variable fat, calcification and soft tissue; fatlfluid
If overlying lung, should be able to visualize layer
normal lung parenchyma through mass Well-defined borders
CT Findings o Immature teratoma
Obliteration of normal fat planes; hemorrhage
CT appearance dependent on site of origin and
and cystic degeneration
pathology
o Mediastinal seminoma
o Lesion should be centered in anterior mediastinum
Usually homogeneous with occasional cysts;
Thyroid lesions
calcifications rare
o Majority are multinodular goiter
o Non-seminomatous germ cell tumor (NSGCT)
o Heterogeneous density in continuity with thyroid
Heterogeneous with hemorrhage, cystic necrosis
gland
and calcifications
Often have punctate, course or ring calcifications
Locally invasive, obliterates adjacent fat planes
and cysts
May have pleural effusion
o High attenuation on NECT due to iodine content
Lipomatosis
o May extend into middle or posterior mediastinum
o Abundant fat tissue without mass effect
Thymic lesions
o Thymoma MR Findings
Oval, lobulated mass Thyroid: Heterogeneous signal with both Tl and T2
Often homogeneous; occasional calcification or Thymoma: Isointense to muscle on Tl; hyperintense
cyst formation to muscle (but less than fat) on T2
Invasive thymoma may have pleural drop Lymphoma: Isointense to muscle on Tl, hyperintense
metastases on T2
o Thymic carcinoma o Multiple involved nodes
Irregular margins with local invasion Teratoma: Hyperintense Tl signal due to regions of fat
 ANTERIOR MEDIASTINAL MASS
1 o Cysts may have variable signal depending on
content (water, fat, protein)
Non-Hodgkin lymphoma: Pruritus, erythema
nodosum, coagulopathy, hypercalcemia
56 Lipomatous lesions: Follows subcutaneous fat on all Thymoma: Myasthenia gravis, red cell aplasia,
sequences hypogammaglobulinemia
Thymic carcinoid: Cushing syndrome, multiple
Imaging Recommendations endocrine neoplasia type 1
Best imaging tool: CT to delineate size, location, o Laboratory abnormalities
morphology and content Goiter: Elevated or decreased thyroid stimulating
Protocol advice: Contrast useful to delineate hormone, T3, T4
relationship to vascular structures Seminoma: Beta human chorionic gonadotropin
Non-seminomatous germ cell tumor: Alpha
feto-protein
I DIFFERENTIAL DIAGNOSIS
Natural History & Prognosis
Primary lung Neoplasm Dependent on histology
Lesion centered in lung parenchyma often with
irregular borders Treatment
Observation: Lipomatosis, goiter, mature teratoma
Cardiovascular lesions Surgical excision: Thymoma, thymolipoma, mature
Ascending aortic aneurysm teratoma (symptomatic)
o Contiguous with transverse aorta; fills in posterior Chemotherapy and/or radiation therapy: Lymphoma
portion of retrosternal clear space on lateral view Combination of approaches: Invasive thymoma,
Right ventricular hypertrophy thymic carcinoma, thymic carcinoid, seminoma,
o Fills in lower aspect of retrosternal clear space on NSGCT
lateral
o Elevates base of heart and left ventricular apex on
frontal view I DIAGNOSTIC CHECKLIST
o May be associated with enlarged central pulmonary
arteries Consider
Always consider vascular lesions in differential for
lesions Arising in Sternum mediastinal masses
Osteomyelitis with abscess Middle mediastinal mass or structure displacing or
o Associated with erosion of cortex or sternal involving anterior mediastinal compartment
dehiscence Lesion in lung invading mediastinum
Fracture with hematoma
o Trauma; fracture line may be difficult to visualize Image Interpretation Pearls
Bone neoplasms No fascial planes separate anterior and middle
o Expansion of sternum or destruction of posterior mediastinum
cortex of sternum o Middle mediastinal lesion may cross into anterior
o May have chondroid or osteoid matrix mediastinum
o Assess location where mass is centered
Middle Mediastinal Mass Assess for connection to thyroid gland in superior
Mass centered posterior to anterior mediastinal anterior mediastinal mass
boundaries Assess whether thymus is involved or separate from
mass
Assess for local invasion and sites of distant disease
IClINICAllSSUES
Presentation
Most common signs/symptoms
I SElECTED REFERENCES
o Benign lesions often incidental finding; occasional 1. Macchiarini Pet al: Uncommon primary mediastinal
symptoms due to mass effect tumours. Lancet Oncol. 5(2):107-]8, 2004
2. Kim JH et al: Cystic tumors in the anterior mediastinum.
o Aggressive lesions usually symptomatic due to
Radiologic-pathological correlation. J Comput Assist
invasion or mass effect Tomogr. 27(5):714-23, 2003
Cough, chest pain 3. Yoneda KYet al: Mediastinal tumors. Curr Opin Pulm Med.
Vocal cord paralysis due to recurrent laryngeal 7(4):226-33,2001
nerve involvement 4. Ronson RS et al: Embryology and surgical anatomy of the
Airway compromise with thymic lymphoma mediastinum with clinical implications. Surg Clin North
Other signs/symptoms Am. 80(1):157-69, x-xi, 2000
o Constitutional symptoms 5. Wood DE: Mediastinal germ cell tumors. Semin Thorac
Cardiovasc Surg. 12(4):278-89,2000
Lymphoma: "B" symptoms (fever, weight loss,
6. Strollo DC et al: Primary mediastinal tumors. Part 1:
night sweats) tumors of the anterior mediastinum. Chest. ]] 2(2):5] 1-22,
Goiter: Hypo- or hyperthyroidism 1997
o Paraneoplastic symptoms
 ANTERIOR MEDIASTINAL MASS
I IMAGE GALLERY 1
57
Typical
(Left) Frontal radiograph
shows non-Hodgkin
lymphoma with anterior
component on left (arrows)
confirmed by loss of lung
interface above thoracic inlet
(open arrow). Note posterior
mediastinal component on
right with preserved lung
interface above thoracic inlet
(curved arrow). (Right)
Sagittal oblique CECT shows
Hodgkin lymphoma with
small cystic component
(while arrow) anterior to
ascending aorta (black
arrow) and innominate
artery (curved arrow).

Typical
(Left) Frontal radiograph
shows mature teratoma
(open arrow) that does not
obscure middle and
posterior mediastinal
structures: Descending aorta,
aortica-pulmonary window
or left pulmonary artery
(;':urow) confirming anterior
location. (Right) Axial CECT
shows non-seminomatous
germ celllumor with
heterogeneous density and
punctate calcification
(arrow). Note also
mediastinal adenopathy
(open arrow) and left pleural
effusion (curved arrow).

(Left) Lateral radiograph

shows large mass in right
breast (arrow) with
elongated soft tissue mass
just posterior to sternum
(open arrows) from internal
mammary adenopathy.
(Right) Axial CECT shows
thymic hyperplasia (open
arrows) with small
multilocular thymic cysts
(curved arrows) and
punctate calcifications
(arrow).
 MIDDLE MEDIASTINAL MASS
1
58
Sagittalgraphic shows middle mediastinal compartment
colored in gold extending from anterior margin of heart
and great vessels to the thoracic vertebrae. Anterior
mediastinum in red; posterior mediastinum in blue.
ITERMINOLOGY
Definitions
Anatomy of middle mediastinum
o Extends from thoracic inlet to diaphragm
o Bounded anteriorly by innominate artery, ascending
aorta and anterior pericardium
Felson considers anterior border to extend along
anterior margin of trachea and posterior
pericardium
o Posterior border formed by anterior 1/3 of vertebral
bodies
o Lateral borders formed by mediastinal pleura and
hilar structures
o Essentially a visceral compartment
Contains heart, pericardium, thoracic aorta,
trachea, esophagus, phrenic and vagus nerves, fat
and lymph nodes
Right para tracheal stripe
o Right paratracheal stripe should not be greater than
4 mm thick
o Azygos vein enters at inferior margin of stripe;
should not exceed 1 em transverse diameter
DDx: Vascular Variants Mimicking Mass
Right Aortic Arch Retroaortic Left Brachiocephalic
Axial CECT shows boundary of middle and anterior
mediastinum at anterior margin of ascending aorta and
main pulmonary artery (arrows) and middle and
posterior mediastinum just behind anterior margin of
vertebralbody (open arrows).
o Interface of lung with superior vena cava should not
be confused with right para tracheal stripe
Left para tracheal reflection
o Contact of left lung with middle mediastinum
o Includes left tracheal wall, left common carotid and
left subclavian arteries
Retrotracheal space
o Radiolucent triangular area defined by posterior
tracheal wall, anterior margin of vertebral bodies
and superior margin of transverse thoracic aorta
o Trachea-esophageal stripe should be less than 6 mm
Aortic-pulmonic window
o Bound by inferior margin of transverse aortic arch
and superior margin of left pulmonary artery
o Normal appearance of aortic-pulmonic window:
Concave toward mediastinum
Azygoesophageal recess
o Posterior or lateral to esophagus and anterior to
spine and azygous vein
o Extends from azygos arch inferiorly to aortic hiatus
o Normal azygoesophageal recess: Smooth interface,
convex to the left
Pleuro-esophageallines
Vein Dilated Superior Intercostal Vein
 MIDDLE MEDIASTINAL MASS
Key Facts
1
Terminology CT for documentation of location and lesion 59
Extends from thoracic inlet to diaphragm morphology
Bounded anteriorly by innominate artery, ascending Contrast necessary for evaluation of the heart and
aorta and anterior pericardium aorta
Posterior border formed by anterior 1/3 of vertebral Contrast helpful for delineating relationship of other
bodies masses to aorta and pulmonary arteries
Right paratracheal stripe should not be greater than 4 Top Differential Diagnoses
mm thick Primary Lung Neoplasm
Trachea-esophageal stripe should be less than 6 mm Vascular Variants
Normal appearance of aortic-pulmonic window: Anterior Mediastinal Mass
Concave toward mediastinum
Normal azygoesophageal recess: Smooth interface, Diagnostic Checklist
convex to the left Mediastinal masses should be considered vascular
until proven otherwise
Imaging Findings
No fascial planes separate middle mediastinum from
Displacement of normal mediastinal lines and anterior and posterior mediastinum
interfaces

o Created by lung contacting wall of air-filled

esophagus
o Infrequently present
I IMAGING FINDINGS
General Features
Best diagnostic clue
o Displacement of normal mediastinal lines and
interfaces
o Mass obscuring normal middle mediastinal
structures
Location
o Lymph nodes usually multiple at various locations
throughout mediastinum
o Bronchogenic cysts usually near tracheal carina
o Pericardial cyst most often at right cardiophrenic
angle
Radiographic Findings
Radiography
o Widening of right paratracheal stripe
Due to adenopathy adjacent to tracheal wall
o Widening of left para tracheal reflection
Adenopathy from neoplasm or infection
Left superior vena cava
o Anterior deviation of trachea
Esophageal mass or dilation due to achalasia or
tumor obstruction
Aberrant subclavian artery with retroesophageal
diverticulum (diverticulum of Kommerell)
o Abnormal contour in aortic-pulmonic window
Most often adenopathy from primary lung
neoplasm or lymphoma
If phrenic nerve involved may be ipsilateral
diaphragmatic elevation
o Abnormal contour of azygo-esophageal recess
Subcarinal mass due to adenopathy or
bronchogenic cyst
Enlarged left atrium may simulate subcarinal mass
Hiatal hernia, esophageal mass and esophageal
varices may cause abnormality in inferior recess
CT Findings
Lymphadenopathy
o Size greater than 1 em short axis considered
abnormal
o Round shape with absence of fatty hilum
o Assess for primary iung neoplasm
Primary lung lesion may not be visible when due
to small cell carcinoma
o Lymphoma may result in conglomeration of nodes
with extension into other compartments
May be indistinguishable from adenopathy due to
small cell carcinoma
Mediastinal cysts
o Bronchopulmonary foregut cyst
Well-circumscribed lesion with imperceptible wall
Variable density of cyst fluid; serous or
proteinaceous, may contain milk-of-calcium
Presence of air in cyst suggests superimposed
infection
o Pericardial cyst
Imaging characteristics similar to
bronchopulmonary foregut cyst
Tracheal lesions
o Predominant intraluminal component with or
without invasion into adjacent mediastinal fat
Esophageal lesions
o Esophageal cancer
Small mucosal lesions usually imperceptible
Large eccentric mass with peri-esophageal or celiac
lymph nodes
o Esophageal leiomyoma
Submucosal mass often with smooth borders
o Esophageal varices
Paraesophageal varices: Serpiginous vessels in
mediastinal fat adjacent to esophagus
Esophageal varices: Collateral vessels in
esophageal wall; seen best with intravenous
contrast in portal venous phase
Aortic lesions
o Aneurysm may be fusiform or saccular
o Saccular aneurysm may be confused with other
masses if contrast not given
 MIDDLE MEDIASTINAL MASS
1 Thyroid lesions
o Goiter arising from posterior aspect of gland may Posterior Mediastinal Mass
60 descend into middle mediastinum Large schwannoma or neurofibroma
o Usually connect to thyroid gland
o Heterogeneous attenuation due to iodine content,
cysts and calcifications I CLINICAL ISSUES
MR Findings Presentation
Mediastinal cysts generally high T2 signal and variable Most common signs/symptoms: Depending on size
T1 signal depending on cyst content and etiology may be asymptomatic
Other signs/symptoms
Fluoroscopic Findings o Symptoms due to compression of structures
Esophagram Dyspnea, stridor or wheezing due to tracheal
o Extrinsic compression of esophagus due to compression
adenopathy, aberrant vasculature Dysphagia due to esophageal compression or
o Internal stricture from esophageal carcinoma esophageal tumor
Jugular venous distension due to superior vena
Nuclear Medicine Findings
cava compression
Tc-99m pertechnetate may show ectopic gastric
Vocal cord paralysis due to involvement of
mucosa in esophageal duplication cyst
recurrent laryngeal nerve
Ultrasonographic Findings Diaphragm dysfunction or hiccups due to phrenic
Mediastinal cysts usually anechoic with imperceptible nerve involvement
wall at trans-esophageal ultrasound o Constitutional symptoms
Abnormal lymph nodes tend to be round and Fever, weight loss and night sweats due to
hypoechoic without fatty hilum at endoscopic lymphoma
ultrasound o Paraneoplastic syndromes
Pruritus, erythema nodosum, coagulopathy,
Imaging Recommendations hypercalcemia in non-Hodgkin lymphoma
Best imaging tool
o CT for documentation of location and lesion
morphology I DIAGNOSTIC CHECKLIST
o Endoscopic ultrasound for local staging of
esophageal carcinoma Consider
Protocol advice Medial lung lesion invading middle mediastinum
o Contrast necessary for evaluation of the heart and
Image Interpretation Pearls
aorta
Contrast helpful for delineating relationship of Mediastinal masses should be considered vascular
other masses to aorta and pulmonary arteries until proven otherwise
No fascial planes separate middle mediastinum from
anterior and posterior mediastinum
I DIFFERENTIAL DIAGNOSIS o Anterior and posterior mediastinal masses may
extend into middle mediastinum
Primary Lung Neoplasm Thyroid lesions may descend into middle
Non-small cell lung cancer invading or abutting mediastinum: Consider as etiology for lesion at
mediastinum thoracic inlet
Irregular lateral borders o Assess for connection to thyroid gland

Vascular Variants
Right aortic arch I SELECTED REFERENCES
Left superior vena cava 1. Abiru H et al: Normal radiographic anatomy of thoracic
Partial anomalous pulmonary venous return from left structures: analysis of 1000 chest radiographs in Japanese
upper lobe population. BrJ Radiol. 78(929):398-404, 2005
Dilated collateral veins with central venous 2. Duwe BVet al: Tumors of the mediastinum. Chest.
obstruction 128(4):2893-909,2005
Large azygos vein in azygos continuation of inferior 3. Franquet T et al: The retrotracheal space: normal anatomic
vena cava and pathologic appearances. Radiographies. 22 5pec
No:S231-46, 2002
Anterior Mediastinal Mass 4. Jeung MYet al: Imaging of cystic masses of the
Large neoplasms may displace or extend into middle mediastinum. Radiographies. 22 Spec No:S79-93, 2002
5. RavenelJG et al: Azygoesophageal recess.J Thorac Imaging.
mediastinum 17(3):219-26,2002
o Thymic lymphoma 6. Strollo DC et al: Primary mediastinal tumors: part II.
o Non-seminomatous germ cell tumor Tumors of the middle and posterior mediastinum. Chest.
112(5):1344-57, 1997
 MIDDLE MEDIASTINAL MASS
I IMAGE GAllERY 1
61
Typical
(Left) Frontal radiograph
shows adenopathy from
sarcoidosis widening the
right paratracheal stripe
(arrow), filling in
aortic-pulmonic window
(curved arrow) and causing
abnormal contour in upper
azygo-esophageal recess
(open arrow). (Right) Frontal
radiograph shows mass in
aortic-pulmonic window
(arrow) displacing left
paratracheaf reflection
(curved arrow) with
paralysis of left diaphragm
(open arrow) due to phrenic
nerve involvement.

(Left) Axial CECT shows

enlarged mediastinal lymph
nodes involving right
paratracheal (black arrow),
left paratracheal (white
arrow), aortic-pulmonic
window (black open arrow)
and prevascular (white open
arrow) node groups due to
small cell carcinoma. (Right)
Coronal CECT shows
esophageal mass below the
level of the carina displacing
azygoesophageal recess to
the right (open arrows) and
causing obstruction
superiorly with dilated
fluid-filled lumen (arrow).

Typical
(Left) Axial CECT shows
heterogeneous mass
involving all compartments
of the middle mediastinum
(white arrows) compressing
superior vena cava (black
arrow) and extending into
the anterior mediastinum
(open arrows). (Right)
Frontal radiograph shows
large well-circumscribed
mass (open arrow) that
obscures the border of the
descending thoracic aorta
(arrow) as a result of a large
saccular aortic aneurysm.
 POSTERIOR MEDIASTINAL MASS
1
62

Sagittalgraphic shows posterior mediastinum colored in Axial CECT shows left posterior homogeneous
blue extending from just behind the anterior margin of enhancing mediastinal mass from neurofibroma
the vertebralbodies to the posterior chest wall. Anterior (arrow). Approximate division between middle and
mediastinum is in red, middle mediastinum is in gold. posterior mediastjnum delineated by open arrows.

ITERMINOlOGY IIMAGING FINDINGS

Definitions
Extent: Classifications differ due to lack of discrete
fascial planes
o Felson classification: Anterior margin of posterior
mediastinum begins 1 em posterior to anterior
longitudinal ligament
o Heitzman classification: Groups middle and
posterior mediastinum into supra-azygous,
infra-azygous, supra-aortic and infra-aortic
components
Advantage of describing lesion in context of
adjacent structures in each compartment
o Gray's anatomy: Posterior pericardium to thoracic
spine and medial posterior chest wall
Contents: Fat, lymph nodes, nerves
o Depending on classification scheme may contain
esophagus and descending thoracic aorta
General Features
Best diagnostic clue
o Cervicothoracic sign: Visible lung/mass interface
above clavicular head denotes posterior location
o Displacement of paras pinal lines
Location: Lesions lie beneath parietal pleura
Size: Variable
Morphology
o Vertical oriented mass suggests pathology in
sympathetic chain, multiple vertebral bodies, or
adenopathy
o Horizontal oriented mass suggests pathology of
nerve sheaths or single vertebral body
Radiographic Findings
Interfaces of posterior mediastinum
o Paraspinalline: Interface of lung with paras pinal fat
Do to oblique orientation, right paraspinalline
usually not seen on frontal radiograph
Visible right paras pinal line abnormal; thoracic
vertebra osteophytes most common cause

