Residents Section Pat tern of the Month

Cantin et al. Bronchiectasis Residents Section Pattern of the Month

Residents

inRadiology
Luce Cantin1 Alexander A. Bankier Ronald L. Eisenberg
Cantin L, Bankier AA, Eisenberg RL

Bronchiectasis
Bronchiectasis is a relatively frequent condition in the United States, with an estimated prevalence of 4.2 per 100,000 persons 1834 years old and 272 per 100,000 persons 75 years or older. It is defined as an irreversible localized or diffuse dilatation, usually resulting from chronic infection, proximal airway obstruction, or congenital bronchial abnormality. On chest radiographs, bronchiectasis manifests as tram tracks, parallel line opacities, ring opacities, and tubular structures. However, chest radiographs lack sensitivity for detecting mild or even moderate disease. CT is substantially more sensitive than chest radiography for showing bronchiectasis, which is characterized by lack of bronchial tapering, bronchi visible in the peripheral 1 cm of the lungs, and an increased bronchoarterial ratio producing the so-called signet-ring sign. According to appearance and severity, bronchiectasis can be classified as cylindric, varicose, or cystic (Fig. 1). The wide differential diagnosis of bronchiectasis can be substantially narrowed by considering both the anatomic location and the distribution of this pathology (Fig 2).

Keywords: airway obstruction, bronchiectasis DOI:10.2214/AJR.09.3053 Received May 15, 2009; accepted after revision May 26, 2009.
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All authors: Department of Radiology, Beth Israel Deaconess Medical Center, Harvard Medical School, 330 Brookline Ave., Boston, MA 02215. Address correspondence to R. L. Eisenberg (rleisenb@bidmc.harvard.edu).

WEB This is a Web exclusive article. AJR 2009; 193:W158W171 0361803X/09/1933W158 American Roentgen Ray Society

Fig. 1Categories of bronchiectasis. AD, Normal bronchus (arrow) (A), cylindric bronchiectasis with lack of bronchial tapering (arrow) (B), varicose bronchiectasis with string-of-pearls appearance (arrow) (C), and cystic bronchiectasis (arrow) (D). (Fig. 1 continues on next page)

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Fig. 1 (continued)Categories of bronchiectasis. AD, Normal bronchus (arrow) (A), cylindric bronchiectasis with lack of bronchial tapering (arrow) (B), varicose bronchiectasis with string-of-pearls appearance (arrow) (C), and cystic bronchiectasis (arrow) (D).

Detection of bronchiectasis

Focal bronchiectasis

Diffuse bronchiectasis

Congenital bronchial atresia Extrinsic compression Endobronchial malignancy Foreign body Broncholithiasis Airway stenosis

Peripheral predominance

Central predominance

Upper lung predominance

Lower lung predominance

Right middle lobe and lingula predominance

Allergic bronchopulmonary aspergillosis Mounier-Kuhns syndrome Williams-Campbell syndrome

Cystic fibrosis Sarcoidosis Postradiation fibrosis

Idiopathic Postinfectious Repeated aspiration Fibrotic lung disease Posttransplant rejection Hypogammaglobulinemia

Atypical mycobacterial infection (MAI) Immotile cilia syndrome

Fig. 2Flowchart shows algorithm for evaluation of bronchiectasis. MAI = Mycobacterium avium-intracellulare.

Focal Bronchiectasis Any cause of airway obstruction can lead to focal bronchiectasis (Fig. 3). In contrast to diffuse bronchiectasis, focal bronchiectasis requires diagnostic bronchoscopy in almost all patients. Bronchial Atresia The most common cause of congenital focal bronchiectasis is bronchial atresia, characterized by obliteration of a bronchus with distal bronchiectasis, mucoid impaction, and air trapping that is most commonly seen in the left upper lobe (Fig. 4). In this rare lesion, the bronchial tree peripheral to the point of obliteration is patent and the lung parenchyma is overinflated because of collateral air drift.

