Approach to

Hepatosplenomega
ly
24/8/2015

Case 1
MA, 2 years old boy
NKMI, NKDA
Presented with fever for 3 weeks associated with

cough and runny nose for 1 week

No abdominal distension noted
Was brought to GP to seek treatment and
completed one course of amoxicillin, however
symptoms was not improved.
Subsequently child was brought to Private Hospital
Unremarkable antenatal, birth and developmental
history

Physical examination
Alert, perfusion good, not tachypnoeic, mild
pallor, not jaundice
Pulse rate: 130
Temp: 37
BP: 98/50
Lungs and CVS: unremarkable
Abdomen: Liver 3cm and spleen 2cm palpable
below costal margin
Shotty cervical and inguinal lymph node
palpable

Working
diagnosis??
Investigation??

Investigation done
Date

12/8/2015
5.2

13/8/2015
5.4

14/8/2015
5.0

Hemoglobin
WBC

7.2

34.35

34.63

Platelet

25

31

28

FBC count showed lymphocyte predominant

Investigation done
Date

12/8/2015
5.2

13/8/2015
5.4

14/8/2015
5.0

Hemoglobin
WBC

7.2

34.35

34.63

Platelet

25

31

28

FBC count showed lymphocyte predominant

Date
Urea

12/8/2015
5.2

13/8/2015
5.5

14/8/2015
4.1

Creatinine

34

31

35

Na

139

136

421

4.6

4.6

4.4

Cl
S.Bilirubin

2.1

Albumin

45

Globulin

32

ALT

10

ALP

88

Total Protein

77

LDH

674

Uric Acid

335

CRP

15

Blood C&S

NG

NG

Any other further

investigation?

 Hep B, Hep C, HIV (13/8/15): NR

PBF urgent: Leukocytosis with

lymphocytosis. Presence of 32% blasts cells

which are small in size with scanty cytoplasm
and inconscpicuous nucleoli suggestive of
Acute Leukemia (most likely ALL).
Peripheral blood Immunophenotyping
was sent showed precursor B-ALL

Case 2
ID, 2 years old malay boys
NKMI, NKDA
presented with 2 months history of abdominal pain

associated with few episodes of passing loose stool

Intermittent pain which partially resolved after abdominal
massage
Brought to District Hosp twice, was treated as gastritis
Mother claimed she felt some mass while massaging the
abdomen
Subsequently child was brought to Private GP
Child was referred to GH as Private GP notice abdominal
mass while performing physical examination.

Physical finding
Alert, perfusion good, warm periphery, mild

pallor
BP: 96/57, PR: 116, SpO2 98% under RA
CVS: DRNM
Lungs: clear, air entry equal
Abdomen: Liver palpable 11 cm with no
spleen felt. Liver was non tender and well
defined margin

Working
diagnosis??
Investigation??

Date

29/7/15

Haemoglobin

10.1

TWC

16.9

Platelet

766

Urea

4.0

Creatinine

43

Na

134

4.2

S.Bilirubin

17

Albumin

43

ALT

13

ALP

182

T.Protein

83

Any further
investigation??

Tumour marker(29/7/2015)
AFP: >16600 after dilution(Iu/mL)
US abdomen (30/7/2015) showed large mass at the
right iliac fossa region extending to the epigastric
region, likely liver in origin.
Urgent CECT thorax/abdomen(6/8/2015) showed
large liver mass most likely a hepatoblastoma with
pulmonary nodules
Subsequently child was referred to PGH for further
management

Approach to Hepatomegaly,
Splenomegaly and
Hepatosplenomegaly

Liver
Situated at right hypochondriac region
Moves down with inspiration
Unable to get above
Enlarged to Right illiac fossa
Dull on percussion

Hepatomegaly
The presence of a palpable liver does not

always indicate hepatomegaly

In general, a liver edge greater than 3.5 cm
in newborns and greater than 2 cm in
children below the right costal margin
suggests enlargement
Normal Range of Liver span
1 week of age is 4.5 to 5 cm.
At 12 years, the normal value for boys is 7 to
8 cm and for girls is 6 to 6.5 cm.