DDx: Paraspinal Mass

Lung Cancer Pleural Metastases Loculated Pleural Fluid

 POSTERIOR MEDIASTINAL MASS
Key Facts
Terminology
Felson classification: Anterior margin of posterior
mediastinum begins 1 em posterior to anterior
longitudinal ligament
Contents: Fat, lymph nodes, nerves
Imaging Findings
Cervicothoracic sign: Visible lung/mass interface
above clavicular head denotes posterior location
Displacement of paraspinallines
Vertical oriented mass suggests pathology in
sympathetic chain, multiple vertebral bodies, or
adenopathy
Horizontal oriented mass suggests pathology of nerve
sheaths or single vertebral body
Paraspinalline: Interface of lung with paraspinal fat
o Posterior junction line: Approximation of right and
left lungs posteriorly
Contains four layers of pleura and variable
amount of fat
o Increased density over posterior 2/3 of vertebral
body or neural foramen
May extend over several vertebral levels
o Incomplete border sign: Similar to extrapleural mass,
only 1 edge of mass tangential to X-ray and thus
border forming
o Cervicothoracic sign: Mass with preserved lung
interface above clavicular heads
Osseous abnormalities
o Erosion or destruction of vertebral body
o Rib abnormalities
Rib expansion in extramedullary hematopoiesis
Rib erosions due to plexiform neurofibromas in
neurofibromatosis-I
o Thoracic spine hemivertebra associated with
neurenteric cyst
o Posterior vertebral scalloping associated with dural
ectasia with neurofibromatosis and lateral
meningoceles
Associated abnormalities
o Cutaneous nodules in neurofibromatosis
o Punctate calcifications in mediastinal mass may be
visible in neuroblastoma
CT Findings
Sympathetic nerve tumors
o Neuroblastoma: Heterogeneous with areas of
hemorrhage, cystic degeneration and variable
enhancement
Majority will have regions of calcification
o Ganglioneuroblastoma and ganglioneuroma
generally homogeneous with and without
contrast-enhancement
o Paraganglioma: Rare, may enhance intensely
following contrast administration
Nerve sheath tumors
o Schwannoma: Often decreased attenuation due to
fluid or lipid content; calcification in 10%
1
Posterior junction line: Approximation of right and 63
left lungs posteriorly
CT to establish presence of mass and confirm
location
MRI to assess for extent into neural foramina and
spinal cord involvement
Top Differential Diagnoses
Pleural metastases
Primary lung neoplasm
Thoracic osteophytes
Diagnostic Checklist
Assess for involvement of neural foramina and
intraspinal extension
Calcifications in mass suggest neuroblastoma in child
May have dumbbell shape with extension into
spinal canal
Variable enhancement
o Neurofibroma: Similar to schwannoma; more often
homogeneously enhance
May have early central contrast blush
Lateral meningocele: Fluid density contiguous with
thecal sac; widens neural foramina
o May occur at multiple levels
o Associated with neurofibromatosis-I
May have multiple plexiform neurofibromas and
skin nodules
Neurenteric cyst
o Vertical fluid-filled structure with associated
vertebral body anomaly superior to lesion
Lymphoma: Enlarged lymph nodes rarely isolated to
posterior mediastinum
Metastases: Rarely isolated to posterior mediastinum
o Hematogenous dissemination to bone
o Lymphatic spread from abdomen: Renal cell
carcinoma and testicular neoplasms
o Direct extension: Lung and esophageal cancer
Aneurysm: Fusiform or saccular dilation of aorta
Extramedullary hematopoiesis: Mixed soft tissue and
fat without calcification or osseus erosion; may be
multiple
o Variable, heterogeneous enhancement with contrast
o Associated with marrow expansion; may be seen in
ribs
Paraspinal abscess
o Centered on intervertebral disc with central low
attenuation
Paraspinal hematoma
o Associated with vertebral body fractures in trauma
o May not be obvious on axial images; sagittal and
coronal reformations may be necessary
MR Findings
Sympathetic nerve tumors
o Neuroblastoma: Heterogeneous with areas of high
Tl signal due to hemorrhage and high T2 signal due
to cystic degeneration
 POSTERIOR MEDIASTINAL MASS
o Ganglioneuroblastoma and ganglioneuroma:
Homogeneous; ganglioneuroma may have whorled
IClINICAllSSUES
64 appearance Presentation
o Paraganglioma: Enhances strongly with gadolinium Most common signs/symptoms
May contain multiple vascular flow voids o Dependent on histology and location
Nerve sheath tumors May be incidental finding particularly for benign
o Signal intensity similar to spinal cord lesion
o May be difficult to see on T2 sequences due to Constitutional symptoms including weight loss
similar signal to cerebrospinal fluid and fatigue
o Neurofibromas may have central high T1 and low
Other signs/symptoms
T2 signal due to dense collagen deposition o Signs from local invasion
Central high T2 signal in neurofibroma
Back pain
Paras pinal abscess Horner syndrome: Ptosis, pupillary constriction,
o High T2 signal in abscess cavity ipsilateral facial anhidrosis, flushing
o Marrow edema due to secondary osteomyelitis o Neurologic deficits from extension through neural
Nuclear Medicine Findings foramina
Tc-99m Sulfur Colloid: Uptake may be seen in o Para neoplastic syndromes
extramedullary hematopoiesis Neuroblastoma: Opsoclonus-myoclonus,
MIBG Scintigraphy vasoactive intestinal peptide
o Uptake dependent on catecholamine production o Symptoms due to catecholamine release
Ancillary diagnostic test for neuroblastoma and Hypertension, tachycardia, flushing, headache,
paraganglioma elevated hematocrit due to vasoconstriction
30% of neuroblastoma are not MIBG avid o Anemia often related to congenital disease such as
Poor sensitivity for ganglioneuroma sickle cell and thalassemia in extramedullary
hematopoiesis
Imaging Recommendations
Demographics
Best imaging tool
o CT to establish presence of mass and confirm Age
location o Neuroblastoma occurs in childhood
o MRI to assess for extent into neural foramina and o Other posterior mediastinal masses typically
spinal cord involvement detected from adolescence through 5th decade of
Protocol advice: May get NECT to assess for presence life
of calcifications Treatment
Depends on etiology
Surgical excision of solitary neurofibroma and
I DIFFERENTIAL DIAGNOSIS schwannoma
Pleural lesions
Pleural metastases
o Multiple small nodules seen elsewhere in pleura;
I DIAGNOSTIC CHECKLIST
associated with pleural effusion Consider
Fibrous tumor of pleura Lesion may be arising from middle mediastinum or
o Difficult to distinguish unless on pedicle adjacent lung
o Pedunculated lesions mobile on serial imaging Extramedullary hematopoiesis in patients with
Loculated pleural fluid chronic anemia and multiple posterior mediastinal
o Lentiform shape; often loculated fluid elsewhere masses
lung lesions Neurofibromatosis-l when multiple neural tumors are
Primary lung neoplasm present
o Irregular borders; acute angle of mass with chest wall o Assess for associated cutaneous nodules and lateral
meningocele
Vertebral Body lesions
Image Interpretation Pearls
Thoracic osteophytes
o Dense ossification arising from vertebral body Assess for involvement of neural foramina and
Primary bone neoplasm intraspinal extension
o Expansile lesion arising in vertebral body; may Sympathetic ganglion tumors have vertical axis; nerve
contain osteoid or chondroid matrix sheath tumors have horizontal axis
o Osteoblastoma and aneurysmal bone cyst in Calcifications in mass suggest neuroblastoma in child
posterior elements
I SElECTED REFERENCES
I. Duwe BV et al: Tumors of the mediastinum. Chest.
128(4):2893-909, 200S
 POSTERIOR MEDIASTINAL MASS
I IMAGE GALLERY 1
65

(Left) Frontal radiograph

shows well-de(ined mass
(arrow) that makes obtuse
margins on the superior and
inFerior ends (open arrows)
indicating extraparenchymal
location. (Right! Lateral
radiograph in same patient
shows mass overlying the
posterior margin of vertebrae
(arrows) confirming location
as posterior mediastinal
mass. Diagnosis is
neurofibroma.

Typical
(Left) Frontal radiograph
shows bilateral symmetric
widening of the paraspinal
fines (white arrows) with
associated loss of disc space
and endplate sclerosis (black
arrow) due to discitis with
paraspinal abscess. (Right!
Axial T2WI MR shows
well-circumscribed posterior
mediastinalschwannoma
(arrow) with homogeneous
increased signal relative to
paraspinal muscles without
extension through neural
foramina.

