1. Dr. Sunil Agrawal
1st year MD Resident
Department of Child health

2. Overview
Introduction –
Hepatosplenomegaly
Hepatomegaly
Splenomegaly
Causes –
HEPATOSPLENOMEGALY
Hepatosplenomegaly-
History
physical examination
 investigations and treatment
Approach in children and neonate -summary

3. 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.

4. 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

5. 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.

6. 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

7. 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 1–2 cm below left
costal margin in infants and children.

8. 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.

9. Hepatosplenomegaly - causes
Infections
Haematological disorders
Vascular congestion
Tumours and Infiltrations
Storage disorders
Miscellaneous causes

10. Infections
i) Acute infections -
a)Protozoal - Malaria, kala-azar, toxoplasmosis
b)Bacterial - Typhoid, sepsis
c)Spirochaetal - Leptospirosis
d)Viral -Infectious mononucleosis, cytomegalo virus

11. 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

12. 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

13. 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

14. Tumours and Infiltrations
i)Leukaemia - Acute lymphocytic leukaemia
ii)Lymphomas — Hodgkin's and non-Hodgkins lymphoma
iii)Metastatic disease - Neuroblastoma
iv)Histiocytosis X

15. 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

16. Miscellaneous causes
i) Serum sickness
ii) Connective tissue disorders
a)Juvenile rheumatoid arthritis
b)SLE
iii) Sarcoidosis

17. CAUSES OF
SPLENOHEPATOMEGALY
1)Malaria
2)Kala azar
3)Chronic haemolytic anaemia
4)Portal hypertension

18. 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

19. Age
Neonates and first few months of life - e.g.
Haemolytic anaemias (Thalassaemia
major), storage disorders
Any age - Malaria, kala azar, sepsis, enteric
fever, etc.

20. CAUSES OF HEPATOSPLENOMEGALY BY AGE
A. NEONATE B. CHILD
COMMON UNCOMMON COMMON UNCOMMON
Congestive heart Hemangiomatosis Hemolytic anaemias Budd-Chiari syndrome
failure Histiocytosis Biliary obstruction Constrictive pericarditis
Maternal diabetes Isoimmunization Congestive heart Gauchers disease
Metabolic failure Hemangiomas
Neuroblastoma
disorders Leukemia/lymphoma Immune deficiencies
Sepsis Parasitic infections Metastaic tumors
Storage disease Sepsis Neiman-Picks disease
TORCH infection Systemic infections Collagen vascular
diseases
Veno-oclusive disease

21. 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.

22. 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.

23. 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

24. 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

25. 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

26. 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

27. 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

28. 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

29. 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

30. T/T Contd….
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

31. T/T Contd….
 Splenectomy:
 If Packed cell requirement is more than
250ml/kg/yr(thalassemia)
 Uncontrolled bleeding or not responding to steroid or iv Ig
(chronic ITP)
 If splenectomy is performed, immunize at least 10 days
prior
–Pneumococci
–Haemophilus influenzae,
 if under 5
–Meningococcal vaccine
–Postsurgical penicillin prophylaxis required

32. Approach in children with Hepatosplenomegaly
To summarize

33. Approach in neonates with Hepatosplenomegaly

34. References
Nelsons text book of pediatrics, 19th edition.
Ghai essential pediatrics.
Ian D. D’Agata and William F. Balistreri, Evaluation
of Liver Disease in the Pediatric Patient, Pediatr. Rev.
1999;20;376
Ann D. Wolf and Joel E. Lavine, Hepatomegaly in
Neonates and Children, Pediatr. Rev. 2000;21;303
Websites : www.prsharma.com.np ;
www.pedsinreview.org

35. Thank you