Pitfalls in hepatomegaly
The liver can be displaced inferiorly by the

diaphragm or thoracic organs, giving the

impression of hepatomegaly
Children who have orthopedic abnormalities
such as narrow chest walls or pectus excavatum
can erroneously appear to have hepatomegaly
A normal variant of the right lobe of the liver,
called a Riedel lobe, may extend far below the
right costal margin and be confused as
pathologic hepatic enlargement

Causes of hepatomegaly
Mechanism
Inflammation
Infiltrative
Inappropriate storage
Vascular congestion
Billiary obstruction
Mneumonic (SHIRT)
Structural, Storage/metabolic
Haematological, Heart
Infection, inflammatory, infiltrative
Reticuloendothelial, Rheumatological
Tumor, Trauma

Mechanism

Disease

Inflammation

Infections
Viral-Hepatitis(ABCDE),
congenital rubella, CMV,
coxsackie, echovirus , infectious
mononucleosis
Bacterial- neonatal
septicaemia, E.coli, TB,
congenital syphilis
Parasite- hydatid disease,
malaria, schistosomiasis,
toxoplasmoxis, visceral larva
migrans
Fungal- Histoplasmosis
Toxins/Drugs
Autoimmune disease
Autoimmune hepatitis
SLE
Systemic onset Juvenile

Mechanism

Disease

Inappropriate Storage

Lipid- gauchers ds, Niemann

Pick
Carbohydrate-glycogen storage
ds, hereditary fructose
intolerance, galactosemia,
Mucopolysaccharidoses
Protein/amino acidtyrosinaemia, urea cycle defect
Mineral- wilson ds,
haemochromatosis
Electrolyte transport- cystic
fibrosis
Nutrition- total parenteral
nutrition
Protease- alpha-1-antitrypsin
deficiency

Mechanism

Disease

Infiltrative

Primary neoplastic tumors:

Hepatoblastoma/Hepatocellular
carcinoma
Primary non-neoplastic
tumors
Hemangioma,
hemangioendothelioma,
teratoma, focal nodular
hyperplasia
Metastatic or disseminated
tumorsLeukemia, lymphoma,
neuroblastoma, histiocytosis
Cysts-Parasitic cyst, choledochal
cyst, polycystic liver disease
Hemophagocytic syndromes
Extramedullary
hematopoiesis-thalasemia,

Mechanism

Disease

Vascular Congestion

Suprahepatic
Congestive heart failure
Restrictive pericardial disease
Suprahepatic web
Hepatic vein thrombosis (BuddChiari syndrome)
Intrahepatic
Veno-occlusive disease

Biliary Obstruction

Cholelithiasis
Choledochal cyst
Biliary atresia
Tumors-Hepatic,
Biliary,Pancreatic,Duodenal

Spleen

Located at left hypochondriac region

Move down with inspiration
Unable to get above
Usually not palpable
Smooth, regular margin
Presence of splenic notch
Traubes space 9, 10 and 11th rib spaces
Enlarged towards RIF (in infants and young
children may be towards LIF)

Splenomegaly
Normal Spleen is palpable below the left

costal margin in nearly one-third of neonates

However, a splenic edge felt more than 2
cm below the ribs definitely is an abnormal
finding

Causes of splenomegaly
Moderate or massive splenomegaly(8cm or

more below left costal margin)

Mneumonic: CHIMPS
C: Cardiac and connective tissue diseases
H: Haematological
I: Infection, Injury
M: Malignancy
P: Portal Hypertension
S: storages diseases

Causes of Splenomegaly
Moderate splenomegaly
Mechanism

Diseases

Infection

Bacterial-Subacute bacterial
endocarditis, Septicaemia,
Splenic abscess, Typhoid fever,
Brucellosis, Leptospirosis,
Tuberculosis, Cat scratch
disease
Viral- acute viral hepatitis,
EBV, CMV, AIDS
Parasitic- Toxoplamosis,
malaria, schistosomiasis,
leishmaniasis, trypanosomiasis
Fungal- Histoplasmosis