(Left! Axial T2WI FS MR

shows high T2 signal cystic
mass (arrow) in continuity
with cerebrospinal fluid
causing smooth widening of
neural Foramina (open
arrows). Lateral meningocele
in neurofibromatosis-I.
(Right! Axial CECT shows
mixed soft tissue and
lipomatous posterior
mediastinal mass (arrows) in
patient with beta thalassemia
due to extramedullary
hematopoiesis.
 INDEX
A Agricu]tura] lung diseases, 1I:4-46i, 1I:4-47
Abscess
achalasia vs., 1I:1-10i, 11:1-11
lung. See Lung abscess
paraspina]
nerve sheath tumor vs., 1I:1-58i, 1I:1-59
sympathetic ganglion tumor vs., II:1-63
spina], fracture vs., IV:2-26
subphrenic, diaphragmatic rupture vs., ]V:2-31
Accessory fissures, IV:1-7 to IV:1-8
Acha]asia, II:1-10 to 11:1-11
aberrant right subclavian artery vs., II:2-8
aortic aneurysm vs., 1I:2-41
differentia] diagnosis, 1I:1-lOi, 1I:1-11
mediastina] fibrosis vs., II:1-7
pneumomediastinum vs., ]V:2-3
Acquired immunodeficiency syndrome. See AIDS
(acquired immunodeficiency syndrome)
Actinomycosis, III:2-28i
Acute respiratory distress syndrome (ARDS)
acute interstitia] pneumonia vs., 1:1-56i, 1:1-57
aspiration vs., IV:5-15
fat embolism vs., 1:1-41
lipoid pneumonia vs., 1:1-69
pulmonary alveolar proteinosis vs., ]:1-73
sickle cell disease vs., IV:6-22i, IV:6-23
Adenocarcinoma
acute interstitial pneumonia vs., 1:1-57
lipoid pneumonia vs., I:1-68i, 1:1-69
metastatic
asbestos related pleura] disease vs., III:1-53
malignant mesothelioma vs., III:1-28i,
III:1-29
sp]enosis vs., IV:2-38i, IV:2-39 to IV:2-40
posttransp]ant, IV:4-42i, IV:4-43
pulmonary carcinoid vs., 1:3-94i, 1:3-95
Adenoid cystic carcinoma, 1:3-94i, 1:3-95
Adenomatoid malformation
focal decreased density lung disease vs., V:1-22
pan]obu]ar emphysema vs., 1:3-73
Adenopathy
idiopathic pulmonary artery dilatation vs.,
1I:4-14
pulmonary artery hypertension vs., 1I:4-54i,
1I:4-55
AIDS (acquired immunodeficiency syndrome),
IV:6-18 to IV:6-20, IV:6-2li
differentia] diagnosis, IV:6-18i, ]V:6-20
Air cyst, paratracheal. See Paratrachea] air cyst
Air embolism, IV:2-42 to IV:2-44, IV:2-45i
differentia] diagnosis, IV:2-42i, IV:2-43
Air trapping, V:1-42i, V:1-43
Airspace, 1:1-2 to 1:1-87
alveolar microlithiasis, 1:1-64 to 1:1-66, I:1-67i
aspergillosis, 1:1-20 to 1:1-22, I:1-23i
blastomycosis, 1:1-24 to 1:1-25
bronchio]oa]veo]ar cell carcinoma, 1:1-84 to
]:1-86,1:1-87i
coccidioidomycosis, I:1-26 to I:1-27
diffuse a]veolar hemorrhage, 1:1-44 to 1:1-46,
I:1-47i
fat pulmonary embolism, 1:1-40 to 1:1-42,
I:1-43i
Goodpasture syndrome, 1:1-48 to 1:1-50, I:1-5li
histoplasmosis, 1:1-16 to 1:1-18, 1:1-19i
lung abscess, ]:1-12 to 1:1-14, I:1-15i
pneumonia
acute interstitia], 1:1-56 to 1:1-58, I:1-59i
bacterial, 1:1-2 to 1:1-4, 1:1-5i
cryptogenic organizing, 1:1-80 to 1:1-82,
I:1-83i
desquamative interstitia], 1:1-76 to 1:1-78,
I:1-79i
eosinophilic, 1:1-52 to 1:1-54, 1:1-55i
lipoid, 1:1-68 to 1:1-70, I:l-71i
mycobacteria], 1:1-8 to 1:1-10, I:l-lli
parasitic, 1:1-28 to 1:1-30, I:1-3li
Staphylococcus, 1:1-6 to 1:1-7
pulmonary alveo]ar proteinosis, 1:1-72 to 1:1-74,
1:1-75i
pulmonary calcification, metastatic, 1:1-60 to
1:1-62,I:1-63i
pulmonary edema
cardiogenic, 1:1-32 to 1:1-34, I:1-35i
noncardiac, 1:1-36 to 1:1-38, I:1-39i
Airway obstruction
cardiac volvu]us vs., IV:4-51
chronic, asthma vs., ]:3-69
foreign body
 INDEX
allergic bronchopulmonary aspergillosis vs.,
1:3-47
asthma vs., 1:3-69
Airways, 1:3-2 to 1:3-99
allergic bronchopulmonary aspergillosis, 1:3-46
to 1:3-48, 1:3-49i
alpha-l antiprotease deficiency, 1:3-22 to 1:3-24,
1:3-25i
amyloidosis, 1:3-88 to 1:3-90, 1:3-9li
anomalous bronchi, 1:3-18 to 1:3-20, 1:3-2li
asthma, 1:3-68 to 1:3-70, 1:3-71i
bronchial atresia, 1:3-14 to 1:3-16, 1:3-17i
bronchiectasis, 1:3-34 to 1:3-36, 1:3-37i
bronchiolitis obliterans, 1:3-64 to 1:3-66,
1:3-67i
bronchitis, chronic, 1:3-30 to 1:3-32, 1:3-33i
carcinoid, 1:3-94 to 1:3-96, 1:3-97i
cystic fibrosis, 1:3-2 to 1:3-4, 1:3-5i
emphysema
centrilobular, 1:3-84 to 1:3-86, 1:3-87i
panlobular, 1:3-72 to 1:3-74, 1:3-75i
immotile cilia syndrome, 1:3-10 to 1:3-12,
1:3-13i
Kaposi sarcoma, 1:3-98 to 1:3-99
Langerhans cell granulomatosis, 1:3-76 to
1:3-78, 1:3-79i
laryngeal papillomatosis, 1:3-42 to 1:3-44,
1:3-45i
middle lobe syndrome, 1:3-58 to 1:3-59
mycobacterial avium complex, 1:3-38 to 1:3-40,
1:3-4li
paratracheal air cyst, 1:3-26 to 1:3-27
relapsing polychondritis, 1:3-54 to 1:3-56,
1:3-57i
respiratory bronchiolitis, 1:3-80 to 1:3-82,
1:3-83i
rhinoscleroma, 1:3-28 to 1:3-29
saber-sheath trachea, 1:3-60 to 1:3-62, 1:3-63i
tracheobronchomalacia, 1:3-50 to 1:3-52,
1:3-53i
tracheobronchomegaly, 1:3-6 to 1:3-8, 1:3-9i
tracheopathia osteochondroplastica, 1:3-92 to
1:3-93
Alpha-l antiprotease deficiency, 1:3-22 to 1:3-24,
1:3-25i
differential diagnosis, 1:3-22i, 1:3-23
as lower zone lung disease, IV:5-12
mosaic pattern of lung attenuation vs., V:I-46i,
V:I-47
Alveolar hemorrhage, diffuse. See Diffuse alveolar
hemorrhage
Alveolar microlithiasis, 1:1-64 to 1:1-66, 1:1-67i
differential diagnosis, 1:1-64i, 1:1-65 to 1:1-66
metastatic pulmonary calcification vs., 1:1-60i,
1:1-62
Alveolar proteinosis. See Pulmonary alveolar
protein osis
Alveolitis, allergic. See Hypersensitivity pneumonitis
Amiodarone toxicity
systemic lupus erythematosus vs., III:I-12i,
III:1-13
talcosis vs., II:4-35
Amyloid, tracheobronchial
amyloidosis vs., 1:3-89
tracheopathia osteochondroplastica vs., 1:3-93
Amyloidosis, 1:3-88 to 1:3-90, 1:3-9li
acute lung consolidation vs., V:1-4
alveolar microlithiasis vs., 1:1-65
differential diagnosis, 1:3-88i, 1:3-89 to 1:3-90
lung ossification vs., 1:2-50i, 1:2-51
lymphatic patterns in, V:1-40
metastatic pulmonary calcification vs., 1:1-61 to
1:1-62
relapsing polychondritis vs., 1:3-55
saber-sheath trachea vs., 1:3-61
talcosis vs., 1I:4-35
Aneurysm
aortic. See Aortic aneurysm
left ventricular
partial absence of pericardium vs., II:3-3
valve and annular calcification vs., 1I:3-24i,
II:3-25
Angiomatosis, bacillary, 1:3-99
Angiosarcoma
cardiac, left atrial myxoma vs., II:3-46i, II:3-47
hemangioma/lymphangioma vs., 11:1-56
pericardial metastases vs., II:3-50i, II:3-51
pleural, hemangioendothelioma vs., III:I-49
Ankylosing spondylitis, III:2-14 to III:2-16,
III:2-17i
differential diagnosis, III:2-14i, III:2-15
Marfan syndrome vs., 1I:2-32i, II:2-33
mycobacterial pneumonia vs., 1:1-9
upper lung zone predominant disease vs., V:I-12
as upper zone lung disease, IV:5-12
Antibasement membrane antibody disease. See
Goodpasture syndrome
Aorta and great vessels, II:2-2 to Il:2-51
aortic aneurysm, II:2-40 to Il:2-42, 1I:2-43i
aortic arch, right, Il:2-2 to Il:2-4, 1I:2-5i
aortic atherosclerosis, Il:2-28 to 1I:2-30, 1I:2-3li
aortic coarctation, II:2-10 to Il:2-12, II:2-13i
aortic dissection, Il:2-36 to 11:2-38, II:2-39i
azygos fissure, Il:2-26 to Il:2-27
inferior vena cava, azygos continuation of,
1:2-22 to 1I:2-24, 1I:2-25i
intralobar sequestration, Il:2-14 to II:2-16,
1I:2-17i
Marfan syndrome, II:2-32 to Il:2-34, II:2-35i
 INDEX
proximal descending, normal fusiform
enlargement vs. transection, IV:2-20i,
IV:2-21
stenosis, aortic valve dysfunction vs., II:3-28i,
II:3-30
subclavian artery, aberrant, II:2-6 to II:2-8,
II:2-9i
superior vena cava
left, II:2-18 to II:2-20, II:2-2li
obstruction of, II:2-48 to II:2-50, II:2-5li
Takayasu disease, II:2-44 to II:2-46, II:2-47i
tortuosity (aging)
aneurysm vs., II:2-40i, II:2-41
dissection vs., II:2-37
Aortic aneurysm, II:2-40 to II:2-42, II:2-43i
ankylosing spondylitis vs., III:2-15
apical pleural cap vs., III:1-10
ascending, anterior mediastinal mass vs., V:1-56
differential diagnosis, II:2-40i, II:2-41
dissecting, 11:1-11, II:2-36i, II:2-37
mediastinal fibrosis vs., II:1-6i, II:1-7
Takayasu disease vs., II:2-44i, II:2-45 to II:2-46
thoracic, atherosclerosis vs., II:2-29
Aortic arch
anomalies of
azygos continuation of inferior vena cava
vs., II:2-24
double vs. left pulmonary artery anomalous
origin, II:4-20i
double vs. right, II:2-2i, II:2-3
tracheobronchomalacia vs., I:3-50i
right, II:2-2 to II:2-4, II:2-5i
aberrant right subclavian artery vs., II:2-6i,
II:2-8
azygos continuation of inferior vena cava
vs., II:2-22i, II:2-23
differential diagnosis, II:2-2i, II:2-3
middle mediastinal mass vs., V:1-58i, V:1-60
Aortic coarctation, II:2-10 to II:2-12, II:2-13i
aortic valve dysfunction vs., II:3-30
differential diagnosis, II:2-lOi, 11:2-11
Takayasu disease vs., II:2-44i, II:2-45
Aortic dissection, II:2-36 to II:2-38, II:2-39i
atherosclerosis vs., II:2-28i, II:2-29
differential diagnosis, II:2-36i, II:2-37 to
II:2-38
median sternotomyvs., IV:4-18i, IV:4-19
Aortic transection, IV:2-20 to IV:2-22, IV:2-23i
aberrant right subclavian artery vs., II:2-7
differential diagnosis, IV:2-20i, IV:2-21
dissection vs., II:2-36i, II:2-37
Aortic valve
calcification
left atrial calcification vs., II:3-16i, II:3-17
ventricular calcification vs., II:3-20i, II:3-21
dysfunction, II:3-28 to II:3-30, II:3-3li
differential diagnosis, II:3-28i, II:3-30
mitral valve dysfunction vs., II:3-32i, II:3-33
stenosis
aneurysm vs., II:2-40i, II:2-41
left atrial calcification vs., II:3-16i, II:3-17
post-stenotic dilation vs. dissection, II:2-37
Aortitis, syphilitic, II:2-45
Aortoocclusive disease, II:2-44i, II:2-45 to II:2-46
Apicolysis, IV:4-26 to IV:4-28, IV:4-29i
differential diagnosis, IV:4-26i, IV:4-28
ARDS.See Acute respiratory distress syndrome
(ARDS)
Arteriovenous malformation
allergic bronchopulmonary aspergillosis vs.,
1:3-47
bronchial atresia vs., 1:3-15
focal increased density lung disease vs., V:1-19
lipomatosis vs., II:1-45
metastasis vs., lV:6-3
middle mediastinal mass vs., V:1-58i, V:1-60
missed lung cancer vs., lV:3-26
iii
multiple pulmonary nodules vs., V:1-28
pulmonary, II:4-2 to II:4-4, II:4-5i
differential diagnosis, II:4-2i, II:4-3
Asbestos related pleural disease, III:1-52 to III:1-54,
III:1-55i
differential diagnosis, III:1-52i, III:1-53 to
III:1-54
Erdheim Chester disease vs., III:l-4Oi, III:1-41
malignant mesothelioma vs., III:1-29
pleurodesis vs., IV:4-15
splenosis vs., IV:2-38i, IV:2-39
yellow-nail syndrome vs., III:l-4i, III:1-5
Asbestosis, 1:2-38 to 1:2-40, I:2-4li
basilar peripheral lung zone disease vs., V:1-15
desquamative interstitial pneumonia vs., 1:1-77
differential diagnosis, I:2-38i, 1:2-39
gravitational gradients in, IV:5-12
idiopathic pulmonary fibrosis vs., I:2-14i,
1:2-15
Iymphangitic carcinomatosis vs., 1:2-53
lymphatic patterns in, V:1-39
metastasis vs., IV:6-4
multiple pulmonary nodules vs., V:1-27
polymyositis-dermatomyositis vs., 1:2-31
rheumatoid arthritis vs., 1:2-24
scleroderma vs., I:2-26i, 1:2-27
upper lung zone predominant disease vs.,
V:l-1Oi
Ascites
diaphragmatic eventration vs., III:3-3
phrenic nerve paralysis vs., III:3-10i, III:3-12
Askin tumor, III:2-28 to III:2-30, III:2-3li
chest wall lymphoma vs., III:2-26i, I1I:2-27
 INDEX
differential diagnosis, III:2-28i, III:2-29 to
III:2-30
elastoma or fibroma vs., III:2-18i, III:2-19
empyema necessitatis vs., III:2-11
Aspergilloma
allergic bronchopulmonary aspergillosis vs.,
1:3-48
apical pleural cap vs., III:l-10i, III:1-11
Aspergillosis, 1:1-20 to 1:1-22, I:I-23i
allergic bronchopulmonary, 1:3-46 to 1:3-48,
1:3-49i
angioinvasive vs., 1:3-48
bronchial atresia vs., 1:3-15 to 1:3-16
cystic fibrosis vs., I:3-2i, 1:3-3
differential diagnosis, I:3-46i, 1:3-47 to
1:3-48
focal increased density lung disease vs.,
V:I-19
immotile cilia syndrome vs., 1:3-11
middle lobe syndrome vs., 1:3-59
tracheobronchomegaly vs., 1:3-7
upper lung zone predominant disease vs.,
iv V:1-11
chronic bronchitis vs., I:3-30i, 1:3-31
differential diagnosis, I:I-20i, 1:1-21
Pancoast tumor vs., III:I-33
parasitic pneumonia vs., I:I-28i, 1:1-29
posttransplant, IV:4-42i, IV:4-43
tumor emboli vs., II:4-75
Aspiration, IV:5-14 to IV:5-16, IV:S-17i
anomalous bronchi vs., 1:3-19
asthma vs., 1:3-69
bacterial pneumonia vs., I:I-2i, 1:1-4
bronchioloalveolar cell carcinoma vs., I:1-85
bronchocentric patterns in, V:I-36
chronic lung consolidation vs., V:1-8
cryptogenic organizing pneumonia vs., I:1-81
differential diagnosis, IV:5-14i, IV:5-15
drug abuse vs., II:4-40
drug reactions vs., IV:6-7
eosinophilic pneumonia vs., I:1-54
fat embolism vs., 1:1-41
high altitude pulmonary edema vs., II:4-62i,
II:4-63
lobar atelectasis vs., IV:I-2i
lobar torsion vs., IV:4-47
lung trauma vs., IV:2-12i, IV:2-14
neurogenic pulmonary edema vs., II:4-66i,
II:4-67
scleroderma vs., 1:2-27
silo-filler's disease vs., II:4-47
smoke inhalation vs., II:4-42i, II:4-43
staphylococcus pneumonia vs., I:I-6i, 1:1-7
viral pneumonia vs., 1:2-3
Asthma, 1:3-68 to 1:3-70, I:3-7li
bronchiectasis vs., 1:3-36
bronchiolitis obliterans vs., 1:3-65
centrilobular emphysema vs., 1:3-85
chronic bronchitis vs., 1:3-31
differential diagnosis, I:3-68i, 1:3-69
Atelectasis, IV:I-2 to IV:1-13
acute lung consolidation vs., V:I-4
aspiration vs., IV:5-15
bacterial pneumonia vs., 1:1-4
bronchiectasis vs., 1:3-36
chronic lung consolidation vs., V:I-8
complete lung thoracotomy vs., IV:4-19i,
IV:4-22
lobar, IV:1-2 to IV:1-4, IV:I-5i
differential diagnosis, IV:I-2i, IV:1-4
phrenic nerve paralysis vs., III:3-11
radiation-induced lung disease vs., IV:6-1Oi
thoracotomyvs., IV:4-19i, IV:4-22
lobar torsion vs., IV:4-46i, IV:4-47
pulmonary embolism vs., II:4-52
round, IV:I-10 to IV:I-12, IV:I-13i
blastomycosis vs., I:I-24i, 1:1-25
differential diagnosis, IV:l-10i, IV:1-11 to
IV:I-12
missed lung cancer vs., IV:3-26
sub segmental vs., IV:1-7
subsegmental, IV:1-6 to IV:1-8, IV:I-9i
differential diagnosis, IV:I-6i, IV:1-7 to
IV: 1-8
thoracoplasty vs., IV:4-26i, IV:4-28
Atherosclerosis, aortic, II:2-28 to II:2-30, II:2-3li
differential diagnosis, II:2-28i, II:2-29
dissection vs.; II:2-36i, II:2-37 to II:2-38
transection vs. ulceration, IV:2-20i, IV:2-21
Athletes, hyperinflation in
centrilobular emphysema vs., 1:3-85
panlobular emphysema vs., 1:3-73
Atrial septal defects, IV:5-6i, IV:5-8
Azygos arch, enlargement of, II:2-22i, II:2-23 to
11:2-24
Azygos fissure, II:2-26 to II:2-27
differential diagnosis, II:2-26i, 11:2-27
sub segmental atelectasis vs., IV:I-7
B
Bacillary angiomatosis, 1:3-99
Bacterial pneumonia, 1:1-2 to 1:1-4, I:l-Si
aspergillosis vs., 1:1-21
chronic bronchitis vs., I:3-30i, 1:3-31
coccidioidomycosis vs., 1:1-26
differential diagnosis, I:I-2i, 1:13 to 1:1-4
diffuse alveolar hemorrhage vs., I:I-44i, 1:1-45
 INDEX
immunosuppression (non-AIDS) vs., IV:6-14i,
IV:6-15
lipoid pneumonia vs., 1:1-69
posttransplant,IV:4-43
radiation-induced lung disease vs., IV:6-10i,
IV:6-11
staphylococcus pneumonia vs., 1:1-6, I:I-6i
staphylococcus vs., I:2-6i, 1:2-7
Basilar peripheral lung zone disease, V:I-14 to
V:I-16, V:I-I7i
differential diagnosis, V:I-14i, V:I-15 to V:I-16
Berylliosis, 1:2-46 to 1:2-48, I:2-49i
differential diagnosis, I:2-46i, 1:2-47
sarcoidosis vs., 1:2-11
Blastoma, pleural, III:1-3 7
Blastomycosis, 1:1-24 to 1:1-25
differential diagnosis, I:I-24i, 1:1-25
histoplasmosis vs., I:I-16i, 1:1-17
Blebs, centrilobular emphysema vs., 1:3-85
Bleomycin toxicity, V:I-15
Blesovsky syndrome. See Atelectasis, round
Bochdalek hernia
chest wall lipoma vs., III:2-22i, III:2-23
eventration vs., III:3-2i, III:3-3
hiatal hernia vs., II:I-15
rupture vs., IV:2-30i, IV:2-31
Body brace, IV:2-6i, IV:2-7
Bone marrow transplantation, 1:2-7
Bone neoplasms
anterior mediastinal mass vs., V:I-56
posterior mediastinal mass vs., V:I-64
BOOP (bronchiolitis obliterans-organizing
pneumonia). See Cryptogenic organizing
pneumonia
Brachiocephalic vein, V:I-S8i, V:1-60
Breast cancer, metastatic, IV:3-6i, IV:3-7
Breast prosthesis, III:2-8i, III:2-9
Bronchi, anomalous, 1:3-18 to 1:3-20, I:3-2li
differential diagnosis, I:3-18i, 1:3-19
Bronchial adenoma. See Carcinoid tumor,
pulmonary
Bronchial atresia, 1:3-14 to 1:3-16, 1:3-17i
allergic bronchopulmonary aspergillosis vs.,
1:3-47
bronchiectasis vs., 1:3-35
differential diagnosis, 1:3-14i, 1:3-15 to 1:3-16
focal decreased density lung disease vs., V:1-22
Bronchial-intercostal trunk, infundibulum of,
IV:2-21
Bronchial neoplasms, II:2-16
Bronchial stump dehiscence, IV:4-51
Bronchiectasis, 1:3-34 to 1:3-36, I:3-37i
asthma vs., I:3-68i, 1:3-69
chronic bronchitis vs., 1:3-31
differential diagnosis, I:3-34i, 1:3-35 to 1:3-36
focal decreased density lung disease vs., V:I-22i
postinfectious, vs. cystic fibrosis, 1:3-3
Bronchiolitis
infectious, V:1-35
inflammatory, V:I-47
proliferative. See Cryptogenic organizing
pneumonia
respiratory. See Respiratory bronchiolitis
Bronchiolitis obliterans, 1:3-64 to 1:3-66, I:3-67i
bronchocentric patterns in, V:I-36
centrilobular emphysema vs., 1:3-85
differential diagnosis, 1:3-64i, 1:3-65
Bronchiolitis obliterans-organizing pneumonia
(BOOP). See Cryptogenic organizing
pneumonia
Bronchioloalveolar cell carcinoma, 1:1-84 to 1:1-86,
1:1-87i
acute interstitial pneumonia vs., 1:1-57
acute lung consolidation vs., V:I-2i, V:I-4
aspiration vs., IV:5-15
bacterial pneumonia vs., 1:1-4
coccidioidomycosis vs., I:I-26i
cryptogenic organizing pneumonia vs., 1:1-81 v
differential diagnosis, 1:1-84i, 1:1-85 to 1:1-86
lipoid pneumonia vs., 1:1-68i, 1:1-69
metastasis vs., IV:6-4
missed lung cancer vs., IV:3-26
mucinous, vs. nonspecific interstitial
pneumonitis, 1:2-35
multiple pulmonary nodules vs., V:I-27
pulmonary alveolar proteinosis vs., 1:1-72i,
1:1-73
tumor emboli vs., II:4-76
Wegener granulomatosis vs., II:4-28i, II:4-29
Bronchitis, chronic, 1:3-30 to 1:3-32, 1:3-33i