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Fig. 3Focal bronchiectasis (idiopathic) in left lower lobe (arrow).

Fig. 4Bronchial atresia. A, Transverse image of focal bronchiectasis (arrow) distal to bronchial atresia associated with hyperlucency and hyperexpansion of left lung. B, Coronal image shows proximal mucoid impaction (arrow), distal bronchiectasis (arrowhead), and widespread air trapping of left lung.

Extrinsic Compression Extrinsic compression is an acquired cause of focal bronchiectasis, most commonly caused by lymphadenopathy, usually from previous granulomatous exposure. Less frequent causes include sarcoidosis, hilar mass, and metastatic lymphadenopathy. Endoluminal Obstruction by Tumor Most carcinoid tumors are primarily endobronchial lesions, occurring in the central, main, or segmental bronchi (Fig. 5). Some small tumors are located entirely within the lumen. How-

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Fig. 5Carcinoid. This predominantly endobronchial tumor, arising before bifurcation of left upper and lower lobe bronchi, causes distal bronchiectasis. A and B, Transverse images of tumor (arrow, A) and distal bronchiectasis (arrows, B). C and D, Coronal oblique image (C) and volume-rendering reformation (D) in similar orientation as A and B show central carcinoid tumor (arrows) and distal bronchiectasis (arrowheads, C).

ever, some display a dominant extraluminal component with only a small part of the tumor lying within the airway (iceberg lesion). A variety of other benign and malignant neoplasms can also result in obstruction leading to focal bronchiectasis. Foreign Body Aspirated foreign material can result in focal bronchiectasis. Persistence of a noncalcified foreign body, such as a vegetable fiber, within a bronchus for a prolonged period of time can serve as a nidus for calcium deposition. Broncholithiasis Calcified or ossified material within the bronchial lumen can cause focal bronchiectasis. By far the most common cause of broncholithiasis is erosion by and extrusion of a calcified

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Fig. 6Broncholithiasis. AC, Calcified left upper lobe endobronchial broncholithiasis (arrow) from previous tuberculosis exposure is seen on transverse image (A), minimumintensity-projection reformation in coronal oblique plane (B), and volume-rendering reformation (C) in similar orientation. In C, arrow points to distal bronchiectasis.

C adjacent lymph node, usually associated with a long-standing focus of necrotizing granulomatous lymphadenitis, especially after tuberculosis (Fig. 6). Nevertheless, the frequency of broncholithiasis complicating granulomatous infection is quite low. The most common sites are the proximal right middle lobe bronchus and the origin of the anterior segmental bronchus of the upper lobes because of airway anatomy and lymph node distribution. Airway Stenosis Airway stenosis causing focal bronchiectasis (Fig. 7) can result from a broad spectrum of entities including infection, intubation stricture, healing of a tracheostomy stoma, tracheobronchopathia osteochondroplastica, amyloidosis, relapsing polychondritis, sarcoidosis, and fibrosing mediastinitis. Diffuse Bronchiectasis: Upper Predominance Cystic Fibrosis The most common cause of congenital upper-lung-predominant bronchiectasis is cystic fibrosis, commonly associated with enlarged lung volumes and interstitial alterations (Fig. 8). An autosomal recessive genetic disorder causing ineffective clearance of secretions, cystic fibrosis presents with recurrent pneumonias, sinusitis, pancreatic insufficiency, and infertility. Milder forms of cystic fibrosis, however, can remain unrecognized until adulthood (Fig. 9).

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Fig. 7Congenital stenosis of left mainstem bronchus. A and B, Transverse images show stenosis (arrow, A), distal bronchiectasis, and mucoid impaction (arrows, B). C, Coronal reformation image shows bronchial stenosis (arrow).

