Dr. Sunil Agrawal1st year MD ResidentDepartment of Child health
OverviewIntroduction – Hepatosplenomegaly Hepatomegaly SplenomegalyCauses – HEPATOSPLENOMEGALYHepatosplenomegaly- History physical examination investigations and treatmentApproach in children and neonate -summary
Hepatosplenomegaly - IntroductionHepatosplenomegaly 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 1–2 cm below left costal margin in infants and children.
Normal variants -splenomegaly Palpable spleen tip due to thinner abdominal musculatureSplenomegaly is usually caused by infection autoimmune disorders hemolysisBecause 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
INVESTIGATIONSComplete haemogram - Infections, anaemiaPeripheral smear - Leukaemia (Blast cells) Thalassaemia (hypochromia, nucleated RBCs, 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 fibrosisWilsons 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 & cystsLiver biopsy- Pathological diagnosisChest X-ray - ECG, echocardiography if cardiac cause suspectedHaemolytic profile in suspected haemolytic anaemiaBlood culture, Widal, Mantoux test - as required
TREATMENT STRATEGIESTherapy is directed at treatment of underlying diseaseInfections –Consider interferon for hepatitis B –Consider interferon and ribaviron for hepatitis CMetabolic disease –Metabolism consultation –Often requires specific restricted formulasCholestasis –Ursodeoxycholic acid –Supplemental fat soluble vitamins A, D, E, K
T/T Contd….Immune suppression for autoimmune hepatitisChemotherapy – Histiocytosis, leukemia,lymphomaSurgical treatment Kasai portoenterostomy for biliary atresia has better outcome if done before 60 days of age
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
ReferencesNelsons 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;376Ann D. Wolf and Joel E. Lavine, Hepatomegaly in Neonates and Children, Pediatr. Rev. 2000;21;303Websites : www.prsharma.com.np ; www.pedsinreview.org