bronchiectasis vs., 1:3-35
differential diagnosis, 1:3-30i, 1:3-31
Broncho-pleural fistula, III:1-20 to III:I-22,
III:I-23i
differential diagnosis, III:I-20i, Ill:I-21
pneumonectomy and, IV:4-30i, IV:4-31
Bronchocentric granulomatosis
allergic bronchopulmonary aspergillosis vs.,
1:3-47
asthma vs., 1:3-69
Bronchocentric patterns, V:I-34 to V:I-36, V:I-37i
differential diagnosis, V:I-34i, V:I-35 to V:I-36
Bronchogenic carcinoma. See also Lung cancer,
non-small cell
allergic bronchopulmonary aspergillosis vs.,
1:3-46i,I:3-47
ankylosing spondylitis vs., III:2-14i, III:2-15
asbestos related pleural disease vs., III:I-53
aspiration vs., IV:5-15
blastomycosis vs., 1:1-24, 1:1-24i
 INDEX
chest wall lymphoma vs., IIl:2-27
lipoid pneumonia vs., 1:1-69
mediastinal fibrosis vs., 11:1-7
metastasis vs., IV:6-3
mycobacterial avium complex vs., 1:3-38i,
1:3-39
mycobacterial pneumonia vs., 1:1-8i, 1:1-9
pericardial cyst vs., 11:3-9
posttransplant, IV:4-43
round atelectasis vs., IV:1-1Oi
screening for, IV:3-20i, IV:3-22
thoracic cyst vs., 11:1-4
Wegener granulomatosis vs., 11:4-29
Bronchogenic cyst
aberrant right subclavian artery vs., 11:2-8
bronchial atresia vs., 1:3-14i, 1:3-15
congenital, vs. lung abscess, 1:1-13
focal decreased density lung disease vs., V:1-23
focal increased density lung disease vs., V:1-19
hemangioma/lymphangioma vs., 1I:1-54i,
11:1-55
hiatal hernia vs., 1I:1-14i, 11:1-15
vi mediastinal goiter vs., II:1-20
pericardial cyst vs., 11:3-9
tracheobronchomalacia vs., 1:3-5Oi
Broncholithiasis
aspiration vs., IV:5-15
metastasis vs., IV:6-3
Bronchomalacia. See Tracheobronchomalacia
Bronchopneumonia. See Bacterial pneumonia
Bronchus, fractured, IV:4-47
Brucellosis
histoplasmosis vs., 1:1-17
mycobacterial pneumonia vs., 1:1-10
Bruton disease, 1:3-11
Bullae
alpha-1 anti protease deficiency vs., 1:3-22i,
1:3-23
apical lung hernia vs., 1I1:1-2i
bronchiectasis vs., 1:3-34i, 1:3-35
panlobular emphysema vs., 1:3-73
Bullectomy, IV:4-34 to IV:4-36, IV:4-37i
c definition of, 11:1-34
Calcification
coronary artery. See Coronary artery
calcification
left atrial. See Left atrial calcification
metastatic pulmonary. See Pulmonary
calcification, metastatic
myocardial. See Ventricular calcification
valvular. See Valve and annular calcification
Cancer screening, IV:3-20 to IV:3-22, IV:3-23i
differential diagnosis, IV:3-20i, IV:3-22
Cancer staging, IV:3-10i, IV:3-1O to IV:3-12,
IV:3-13i
Candidiasis
aspergillosis vs., 1:1-21
tumor emboli vs., 11:4-75
Wegener granulomatosis vs., 1I:4-28i, 11:4-29
Capillary hemangiomatosis, pulmonary, 11:4-78 to
11:4-79
differential diagnosis, 1I:4-78i, 11:4-79
veno-occlusive disease vs., 1I:4-32i
Caplan syndrome, 1:1-13
Carcinoid syndrome, 1:3-69
Carcinoid tumor
allergic bronchopulmonary aspergillosis vs.,
1:3-46i,I:3-47
bronchial, aspiration vs., IV:5-15
bronchial atresia vs., 1:3-16
non-small cell lung cancer vs., IV:3-2i, IV:3-3
pulmonary, 1:3-94 to 1:3-96, 1:3-97i
differential diagnosis, 1:3-94i, 1:3-95
pulmonary arteriovenous malformation vs.,
1I:4-2i, 11:4-3
pulmonary artery aneurysm vs., 1I:4-58i, 11:4-59
screening for, IV:3-20i, IV:3-22
thymic
germ cell tumorvs., 11:1-41
thymoma vs., 11:1-32
Carcinoma. See also Lymphangitic carcinomatosis
adenoid cystic, 1:3-94i, 1:3-95
bronchioloalveolar cell. See Bronchioloalveolar
cell carcinoma
bronchogenic. See Bronchogenic carcinoma
esophageal
achalasia vs., 11:1-11
mediastinal varices vs., 11:1-71
rupture vs., IV:2-34i, IV:2-35
mucoepidermoid, vs. pulmonary carcinoid,
1:3-95
oat cell. See Lung cancer, small cell
renal, hemorrhage vs. metastasis, IV:6-2i, IV:6-3
squamous cell, vs. cystic lung disease, V:1-30i,
V:1-31
thymic
thymoma vs., 11:1-31
thyroid, vs. mediastinal goiter, 1I:1-18i, 11:1-19
Cardiac pacemakers, IV:4-10 to IV:4-12, IV:4-13i
abnormal lead positioning, IV:4-6i, IV:4-10i
complications of, IV:4-10i
differential diagnosis, IV:4-12
dual lead pacer, IV:4-2i
Cardiac tamponade
cardiac volvulus vs., IV:4-51
superior vena cava obstruction vs., 1I:2-48i
 INDEX
Cardiac tumors
fibroma, ventricular calcification vs., IT:3-22
left atrial calcification vs., 11:3-17
left atrial myxoma vs., 1l:3-46i, 11:3-47 to
11:3-48
pericardia I cyst vs., 1l:3-8i
pericardial metastases vs., 1l:3-SOi, 11:3-51
Cardiac volvulus, IV:4-50 to IV:4-52, IV:4-S3i
differential diagnosis, IV:4-SOi, IV:4-51
heterotaxy syndrome vs., 1l:3-4i, 11:3-6
pneumonectomy and, IV:4-31
Cardiogenic pulmonary edema. See Pulmonary
edema, cardiogenic
Cardiomegaly, 11:3-44
Cardiomyopathy
mitral valve dysfunction vs., 1l:3-32i, 11:3-33
postcardiac injury syndrome vs., 11:3-42
restrictive, vs. constrictive pericarditis, 11:3-38
Castleman disease, 11:1-48to 11:1-50, II:I-Sli
angioimmunoblastic lymphadenopathy vs.,
II:I-52i,II:1-53
differential diagnosis, II:I-48i, II:1-49 to
IT:I-50
hemangioma/lymphangioma vs., II:I-55
lymphocytic interstitial pneumonia vs., 1:2-57
mediastinal goiter vs., 11:1-19
Cellulose granulomatosis, II:4-35
Chest trauma, blunt
drug abuse vs., II:4-40
lobar torsion vs., IV:4-47
Chest wall, III:2-2 to III:2-31
ankylosing spondylitis, III:2-14 to III:2-16,
III:2-17i
Askin tumor, III:2-28 to III:2-30, III:2-3li
defects of, V:I-22
elastoma, III:2-18 to 1Il:2-20, III:2-2li
empyema necessitatis, 1Il:2-1Oto 1Il:2-12,
III:2-13i
fibroma and fibromatosis, III:2-18 to 1Il:2-20,
III:2-2li
kyphoscoliosis, 1ll:2-4 to 1ll:2-6, III:2-7i
lipoma, III:2-22 to III:2-24, III:2-2Si
lymphoma, III:2-26 to III:2-27
mass
pleurodesis vs., IV:4-15
soft tissue, vs. Poland syndrome, 1ll:2-9
pectus deformity, III:2-2 to III:2-3
Poland syndrome, III:2-8 to III:2-9
resection vs. thoracoplasty, IV:4-26i, IV:4-28
Chicago disease. See Blastomycosis
Chondroid tumors, 1:1-10
Chondrosarcoma
chest wall lymphoma vs., III:2-27
empyema necessitatis vs., III:2-11
mycobacterial pneumonia vs., 1:1-10
Chronic obstructive pulmonary disease. See
Bronchitis, chronic
Churg-Strauss syndrome
asthma vs., 1:3-69
eosinophilic pneumonia vs., I:I-S2i, 1:1-53
Goodpasture syndrome vs., 1:1-49 to 1:1-50
pneumocystis pneumonia vs., 1:2-7
Wegener granulomatosis vs., II:4-29
Chylothorax
pneumonectomy and, IV:4-30i, IV:4-32
traumatic. See Thoracic duct tear
Ciliary dyskinesia syndrome, primary. See Immotile
cilia syndrome
Coal worker pneumoconiosis. See Silicosis
Coccidioidomycosis, 1:1-26 to 1:1-27
aspergillosis vs., 1:1-21
differential diagnosis, 1:1-26i, 1:1-27
mycobacterial pneumonia vs., 1:1-9
sarcoidosis vs., 1:2-11
Collagen vascular disease, IV:6-7
Colonic interposition, II:I-14i, II:I-15
Computed tomography, high-resolution
bronchocentric pattern, V:I-34 to V:I-36, vii
V:I-37i
differential diagnosis, V:I-34i, V:I-35 to
V:I-36
ground-glass opacities, V:1-42 to V:I-44,
V:I-4Si
differential diagnosis, V:I-42i, V:I-43
lymphatic pattern, V:I-38 to V:I-40, V:I-4li
differential diagnosis, V:I-38i, V:I-39 to
V:1-40
mosaic pattern of lung attenuation, V:I-46 to
V:I-48, V:I-49i
differential diagnosis, V:I-46i, V:I-47
tree-in-bud pattern, V:1-50 to V:I-52, V:I-53i
differential diagnosis, V:I-SOi, V:1-5 1
Congestive heart failure. See Pulmonary edema,
cardiogenic
Contusions, vs. neurogenic pulmonary edema,
Il:4-67
Coronary artery calcification, II:3-12 to II:3-14,
II:3-ISi
differential diagnosis, 1l:3-12i, 11:3-13 to
II:3-14
left atrial calcification vs., II:3-18
valve and annular calcification vs., II:3-24i,
11:3-26
ventricular calcification vs., 1:3-22
Costochondral junction, first, IV:3-26
Costochondritis, llI:2-10i, III:2-11
Costosternal spur, IV:3-16i, IV:3-18
Cryptococcus
mycobacterial avium complex vs., I:3-38i,
1:3-39
sarcoidosis vs., 1:2-11
 INDEX
Cryptogenic organizing pneumonia, 1:1-80 to
1:1-82,I:1-83i
acute lung consolidation vs., V:1-4
aspiration vs., IV:5-15
bacterial pneumonia vs., 1:1-4
basilar peripheral lung zone disease vs., V:1-15
bronchioloalveolar cell carcinoma vs., I:1-84i,
1:1-85
desquamative interstitial pneumonia vs., I:1-77
differential diagnosis, I:1-80i, 1:1-81
eosinophilic pneumonia vs., I:1-52i, 1:1-53
lipoid pneumonia vs., 1:1-69
metastasis vs., IV:6-4
nonspecific inter.stitial pneumonitis vs., 1:2-35
parasitic pneumonia vs., 1:1-29
posttransplant, IV:4-42i, IV:4-43
pulmonary alveolar proteinosis vs., 1:1-72i,
1:1-73
rheumatoid arthritis vs., 1:2-24
tumor emboli vs., 1T:4-76
vasculitis vs., II:4-26i, 11:4-27
viral pneumonia vs., 1:2-3
Cutis laxa, 1:3-7
Cyanotic pseudofibrosis, IV:5-8
Cystic adenomatoid malformation. See also
Thoracic cysts
anomalous bronchi vs., 1:3-18i, 1:3-19
diaphragmatic hernia vs .., 111:3-8
intralobar sequestration vs.., 11:2-15
Cystic fibrosis, 1:3-2 to 1:3-4, I:3-5i
allergic bronchopulmonary aspergillosis vs.,
I:3-46i,I:3-47
asthma vs., 1:3-68i, 1:3-69
differential diagnosis, 1:3-2i, 1:3-3 to 1:3-4
immotile cilia syndrome vs., 1:3-10i, 1:3-11
middle lobe syndrome vs., 1:3-59
tracheobronchomegaly vs., 1:3-7
upper lung zone predominant disease vs., V:1-11
as upper zone lung disease, IV:5-12
Cystic foregut malformations, 11:1-7
Cystic lung disease, V:1-30 to V:1-32, V:1-33i
differential diagnosis, V:1-30i, V:1-30 to V:1-31
focal decreased density lung disease vs., V:1-22
to V:1-23
Cysts
air, paratrachea!. See Paratracheal air cyst
bronchogenic. See Bronchogenic cyst
dermoid., II:1-2i, 11:1-4
duplication. See Duplication cyst
foregut, V:1-19
hydatid
pericardial cyst vs., 11:3-9
septic emboli vs., 11:4-23
ventricular calcification vs., 11:3-22
neurenteric
nerve sheath tumor vs., II:1-59
sympathetic ganglion tumor vs., 1I:1-63
pericardia!. See Pericardial cyst
thoracic. See Cystic adenomatoid malformation;
Thoracic cysts
thymic. See Thymic cyst
trilobular emphysema vs., 1:3-85
Cytomegalovirus infections, IV:6-18i
D
Da Nang lung. See Pneumonia, acute interstitial
Dehiscence
of bronchial stump, IV:4-51
median sternotomy vs., IV:4-18i
Dermatologic manifestations, pulmonary disease,
III:1-4
Dermatomyositis-polymyositis. See Polymyositis-
dermatomyositis
Dermoid cyst, II:1-2i, 11:1-4
Desmoid tumor, III:2-11
Desquamative interstitial pneumonia, 1:1-76 to
1:1-78,I:1-79i
bronchiolitis obliterans vs., 1:3-65
differential diagnosis, 1:1-76i, 1:1-77 to 1:1-78
nonspecific interstitial pneumonitis vs., 1:2-34i,
1:2-35
respiratory bronchiolitis vs., I:3-80i, 1:3-81
Diaphragm, III:3-2 to 111:3-13
elevated, IIl:1-45
eventration of, III:3-2 to IIl:3-4, III:3-5i
differential diagnosis, III:3-2i, III:3-3 to
III:3-4
hernia vs., III:3-6i, III:3-7
phrenic nerve paralysis vs., II1:3-11
rupture vs., IV:2-30i, IV:2-31
hernias. See Diaphragmatic hernias
normal variant, III:3-2i, III:3-3 to 11I:3-4
paralysis vs. rupture, IV:2-31
phrenic nerve paralysis, III:3-10 to III:3-12,
II1:13i
rupture, IV:2-30 to IV:2-32, IV:2-33i
differential diagnosis, IV:2-30i, IV:2-31
esophageal tear vs., IV:2-35
eventration vs., III:3-2i, III:3-3 to III:3-4
hernia vs .., III:3-6i, III:3-7 to 111:3-8
phrenic nerve paralysis vs., III:3-11
tumors, III:3-4
Diaphragmatic hernias, III:3-6 to IIl:3-8, III:9i. See
also Bochdalek hernia; Morgagni hernia
differential diagnosis, III:3-6i, III:3-7 to 111:3-8
false. See Diaphragm, eventration of
hiatal hernia vs., 11:1-15
lipoid pneumonia vs., 1:1-69
pectus deformity vs., III:2-2i, III:2-3
phrenic nerve paralysis vs., 11I:3-]1
 INDEX
Diffuse alveolar hemorrhage, 1:1-44 to 1:1-46,
I:l--47i. See a/50 Goodpasture syndrome
acute interstitial pneumonia vs., I:1-56i, 1:1-57
differential diagnosis, l:l--44i, 1:1-45 to 1:1-46
drug reactions vs., IV:6-6i, IV:6-7
parasitic pneumonia vs., 1:1-28i, 1:1-29
Discitis
kyphoscoliosis vs., III:2--4i, III:2-6
sympathetic ganglion tumor vs., II:1-62i,
II:1-63
Disseminated intravascular coagulation, I:1-46
Diverticulum
ductus, IV:2-21
epiphrenic
diaphragmatic hernia vs.., III:3-8
esophageal tear vs., IV:2-35
hiatal hernia vs., II:1-14i, II:1-15
esophageal, II:1-12 to II:l-13
differential diagnosis, II:1-12i, II:l-13
Killian-Jamieson, III:1-3
pharyngeal, III:1-3
tracheal. See Paratracheal air cyst
Zenker
achalasia vs., II:1-lOi, II:1-11
apical lung hernia vs., III:1-2i
paratracheal air cyst vs., 1:3-26i
Drug abuse, 11:4-38 to 11:4-40, II:4--4li
differential diagnosis, II:4-38i, 11:4-39 to
11:4--40
Drug reactions
amiodarone
systemic lupus erythematosus vs., III:1-12i
takosis vs., 11:4-35
bleomycin, V:1-15
cytotoxic, 1:2-39
desquamative interstitial pneumonia vs.,
I:1-76i,I:1-77
intrathoracic, IV:6-6 to IV:6-8, IV:6-9i. See a/50
Hypersensitivity pneumonitis
desquamative interstitial pneumonia vs.,
I:1-76i,I:1-77
differential diagnosis, IV:6-6i, IV:6-7
lymphangitic carcinomatosis vs., 1:2-53
Paraquat inhalation, II:4--46i, II:4-47
pericardial metastases vs., 11:3-51
polymyositis-dermatomyositis vs., 1:2-30i,
1:2-31
pulmonary fibrosis vs., 1:2-15
radiation-induced lung disease vs., IV:6-12
scleroderma vs., 1:2-28
systemic lupus erythematosus vs., III:1-13
Ductus diverticulum, IV:2-21
Duplication cyst
esophageal
aberrant right subclavian artery vs., II:2-8
nerve sheath tumor vs., 11:1-59
pericardial cyst vs., 11:3-9
sympathetic ganglion tumor vs., II:1-63
hiatal hernia vs., 11:1-15
thoracic cyst vs., II:I-2i, 11:1-4
E
Edema. See a/50 Pulmonary edema
negative pressure, 1:1-32i
Ehlers-Danlos syndrome
Marfan syndrome vs., II:2-34
tracheobronchomegaly vs., 1:3-7
Elastofibroma, III:2-11
Elastoma, III:2-18 to III:2-20, III:2-2li
differential diagnosis, III:2-18i, III:2-19
Embolism. See a/50 Pulmonary embolism
air, IV:2-42 to IV:2-44, IV:2--45i
differential diagnosis, IV:2--42i, IV:2-43
iodinated oil, vs. septic emboli, II:4-23
septic
focal increased density lung disease vs.,
V:I-19
immunosuppression (non-AIDS) vs., IV:6-16 IX
multiple pulmonary nodules vs., V:I-27
tumor-related
pulmonary embolism vs., 11:4-52
septic emboli vs., II:4-23
Emphysema
bullous
Langerhans cell granulomatosis vs., 1:3-77
pneumothorax vs., IV:2-7
centrilobular, 1:3-84 to 1:3-86, I:3-87i
alpha-l antiprotease deficiency vs., I:3-22i,
1:3-23
bronchocentric patterns vs., V:I-34i, V:I-36
differential diagnosis, I:3-84i, 1:3-85
laryngeal papillomatosis vs., 1:3-43
panlobular emphysema vs., I:3-72i, 1:3-73
upper lung zone predominant disease vs.,
V: 1-11
chronic bronchitis vs., 1:3-31
congenital lobar, vs. bronchial atresia, 1:3-15
focal decreased density lung disease vs., V:1-22,
V:1-23
panlobular, 1:3-72 to 1:3-74, I:3-75i
bronchiolitis obliterans vs., I:3-64i, 1:3-65
centrilobular vs., 1:3-85
differential diagnosis, 1:3-72i, 1:3-73
Iymphangiomyomatosis vs., I:2-60i, 1:2-61
mosaic pattern of lung attenuation vs.,
V: 1-47
paraseptal, V:I-15
pulmonary interstitial. See Pneumomediastinum
as upper zone lung disease, IV:5-12
 INDEX
Empyema, III:1-16 to III:1-18, III:1-19i
asbestos related pleural disease vs., III:1-S2i,
111:1-53
differential diagnosis, III:1-16i, III:1-17
dormant, thoracoplasty vs., IV:4-26i, IV:4-28
hemangioendothelioma vs., III:1-49
malignant mesothelioma vs., III:1-29
pleurodesisvs., IV:4-14i, IV:4-15
postpneumonectomy, IV:4-32
transudative pleural effusion vs., III:1-44i
tuberculosis dormant, III:1-29
Empyema necessitatis, III:2-10 to III:2-12, III:2-13i
Askin tumor vs., III:2-30
broncho-pleural fistula vs., III:1-21
differential diagnosis, III:2-10i, III:2-11
elastoma or fibroma vs., III:2-19
Endobronchial obstruction
anomalous bronchi vs., I:3-18i, 1:3-19
cystic fibrosis vs., 1:3-4
Endocarditis, 1:1-13
Endometriosis, III:1-17
Endotracheal tubes. See Tubes and catheters
x Eosinophilic granuloma. See Langerhans cell
granulomatosis
Eosinophilic pneumonia, 1:1-52 to 1:1-54, I:1-SSi
acute, vs. chronic lung consolidation., V:1-6i,
V:1--S
asthma vs., 1:3-69
bacterial pneumonia vs., 1:1-4
cardiogenic pulmonary edema vs., 1:1-33
chronic
acute lung consolidation vs., V:1-2i, V:1-4
basilar peripheral lung zone disease vs.,
V:1-14i, V:1-16
upper lung zone predominant disease vs.,
V:1-11
cryptogenic organizing pneumonia vs., 1:1-81
differential diagnosis, I:1-S2i, 1:1-53 to 1:1-54
drug reactions vs., IV:6-6i, IV:6-7
gravitational gradients in, IV:5-12
parasitic pneumonia vs., 1:1-29
Epicardial fat pad, II:3-51
Epiphrenic diverticulum
diaphragmatic hernia vs., III:3-8
esophageal tear vs., IV:2-35
hiatal hernia vs., II:1-14i, II:1-15
Erdheim Chester disease, III:1-40 to III:1-42,
III:1-43i
differential diagnosis, III:1-40i, III:1-41 to
III:1-42
diffuse pulmonary Iymphangiomatosis vs.,
I:2-64i, 1:2-65
lymphatic patterns in, V:1-40
mediastinal fibrosis vs., II:1-8
Esophagectomy, IV:4-24 to IV:4-2S
Esophagitis, IV:2-34i, IV:2-35
Esophagopleural fistula
bronchopleural fistula vs., III:1-20i, III:1-21
pneumonectomy and, IV:4-32
Esophagus
air distention of, IV:2-3
diverticuli of, II:1-12 to 11:1-13
differential diagnosis, II:1-12i, 11:1-12 to
II:1-13
fistula vs. rupture, IV:2-35
intubation of, IV:2-11
resection of, IV:4-24 to IV:4-25
rupture of, IV:2-34 to IV:2-36, IV:2-37i
differential diagnosis, IV:2-34i, IV:2-35
tracheobronchial tear vs., IV:2-10i, IV:2-11
varices, II: 1-66i, II:1-67
webs, II:1-12i, 11:1-13
Eventration, III:1-44i, III:1-45. See also
Diaphragm, eventration of
Ewing sarcoma, III:2-29
Exhalation
diaphragmatic eventration vs., III:3-4
normal, V:1-42i, V:1-43
Extrapleural fat, III:1-11
F
Fallen lung. See Tracheobronchial tear
Farmer's lung
bacterial pneumonia vs., 1:1-4
Langerhans cell granulomatosis vs., 1:3-77
silicosis vs., I:2-42i, 1:2-43 to 1:2-44
viral pneumonia vs., 1:2-3
Fasciitis, necrotizing, III:2-10i, III:2-11
Fat, extrapleural, III:1-11
Fat pad
diaphragmatic hernia vs .., 111:3-8
