Approach To A Child

With
Hepatosplenomegal
y

DR. RABI DHAKAL

1ST YEAR MD RESIDENT
DEPARTMENT OF
PEDIATRIC
 Hepatosplenomegaly - Introduction

Hepatosplenomegaly is enlargement of both

the spleen and liver.

Hepatomegaly :
Represents the clinical appearance of
liver enlargement
Enlarged liver, indicates potentially
reversible primary or secondary liver
disease.
Hepatomegaly may be confirmed by
palpation, percussion, or radiologic tests .

May be mistaken for

displacement of the liver by the diaphragm
abdominal tumor
spinal deformity
fecal material
 can occur via five mechanisms,
Inflammation,
Excessive storage,
Infiltration,
Congestion, and
Obstruction.
Presence of a palpable liver does not always
represent hepatomegaly .

Determined on the basis of liver span and

degree of extension below the right costal
margin.
Normal liver spans range from 5
to 9 cm depending on age.
The normal range for liver span
by percussion at
1 week of age - 4.5 to 5 cm.
12 years, boys - 7 to 8 cm
girls - 6 to 6.5 cm
SPLENOMEGALY :
Primary functions is to filter defective and/or
foreign cells.

Splenomegaly is usually caused by systemic

disease and not by primary splenic disease.

Normal spleen may be palpable 12 cm below

left costal margin in infants and children.
 Normal variants -splenomegaly
Palpable spleen tip due to thinner abdominal musculature
Splenomegaly is usually caused by
infection
autoimmune disorders
hemolysis
Because of exposure below the protective rib
cage, splenomegaly results in increased risk of
splenic injury or rupture.
Hepatosplenomega
ly
Infections
Haematological disorders
Vascular congestion
Tumours and Infiltrations
Storage disorders
Miscellaneous causes
 Infections

i) Acute infections -
a)Protozoal - Malaria, kala-azar, toxoplasmosis
b)Bacterial - Typhoid, sepsis
c)Spirochaetal - Leptospirosis
d)Viral -Infectious mononucleosis, cytomegalo
virus
 ii) Chronic infections -
a)Mycobacterial - Disseminated tuberculosis
b)Protozoal -Malaria,kala-azar,toxoplasmosis
c)Spirochaetal - Congenital syphilis
d)Viral - HIV, Rubella, herpes, cytomegalovirus
infection
e)Bacterial - Brucellosis
f)Fungal - Histoplasmosis
Haematological disorders

i)Iron-deficiency anaemia
ii) Haemolytic disorders -
a)Thalassaemia
b)Hereditary spherocytosis
c)Sickle cell anaemia
d)Autoimmune haemolytic
anaemias
e)Isoimmunization disorders - Rh
and ABO incompatibility
 Vascular congestion
i)Congestive cardiac failure
ii)Constrictive pericarditis
iii)Cirrhosis -
a) Hepatitis
b) Chronic active hepatitis
c)Biliary atresia
d)Cystic fibrosis
e)Wilson's disease
f)Galactosemia
g)Alpha-l-antitrypsin deficiency
h)Haemosiderosis
Tumours and Infiltrations

i)Leukaemia - Acute lymphocytic leukaemia

ii)Lymphomas Hodgkin's and non-Hodgkins
lymphoma
iii)Metastatic disease - Neuroblastoma
iv)Histiocytosis X
 Storage disorders
i) Lipid storage diseases -
a)Gaucher disease
b)Niemann-Pick disease
c)Gangliosidoses
d)Mucolipidoses
ii) Mucopolysaccharoidoses
a)Hurler's syndrome
b)Hunter's syndrome
iii) Glycogen storage disease - Type IV
iv) Amyloidosis
 Miscellaneous causes

i) Serum sickness
ii) Connective tissue disorders
a)Juvenile rheumatoid arthritis
b)SLE
iii) Sarcoidosis
History
Age at onset
Sex
Fever, jaundice
Acute illness, dyspnea, fatigue, diarrhea, vomiting
Signs of malignancy- proptosis, subcutaneous nodules
Travel history endemic diseases
Developmental milestones
Nutrition history (neonatal formula)
Medical history: umbilical catheter, weight loss, failure
to thrive, bleeding, bruising, Pruritis, pallor, heart
disease , rashes, joint pain.
Family history: Early cholecystectomy, gallstones,
anemias, ethnic heritage, liver disease, maternal HBV,
HCV
 Age