Sarcoidosis Parenchymal involvement by sarcoidosis can lead to upper and mid lung fibrosis and traction bronchiectasis, typically associated with multiple nodules in a perilymphatic distribution (Fig. 10). Mediastinal and bilateral symmetric lymphadenopathy is common, although it can regress as the interstitial disease worsens. Postradiation Fibrosis Another important cause of upper-lung-predominant bronchiectasis is postradiation fibrosis, in which traction bronchiectasis is usually limited to the radiation port (Fig. 11). A straight interface between the irradiated field and normal lung is often seen. This does not respect anatomic borders, such as fissures and lobes. Postradiation fibrosis may also be bilateral after mediastinal radiation for lymphoma.

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Fig. 8Cystic fibrosis. A and B, Transverse (A) and coronal (B) images show upper lobe predominance of cystic bronchiectasis (arrows) and volume loss, enlarged lung volumes, and diffuse heterogeneous attenuation.

Fig. 9Adult cystic fibrosis. In this milder case, there is upper lobe predominance of cylindric bronchiectasis (white arrows) and bronchiolitis (black arrows).

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Fig. 10Sarcoidosis. A and B, Transverse images show fibrosis and traction bronchiectasis (arrows, B) that predominantly involve upper lobes.

Diffuse Bronchiectasis: Lower Predominance Lower-lung-predominant bronchiectasis, which is the most frequent pattern, is most commonly idiopathic (Fig. 12). However, some cases have known causes. Recurrent Childhood Infections Postinfectious bronchiectasis is a frequent cause of lower-lung-predominant bronchiectasis. It is less seen today because of better control of tuberculosis, earlier treatment of pneumonia, and immunization. However, recurrent infections still remain a frequent cause of bronchiectasis in immune-suppressed patients. Aspiration Any predisposition for repeated aspiration is associated with an increased risk of developing bibasilar bronchiectasis. Common causes include a large hiatal hernia with gastroesophageal reflux, scleroderma (Fig. 13) and other causes of a patulous esophagus, and esophageal motility disorders.

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Fig. 11Postradiation fibrosis. A and B, Right paramediastinal fibrotic changes, which developed after treatment of lung cancer, are associated with traction bronchiectasis (arrows).

Fig. 12Lower lobe predominance of bronchiectasis. A, Subtle idiopathic bibasilar cylindric bronchiectasis shows signet-ring sign (arrows). B, In another patient, there is marked idiopathic left lower bronchiectasis with volume loss, bronchial wall thickening, and diffuse opacity.

Fibrotic Lung Disease Bronchiectasis predominantly involving lung bases is a common finding in fibrotic lung disease. In usual interstitial pneumonia (UIP), coarse reticulation, honeycombing, parenchymal distortion, and traction bronchiectasis are typically predominant in a subpleural and

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Fig. 13Scleroderma. There is patulous esophagus (white arrow), recurrent aspiration with subsequent bibasilar bronchiectasis, and chronic ground-glass opacities. Black arrow points to bronchus visible in peripheral 1 cm of lung.

Fig. 14Usual interstitial pneumonia. Bibasilar and subpleural reticulation and traction bronchiectasis are seen in areas of fibrosis (arrows).

bibasilar distribution with geographic heterogeneity (Fig. 14). Although often idiopathic, UIP occurs in asbestosis, drug toxicity, and collagenvascular disease. Nonspecific interstitial pneumonia has almost the same differential diagnosis but typically occurs in younger patients and carries a better prognosis. Ground-glass opacity is more frequent than in UIP and honeycombing usually remains minimal. Rare Causes Bronchiolitis obliterans from posttransplantation rejection is a rare cause of bronchiectasis associated with patchy mosaic perfusion, air trapping, and bronchiolar obstruction (Fig. 15). Hypogammaglobulinemia can also be a cause of lower-lung-predominant bronchiectasis, most often caused by recurrent infections.

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Fig. 15Bronchiolitis obliterans after lung transplantation. A and B, Transverse images of right lung in deep inspiration (A) and end expiration (B) show subtle basilar cylindric bronchiectasis (arrows, A) and widespread air trapping (arrows, B).

Fig. 16Mycobacterium avium-intracellulare infection. Bronchiectasis (arrows) predominantly involves right middle lobe and lingula.