epicardial, vs. phrenic nerve paralysis, 1ll:3-12
pericardial, vs. cyst, II:3-9
Fat pulmonary embolism, 1:1-40 to 1:1-42, I:1-43i
air embolism vs., IV:2-43
differential diagnosis, I:1-40i, 1:1-41
septic emboli vs., 11:4-23
Fibrin ball, IV:2-40
Fibroelastoma, papillary, 11:3-48
Fibrohistiocytoma, III:2-19
Fibroma and fibromatosis, III:2-18 to II1:2-20,
III:2-21i
differential diagnosis, III:2-18i, 111:2-19
Fibromuscular dysplasia, 11:2-45
Fibrosarcoma
elastoma or fibroma vs., III:2-18i, 111:2-19
partial absence of pericardium vs., II:3-2i
Fibrothorax
exudative pleural effusion vs., 1Il:1-7
pleural metastasis vs., III:1-24i, 111:1-25
Flail chest, IV:2-16 to IV:2-18, IV:2-19i
 INDEX
differential diagnosis, IV:2-16i, IV:2-17
Flow artifacts, II:4:50i, 11:4-52
Focal decreased density lung disease, V:1-22 to
V:1-24, V:1-25i
differential diagnosis, V:1-22i, V:1-22 to V:1-23
Focal increased density lung disease, V:1-18 to
V:1-20, V:1-2li
differential diagnosis, V:1-18i, V:1-19 to V:1-20
Foregut cyst, V:1-19
Foreign bodies
allergic bronchopulmonary aspergillosis vs.,
1:3-47
esophageal, 11:2-8
metastasis vs., IV:6-3
septic emboli vs., 11:4-23
splenosis vs., IV:2-39
vertebroplasty glue, II:4-52
Fungal infections
coccidioidomycosis vs., 1:1-27
cryptogenic organizing pneumonia vs., 1:1-80i,
1:1-81
histoplasmosis vs., 1:1-17
immunosuppression (non-AIDS) vs., IV:6-14i,
IV:6-15 to IV:6-16
lung abscess vs., 1:1-13
multiple pulmonary nodules vs., V:1-27
mycobacterial pneumonia vs., 1:1-9
tree-in-bud pattern vs., V:1-51
G lymphocytic interstitial pneumonia vs.,
Ganglioneuroblastoma, III:2-29
Ganglioneuroma, 11I:2-29
Gangrene, pulmonary. See Lung abscess
Gas-forming infection, pleural, III:1-21
Gastric pull-up, II:1-14i, 11:1-15
Gastric volvulus, III:3-6i, III:3-7
Gastrointestinal stromal tumor, esophageal, 11:1-20
Gastrointestinal tract, stromal tumors of, II:1-70i,
11:1-71
Gaucher disease, IV:6-23
Germ cell tumors, 11:1-40 to 11:1-42, II:1-43i
aortic dissection vs., 11:2-38
differential diagnosis, II:1-40i, 11:1-41
Hodgkin lymphoma vs., 11:1-23
lipoid pneumonia vs., 1:1-69
mediastinal, II:4-14
mediastinal fibrosis vs., 11:1-7
non-seminomatous, V:1-60
thoracic cyst vs., 11:1-4
thymic tumor vs., II:1-34i, 1l:1-35
thymoma vs., II:1-30i, 11:1-31
Giant cell arteritis, 11:2-45
Gilchrist disease. See Blastomycosis
Glomerulonephritis. See Goodpasture syndrome
Goiter
mediastinal, 11:1-18 to 11:1-20, II:1-2li
differential diagnosis, II:1-18i, 11:1-19 to
11:1-20
fibrosis vs., II:1-7
thyroid
germ cell tumor vs., II:1-40i, II:1-41
Hodgkin lymphoma vs., 11:1-24
thymoma vs., II:1-30i, 11:1-31
tracheobronchomalacia vs., 1:3-5Oi
Goodpasture syndrome, 1:1-48 to 1:1-50, 1:1-5li
differential diagnosis, 1:1-48i, 1:1-49 to 1:1-50
Pneumocystis pneumonia vs., 1:2-7
systemic lupus erythematosus vs., III:1-13
Wegener granulomatosis vs., 11:4-29
Granuloma
eosinophilic. See Langerhans cell granulomatosis
focal increased density lung disease vs., V:1-19
foreign body vs. pulmonary carcinoid, 1:3-95
mediastinal. See Mediastinal fibrosis
metastasis vs., IV:6-3
missed lung cancer vs., IV:3-26
screening for, IV:3-20i, IV:3-22 XI
Granulomatosis
bronchocentric
allergic bronchopulmonary aspergillosis vs.,
1:3-47
asthma vs., 1:3-69
cellulose, talcosis vs., 11:4-35
lymphomatoid
1:2-56i,I:2-57
Wegener granulomatosis vs., 11:4-29
Wegener. See Wegener granulomatosis
Great vessels. See Aorta and great vessels
Ground-glass opacities, V:1-42 to V:1-44, V:1-45i
differential diagnosis, V:1-42i, V:1-43
in focal increased density lung disease, V:1-18i,
V:1-19
H
Haemophilus infections, II:4-28i, 11:4-29
Hamartoma
lipoid pneumonia vs., 1:1-69
missed lung cancer vs., IV:3-24i, lV:3-26
multiple primary, V:1-28
non-small cell lung cancer vs., IV:3-2i, IV:3-3
pulmonary carcinoid vs., 1:3-94i, 1:3-95
screening for, IV:3-20i, IV:3-22
Hamman-Rich syndrome. See Pneumonia, acute
interstitial
Heart, 11:3-2 to 11:3-53. See also Cardiac terms;
Pericardium
aortic valve dysfunction, 11:3-28 to 1l:3-30,
II: 3-3 li
 INDEX
constrictive pericarditis, II:3-36 to II:3-38,
II:3-39i
coronary artery calcification, II:3-12 to II:3-14,
II:3-15i
heterotaxy syndrome, II:3-4 to II:3-6, II:3-7i
left atrial calcification, II:3-16 to II:3-18,
II:3-19i
left atrial myxoma, II:3-46 to II:3-48, II:3-49i
mitral valve dysfunction, II:3-32 to II:3-34,
II:3-35i
myxedema, II:3-44 to II:3-45
neoplasms of. See Cardiac tumors
normal contour of, IV:4-50i, IV:4-51
postcardiac injury syndrome, II:3-40 to II:3-42,
II:3-43i
valve and annular calcification, 11:3-24 to
II:3-26,II:3-27i
ventricular calcification, II:3-20 to II:3-22,
II:3-23i
Heart failure, left, II:3-44, II:3-44i
Hemangioendothelioma, III:1-48 to III:1-50,
III:1-5li
xii differential diagnosis, III:1-48i, III:I-49
epithelioid
hemangioma/lymphangioma vs., I1:1-56
pleural metastasis vs., III:1-25
malignant mesothelioma vs., III:1-29
Hemangioma, 11:1-54 to II:I-56, 1I:1-57i
differential diagnosis, II:1-54i, 11:1-55 to
I1:1-56
focal increased density lung disease vs., V:1-19
Hemangiomatosis, capillary
pulmonary, I1:4-78 to 11:4-79
differential diagnosis, II:4-78i, 11:4-79
veno-occlusive disease vs., II:4-32i
Hematoma
focal increased density lung disease vs., V:I-19
median sternotomy vs., IV:4-18i
missed lung cancer vs., IV:3-24i, IV:3-26
paraspinal
nerve sheath tumor vs., 1I:1-58i, 11:1-59
sympathetic ganglion tumor vs., 11:1-63
pericardial cyst vs., 11:3-9
pleurodesis vs., IV:4-14i, IV:4-16
Hematopoiesis, extramedullary, 11:1-66 to 11:1-68,
II:1-69i
differential diagnosis, 1I:1-66i, II:I-67
lipoid pneumonia vs., 1:1-69
lipomatosis vs., II:1-44i, II:I-45
sympathetic ganglion tumor vs., II:1-63
Hemivertebra, III:2-4i, III:2-6
Hemoglobinuria, paroxysmal nocturnal, IV:6-23
Hemoptysis, 11:4-76
Hemorrhage
bronchial, 1:3-68i, 1:3-69
diffuse alveolar. See Diffuse alveolar hemorrhage
intrathoracic, IV:4-51
postoperative,IV:4-47
pulmonary. See Pulmonary hemorrhage
Hemosiderosis, idiopathic pulmonary, 1:1-49
Hemothorax, III:1-53
Henoch-Schbnlein purpura, 1:1-46
Hepatomegaly
diaphragmatic eventration vs., 11I:3-4
diaphragmatic rupture vs., IV:2-30i, IV:2-31
phrenic nerve paralysis vs., III:3-10i, III:3-12
Hepatopulmonary syndrome, IV:5-8
Hernia. See also Diaphragmatic hernias; Hiatal
hernia
apical, of lung, I:3-26i
paraesophageal
diaphragmatic rupture vs., IV:2-31
esophageal tear vs., IV:2-35
Heroin overdose, I:1-40i
Heterotaxy syndrome, 11:3-4 to I1:3-6, II:3-7i
differential diagnosis, II:3-4i, 11:3-5 to I1:3-6
Hiatal hernia, 11:1-14 to 11:1-16, II:1-17i
achalasia vs., II:I-IOi, 11:1-11
diaphragmatic hernia vs., III:3-6i, 11I:3-7
differential diagnosis, II:1-14i, I1:1-15 to
11:1-16
esophageal rupture vs., IV:2-35
lipomatosis vs., II:1-44i, 11:1-45
mediastinal varicesvs., II:1-70i, 11:1-71
Hibernoma
chest wall lipoma vs., III:2-23
lipomatosis vs., II:1-45
Hilar adenopathy, II:4-71
Hilar fibrosis, II:4-70i, 11:4-71
Histiocytosis, Langerhans cell (histiocytosis X). See
Langerhans cell granulomatosis
Histoplasmoma, IV:3-3
Histoplasmosis, 1:1-16 to 1:1-18, I:1-19i
differential diagnosis, 1:1-16i, 1:117 to 1:1-18
mycobacterial pneumonia vs., 1:1-9
sarcoidosis vs., I:2-10i, 1:2-11
HIV infections. See AIDS (acquired
immunodeficiency syndrome)
Hodgkin lymphoma, 11:1-22 to 11:1-24, 1I:1-25i
differential diagnosis, II:1-22i, 11:1-23 to
11:1-24
esophageal rupture vs., IV:2-34i
mediastinal fibrosis vs., II:1-6i, 11:1-7
non-Hodgkin lymphoma vs., I1:1-27
sarcoidosis vs., 1:2-10i, 1:2-11
thoracic cyst vs., 11:1-4
thymoma vs., II:1-30i, 11:1-31
Hydatid cyst
pericardial cyst vs., 11:3-9
septic emboli vs., II:4-23
ventricular calcification vs., I1:3-22
 INDEX
Hydropneumothorax
broncho-pleural fistula vs., III:1-21
extrapleural apicolysis vs., IV:4-28
Hygroma, cystic, Il:1-36
Hyperemia, V:1-43
Hypereosinophilic syndrome, 1:1-53
Hyperinflation, compensatory, IV:5-2i, IV:5-3
Hypersensitivity pneumonitis, 1:2-18 to 1:2-20,
1:2-211. See also Drug reactions, intrathoracic
asbestosis vs., 1:2-39
berylliosis vs., 1:2-47
bronchiolitis obliterans vs., 1:3-64i, 1:3-65
bronchocentric patterns in, V:1-35
differential diagnosis, 1:2-18i, 1:2-19
drug abuse vs., 1I:4-38i, II:4-40
idiopathic pulmonary fibrosis vs., 1:2-14i,
1:2-15
Langerhans cell granulomatosis vs., 1:3-77
lymphangiomyomatosis vs., 1:2-61
lymphangitic carcinomatosis vs., 1:2-53
lymphocytic interstitial pneumonia vs., 1:2-58
mosaic pattern of lung attenuation vs., V:1-46i,
V:1-47
nonspecific interstitial pneumonitis vs., 1:2-35
Pneumocystis pneumonia vs., 1:2-6i, 1:2-7
polymyositis-dermatomyositis vs., 1:2-31
respiratory bronchiolitis vs., 1:3-80i, 1:3-81
sarcoidosis vs., 1:2-11
silicosis vs., 1:2-43
tree-in-bud pattern vs., V:1-51
tumor emboli vs., Il:4-76
upper lung zone predominant disease vs., V:1-11
Hypertension
pulmonary artery, Il:4-54 to Il:4-56, 1I:4-57i
aneurysm vs., Il:4-59
capillary hemangiomatosis vs., 1I:4-78i,
Il:4-79
differential diagnosis, 1I:4-54i, Il:4-55
idiopathic dilatation vs., 1I:4-12i, Il:4-13
veno-occlusive disease vs., 1I:4-32i, Il:4-33
pulmonary venous. See Pulmonary edema,
cardiogenic
venous, diffuse pulmonary lymphangiomatosis
vs., 1:2-64i, 1:2-65
Hypothyroidism. See Myxedema
I 1:2-63i
Immotile cilia syndrome, 1:3-10 to 1:3-12, 1:3-13i
allergic bronchopulmonary aspergillosis vs.,
1:3-47
cystic fibrosis vs., 1:3-2i, 1:3-3
differential diagnosis, 1:3-101, 1:3-11
heterotaxy syndrome vs., 1I:3-4i, II:3-6
Immunosuppression (non-AIDS), IV:6-14 to
IV:6-16,IV:6-17i
differential diagnosis, IV:6-14i, IV:6-15 to
IV:6-16
immotile cilia syndrome vs., 1:3-10i, 1:3-11
Inferior vena cava
azygos continuation of, Il:2-22 to Il:2-24,
1I:2-25i
aberrant right subclavian artery vs., 1I:2-6i,
II:2-7
differential diagnosis, 1I:2-22i, II:2-23 to
Il:2-24
superior vena cava obstruction vs., Il:2-49
interrupted, vs. left pulmonary artery
anomalous origin, 1I:4-20i
intrahepatic, occlusion of, Il:2-24
Inhalation injury, 1:2-31
Intercostal vein
partial anomalous venous return vs., 1I:4-6i,
II:4-7
superior
left superior vena cava vs., 1I:2-18i, Il:2-19
middle mediastinal mass vs., V:1-58i, V:1-60
Interlobular septa, IV:1-6i, IV:1-7
Interstitial lung disease XIII
respiratory bronchiolitis-associated (RB-ILD).See
Respiratory bronchiolitis
rheumatoid arthritis vs., 1:2-22i, 1:2-23
Interstitial pneumonitis, nonspecific, 1:2-34 to
1:2-36,1:2-37i
desquamative interstitial pneumonia vs., 1:1-77
differential diagnosis, 1:2-34i, 1:2-35
hypersensitivity pneumonitis vs., 1:2-18i, 1:2-19
lipoid pneumonia vs., 1:1-69
lymphocytic interstitial pneumonia vs., 1:2-57
scleroderma vs., 1:2-26i, 1:2-27
Interstitium, 1:2-2 to 1:2-65
asbestosis, 1:2-38 to 1:2-40,1:2-411
berylliosis, 1:2-46 to 1:2-48, 1:2-49i
hypersensitivity pneumonitis, 1:2-18 to 1:2-20,
1:2-211
interstitial pneumonia, lymphocytic, 1:2-56 to
1:2-58, 1:2-59i
interstitial pneumonitis, nonspecific, 1:2-34 to
1:2-36,1:2-37i
lung ossification, 1:2-50 to 1:2-51
lymphangiomatosis, diffuse pulmonary, 1:2-64
to 1:2-65
lymphangiomyomatosis, 1:2-60 to 1:2-62,
Iymphangitic carcinomatosis, 1:2-52 to 1:2-54,
1:2-55i
Pneumocystis pneumonia, 1:2-6 to 1:2-8, 1:2-9i
polymyositis-dermatomyositis, pulmonary,
1:2-30 to 1:2-32, 1:2-33i
pulmonary fibrosis, idiopathic, 1:2-14 to 1:2-16,
1:2-17i
rheumatoid arthritis, 1:2-22 to 1:2-24, 1:2-25i
 INDEX
sarcoidosis, pulmonary, 1:2-10 to 1:2-12, 1:2-13i
scleroderma, pulmonary, 1:2-26 to 1:2-28,
1:2-29i
silicosis (coal worker pneumoconiosis), 1:2-42 to
[:2-44,1:2-45i
viral pneumonia, 1:2-2 to 1:2-4, I:2-5i
[ntralobar sequestration, 1I:2-14 to 1I:2-16,
II:2-17i
anomalous bronchi vs., 1:3-18i, 1:3-19
bronchial atresia vs., 1:3-14i, 1:3-15
differential diagnosis, II:2-14i, 1I:2-15 to
11:2-16
hiatal hernia vs., 1I:1-15
lung abscess vs., [:1-13
Intravascular coagulation, disseminated, [:1-46
J lymphocytic, angioimmunoblastic
]uxtaphrenic peak, IV:I-6i, IV:1-7 to [V:I-8
K lipomatosis vs., ]]:1-45
XIV Kaposi sarcoma, 1:3-98 to [:3-99
in AIDS, IV:6-18i, [V:6-20
Castleman disease vs., II:I-48i, II:1-49
differential diagnosis, 1:3-98i, 1:3-98 to 1:3-99
Kartagener syndrome. See [mmotile cilia syndrome
Kawasaki disease, 1I:2-45
Kidney disorders
acute failure, 1:I-4Oi
carcinoma, IV:6-2i, [V:6-3
thrombus, IV:6-2i
Klebsiella rhinoscleromatis. See Rhinoscleroma
Kulchitsky cell tumor. See Lung cancer, small cell
Kyphoscoliosis, 11I:2-4 to 11I:2-6, III:2-7i
differential diagnosis, III:2-4i, 11I:2-6
spinal fracture vs., IV:2-24i, [V:2-26
L splenosis vs., IV:2-39
Langerhans cell granulomatosis, [:3-76 to 1:3-78,
I:3-79i
alpha-1 anti protease deficiency vs., I:3-22i,
[:3-23
berylliosis vs., 1:2-46i, 1:2-47
bronchiectasis vs., I:3-34i, 1:3-35
bronchiolitis obliterans vs., 1:3-64i, 1:3-65
bronchocentric patterns in, V:1-35
centrilobular emphysema vs., 1:3-84i, [:3-85
differential diagnosis, 1:3-76i, [:3-77
Erdheim Chester disease vs., III:I-40i, 11I:1-41,
11I:1-41 to 11I:1-42
laryngeal papillomatosis vs., I:3-42i, [:3-43
Iymphangiomyomatosis vs., I:2-60i, [:2-61
lymphocytic interstitial pneumonia vs., I:2-56i,
1:2-58
panlobular emphysema vs., I:3-72i, 1:3-73
respiratory bronchiolitis vs., I:3-80i, 1:3-81
sarcoidosis vs., [:2-11
silicosis vs., I:2-42i, [:2-43
upper lung zone predominant disease vs., V:1-11
as upper zone lung disease, ]V:5-12
Laryngocele, 11I:1-3
Left atrial calcification, 1I:3-16 to 11:3-18, II:3-19i
differential diagnosis, II:3-16i, 11:3-17 to
]]:3-18
valve and annular calcification vs., 11:3-25
ventricular calcification vs., II:3-20i, 11:3-22
Leiomyosarcoma, 11I:1-25
Leukemia
Castleman disease vs., 11:1-49
lymphadenopathy vs., II: 1-52i
Lipoblastoma
chest wall lipoma vs., 11I:2-23
Lipoid pneumonia, ]:1-68 to [:1-70, 1:1-71i
cryptogenic organizing pneumonia vs., I:1-81
differential diagnosis, I:I-68i, 1:1-69
intralobar sequestration vs., Il:2-14i, 11:2-15
nonspecific interstitial pneumonitis vs., 1:2-35
Lipoma
apical pleural cap vs., 111:1-11
cardiac, vs. left atrial myxoma, 1I:3-47
chest wall, 11I:2-22 to 11I:2-24, III:2-25i
differential diagnosis, III:2-22i, 11I:2-23
lymphoma vs., 11I:2-27
extrap]eural fat, vs. Pancoast tumor, III:1-33
pleural
chest wall lipoma vs., III:2-22i, 11I:2-23
fibrous tumor of pleura vs., III:1-37
lipoid pneumonia vs., 1:1-69
spindle cell, vs. chest wall lipoma, 111:2-23
Lipomatosis, ]]:1-44 to 1I:1-46, II:I-47i
differential diagnosis, II:I-44i, 11:1-45
lipoid pneumonia vs., ]:1-69
lobar atelectasis vs., IV:1-4
mediastinal varices vs., II:I-70i, 11:1-71
Liposarcoma
chest wall lipoma vs., III:2-22i, 11I:2-23
lipoid pneumonia vs., 1:1-69
lipomatosis vs., Il:I-44i, 11:1-45
thymic tumor vs., II:I-34i, 11:1-36
Lobar pneumonia. See Bacterial pneumonia
Lobar torsion, lung, IV:4-46 to IV:4-48, IV:4-49i
differential diagnosis, IV:4-46i, IV:4-47
Lobectomy, postoperative, IV:1-4
Laffler syndrome. See Eosinophilic pneumonia
 INDEX
Lung abscess, 1:1-12 to 1:1-14, I:1-15i
aspergillosis vs., I:1-20i, 1:1-21
differential diagnosis, I:1-12i, 1:1-13
focal decreased density lung disease vs., V:1-22
staphylococcus vs. coccidioidomycosis, 1:1-26i
trauma vs., IV:2-12i, 1V:2-14
Wegener granulomatosis vs., I1:4-28i
Lung cancer, 1V:3-2 to 1V:3-27
abscess vs., I:1-12i, 1:1-13
anterior mediastinal mass vs., V:1-54i, V:1-56
bronchioloalveolar cell carcinoma vs., I:1-84i,
1:1-85
calcified vs. missed, IV:3-26
carcinoma vs. pulmonary carcinoid, 1:3-95
cryptogenic organizing pneumonia vs., I:1-81
cystic, 1V:3-26
empyema necessitatis vs., III:2-10i, III:2-11
focal decreased density lung disease vs., V:1-23
focal increased density lung disease vs., V:1-19
to V:1-20
histoplasmosis vs., 1:1-16i, 1:1-17, 1:117
middle mediastinal mass vs., V:1-60
missed, IV:3-24 to 1V:3-26, 1V:3-27i
differential diagnosis, IV:3-24i, 1V:3-26
mycobacterial avium complex vs., 1:3-38i,
1:3-39
mycobacterial pneumonia vs., 1:1-8i, 1:1-9
non-small cell, 1V:3-2 to 1V:3-4, IV:3-5i. See also
Bronchogenic carcinoma
aspergillosis vs., I:l-20i, 1:1-21
differential diagnosis, IV:3-2i, 1V:3-3 to
1V:3-4
small cell vs., IV:3-6i, 1V:3-7
partial absence of pericardium vs., II:3-3
posterior mediastinal mass vs., V:1-62i, V:1-64
radiation-induced lung disease vs., 1V:6-10i,
1V:6--11
regional lymph node classification, 1V:3-14 to
1V:3-15
round atelectasis vs., IV:1-10i, 1V:1-11
screening, 1V:3-20 to 1V:3-22, 1V:3-23i
differential diagnosis, IV:3-20i, 1V:3-22
septic emboli vs., II:4-22i, II:4-23
small cell, 1V:3-6 to 1V:3-8, IV:3-9i
differential diagnosis, IV:3-6i, 1V:3-7
solitary pulmonary nodule vs., 1V:3-7
solitary pulmonary nodule in, 1V:3-16 to
1V:3-18,IV:3-19i
mimics of, 1V:3-16i, IV:3-18
staging, IV:3-10i, 1V:3-10 to 1V:3-12, IV:3-13i
Lung diseases. See also Pulmonary terms
aspiration. See Aspiration
consolidation
acute, V:1-2 to V:1-4, V:1-5i
chronic lung consolidation vs., V:1-6i,
V:1-S
differential diagnosis, V:1-2i, V:1-4
chronic, V:1-6 to V:1-8, V:1-9i
acute vs., V:1-2i, V:1-4
differential diagnosis, V:1-6i, V:1-8
focal decreased density, V:1-22 to V:1-24,
V:1-25i
differential diagnosis, V:1-22i, V:1-22 to
V:1-23
focal increased density, V:1-18 to V:1-20,
V:1-2li
differential diagnosis, V:1-18i, V:1-19 to
V:1-20
hernia, apical, 1lI:1-2 to III:3
differential diagnosis, III:1-2i, 11I:1-2 to 1lI:3
paratracheal air cyst vs., 1:3-26i
ossification, 1:2-50 to 1:2-51
differential diagnosis, 1:2-50i, 1:2-50 to
1:2-51
radiation-induced, 1V:6-10 to 1V:6-12, IV:6-13i
differential diagnosis, IV:6-10i, 1V:6-11 to
