Dr. Sunil Agrawal1st year MD ResidentDepartment of Child health

OverviewIntroduction –

Hepatosplenomegaly Hepatomegaly Splenomegaly

Causes –HEPATOSPLENOMEGALY

Hepatosplenomegaly- Historyphysical examination investigations and treatment

Approach in children and neonate -summary

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 diaphragmabdominal tumorspinal deformityfecal 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 1–2 cm below left costal margin in infants and children.

Normal variants -splenomegalyPalpable 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.

Hepatosplenomegaly - causesInfectionsHaematological disordersVascular congestionTumours and InfiltrationsStorage disordersMiscellaneous 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 disordersi)Iron-deficiency anaemiaii) Haemolytic disorders - a)Thalassaemia b)Hereditary spherocytosis c)Sickle cell anaemia d)Autoimmune haemolytic anaemias e)Isoimmunization disorders - Rh and ABO

incompatibility

Vascular congestioni)Congestive cardiac failureii)Constrictive pericarditisiii)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 leukaemiaii)Lymphomas — Hodgkin's and non-Hodgkins lymphomaiii)Metastatic disease - Neuroblastomaiv)Histiocytosis X

Storage disordersi) Lipid storage diseases - a)Gaucher disease b)Niemann-Pick disease c)Gangliosidoses d)Mucolipidosesii) Mucopolysaccharoidoses a)Hurler's syndrome b)Hunter's syndromeiii) Glycogen storage disease - Type IViv) Amyloidosis

Miscellaneous causesi) Serum sicknessii) Connective tissue disorders a)Juvenile rheumatoid arthritis b)SLEiii) Sarcoidosis

CAUSES OF SPLENOHEPATOMEGALY1)Malaria2)Kala azar3)Chronic haemolytic anaemia4)Portal hypertension

Age at onsetSex Fever, jaundiceAcute illness, dyspnea, fatigue, diarrhea, vomitingSigns of malignancy- proptosis, subcutaneous

nodulesTravel history – endemic diseasesDevelopmental 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

AgeNeonates and first few months of life

- e.g. Haemolytic anaemias (Thalassaemia major), storage disorders

Any age - Malaria, kala azar, sepsis, enteric fever, etc.

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

Hepatosplenomegaly withFever - Infection - Malaria, kala-azar, enteric fever,

malignancyJaundice, anorexia, vomiting, haematemesis, malena -

liver disease especially cirrhosis with portal hypertensionRecurrent Jaundice - Liver disease, Hemolytic anemiaDyspnoea / difficulty in feeding - cardiac causes e.g. CCFDelayed development - Carbohydrate / Lipid storage

disordersFamily history - Congenital hemolytic anemia, storage

disorders etc.

CLINICAL EXAMINATION

CAUSES OF HEPATOSPLENOMEGALY WITH PALLOR –

1)Infections - Malaria, kala-azar, bacteremia2)Haemolytic anaemia - Hereditary

spherocytosis, sickle cell anaemia, thalassaemia, autoimmune haemolytic anaemia.

3)Nutritional - Iron deficiency anaemia.4)Leukaemia and lymphomas.

CLINICAL EXAMINATIONGeneral examination

Pallor - Already discussedPetechiae, purpura, ecchymosis, lymphadenopathy etc.

- LeukaemiaJaundice - Liver disease / haemolytic anaemiaKoilonychia, platynychia - Iron deficiencyMental retardation - Mucopolysaccharoidoses

Systemic examinationAbdomen

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

INVESTIGATIONSComplete haemogram - Infections, anaemiaPeripheral 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 diseasesReticulocyte count - High in haemolytic anaemia

Liver Function TestSerum proteins - Low in kwashiorkorSGOT/SGPT - Raised in hepatitis & hepatic

necrosisAlkaline phosphatase - Elevated in

hepatobiliary obstruction & liver abscessBilirubin (total, direct) - Haemolytic

anaemias

Miscellaneous testsRaised alpha foeto protein- HepatoblastomaHbs Ag - Hepatitis BHigh prothrombin time - Liver parenchymal dysfunctionHigh sweat chlorides - Cystic fibrosisWilson's disease - Low ceruloplasminLiver scan - To differentiate biliary atresia from neonatal hepatitisUrine and stool examination - In case of jaundice

USG abdomen - Cirrhosis with portal hypertension, Ascites, Tumors & cysts

Liver biopsy- Pathological diagnosisChest X-ray - ECG, echocardiography if cardiac cause

suspectedHaemolytic profile in suspected haemolytic anaemiaBlood culture, Widal, Mantoux test - as required

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

Immune suppression for autoimmune hepatitisChemotherapy – Histiocytosis, leukemia, lymphoma

Surgical treatment

Kasai portoenterostomy for biliary atresia has better outcome if done before 60 days of age

T/T Contd….

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

Approach in children with Hepatosplenomegaly

To summarize

Approach in neonates with Hepatosplenomegaly

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

Thank you