Mechanism

Causes

Inflammation/disordered
immunoregulation

SLE, Rheumatoid arthritis,

Inflammatory bowel disease,
coeliac disease, chronic
granulomatous disease, ITP, Drug
reaction

Extramedullary haematopoeisis

Hereditary spherocytosis,
haemoglobinopathies,
thalasaemia major

Congestive

Extrahepatic- post nenonatal

umbilical vessel catheterisation or
sepsis
Hepatic- cirhosis, congenital
hepatic fibrosis
Suprahepatic- Budd Chiari
syndrome
Splenic vein thrombosis

Mechanism

Disease

Malignancy

Leukemia, Hodgkins lymphoma,

Non Hodgkins lymphoma

Storage disease

Lipidoses
Mucopolysaccharidoses
Amyloidoses

Structural

Hematoma
Splenic cyst/pseudocyst
Hemangioma
Lymphangioma

Massive Splenomegaly
Mechanisms

Diseases

Infections

Chronic malaria
Schistosomiasis
Visceral leshmananiasis(kalaazar)

Extramedulary haematopoiesis

Hereditary spherocytosis
Thalasaemia Major

Congestive

Non cirrhotic portal fibrosis

Malignancy

Non-hodgkins lymphoma
Metastatic disease
CML

Storage disease

Lipidoses disease

Hepatosplenomegaly
Mechanism

Disease

Hematological

Thalasaemia,myeloproliferative
ds and lymphoproliferative ds

Infection

EBV, TORCHES, Typhoid

Malignancy

Leukemia, lymphoma

Storage disease

Lipidoses ds,
mucopolysaccharidoses

Structural

Liver cirhosis with portal

hypertension, congenital hepatic
fibrosis

Important history
Neonates
Important history

Associated diseases/problems

Prolonged jaundice

Extrahepatic biliary atresia

Alpha-1 antitrypsin deficiency
Galactosemia

Vomitting and diarhhea

Metabolic/storage disorder
septicaemia

Family history of early infant

Metabolic diseases
death or hepatic,
neurodegenerative, or psychiatric
disease
Antenatal history

TORCHES infection
RH or ABO incompatibility

History of umbilical
catheterization

Hepatic abscess

Delayed passing meconium

Cystic fibrosis

Child
Important history

Possible diseases/problems

Fever

Infective causes

Foreign travelling, ingestion of

shellfish or drugs

Viral hepatitis
Parasitic infection

history of poor weight gain,

vomiting, diarrhea, distinctive
odors, loss of developmental
milestones, complex seizure
disorder, or hypotonia

Metabolic disorder

Acholic stool or dark color urine

Hepatic dysfunction

History of blood transfusion

Viral Hepatitis B or C, HIV

Drug history

Eg: NSAIDS, Isoniazid,

propylthiouracil, and
sulfonamides.

history of inflammatory
bowel disease or
immunodeficiency

Primary sclerosing cholangitis

Associated physical
findings
Associated physical findings

Possible problem/diseases

Anemia

Hematological
disorder/malignancy
Infection

Jaundice

Hemolytic anemia
Hepatic dysfunction
Billiary tree dysfunction

Eye Findings
Cataracts
Kayser-Fleischer rings
Chorioretinitis
Posterior embryotoxon

TORCH infections
Wilson disease
Alagille syndrome

Dysmorphic Features

Metabolic and storage diseases

Alagille syndrome

Ascites

Portal hypertension

Coarse facial features

Metabolic & Storage disorder

Investigation

Thank you

References
Hepatomegaly in Neonates and Children,

Ann D. Wolf and Joel E. Lavine, Pediatrics in

Review 2000;21;303
Examination Paediatrics 3rd edition,
Wayne Harris
MRCPCH Clinical Exam Made Simple 1st
edition, Stanley Tamuka