1V:6--12
torsion. See Lobar torsion, lung
trauma, 1V:2-12 to 1V:2-14, IV:2-15i xv
differential diagnosis, IV:2-12i, 1V:2-14
intralobar sequestration vs.., I1:2-14i, II:2-15
by zone
basilar peripheral, V:1-14 to V:1-16, V:1-17i
differential diagnosis, IV:5-10i, 1V:5-12
upper predominant, V:1-10 to V:1-12,
V:l-13i
Lung physiology, 1V:5-2 to 1V:5-17
gravitational lung gradients, 1V:5-10 to 1V:5-12,
IV:5-13i
differential diagnosis, 1V:5-lOi, IV:5-12
ground-glass opacities vs., V:1-43
perfusion, 1V:5-6 to IV:5-8, IV:5-9i
differential diagnosis, IV:5-6i, 1V:5-8
ventilation and volumes in, IV:5-2 to IV:5-4,
1V:5-5i
volume reduction, IV:4-34 to IV:4-36, IV:4-37i
Lung transplantation, 1V:4-38 to 1V:4-40, IV:4-4li
lymphoproliferative disease following, IV:4-42
to 1V:4-44, IV:4-45i
differential diagnosis, IV:4-42i, IV:4-43
Lupus erythematosus, systemic, 1lI:1-12 to III:1-14,
III:1-15i
differential diagnosis, III:1-12i, III:1-13
polymyositis-dermatomyositis vs., I:2-30i
Wegener granulomatosis vs., II:4-29
Lymph nodes
azygos fissure vs., I1:2-26i
hilar, vs. pulmonary embolism, I1:4-50i, II:4-52
left superior vena cava vs., II:2-19
pericardia I
cyst vs., 11:3-9
metastases vs., 11:3-52
 INDEX
regional classification of, IV:3-14 to IV:3-15
Lymphadenopathy
AIDS vs., IV:6-18i, IV:6-20
angioimmunoblastic, 11:1-52 to 11:1-54, II:1-55i
Castleman disease vs., II:1-50
differential diagnosis, II:1-52i, II:I-52 to
11:1-53
lymphocytic interstitial pneumonia vs.,
1:2-57
histoplasmosis vs., 1:1-17
pulmonary embolism vs., If:4-52
Lymphangioleiomyomatosis. See
Lymphangiomyomatosis
Lymphangioma, 11:1-54 to 11:1-56, II:1-57i
differential diagnosis, II:1-54i, 11:1-55 to
11:1-56
thoracic cyst vs., II:1-2i, 11:1-4
thymic tumor vs., 11:1-36
Lymphangiomatosis, diffuse pulmonary, 1:2-64 to
1:2-65
differential diagnosis, 1:2-64i, 1:2-65
Erdheim Chester disease vs., 1ll:1-41
XVI lymphatic patterns in, V:1-38i, V:1-40
Lymphangiomyomatosis, 1:2-60 to 1:2-62, 1:2-63i
alpha-l anti protease deficiency vs., 1:3-23
bronchiectasis vs., 1:3-34i, 1:3-35
differential diagnosis, 1:2-60i, 1:2-61
Langerhans cell granulomatosis vs., 1:3-76i,
1:3-77
laryngeal papillomatosis vs., 1:3-42i, 1:3-43
lymphocytic interstitial pneumonia vs., 1:2-S6i
panlobular emphysema vs., 1:3-72i, 1:3-73
thoracic duct tear vs., IV:2-46i, IV:2-47
yellow-nail syndrome vs., III:l-4i, 111:1-5
Lymphangitic carcinomatosis, 1:2-52 to 1:2-54,
1:2-SSi
asbestosis vs., 1:2-39
cardiogenic pulmonary edema vs., 1:1-33
differential diagnosis, 1:2-52i, 1:2-53 to 1:2-54
diffuse pulmonary Iymphangiomatosis vs.,
1:2-64,1:2-64i
Erdheim Chester disease vs., 1ll:1-41
Kaposi sarcoma vs., 1:3-98i, 1:3-99
lymphatic patterns in, V:1-39
subsegmental atelectasis vs., IV:1-7
tree-in-bud pattern vs., V:I-51
Lymphangitic tumor. See Lymphangitic
carcinomatosis
Lymphatic patterns, V:I-38 to V:1-40, V:l-4li
differential diagnosis, V:1-38i, V:I-39 to V:I-40
Lymphocytic interstitial pneumonia, 1:2-56 to
1:2-58,1:2-S9i
Castleman disease vs., II:1-50
desquamative interstitial pneumonia vs., 1:1-77
differential diagnosis, 1:2-56i, 1:2-57 to 1:2-58
lymphatic patterns in, V:1-39
Lymphoepithelial cyst. See Para tracheal air cyst
Lymphoma. See also Hodgkin lymphoma; Non-
Hodgkin lymphoma (NHL)
acute lung consolidation vs., V:I-4
amyloidosis vs., 1:3-90
angioimmunoblastic lymphadenopathy vs.,
II:I-52
ankylosing spondylitis vs., 1ll:2-15
aortic dissection vs., 11:2-37
bronchioloalveolar cell carcinoma vs., 1:1-84i,
1:1-85
cardiac, left atrial myxoma vs., II:3-46i, II:3-47
Castleman disease vs., II:1-49
chest wall, 1ll:2-26 to III:2-27
differential diagnosis, III:2-26i, III:2-27
cryptogenic organizing pneumonia vs., 1:1-81
extramedullary hematopoiesis vs., II:1-67
germ cell tumor vs., II:1-40i, II:I-41
histoplasmosis vs., 1:1-16i, 1:117
Kaposi sarcoma vs., 1:3-98i
Iymphangitic carcinomatosis vs., 1:2-53
malignant mesothelioma vs., III:I-29
mediastinal goiter vs., II:1-18i, II:I-19
metastasis vs., IV:6-4
multiple pulmonary nodules vs., V:I-27
myxedema vs., II:3-44, II:3-44i
Pancoast tumor vs., III:I-32i, III:I-33 to
1ll:1-34
pleural, III:2-29
Poland syndrome vs., III:2-8i, III:2-9
posttransplant. See Lymphoproliferative disease,
posttransplant
pulmonary, vs. mycobacterial pneumonia,
1:1-8i,I:I-9
small cell lung cancer vs., IV:3-6i, IV:3-7
thymic, vs. middle mediastinal mass, V:I-60
thymic rebound vs., II:1-38i, II:1-39
thymic tumor vs., II:1-34i, II:I-35
Lymphoproliferative disease
immunosuppression (non-AIDS) vs., IV:6-15
posttransplant, IV:4-42 to IV:4-44, IV:4-45i
differential diagnosis, IV:4-42i, IV:4-43
multiple pulmonary nodules vs., V:I-27
M
Mach band, IV:2-3
Malignant effusion, IV:2-46i, IV:2-47
Marfan syndrome, II:2-32 to II:2-34, II:2-3Si
differential diagnosis, II:2-32i, II:2-33 to
II:2-34
tracheobronchomegaly vs., 1:3-6i
Mastectomy, 1ll:2-9
Measles
histoplasmosisvs., 1:1-17
mycobacterial pneumonia vs., 1:1-10
 INDEX
Mediastinal fibrosis, II:1-6 to II:1-8, 1I:1-9i
congenital interruption of pulmonary artery vs.,
1I:4-16i, II:4-17
differential diagnosis, 1I:1-6i, II:I-7 to II:1-8
histoplasmosis vs., 1:1-17
Scimitar syndrome vs., II:4-10
small cell lung cancer vs., IV:3-7
superior vena cava obstruction vs., 1I:2-48i
veno-occlusive disease vs., 1I:4-32i
Mediastinal mass
aberrant right subclavian artery vs., II:2-7
ankylosing spondylitis vs., II1:2-1S
anterior, V:l-S4 to V:l-S6, V:l-S7i
differential diagnosis, V:I-54i, V:I-56
lipomatosis vs., II:I-4S
middle mass vs., V:I-60
focal increased density lung disease vs., V:1-20
left pulmonary artery anomalous origin vs.,
II:4-21
lobar atelectasis vs., IV:I-2i, IV:1-4
middle, V:l-S8 to V:1-60, V:I-61i
anteriorvs., V:l-S6
differential diagnosis, V:I-58i, V:1-60
pleurodesis vs., IV:4-15
posterior, V:1-62 to V:1-64, V:I-65i
diaphragmatic hernia vs.., III:3-8
differential diagnosis, V:I-62i, V:1-64
middle mass vs., V:1-60
right aortic arch vs., 1I:2-2i, II:2-3
splenosis vs., IV:2-39
varices vs., II:l-71
Mediastinitis
air embolism vs., IV:2-42i, IV:2-43
fibrosing. See Mediastinal fibrosis
lipomatosis vs., II:1-45
Mediastinum, II:1-2 to II:1-73
achalasia, II:1-10 to II:1-11
adenopathy of
lipomatosis vs., II:1-45
varices vs., II:I-7l
Castleman disease, II:1-48 to II:I-SO, 1I:1-5li
esophageal diverticuli, II:1-12 to II:1-13
extramedullary hematopoiesis, II:1-66 to
II:1-68,1I:1-69i
germ cell tumors, II:1-40 to II:1-42, 1I:1-43i
goiter, II:1-18 to II:1-20, 1I:1-2li
hemangioma/lymphangioma, 1I:1-S4 to II:I-56,
1I:1-57i
hiatal and paraesophageal hernias, II:I-14 to
II:1-16,1I:1-17i
Hodgkin lymphoma, II:1-22 to II:1-24, 1I:1-25i
lipomatosis, II:1-44 to II:1-46, 1I:1-47i
mediastinal fibrosis, II:I-6 to II:1-8, 1I:1-9i
nerve sheath tumors, II:1-S8 to II:1-60, 1I:1-6li
non-Hodgkin lymphoma, II:1-26 to II:1-28,
1I:1-29i
sympathetic ganglion tumors, II:I-62 to II:1-64,
1I:1-65i
thoracic cysts, II:1-2 to II:I-4, 1I:1-5i
thymic rebound, II:1-38 to II:1-39
thymic tumors, non-thymoma, II:I-34 to
II:1-36, 1I:1-37i
thymoma, II:1-30 to II:1-32, 1I:1-33i
varices, mediastinal, II:1-70 to II:1-72, 1I:1-73i
widened, vs. aortic transection, IV:2-21
Meningocele, lateral
extramedullary hematopoiesis vs., II:1-67
nerve sheath tumor vs., 1I:1-58i, II:I-59
sympathetic ganglion tumor vs., II:1-63
Mesothelioma
amyloidosis vs., 1:3-90
apical pleural cap vs., 1lI:1-11
asbestos related pleural disease vs., 1I1:1-52i,
III:1-53
chest wall lymphoma vs., III:2-26i, III:2-27
empyema vs., Ill:1-17
Erdheim Chester disease vs., III:1-41
extramedullary hematopoiesis vs., II:I-67
hemangioendothelioma vs., 1I1:1-48i, III:1-49 xvii
malignant, Ill: 1-28 to III:1-30, III:I-3li
differential diagnosis, III:I-28i, III:1-29
pleural metastasis vs., 1I1:1-24i, III:1-25
round atelectasis vs., IV:1-11
splenosis vs., IV:2-39
thoracoplasty vs., IV:4-28
Metastasis (metastatic disease)
amyloidosis vs., 1:3-90
angiocentric hematogenous, V:1-50i, V:1-5 1
aortic, vs. atherosclerosis, 1I:2-28i, II:2-29
cardiac
left atrial myxoma vs., 1I:3-46i, II:3-47
ventricular calcification vs., II:3-22
centrilobular emphysema vs., 1:3-84i, [:3-85
to chest, lV:6-2 to lV:6-4, IV:6-5i
differential diagnosis, IV:6-2i, lV:6-3 to
IV:6-4
to chest wall
fibrous tumor of pleura vs., 11I:1-36i,
IlI:I-37
lymphoma vs., 1lI:2-27
cystic lung disease vs., V:I-30i, V:I-31
empyema necessitatis vs., Ill:2-11
endobronchial, vs. aspiration, IV:S-15
extravascular involvement in, II:4-76
hematogenous
basilar peripheral lung zone disease vs.,
V:l-1S
gravitational gradients in, IV:S-12
multiple pulmonary nodules vs., V:1-27
Hodgkin lymphoma vs., II:I-24
immunosuppression (non-AIDS) vs., IV:6-1S to
[V:6-16
 INDEX
intravascular, vs. pulmonary artery sarcoma,
1I:4-70i,II:4-71
laryngeal papillomatosis vs., 1:3-42i, 1:3-43
lymphangitic, as lower zone lung disease,
. IV:5-12
mediastinal, vs. hemangioma/lymphangioma,
1I:1-54i,II:1-56
mediastinal goiter vs., 1I:1-18i
non-Hodgkin lymphoma vs., 1I:1-26i, 11:1-27
pericardial, 11:3-50 to 11:3-52, 1I:3-53i
differential diagnosis, 1I:3-50i, 11:3-51 to
11:3-52
left atrial myxoma vs., 11:3-48
pericardia I cyst vs., 1I:3-8i, 11:3-9
pleural. See Pleural metastasis
pulmonary, vs. arteriovenous malformation,
1I:4-2i, 11:4-3
pulmonary calcification. See Pulmonary
calcification, metastatic
rib fractures vs., IV:2-16i, IV:2-17
screening for, IV:3-22
septic emboli vs., 11:4-23
xviii small cell lung cancer vs., IV:3-6i, IV:3-7
spinal fracture vs., IV:2-24i, IV:2-26
sternal fracture vs., IV:2-28i
sympathetic ganglion tumor vs., 1I:1-62i,
11:1-63
thoracic cyst vs., 11:1-4
thymoma vs., 11:1-32
Wegener granulomatosis vs., 11:4-29
Microlithiasis, alveolar. See Alveolar microlithiasis
Middle aortic syndrome, 11:2-45
Middle lobe syndrome, 1:3-58 to 1:3-59
differential diagnosis, 1:3-58i, 1:3-59
pectus deformity vs., III:2-2i, III:2-3
Mislabeled films, 11:3-5
Mitral regurgitation. See Mitral valve dysfunction
Mitral stenosis
alveolar microlithiasis vs., 1:1-65
metastatic pulmonary calcification vs., 1:1-61
Mitral valve dysfunction, 11:3-32 to 11:3-34,
1I:3-35i
aortic valve dysfunction vs., 11:3-30
calcification, vs. left atrial calcification, 11:3-17
differential diagnosis, 1I:3-32i, 11:3-33 to
11:3-34
high altitude pulmonary edema vs., 1I:4-62i,
11:4-63
neurogenic pulmonary edema vs., 1I:4-66i,
11:4-67
smoke inhalation vs., 1I:4-42i, 11:4-43
Moles,IV:3-18
Mononucleosis, infectious
histoplasmosis vs., 1:1-17
mycobacterial pneumonia vs., 1:1-10
Morgagni hernia
eventration vs., III:3-3
hiatal hernia vs., 11:1-16
lipomatosis vs., III:3-45
partial absence of pericardium vs., 1I:3-2i
pericardial cyst vs., 11:3-9
pericardial metastases vs., 11:3-52
Mosaic pattern of lung attenuation
bronchocentric patterns vs., V:I-34i
differential diagnosis, V:I-46i, V:I-47
ground-glass opacities vs .., V:I-42i, V:1-43
pulmonary vascular disease vs., IV:5-2i, IV:5-3
Mounier-Kuhn syndrome. See
Tracheobronchomegaly
Mucoepidermoid carcinoma, 1:3-95
Mucoid impaction
allergic bronchopulmonary aspergillosis vs.,
1:3-47
bronchial atresia vs., 1:3-14i, 1:3-15
pulmonary embolism vs., 11:4-52
Mucormycosis, 1:1-21
Multiple myeloma, III:2-11
Mycobacteria, atypical, IV:3-2i
Mycobacterial avium complex, 1:3-38 to 1:3-40,
1:3-411
differential diagnosis, 1:3-38i, 1:3-39
middle lobe syndrome vs., 1:3-58i, 1:3-59
Mycobacterial pneumonia, 1:1-8 to 1:1-10, l:l-11i
aspergillosis vs., 1:1-21
coccidioidomycosis vs., 1:1-27
differential diagnosis, 1:1-8i, 1:1-9 to 1:1-10
drug reactions vs., IV:6-6i, IV:6-7
lung abscess vs., 1:1-12i, 1:1-13
mycobacterial avium complex vs., 1:3-38i,
1:3-39
small cell lung cancer vs., IV:3-7
Myocardial calcification (infarction). See
Ventricular calcification
Myxedema, 11:3-44 to 11:3-45
differential diagnosis, 11:3-44, II:3-44i
Myxoma, left atrial, 11:3-46 to 11:3-48, 1I:3-49i
calcification vs., 11:3-17
differential diagnosis, 1I:3-46i, 11:3-47 to
11:3-48
mitral valve dysfunction vs., 11:3-34
N
Necrotizing fasciitis, 1I1:2-10i, III:2-11
Nerve sheath tumors, 11:1-58 to 11:1-60, 11:1-611.
See also specific tumor types
differential diagnosis, 1I:1-58i, 11:1-59
extramedullary hematopoiesis vs., 11:1-67
Pancoast tumor vs., III:I-33
sympathetic ganglion tumor vs., 11:1-63
 INDEX
Neurenteric cyst
nerve sheath tumor vs., II:1-59
sympathetic ganglion tumor vs., II:1-63
Neuroblastoma
Askin tumor vs., III:2-28i, III:2-29
extramedullary hematopoiesis vs., 1I:1-66i,
II:1-67
hemangioma/lymphangioma vs., II:1-56
Neuroendocrine tumor
thymic, definition of, TI:1-34
thymoma vs., II:1-32
Neurofibroma
Castleman disease vs., II:1-49
hiatal hernia vs., 11:1-16
middle mediastinal mass vs., V:1-60
mimicking solitary pulmonary nodule, IV:3-18
Pancoast tumor vs., 11I:1-32i, III:1-33 to
III:1-34
pericardial cyst vs., II:3-9
sympathetic ganglion tumor vs., 1I:1-62i,
11:1-63
Neurofibromatosis
alpha-l antiprotease deficiency vs., 1:3-23
aortic coarctation vs., II:2-11
kyphoscoliosis vs., III:2-6
lymphangiomyomatosis vs., 1:2-61
Madan syndrome vs., 1I:2-32i, II:2-33
multiple pulmonary nodules vs., V:1-26i,
V:1-28
Takayasu disease vs., II:2-45
talcosis vs., II:4-35
Neurogenic tumor
fibrous tumor of pleura vs., 11I:1-36i, III:1-37
mediastinal, vs. Castleman disease, II:1-49
Neuromuscular disorders, kyphoscoliosis vs., III:2-6
Nipple shadow, IV:3-16i, IV:3-18
Nocardia,IV:6-16
Non-Hodgkin lymphoma (NHL)
mediastinal, 11:1-26 to II:1-28, 1I:1-29i
differential diagnosis, 1I:1-26i, II:1-27 to
II:1-28
pulmonary, vs. cryptogenic organizing
pneumonia, 1:1-80i, 1:1-81
small cell lung cancer vs., IV:3-6i, IV:3-7
o extramedullary hematopoiesis vs., 1I:1-66i,
Oat cell carcinoma. See Lung cancer, small cell
Opportunistic pulmonary infections, 1:1-37
Ossification, idiopathic
alveolar microlithiasis vs., 1:1-65
metastatic pulmonary calcification vs., 1:1-60i,
1:1-62
Osteochondroma, 1:1-10
Osteomyelitis
Askin tumor vs., 11I:2-30
sternal fracture vs., IV:2-28i
with abscess, vs. anterior mediastinal mass,
V:1-56
Osteophytes, thoracic, V:1-64
Osteosarcoma
Askin tumor vs., I1I:2-29
chest wall lymphoma vs., I1I:2-27
empyema necessitatis vs., III:2-11
left atrial calcification vs., II:3-17
p
Pacemakers, cardiac. See Cardiac pacemakers
Pan bronchiolitis, diffuse, 1:3-11
Pancoast tumor, III:1-32 to 11I:1-34, III:1-35i
apical pleural cap vs., III:l-lO, III:l-lOi
chest wall lymphoma vs., III:2-26i, I1I:2-27
differential diagnosis, III:1-32i, I1I:1-33 to
III:1-34
extrapleural apicolysis vs., IV:4-28
exudative pleural effusion vs., 1I1:1-6i, III:1-7
Pancreatic pseudocyst
broncho-pleural fistula vs., 11I:1-20i, III:1-21 xix
empyema vs., 11I:1-16i, III:1-17
pericardial cyst vs., II:3-9
thoracic cyst vs., II:1-4
Papillary fibroelastoma, II:3-48
Papillomatosis
laryngeal, bronchocentric patterns in, V:1-36
laryngotracheal, 1:3-42 to 1:3-44, 1:3-45i
bronchiectasis vs., 1:3-35
differential diagnosis, 1:3-42i, 1:3-43
Langerhans cell granulomatosis vs., 1:3-76i,
1:3-77
lymphangiomyomatosis vs., 1:2-61
lymphocytic interstitial pneumonia vs.,
1:2-56i,I:2-58
relapsing polychondritis vs., 1:3-55
rhinoscleroma vs., 1:3-28i
tracheopathia osteochondroplastica vs.,
1:3-92i,I:3-93
tracheobronchial, vs. centrilobular emphysema,
1:3-84i, 1:3-85
Paraesophageal hernia. See Hiatal hernia
Paraganglioma
Castleman disease vs., 11:1-50
11:1-67
nerve sheath tumor vs., II:1-59
Paraquat inhalation, 1I:4-46i, TI:4-47
Paratracheal air cyst, 1:3-26 to 1:3-27
apical lung hernia vs., 11I:1-2i
differential diagnosis, 1:3-26i
tracheobronchomegaly vs., 1:3-6i, 1:3-7
Patent ductus arteriosus
aortic aneurysm vS., 11:2-401, II:2-41
 INDEX
perfusion abnormalities in, IV:S-8
Patterns, V:I-2 to V:I-6S
basilar peripheral lung zone disease, V:I-14 to
V:I-16, V:1-17i
cystic lung disease, V:1-30 to V:1-32, V:1-33i
focal decreased density lung disease, V:I-22 to
V:I-24, V:1-2Si
focal increased density lung disease, V:I-18 to
V:1-20, V:1-2li
high-resolution computed tomography
bronchocentric pattern, V:I-34 to V:I-36,
V:1-37i
ground-glass opacities, V:1-42 to V:1-44,
V:l--4Si
lymphatic pattern, V:I-38 to V:1-40, V:l--4li
mosaic pattern of lung attenuation, V:I-46
to V:I-48, V:l--49i
tree-in-bud pattern, V:l-S0 to V:l-S2,
V:l-S3i
lung consolidation
acute, V:I-2 to V:1--4,V:l-Si
chronic, V:I-6 to V:I-8, V:1-9i
xx mediastinal mass
anterior, V:l-S4 to V:l-S6, V:l-S7i
middle, V:l-S8 to V:1-60, V:1-6li
posterior, V:1-62 to V:1-64, V:1-6Si
multiple pulmonary nodules, V:1-26 to V:I-28,
V:1-29i
upper lung zone predominant disease, V:I-I0 to
V:I-12, V:l-13i
PEARL(unilateral lung disease), 1:2-S4
Pectus deformity, 1lI:2-2 to III:2-3
differential diagnosis, II1:2-2i, III:2-3
pectus excavatum vs. middle lobe syndrome,
1:3-S8i, 1:3-S9
Pericardial cyst, Il:3-8 to Il:3-1O, II:3-11i
diaphragmatic hernia vs.., IlI:3-8
differential diagnosis, II:3-8i, Il:3-9 to Il:3-10
hiatal hernia vs., Il:I-16
lipomatosis vs., III:3-4S
metastases vs., II:3-S0i, II:3-S 1
partial absence of pericardium vs., II:3-2i, Il:3-3
postcardiac injury syndrome vs., II:3--40i,
Il:3--42
Pericardial effusion, malignant. See Pericardium,
metastases
Pericarditis
constrictive, Il:3-36 to Il:3-38, II:3-39i
differential diagnosis, II:3-36i, II:3-37 to
II:3-38
superior vena cava obstruction vs., II:2--48i
treatment-induced, II:3-S 1
without constriction, II:3-38
Pericardium
calcification
constrictive pericarditis vs., Il:3-37
coronary artery calcification vs., II:3-12i,
11:3-13
valve and annular calcification vs., II:3-24i,
