Approach to a child with Hepatosplenomegaly

1. Dr. Sunil Agrawal
1
st
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. 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
History

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

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. 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
TREATMENT STRATEGIES

30. 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….

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, 19
th
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