Neonates and first few

months of life - e.g.
Haemolytic anaemias
(Thalassaemia major),
storage disorders
Anyage - Malaria, kala azar,
sepsis, enteric fever, etc.
Hepatosplenomegaly
with
Fever - Infection - Malaria, kala-azar, enteric
fever, malignancy
Jaundice, anorexia, vomiting, haematemesis,
malena - liver disease especially cirrhosis with
portal hypertension
Recurrent Jaundice - Liver disease, Hemolytic
anemia
Dyspnoea / difficulty in feeding - cardiac
causes e.g. CCF
Delayed development - Carbohydrate / Lipid
storage disorders
Family history - Congenital hemolytic anemia,
storage disorders etc.
 CLINICAL EXAMINATION

CAUSES OF HEPATOSPLENOMEGALY
WITH PALLOR
1)Infections - Malaria, kala-azar,
bacteremia
2)Haemolytic anaemia - Hereditary
spherocytosis, sickle cell anaemia,
thalassaemia, autoimmune haemolytic
anaemia.
3)Nutritional - Iron deficiency anaemia.
4)Leukaemia and lymphomas.
CLINICAL
EXAMINATION
General examination
Pallor - Already discussed
Petechiae, purpura, ecchymosis,
lymphadenopathy etc. - Leukaemia
Jaundice - Liver disease / haemolytic anaemia
Koilonychia, platynychia - Iron deficiency
Mental retardation - Mucopolysaccharoidoses
Systemic examination

Abdomen
Tender hepatomegaly- Viral hepatitis, CCF,
liver abscess, enteric fever
Firm consistency liver with sharp edge -
Cirrhosis, constrictive pericarditis
Just palpable soft spleen - Enteric fever,
infective endocarditis, etc.
Ascites - Suggests cirrhosis with portal
hypertension, malignancy, TB
CVS - Raised JVP - CCF, constrictive pericarditis
 INVESTIGATIONS

Complete haemogram - Infections,

anaemia
Peripheral smear -
Leukaemia (Blast cells)
Thalassaemia (hypochromia, nucleated RBC's, target
cells)
Sickle cell anaemia (sickling on treatment with 2%
sodium metabisulphite)
Parasitic diseases (Eosinophilia)
ESR - Elevated in inflammatory diseases
Reticulocyte count - High in haemolytic
anaemia
 Liver Function Test
Serum proteins - Low in kwashiorkor
SGOT/SGPT - Raised in hepatitis & hepatic
necrosis
Alkaline phosphatase - Elevated in
hepatobiliary obstruction & liver abscess
Bilirubin (total, direct) - Haemolytic
anaemias
 Miscellaneous tests
Raised alpha foeto protein- Hepatoblastoma
Hbs Ag - Hepatitis B
High prothrombin time - Liver parenchymal
dysfunction
High sweat chlorides - Cystic fibrosis
Wilson's disease - Low ceruloplasmin
Liver scan - To differentiate biliary atresia from
neonatal hepatitis
Urine and stool examination - In case of jaundice
 USG abdomen - Cirrhosis with portal
hypertension, Ascites, Tumors & cysts
Liver biopsy- Pathological diagnosis
Chest X-ray - ECG,
echocardiography if cardiac cause
suspected
Haemolytic profile in suspected
haemolytic anaemia
Blood culture, Widal, Mantoux test -
as required
TREATMENT STRATEGIES
Therapy is directed at treatment of
underlying disease
Infections
Consider interferon for hepatitis B
Consider interferon and ribaviron for hepatitis C
Metabolic disease
Metabolism consultation
Often requires specific restricted formulas
Cholestasis
Ursodeoxycholic acid
Supplemental fat soluble vitamins A, D, E, K
 Immune suppression for autoimmune hepatitis
Chemotherapy Histiocytosis, leukemia,
lymphoma

Surgical treatment

Kasai portoenterostomy for biliary atresia has

better outcome if done before 60 days of age
T/T Contd.
Splenectomy:
IfPacked cell requirement is more than
250ml/kg/yr(thalassemia)
Uncontrolled bleeding or not responding to
steroid or iv Ig (chronic ITP)
Ifsplenectomy is performed, immunize at least
10 days prior
Pneumococci
Haemophilus influenzae,
if under 5

Meningococcal vaccine
Postsurgical penicillin prophylaxis required
THANK YOU