II:3-26
ventricular calcification vs., II:3-20i, II:3-21
cysts. See Pericardia I cyst
fat pad vs. cyst, II:3-9
metastases, II:3-S0 to Il:3-S2, I1:3-S3i
differential diagnosis, I1:3-S0i, Il:3-S 1 to
II:3-S2
left atrial myxoma vs., Il:3-48
neoplasms
cardiac volvulus vs., IV:4-S0i, IV:4-S1
constrictive pericarditis vs., I1:3-36i, Il:3-38
left atrial myxoma vs., II:3-48
partial absence vs., II:3-3
partial absence of, II:3-2 to II:3-3
differential diagnosis, II:3-2i, Il:3-3
left, cardiac volvulus vs., IV:4-S0i, IV:4-S 1
metastases vs., I1:3-S0i, Il:3-S2
Pesticide exposure, I1:4--46i, 11:4-47
Pheochromocytoma, I1:1--48i, Il:l-S0
Phlegmon. See Lung abscess
Phrenic ampulla, II:1-12i, II:1-13
Phrenic nerve paralysis, III:3-10 to 111:3-12,II1:13i
diaphragmatic eventration vs., 1lI:3-3
differential diagnosis, II1:3-10i, IlI:3-11 to
III:3-12
Plague
histoplasmosis vs., 1:1-17
mycobacterial pneumonia vs., 1:1-10
Plasmacytoma
anterior mediastinal mass vs., V:l-S4i, V:l-S6
empyema necessitatis vs., IlI:2-11
Pleura, Ill: 1-2 to III:1-SS
apical lung hernia, Ill: 1-2 to IIl:3
apical pleural cap, 111:1-10to IlI:l-11
asbestos related pleural disease, IIl:1-S2 to
IIl:1-S4, II1:1-SSi
broncho-pleural fistula, IlI:1-20 to 1II:1-22,
II1:1-23i
empyema, III:1-16 to IlI:1-18, II1:1-19i
Erdheim Chester disease, III:1-40 to Ill: 1-42,
II1:1--43i
exudative pleural effusion, Ill: 1-6 to IlI:1-8,
II1:1-9i
fibrous tumor of, Ill: 1-36 to 1lI:1-38, III:1-39i
Askin tumor vs., III:2-28i, IlI:2-29
differential diagnosis, II1:1-36i, IIl:1-37
hemangioendothelioma vs., Ill: 1-49
pleural metastasis vs., 1lI:1-2S
posterior mediastinal mass vs., V:1-64
splenosis vs., IV:2-39
transudative pleural effusion vs., III:l--44i,
IlI:1-4S
 INDEX
hemangioendothelioma, III:1-48 to III:1-50,
III:I-5li
malignant mesothelioma, III:1-28 to III:1-30,
III:I-3li
metastasis. See Pleural metastasis
Pancoast tumor, III:I-32 to III:1-34, III:I-35i
sclerosis. See Pleurodesis
systemic lupus erythematosus, III:1-12 to
III:1-14, III: 1-15i
transudative pleural effusion, III:1-44 to
III:1-46, III: 1-4 7i
tumors, IV:l-lOi, IV:l-ll to IV:I-12
yellow-nail syndrome, III:1-4 to III:1-5
Pleural cap, apical, III:1-10 to III:1-11
differential diagnosis, III:I-I0i, III:l-ll
Pancoast tumor vs., 1I1:1-32i, III:I-33 to
III:I-34
Pleural disease, benign vs. malignant, III:1-29
Pleural effusion
chronic, III:I-4i, III:1-5
diaphragmatic rupture vs., IV:2-31
exudative, III:I-6 to III:1-8, III:I-9i
differential diagnosis, III:I-6i, III:I-7
transudative effusion vs., III:I-45
immunosuppression (non-AIDS) vs., IV:6-16
interlobar, vs. focal increased density lung
disease, V:1-20
lobar atelectasis vs., IV:I-2i, IV:I-4
loculated, vs. pleural metastasis, 1I1:1-24i,
III:1-25
malignant, III:I-25
partial absence of pericardium vs., II:3-3
pericardial cyst vs., 1I:3-8i, II:3-9 to II:3-10
pericardial metastases vs., II:3-52
posterior mediastinal mass vs., V:I-62i, V:I-64
subpulmonic, III:3-3
supine
apical pleural cap vs., III:1-10, III:l-IOi
Pancoast tumor vs., III:I-33
thoracic duct tear vs., IV:2-46i, IV:2-47
thoracotomy vs., IV:4-19i, IV:4-22
transudative, III:1-44 to III:I-46, III:I-47i
Pleural masses, multiple
focal increased density lung disease vs., V:I-20
splenosis vs., IV:2-39
Pleural metastasis, III:1-24 to III:1-26, 1I1:1-27i
apical pleural cap vs., III:1-11
differential diagnosis, III:I-24i, IlI:I-25
empyema vs., 11I:1-16i, III:I-17
extramedullary hematopoiesis vs., II:I-67
exudative pleural effusion vs., 11I:1-6i, III:I-7
fibrous tumor of pleura vs., 11I:1-36i, III:I-37
hemangioendothelioma vs., 1I1:1-48i, III:I-49
multiple pulmonary nodules vs., V:I-26i,
V:1-28
Pancoast tumor vs., III:1-34
pleurodesis vs., IV:4-14i, IV:4-15
posterior mediastinal mass vs., V:I-62i, V:I-64
splenosis vs., IV:2-38i, IV:2-39
Pleural tent. See Apicolysis
Pleurodesis, IV:4-14 to IV:4-16, IV:4-17i
differential diagnosis, IV:4-14i, IV:4-15 to
IV:4-16
empyema vs., 1I1:1-16i, III:I-17
Pleuropericarditis
postcardiac injury syndrome vs., 1I:3-40i,
II:3-41 to II:3-42
viral, III:1-13
Plombage, extra pleural. See Thoracoplasty
Pneumatoceles
laryngeal papillomatosis vs., 1:3-43
lung abscess vs., 1:1-13
trauma-related, 1I:2-14i, II:2-15
Pneumoconiosis. See Asbestosis
Pneumocystis pneumonia, 1:2-6 to 1:2-8, 1:2-9i
acute interstitial pneumonia vs., 1:1-57
aspiration vs., IV:5-14i, IV:5-15
cardiogenic pulmonary edema vs., 1:1-32i xxi
desquamative interstitial pneumonia vs.,
1:1-76i,I:I-78
differential diagnosis, 1:2-6i, 1:2-7
diffuse alveolar hemorrhage vs., 1:1-44i
drug reactions vs., IV:6-7
fat embolism vs., 1:1-40i
Goodpasture syndrome vs., 1:1-48i, 1:1-50
Langerhans cell granulomatosis vs., 1:3-77
lipoid pneumonia vs., 1:1-69
lung abscess vs., 1:1-13
mosaic pattern of lung attenuation vs., V:I-46i,
V: 1--47
noncardiac pulmonary edema vs., 1:1-36i,
1:1-37
pulmonary alveolar proteinosis vs., I:1-73
vasculitis vs., 1I:4-26i, II:4-27
Pneumomediastinum, IV:2-2 to IV:2-4, IV:2-5i
air embolism vs., IV:2-42i, IV:2-43
differential diagnosis, IV:2-2i, IV:2-3
pneumothorax vs., IV:2-6i, IV:2-7
tracheobronchial tear vs., IV:2-1Oi
Pneumonectomy, IV:4-30 to IV:4-32, IV:4-33i
differential diagnosis of complications,
IV:4-30i, IV:4-31 to IV:4-32
Pneumonectomy syndrome, IV:4-30i, IV:4-32
Pneumonia
acute interstitial, 1:1-56 to 1:1-58, 1:1-59i
differential diagnosis, 1:1-56i, 1:1-57
respiratory bronchiolitis vs., 1:3-80i, 1:3-81
alveolar macrophage. See Desquamative
interstitial pneumonia
Aspergillus, 1:3-48
bacterial. See Bacterial pneumonia
 INDEX
blastomycosis vs., [:1-24, 1:1-24i
bronchiectasis vs., [:3-35
bronchioloalveolar cell carcinoma vs., 1:1-84i,
[:1-85
cardiogenic pulmonary edema vs., I:1-33
cryptogenic organizing. See Cryptogenic
organizing pneumonia
desquamative interstitial. See Desquamative
interstitial pneumonia
drug abuse vs., II:4-38i, 11:4-39
eosinophilic. See Eosinophilic pneumonia
focal increased density lung disease vs., V:I-18i,
V:I-19
fungal, posttransplant, IV:4-43
Goodpasture syndrome vs., I: 1-48i, I:1-50
high altitude pulmonary edema vs., 11:4-63
hypersensitivity. See Hypersensitivity
pneumonitis
infectious, mosaic pattern of lung attenuation
in, V:I-47
intralobar sequestration vs.., II:2-14i, 11:2-15
lipoid. See Lipoid pneumonia
xxii lobar atelectasis vs., IV:1-4
lobar torsion vs., IV:4-46i, IV:4-47
lung trauma vs., IV:2-12i, IV:2-14
lymphocytic interstitial. See Lymphocytic
interstitial pneumonia
middle lobe syndrome vs., 1:3-58i
mycobacterial. See Mycobacterial pneumonia
necrotizing. See Lung abscess
neurogenic pulmonary edema vs., 11:4-67
nonspecific interstitial. See Interstitial
pneumonitis, nonspecific
parasitic, 1:1-28 to 1:1-30, 1:1-3li
differential diagnosis, 1:1-28i, 1:1-29
pectus deformity vs., 1II:2-2i, III:2-3
pneumococcal vs. staphylococcus, 1:I---6i
Pneumocystis]iroveci. See Pneumocystis
pneumonia
pneumomediastinum vs., IV:2-3
post-obstructive, vs. intralobar sequestration,
11:2-16
pulmonary alveolar proteinosis vs., 1:1-73
pulmonary embolism vs., 11:4-52
round, vs. missed lung cancer, IV:3-26
septic emboli vs., II:4-22i, 11:4-23
sickle cell disease vs., IV:6-22i, IV:6-23
silo-filler's disease vs., II:4-46i, 11:4-47
smoke inhalation vs., 11:4-43
staphylococcus, 1:1-6 to 1:1-7
differential diagnosis, 1:1-6i, 1:1-7
systemic lupus erythematosus vs., III:I-12i,
111:1-13
vasculitis vs., II:4-26i, II:4-27
viral, 1:2-2 to [:2-4, 1:2-5i
differential diagnosis, 1:2-2i, 1:2-3
drug reactions vs., IV:6-6i, IV:6-7
Pneumonitis. See Bacterial pneumonia;
Hypersensitivity pneumonitis
Pneumopericardium
air embolism vs., IV:2-42i, IV:2-43
pneumomediastinum vs., IV:2-2i, [V:2-3
Pneumothorax, lV:2-6 to IV:2-8, IV:2-9i
differential diagnosis, IV:2-6i, IV:2-7
focal decreased density lung disease vs., V:I-22i
pneumomediastinum vs., IV:2-2i, [V:2-3
tracheobronchial tear vs., IV:2-10
Poland syndrome, III:2-8 to 11I:2-9
differential diagnosis, 1II:2-8i, 11I:2-9
Polyangiitis, microscopic
Goodpasture syndrome vs., 1:1-48i, [:1-49
Pneumocystis pneumonia vs., [:2-7
Wegener granulomatosis vs., 11:4-29
Polyarteritis nodosa
asthma vs., 1:3-69
Wegener granulomatosis vs., 11:4-29
Polychondritis, relapsing, [:3-54 to [:3-56, 1:3-57i
amyloidosis vs., [:3-89
differential diagnosis, 1:3-54i, [:3-55
Marfan syndrome vs., II:2-32i, 11:2-34
rhinoscleroma vs., 1:3-28i
saber-sheath trachea vs., [:3-61
tracheopathia osteochondroplastica vs., 1:3-92i,
[:3-93
Polymyositis-dermatomyositis, [:2-30 to [:2-32,
1:2-33i
differential diagnosis, I:2-30i, [:2-31
Post-intubation stenosis
relapsing polychondritis vs., [:3-55
rhinoscleroma vs., [:3-28
Postcardiac injury syndrome, 11:3-40 to 11:3-42,
II:3-43i
differential diagnosis, II:3-40i, 11:3-41 to
11:3-42
Pott disease
kyphoscoliosis vs., III:2-6
spinal fracture vs., IV:2-24i, [V:2-26
Pseudoaneurysm
aortic coarctation vs., II:2-10i, 11:2-11
aortic transection vs., IV:2-20i, [V:2-21
Pseudochylothorax, IV:2-46i, [V:2-47
Pseudocoarctation, II:2-10i, 11:2-11
Pseudocollaterals, 11:2-49
Pseudocyst, pancreatic. See Pancreatic pseudocyst
Pseudodiverticu[osis, II:1-12i, 11:1-12to 11:1-13
Pseudofibrosis, cyanotic, IV:5-8
Pulmonary alveolar proteinosis, [:1-72 to [:1-74,
1:1-75i
acute interstitial pneumonia vs., 1:1-57
acute lung consolidation vs., V:1-4
alveolar microlithiasis vs., [:1-66
aspiration vs., IV:5-14i, [V:5-15
 INDEX
bronchioloalveolar cell carcinoma vs., 1:1-86
cardiogenic pulmonary edema vs., 1:1-33
differential diagnosis, I:l-72i, 1:1-73
lipoid pneumonia vs., I:1-68i, 1:1-69
metastasis vs., IV:6-4
nonspecific interstitial pneumonitis vs., I:2-34i,
1:2-35
Pneumocystis pneumonia vs., 1:2-7
viral pneumonia vs., I:2-2i, 1:2-3
Pulmonary artery
absent left, vs. anomalous origin, II:4-20i,
11:4-21
aneurysm, 11:4-58to 11:4-60, II:4-6li
differential diagnosis, II:4-S8i, 11:4-59
congenital interruption of, 11:4-16 to 11:4-18,
II:4-19i
differential diagnosis, II:4-16i, 11:4-17 to
11:4-18
Scimitar syndrome vs., II:4-8i, 11:4-9
dilatation, idiopathic, 11:4-12 to 11:4-14,
II:4-1Si
aneurysm vs., II:4-S8i, 11:4-59
differential diagnosis, II:4-12i, 11:4-13 to
11:4-14
hypertension vs., II:4-S4i, 11:4-55
hypertension. See Hypertension, pulmonary
artery
left, anomalous origin of, 11:4-20 to 11:4-21
differential diagnosis, II:4-20i, 11:4-21
sarcoma. See Sarcoma, pulmonary artery
stenosis, perfusion abnormalities in, IV:S-6i,
IV:5--8
Pulmonary calcification, metastatic, 1:1-60 to
1:1-62,I:1-63i
alveolar microlithiasis vs., I:1-64i, 1:1-65
differential diagnosis, I:1-60i, 1:1-61 to 1:1-62
lung ossification vs., 1:2-51
talcosis vs., II:4-34i, 11:4-35
upper lung zone predominant disease vs., V:1-12
as upper zone lung disease, IV:5-12
Pulmonary edema
bacterial pneumonia vs., I:1-2i, 1:1-3
cardiogenic, 1:1-32 to 1:1-34, I:1-3Si
asthma vs., 1:3-69
differential diagnosis, I:1-32i, 1:1-33
Erdheim Chester disease vs., III:1-41
in lung cancer staging, IV:3-10i
lymphatic patterns in, V:1-38i, V:1-39
neurogenic pulmonary edema vs., 11:4-67
noncardiac vs., 1:1-36i, 1:1-37
radiation-induced lung disease v s., lV:6-11
systemic lupus erythematosus vs., III:1-13
chronic lung consolidation vs., V:1-6i, V:1-8
diffuse alveolar hemorrhage vs., I:1-44i, 1:1-45
drug abuse vs., 11:4-40
drug reactions vs., IV:6-6i, IV:6-7
Erdheim Chester disease vs., III: 1-40i, III:1-41
fat embolism vs., 1:1-41
high altitude, 11:4-62 to 11:4-64, II:4-6Si
differential diagnosis, 1I:4-62i, 11:4-63
neurogenic pulmonary edema vs., 11:4-67
hydrostatic
acute interstitial pneumonia vs., I:1-S6i,
1:1-57
fat embolism vs., 1:1-41
smoke inhalation vs., 11:4-43
lymphangitic carcinomatosis vs., I:2-S2i, 1:2-53
negative pressure
high altitude pulmonary edema vs., 11:4-63
neurogenic pulmonary edema vs., 11:4-67
smoke inhalation vs., II:4-42i, 11:4-43
neurogenic, 11:4-66 to 11:4-68, 1I:4-69i
differential diagnosis, 1I:4-66i, 11:4-67
high altitude pulmonary edema vs., 1I:4-62i,
11:4-63
smoke inhalation vs., 11:4-43
upper lung zone predominant disease vs.,
V:1-12
as upper zone lung disease, IV:5-12 xxiii
noncardiac, 1:1-36 to 1:1-38, 1:1-39i
cardiogenic pulmonary edema vs., 1:1-33
differential diagnosis, I:1-36i, 1:1-37
Goodpasture syndrome vs., 1:1-50
parasitic pneumonia vs., I:1-28i, 1:1-29
pneumocystis pneumonia vs., I:2-6i, 1:2-7
pulmonary alveolar proteinosis vs., 1:1-73
sickle cell disease vs., IV:6-23
subsegmental atelectasis vs., IV:1-7
vasculitis vs., 1I:4-26i, 11:4-27
viral pneumonia vs., I:2-2i, 1:2-3
Pulmonary embolism, 11:4-50 to 11:4-52, 1I:4-S3i.
See also Embolism
air embolism vs., IV:2-43
aspergillosis vs., 1:1-13
cardiac volvulus vs., IV:4-51
congenital interruption of pulmonary artery vs.,
1I:4-16i,II:4-17
cryptogenic organizing pneumonia vs., 1:1-81
differential diagnosis, 1I:4-S0i, 11:4-52
fat. See Fat pulmonary embolism
high altitude pulmonary edema vs., 11:4-63
idiopathic pulmonary artery dilatation vs.,
II:4-13
metastasis vs., lV:6-4
pulmonary artery sarcoma vs., 1I:4-70i, 11:4-71
recurrent, vs. asthma, 1:3-69
septic, 11:4-22 to 11:4-24, 1I:4-2Si
differential diagnosis, 1I:4-22i, 11:4-23
lung abscess vs., 1:1-13
pulmonary arteriovenous malformation vs.,
1I:4-2i, 11:4-3
Wegener granulomatosis vs., 11:4-29
 INDEX
sickle cell disease vs., IV:6-23
tumor-related, 11:4-74 to 11:4-76, 1I:4-77i
differential diagnosis, 1I:4-74i, 11:4-75 to
11:4-76
with infarction, vs. round atelectasis, IV:I-I0i,
lV:I-12
Pulmonary fibrosis
idiopathic, 1:2-14 to 1:2-16, 1:2-17i
accelerated, acute interstitial pneumonia vs.,
1:1-57
asbestosis vs., 1:2-38i, 1:2-39
basilar peripheral lung zone disease vs.,
V:I-15
berylliosis vs., 1:2-47
desquamative interstitial pneumonia vs.,
1:1-77
differential diagnosis, 1:2-14i, 1:2-15
drug reactions vs., IV:6-6i, IV:6-7
gravitational gradients in, IV:5-12
hypersensitivity pneumonitis vs., 1:2-18i,
1:2-19
lymphangiomyomatosis vs., 1:2-61
XXIV lymphangitic carcinomatosis vs., 1:2-52i,
1:2-53
metastasis vs., IV:6-3
nonspecific interstitial pneumonitis vs.,
1:2-35
polymyositis-dermatomyositis vs., 1:2-31
scleroderma vs., 1:2-26i, 1:2-27
systemic lupus erythematosus vs., 11I:1-12i,
111:1-13
upper lung zone predominant disease vs.,
V:l-lOi
progressive massive
mycobacterial avium complex vs., 1:3-39
mycobacterial pneumonia vs., 1:1-8i, 1:1-9
radiation-induced, 1:3-2i, 1:3-4
tracheobronchomegaly vs., 1:3-6i, 1:3-7
Pulmonary hemorrhage
aspiration vs., IV:5-14i, IV:5-15
bacterial pneumonia vs., 1:1-2i, 1:13
cardiogenic pulmonary edema vs., 1:1-32i,
1:1-33
chronic lung consolidation vs., V:I-6i, V:I-8
diffuse
posttransplant,IV:4-43
pulmonary alveolar proteinosis vs., 1:1-72i,
1:1-73
eosinophilic pneumonia vs., 1:1-54
fat embolism vs., 1:1-41
immunosuppression (non-AIDS) vs., IV:6-15
lipoid pneumonia vs., 1:1-69
noncardiac pulmonary edema vs., 1:1-36i,
1:1-37
pneumocystis pneumonia vs., 1:2-7
sickle cell disease vs., IV:6-22i, IV:6-23
viral pneumonia vs., 1:2-2i, 1:2-3
Pulmonary hemosiderosis, idiopathic, 1:2-7
Pulmonary infarct
bacterial pneumonia vs., 1:1-4
coccidioidomycosis vs., 1:1-26i
eosinophilic pneumonia vs., 1:1-52i, 1:1-53
focal increased density lung disease vs., V:1-19
immunosuppression (non-AIDS) vs., IV:6-15
lung abscess vs., 1:1-13
non-small cell lung cancer vs., IV:3-4
phrenic nerve paralysis vs., III:3-11
septic emboli vs., 1I:4-22i, 11:4-23
Pulmonary injuries
fat embolism vs., 1:1-41
lobar torsion vs., IV:4-46i, IV:4-47
staphylococcus pneumonia vs., 1:1-6i, 1:1-7
subsegmental atelectasis vs., IV:I-7
Pulmonary nodules
multiple, V:I-26 to V:I-28, V:I-29i
differential diagnosis, V:I-26i, V:I-27 to
V:1-28
splenosis vs., IV:2-39
solitary, IV:3-16 to IV:3-18, IV:3-19i
arteriovenous malformation vs., IV:4-3
bronchial atresia vs., 1:3-16
mimics of, IV:3-16i, IV:3-18
small cell lung cancer vs., IV:3-7
Pulmonary obstruction, unilateral, 11:2-11
Pulmonary sequestration. See also Intralobar
sequestration
extralobar vs. intralobar, 1I:2-14i, 11:2-15
focal increased density lung disease vs., V:1-19
missed lung cancer and, IV:3-24i, IV:3-26
Scimitar syndrome vs., 1I:4-8i, 11:4-9
Pulmonary valve stenosis
idiopathic pulmonary artery dilatation vs.,
1I:4-12i,II:4-13
pulmonary artery aneurysm vs., 1I:4-58i, 11:4-59
pulmonary artery hypertension vs., 1I:4-54i,
11:4-55
Pulmonary vasculature, 11:4-2 to 11:4-79. See also
Pulmonary artery
anomalous venous return, partial, 11:4-6 to
11:4-7
left superior vena cava vs., 1I:2-18i, 11:2-19
arteriovenous malformations, 11:4-2 to 11:4-4,
1I:4-5i
capillary hemangiomatosis, 11:4-78 to 11:4-79
drug abuse and, 11:4-38 to 11:4-40, 1I:4-4li
mosaic patterns, IV:5-2i, IV:5-3
veins
confluence of, mimicking solitary pulmonary
nodule,IV:3-18
varices, vs. focal increased density lung
disease, V:I-19
wandering, 11:4-9
 INDEX
Pulmonary venous hypertension. See Pulmonary
edema, cardiogenic
R s
Radiation injuries. See also Lung diseases, radiation-
induced
extrapleural fluid vs. Pancoast tumor, Ill: 1-33
fibrosis
apical pleural cap vs., Ill: 1-10
Pancoast tumor vs., Ill:I-33
pleural metastasis vs., Ill: 1-25
kyphoscoliosis vs., Ill:2-6
pericardial metastases vs., II:3-5 1
Radiation therapy
immunosuppression (non-AIDS) vs., lV:6-15
thoracotomy vs., IV:4-22
Radiographic artifacts, Ill:2-9
Renal carcinoma, IV:6-2i, IV:6-3
Renal failure, acute, I:I-4Oi
Renal thrombus, IV:6-2i
Respiratory bronchiolitis, 1:3-80 to 1:3-82, I:3-83i
bronchocentric patterns in, V:I-35
desquamative interstitial pneumonia vs., 1:1-77