Diffuse Bronchiectasis: Middle Lobe and Lingula Predominance Nontuberculous Mycobacterial Infection The most common acquired cause of bronchiectasis predominantly involving the right middle lobe and lingula is nontuberculous mycobacterial infection, such as Mycobacterium avium-intracellulare (MAI). This infection, typically seen in women over 60 years old, presents with chronic infection, bronchiectasis, mucoid impaction, and bronchiolitis (Fig. 16). The disease often has an indolent but progressive course requiring long-term antibiotics. Immobile Cilia Syndrome This rare congenital cause of bronchiectasis, which primarily involves the middle lung, is characterized by ineffective clearing of secretions, causing bronchiectasis, recurrent pneumonias, sinusitis, and infertility. In 50% of cases, total situs inversus is present, a condition known as Kartageners syndrome (Fig. 17).

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Fig. 17Kartageners syndrome. A, Chest radiograph shows cardiomegaly, dextrocardia, left middle lobe bronchiectasis, and volume loss. Arrow points to wrong-sided left marker. B, Transverse CT image confirms dextrocardia (asterisk is in left ventricle) and bronchiectasis (arrows) that predominantly affects midportion of lungs.

Fig. 18Allergic bronchopulmonary aspergillosis. A, Chest radiograph shows central bronchiectasis and mucoid impaction, so-called finger-in-glove appearance (arrows). B, Transverse CT image shows central bronchiectasis, mucoid impaction (large arrow), and distal bronchiolitis (small arrow).

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Fig. 19Mounier-Kuhns syndrome. A, Enlarged trachea (arrow). B, Enlarged mainstem bronchi (black arrows) and distal bronchiectasis (white arrows).

Fig. 20Williams-Campbell syndrome. There is mostly varicose and cystic central bronchiectasis (arrows).

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Bronchiectasis Diffuse Bronchiectasis: Central Predominance Allergic Bronchopulmonary Aspergillosis Allergic bronchopulmonary aspergillosis is an immune reaction to Aspergillus species that damages the bronchial wall, causing central bronchiectasis and mucous plugs that contain fungus and inflammatory cells. On chest radiography, this produces a characteristic finger-inglove appearance. On CT, central bronchiectasis and mucoid impactions are often associated with areas of peripheral bronchiolitis, manifesting as bronchiolar nodules or tree-in-bud opacities (Fig. 18). Cartilage-Deficiency Disorders Several rare disorders characterized by cartilage deficiency can cause bronchiectasis that primarily involves the central regions of the lung. One is tracheobronchomegaly, also known as Mounier-Kuhns syndrome, which manifests as diffuse enlargement of the trachea and mainstem bronchi with more distal bronchiectasis (Fig. 19). An even rarer cartilage deficiency disorder is the Williams-Campbell syndrome, in which central bronchiectasis, mimicking the appearance of parenchymal cysts, is associated with widespread air trapping (Fig. 20). Suggested Reading
1. Barker AF. Bronchiectasis. New Engl J Med 2002; 346:13831393 2. Baydarian M, Walter RN. Bronchiectasis: introduction, etiology, and clinical features. Dis Mon 2008; 54:516526 3. Hansell DM, Bankier AA, MacMahon TC, McLoud TC, Mller NL, Remy J. Fleischner Society: glossary of terms for thoracic imaging. Radiology 2008; 246:697722 4. Javidan-Nejad C, Bhalla S. Bronchiectasis. Radiol Clin North Am 2009; 47:289306 5. Kim JS, Mller NL, Park CS, Grenier P, Herold CJ. Cylindrical bronchiectasis: diagnostic findings on thinsection CT. AJR 1997; 168:751754 6. ODonnell AE. Bronchiectasis. Chest 2008; 134:815823 7. Quast TM, Self AR, Browning radiofrequency: diagnostic evaluation of bronchiectasis. Dis Mon 2008; 54:527539 8. Reid LM. Reduction in bronchial subdivision in bronchiectasis. Thorax 1950; 5:233247

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