differential diagnosis, I:3-80i, 1:3-81
hypersensitivity pneumonitis vs., I:2-18i, 1:2-19
tree-in-bud pattern vs., V:I-51
Retroperitoneal fibrosis, Ill: 1-41
Rhabdomyosarcoma
Askin tumor vs., III:2-29
elastoma or fibroma vs., Ill:2-19
Rheumatoid arthritis, 1:2-22 to 1:2-24, I:2-2Si
asbestosis vs., 1:2-38i, 1:2-39
basilar peripheral lung zone disease vs., V:1-15
differential diagnosis, 1:2-22i, 1:2-23 to 1:2-24
focal decreased density lung disease vs., V:1-23
idiopathic pulmonary fibrosis vs., 1:2-15
polymyositis-dermatomyositis vs., 1:2-31
scleroderma vs., 1:2-27
systemic lupus erythematosus vs., Ill:I-13
yellow-nail syndrome vs., III:I-4i, III:1-5
Rheumatoid nodules, necrotic
focal increased density lung disease vs., V:I-19
lung abscess vs., 1:1-13
multiple pulmonary nodules vs., V:I-27
Wegener granulomatosis vs., II:4-29
Rhinoscleroma, 1:3-28 to 1:3-29
amyloidosis vs., 1:3-89
differential diagnosis, 1:3-28i
relapsing polychondritis vs., 1:3-S4i, 1:3-55
saber-sheath trachea vs., 1:3-61
Rhodococcus, 1:1-24, 1:1-24i
Rib fractures, IV:2-16 to IV:2-18, IV:2-19i
asbestos related pleural disease vs., III:1-53
differential diagnosis, IV:2-16i, IV:2-17
mimicking solitary pulmonary nodule, TV:3-16i
multiple pulmonary nodules vs., V:I-26i,
V:1-28
Saber-sheath trachea, 1:3-60 to 1:3-62, 1:3-63i
differential diagnosis, 1:3-60i, 1:3-61
relapsing polychondritis vs., 1:3-55
Sarcoid
amyloidosis vs., 1:3-90
endobronchial, 1:3-93
multiple pulmonary nodules vs., V:I-27
Sarcoidosis, 1:2-10 to 1:2-12, I:2-13i
acute lung consolidation vs., V:1-2i, V:I-4
alveolar microlithiasis vs., 1:1-65
amyloidosis vs., 1:3-88i, 1:3-90
ankylosing spondylitis vs., III:2-14i, III:2-15
asthma vs., 1:3-69
basilar peripheral lung zone disease vs., V:1-14i,
V:I-15
berylliosis vs., 1:2-46i, 1:2-47
cryptogenic organizing pneumonia vs., I:1-80i,
1:1-81 xxv
desquamative interstitial pneumonia vs., 1:1-78
differential diagnosis, I:2-10i, 1:2-11
drug reactions vs., IV:6-6i, IV:6-7
Erdheim Chester disease vs., Ill:I-41
hypersensitivity pneumonitis vs., 1:2-19
idiopathic pulmonary fibrosis vs., 1:2-14i,
1:2-15
Kaposi sarcoma vs., I:3-98i
Langerhans cell granulomatosis vs., 1:3-77
lung cancer staging and, IV:3-lOi
lung ossification vs., 1:2-50
Iymphangitic carcinomatosis vs., 1:2-53
lymphatic patterns in, V:1-38i, V:I-39
metastatic pulmonary calcification vs., 1:1-61
non-Hodgkin lymphoma vs., II:1-26i, II:I-27
nonspecific interstitial pneumonitis vs., 1:2-35
pulmonary alveolar proteinosis vs., 1:1-73
relapsing polychondritis vs., 1:3-55
saber-sheath trachea vs., 1:3-61
scleroderma vs., 1:2-28
silicosis vs., 1:2-42i, 1:2-43
small cell lung cancer vs., IV:3-7
talcosis vs., II:4-34i, II:4-35
tree-in-bud pattern vs., V:l-SOi, V:I-51
upper lung zone predominant disease vs., V:l-ll
as upper zone lung disease, IV:5-12
Sarcoma. See also Angiosarcoma; Chondrosarcoma;
Liposarcoma; Osteosarcoma
aortic, II:2-29
atrial metastasis, V:l-SOi, V:I-5 1
of diaphragm, Ill:3-4
Ewing, III:2-29
fibrosarcoma
 INDEX
elastoma or fibroma vs., III:2-18i, III:2-19
partial absence of pericardium vs., II:3-2i
Kaposi's. See Kaposi sarcoma
leiomyosarcoma, III:1-25
pericardial, IV:4-S0i, IV:4-51
pleural
fibrous tumor of pleura vs., III:1-37
hemangioendothelioma vs., III:I-48i,
III:1-49
pleural metastasis vs., Ill: 1-25
pulmonary artery, 11:4-70 to 11:4-72, II:4-73i
differential diagnosis, II:4-70i, 11:4-71
idiopathic pulmonary artery dilatation vs.,
II:4-12i,II:4-14
metastasis vs., IV:6-2i, IV:6-4
pulmonary embolism vs., 11:4-52
tumor emboli vs., II:4-74i, 11:4-75
rhabdomyosarcoma
Askin tumor vs., II1:2-29
elastoma or fibroma vs., Ill:2-19
soft tissue, III:2-18i, III:2-19
Scarring, IV:I-6i, IV:I-7 to IV:1-8
XXVI Schwan noma
Castleman disease vs., II:I-48i, 11:1-49
cystic, 11:1-4
elastoma or fibroma vs., III:2-18i, Ill:2-19
hiatal hernia vs., 11:1-16
middle mediastinal mass vs., V:1-60
Scimitar syndrome, 11:4-8to 11:4-10, II:4-1li
congenital interruption of pulmonary artery vs.,
II:4-16i,II:4-18
differential diagnosis, II:4-8i, 11:4-9 to 11:4-10
heterotaxy syndrome vs., II:3-4i, 11:3-6
Scleroderma
achalasia vs., 11:1-11
asbestosis vs., I:2-38i, 1:2-39
basilar peripheral lung zone disease vs., V:I-15
gravitational gradients in, IV:5-12
hypersensitivity pneumonitis vs., 1:2-19
idiopathic pulmonary fibrosis vs., 1:2-15
lymphangitic carcinomatosis vs., I:2-S2i, 1:2-53
metastasis vs., IV:6-4
polymyositis-dermatomyositis vs., I:2-30i,
1:2-31
pulmonary, 1:2-26 to 1:2-28, I:2-29i
differential diagnosis, I:2-26i, 1:2-27 to
1:2-28
rheumatoid arthritis vs., I:2-22i, 1:2-23
upper lung zone predominant disease vs.,
V:I-I0i
Scleroma. See Rhinoscleroma
Sclerosis. See Pleurodesis; Scleroderma, pulmonary
Scoliosis, 111:3-11
Seminoma, II:I-22i, 11:1-23
Septal defects
atrial, perfusion abnormalities in, IV:S-6i,
IV:5-8
thickening vs. subsegmental atelectasis, IV:I-6i,
IV:I-7
ventricular
mitral valve dysfunction vs., 11:3-34
perfusion abnormalities in, IV:5-8
Septic emboli. See Pulmonary embolism, septic
Serratus anterior muscle, Ill: 1-54
Shunt, right-to-left, IV:4-32
Sickle cell disease, IV:6-22 to IV:6-24, IV:6-2Si
differential diagnosis, IV:6-22i, IV:6-23
Silicosis, 1:2-42 to 1:2-44, I:2-4Si
alveolar microlithiasis vs., 1:1-65
amyloidosis vs., I:3-88i, 1:3-90
ankylosing spondylitis vs., Ill:2-15
basilar peripheral lung zone disease vs., V:I-14i,
V:I-15
berylliosis vs., I:2-46i, 1:2-47
bronchocentric patterns in, V:I-36
differential diagnosis, I:2-42i, 1:2-43 to 1:2-44
focal increased density lung disease vs., V:I-19
hypersensitivity pneumonitis vs., 1:2-19
Langerhans cell granulomatosis vs., I:3-76i,
1:3-77
lung ossification vs., 1:2-50, I:2-S0i
metastatic pulmonary calcification vs., 1:1-61
sarcoidosis vs., 1:2-11
talcosis vs., II:4-34i, 11:4-35
upper zone lung disease and, IV:5-12, V:l-ll
Silo-filler's disease, 11:4-46 to 11:4-48, II:4-49i
differential diagnosis, II:4-46i, 11:4-47
Sinusitis, allergic aspergillus, 1:3-11
Sjogren syndrome
laryngeal papillomatosis vs., 1:3-43
lymphangiomyomatosis vs., 1:2-61
polymyositis-dermatomyositis vs., 1:2-31
Skin fold
pneumomediastinum vs., lV:2-3
pneumothorax vs., IV:2-6i, lV:2-7
Smoke inhalation, 11:4-42 to 11:4-44, II:4-4Si
differential diagnosis, II:4-42i, 11:4-43
drug abuse vs., II:4-38i, 11:4-40
high altitude pulmonary edema vs., 11:4-63
neurogenic pulmonary edema vs., II:4-66i,
11:4-67
silo-filler's disease vs., II:4-46i, 11:4-47
upper lung zone predominant disease vs., V:1-12
as upper zone lung disease, IV:5-12
Spinal abscess, IV:2-26
Spinal fracture, IV:2-24 to IV:2-26, IV:2-27i
differential diagnosis, IV:2-24i, IV:2-26
Splenosis, IV:2-38 to IV:2-40, IV:2-4li
differential diagnosis, IV:2-38i, IV:2-39 to
IV:2-40
 INDEX
malignant mesothelioma vs., 1I1:1-28i, III:1-29
pleural metastasis vs., 11I:1-24i, III:I-25
Spondylitis
ankylosing. See Ankylosing spondylitis
infectious, III:2-6
Sporotrichosis
histoplasmosis vs., I:1-17
mycobacterial pneumonia vs., 1:1-9
Squamous cell carcinoma, V:I-30i, V:I-31
Staphylococcus infections, IV:6-16
Stents, coronary artery, IV:4-2i
Sternal fracture, IV:2-28 to IV:2-29
differential diagnosis, IV:2-28i
Sternal mass, V:I-54i, V:I-56
Sternotomy, median, IV:4-18 to IV:4-19
differential diagnosis, IV:4-18i, IV:4-19
Stromal tumors, gastrointestinal, 1I:1-70i, II:1-71
Subclavian artery
aberrant right, 11:2-6 to 11:2-8, 1I:2-9i
differential diagnosis, 1I:2-6i, II:2-7 to II:2-8
aneurysm vs. Pancoast tumor, III:I-33
Subglottic stenosis, 1:3-69
Subphrenic abscess, IV:2-31
Subpleural bleb, 1:3-26i
Subpleural fat, III:I-53
Subpulmonic effusion, III:3-lOi, II1:3-11 to
III:3-12
Superior vena cava
left, 11:2-18 to 11:2-20, 1I:2-2li
differential diagnosis, 1I:2-18i, II:2-19
obstruction, 11:2-48 to II:2-50, 1I:2-51i
aortic coarctation vs., II:2-11
azygos continuation of inferior vena cava
vs., 1I:2-22i, I1:2-23
differential diagnosis, 1I:2-48i, 11:2-49
partial anomalous venous return vs., 1I:4-6i
Swyer-Jamessyndrome
congenital interruption of pulmonary artery vs.,
II:4-17
Poland syndrome vs., 1I1:2-8i, III:2-9
Scimitar syndrome vs., II:4-10
Sympathetic ganglion tumors, II:1-62 to 11:1-64,
1I:1-6Si
differential diagnosis, 1I:1-62i, II:I-63
extramedullary hematopoiesis vs., II:I-67
nerve sheath tumor vs., I1:1-59
Syphilis
aortitis vs. Takayasu disease, [[:2-45
histoplasmosis vs., 1:1-17
Systemic lupus erythematosus. See Lupus
erythematosus, systemic
T thymic tumor vs., II:I-36
Takayasu disease, 1I:2-44 to II:2-46, 1I:2-47i
aortic atherosclerosis vs., 1I:2-28i, II:2-29
aortic coarctation vs., 1I:2-10i, [[:2-11
congenital interruption of pulmonary artery vs.,
II:4-17
differential diagnosis, 1I:2-44i, II:2-45 to
II:2-46
Erdheim Chester disease vs., III:1-41
Talcosis, II:4-34 to II:4-36, 1I:4-37i
alveolar microlithiasis vs., 1:1-64i, 1:1-65
differential diagnosis, 1I:4-34i, II:4-35
metastatic pulmonary calcification vs., 1:1-60i,
1:1-61
silicosis vs., 1:2-43
Temporal arteritis, II:2-45
Teratoma
hemangioma/lymphangioma vs., 1I:1-54i,
II:I-56
Hodgkin lymphoma vs., II:I-23
mediastinal goiter vs., II:1-19
thymic rebound vs., 1I:1-38i, II:I-39
Thoracic aneurysm. See Aortic aneurysm
Thoracic cysts, 1I:1-2 to II:I-4, 1I:1-Si. See also
Cystic adenomatoid malformation
aberrant right subclavian artery vs., II:2-8 xxvii
differential diagnosis, 1I:1-2i, II:I-4
Thoracic duct tear, lV:2-46 to lV:2-48, IV:2-49i
differential diagnosis, IV:2-46i, IV:2-47
Thoracic outlet syndrome, III:1-33
Thoracoplasty, IV:4-26 to IV:4-28, IV:4-29i
differential diagnosis, IV:4-26i, IV:4-28
kyphoscoliosis vs., III:2-4i, III:2-6
Thoracotomy, IV:4-19 to IV:4-22, IV:4-23i
differential diagnosis, IV:4-19i, IV:4-22
rib fracture vs., IV:2-16i, IV:2-17
Thromboembolism
chronic pulmonary
capillary hemangiomatosis vs., 1I:4-78i,
I1:4-79
congenital interruption of pulmonary artery
vs., 1I:4-16i, II:4-17
Scimitar syndrome vs., 1I:4-8i, Il:4-10
septic emboli vs., II:4-23
Thrombosis
in-situ, vs. tumor emboli, 1I:4-74i, [[:4-75
intracardiac vs. left atrial myxoma, Il:3-47
pulmonary embolism vs., II:4-52
Thymic cyst
definition of, II:1-34
Hodgkin lymphoma vs., 1I:1-22i
metastases vs., II:3-52
partial absence of pericardium vs., II:3-3
pericardial cyst vs., II:3-9
Thymic rebound, II:I-38 to II:1-39
differential diagnosis, 1I:1-38i, 11:1-39
 INDEX
Thymic tumors, 11:1-34 to 11:1-36, I1:1-37i. See also
specific tumors
carcinoma
definition of, 11:1-34
thymoma vs., 11:1-31
differential diagnosis, I1:1-34i, 11:1-35 to
11:1-36
Thymolipoma
definition of, 11:1-34
lipoid pneumonia vs., 1:1-69
myxedema vs., 11:3-44, I1:3-44i
partial absence of pericardium vs., 11:3-3
pericardial cyst vs., 11:3-9
postcardiac injury syndrome vs., I1:3-40i,
11:3-42
thymoma vs., 11:1-32
Thymoma, 11:1-30 to 11:1-32, I1:1-33i
differential diagnosis, I1:1-30i, 11:1-31 to
11:1-32
fibrous tumor of pleura vs., III:1-36i, III:1-37
germ cell tumor vs., I1:1-40i, 11:1-41
Hodgkin lymphoma vs., I1:1-22i, 11:1-23
xxviii malignant mesothelioma vs., III:1-28i, 11I:1-29
mediastinal goiter vs., 11:1-19
other thymic tumors vs., 11:1-35
splenosis vs., IV:2-38i, IV:2-39
thoracic cyst vs., 11:1-4
Thymus
hyperplasia. See Thymic rebound
normal, I1:1-38i
Thyroid, substernal, 11:2-8
Tracheal diverticulum. See Paratracheal air cyst
Tracheal stenosis, I:3-60i, 1:3-61
Tracheal wall calcification, 1:3-55
Tracheobronchial tear, IV:2-10 to IV:2-11
differential diagnosis, IV:2-10i, IV:2-11
Tracheobronchomalacia, 1:3-50 to 1:3-52, I:3-53i
differential diagnosis, I:3-50i, 1:3-51
saber-sheath trachea vs., I:3-60i, 1:3-61
tracheobronchomegaly vs., 1:3-7
underlying causes of, 1:3-51
Tracheobronchomegaly, 1:3-6 to 1:3-8, 1:3-9i
differential diagnosis, I:3-6i, 1:3-7
saber-sheath trachea vs., I:3-60i, 1:3-61
Tracheocele. See Paratracheal air cyst
Tracheopathia osteochondroplastica, 1:3-92 to
1:3-93
amyloidosis vs., 1:3-89
chronic bronchitis vs., I:3-30i, 1:3-31
differential diagnosis, I:3-92i, 1:3-93
relapsing polychondritis vs., 1:3-54i, 1:3-55
saber-sheath trachea vs., 1:3-61
Tracheostomy tubes. See Tubes and catheters
Transplantation
bone marrow, 1:2-7
lung. See Lung transplantation
Trauma, IV:2-2 to IV:2-49
air embolism, IV:2-42 to IV:2-44, IV:2-45i
aortic transection, IV:2-20 to IV:2-22, IV:2-23i
diaphragmatic tear, IV:2-30 to lV:2-32, IV:2-33i
esophageal tear, IV:2-34 to IV:2-36, IV:2-37i
flail chest, IV:2-16 to IV:2-18, IV:2-19i
lung, IV:2-12 to IV:2-14, IV:2-15i
pneumomediastinum, IV:2-2 to IV:2-4, IV:2-5i
pneumothorax, IV:2-6 to IV:2-8, IV:2-9i
rib fracture, IV:2-16 to IV:2-18, IV:2-19i
spinal fracture, IV:2-24 to IV:2-26, IV:2-27i
splenosis, IV:2-38 to IV:2-40, IV:2-4li
sternal fracture, IV:2-28 to IV:2-29
thoracic duct tear, IV:2-46 to IV:2-48, IV:2-49i
tracheobronchial tear, IV:2-10 to IV:2-11
Tree-in-bud pattern
bronchocentric patterns vs., V:1-34i
differential diagnosis, V:1-50i, V:I-51
Tuberculoma
non-small cell lung cancer vs., IV:3-3
tumor emboli vs., 11:4-76
Tuberculosis. See also Mycobacterial pneumonia
allergic bronchopulmonary aspergillosis vs.,
1:3-47
alveolar microlithiasis vs., I:1-64i, 1:1-65
angioimmunoblastic lymphadenopathy vs.,
II:1-52i,II:1-53
ankylosing spondylitis vs., III:2-14i, 11I:2-15
berylliosis vs., 1:2-47
bronchocentric patterns in, V:1-35
cystic fibrosis vs., 1:3-4
histoplasmosis vs., 1:1-17
Langerhans cell granulomatosis vs., 1:3-77
in lung cancer staging, IV:3-10i
lung ossification vs., 1:2-50, I:2-50i
metastatic pulmonary calcification vs., 1:1-61
miliary
amyloidosis vs., I:3-88i, 1:3-90
sarcoidosis vs., 1:2-11
tree-in-bud pattern vs., V:I-50i, V:I-5 1
multiple pulmonary nodules vs., V:I-27
non-Hodgkin lymphoma vs., II:1-26i, 11:1-28
pleural, IV:4-14i, IV:4-15
post-primary
mycobacterial avium complex vs., I:3-38i,
1:3-39
upper lung zone predominant disease vs.,
V: 1-11
as upper zone lung disease, IV:5-12
primary, vs. sarcoidosis, I:2-10i, 1:2-11
reactivation of, vs. Pancoast tumor, III:1-33
silicosis vs., 1:2-43
Tubes and catheters
abnormal, IV:4-6 to IV:4-8, IV:4-9i
 INDEX
differential diagnosis, IV:4-6i, IV:4-8
normal, IV:4-2 to IV:4-4, IV:4-5i
differential diagnosis, IV:4-2i, IV:4-4
Tularemia
histoplasmosis vs., 1:1-17
mycobacterial pneumonia vs., 1:1-10
Twiddler syndrome, IV:4-10i
u ":3-22
Ulcer, esophageal, 1I:1-12i, 11:1-13
Upper lobe collapse, peripheral
apical pleural cap vs., "1:1-11
Pancoast tumor vs., 11I:1-34
Upper lung zone predominant disease, V:I-I0 to
V:I-12, V:l-13i
differential diagnosis, V:l-lOi, V:l-11 to V:I-12
Uremia, [:1-46
v
Valve and annular calcification, ":3-24 to ":3-26,
1I:3-27i
differential diagnosis, I1:3-24i, 11:3-25 to
11:3-26
left atrial calcification vs., 1I:3-16i, ":3-17
mitral valve, vs. coronary artery calcification,
1I:3-12i
mitral valve dysfunction vs., 1I:3-32i, 11:3-33
ventricular calcification vs., 11:3-201, 11:3-21
Varices
mediastinal, II:1-70 to II:1-72, 1I:1-73i
differential diagnosis, 1I:1-70i, ":1-71
pulmonary venous, 1I:4-6i
Vascular ring, 11:4-21
Vasculitis
connective tissue, 1:2-7
Goodpasture syndrome vs., 1:1-48i, 1:1-49
pulmonary, ":4-26 to ":4-27
differential diagnosis, 1I:4-26i, 11:4-27
Vena cavae. See Inferior vena cava; Superior vena
cava
Veno-occlusive disease, pulmonary, 11:4-32to
11:4-33
capillary hemangiomatosis vs., 1I:4-78i
differential diagnosis, 1I:4-32i, ":4-33
diffuse pulmonary Iymphangiomatosis vs.,
1:2-64i, 1:2-65
Erdheim Chester disease vs., 11I:1-41
lymphatic patterns in, V:I-39
pulmonary embolism vs., ":4-52
Venous hypertension, 1:2-64i, 1:2-65
Venous return, partial anomalous, 11:4-6 to ":4-7
differential diagnosis, 1I:4-6i, 11:4-7
Venous thrombosis, 1:1-41
Ventilation and lung volumes, IV:5-2 to IV:5-4,
IV:5-5i
differential diagnosis, IV:5-2i, IV:5-3
Ventricular calcification, 11:3-20 to ":3-22, 1I:3-23i
constrictive pericarditis vs., ":3-37
coronary artery calcification vs., 1I:3-12i,
":3-14
differential diagnosis, 11:3-201, 11:3-21 to
left atrial calcification vs., 1I:3-12i
valve and annular calcification vs., 11:3-25
Ventricular hypertrophy, right, V:I-54i, V:I-56
Ventricular septal defect
mitral valve dysfunction vs., 11:3-34
perfusion abnormalities in, IV:5-8
Vertebrae, H-shaped, IV:6-23
Vertical fissure of Davis, IV:1-8
Vocal cord dysfunction, 1:3-69
Volume, lung. See Ventilation and lung volumes
w
Wegener granulomatosis, 11:4-28 to ":4-30,
11:4-311
aspergillosis vs., 1:1-201,1:1-21
bronchioloalveolar cell carcinoma vs., I:1-85
cystic lung disease vs., V:I-30i, V:I-31
differential diagnosis, 1I:4-28i, 11:4-29
focal decreased density lung disease vs., V:1-23
focal increased density lung disease vs., V:I-19
Goodpasture syndrome vs., 1:1-49
laryngeal papillomatosis vs., 1:3-43
left atrial myxoma vs., ":3-48
lung abscess vs., 1:1-12i, 1:1-13
metastasis vs., IV:6-3
multiple pulmonary nodules vs., V:1-28
pneumocystis pneumonia vs., 1:2-7
relapsing polychondritis vs., 1:3-54i, 1:3-55
rhinoscleroma vs., 1:3-28i
saber-sheath trachea vs., 1:3-61
septic emboli vs., ":4-23
tracheopathia osteochondroplastica vs., 1:3-92i,
1:3-93
tumor emboli vs., 11:4-76
Williams-Campbell syndrome, 1:3-7
Williams syndrome, 11:2-45
y
Yellow-nail syndrome, 11I:1-4 to "1:1-5
differential diagnosis, III:I-4i, 111:1-5
immotile cilia syndrome vs., 1:3-11
Young syndrome, 1:3-101, 1:3-11
 INDEX
z
Zenker diverticulum
achalasia vs., II:I-IOi, 11:1-11
apical lung hernia vs., III:I-2i
paratracheal air cyst vs., I:3-26i

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