Approach to a child with Hepatosplenomegaly

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Approach to a child with Hepatosplenomegaly

  1. 1. Dr. Sunil Agrawal1st year MD ResidentDepartment of Child health
  2. 2. OverviewIntroduction – Hepatosplenomegaly Hepatomegaly SplenomegalyCauses – HEPATOSPLENOMEGALYHepatosplenomegaly- History physical examination  investigations and treatmentApproach in children and neonate -summary
  3. 3. 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.
  4. 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. 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. 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. 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. 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. 9. Hepatosplenomegaly - causesInfectionsHaematological disordersVascular congestionTumours and InfiltrationsStorage disordersMiscellaneous causes
  10. 10. Infections i) Acute infections -a)Protozoal - Malaria, kala-azar, toxoplasmosisb)Bacterial - Typhoid, sepsisc)Spirochaetal - Leptospirosisd)Viral -Infectious mononucleosis, cytomegalo virus
  11. 11. ii) Chronic infections -a)Mycobacterial - Disseminated tuberculosisb)Protozoal -Malaria,kala-azar,toxoplasmosisc)Spirochaetal - Congenital syphilisd)Viral - HIV, Rubella, herpes, cytomegalovirus infectione)Bacterial - Brucellosisf)Fungal - Histoplasmosis
  12. 12. 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
  13. 13. Vascular congestioni)Congestive cardiac failureii)Constrictive pericarditisiii)Cirrhosis - a) Hepatitis b) Chronic active hepatitis c)Biliary atresia d)Cystic fibrosis e)Wilsons disease f)Galactosemia g)Alpha-l-antitrypsin deficiency h)Haemosiderosis
  14. 14. Tumours and Infiltrationsi)Leukaemia - Acute lymphocytic leukaemiaii)Lymphomas — Hodgkins and non-Hodgkins lymphomaiii)Metastatic disease - Neuroblastomaiv)Histiocytosis X
  15. 15. Storage disordersi) Lipid storage diseases - a)Gaucher disease b)Niemann-Pick disease c)Gangliosidoses d)Mucolipidosesii) Mucopolysaccharoidoses a)Hurlers syndrome b)Hunters syndromeiii) Glycogen storage disease - Type IViv) Amyloidosis
  16. 16. Miscellaneous causesi) Serum sicknessii) Connective tissue disorders a)Juvenile rheumatoid arthritis b)SLEiii) Sarcoidosis
  17. 17. CAUSES OFSPLENOHEPATOMEGALY1)Malaria2)Kala azar3)Chronic haemolytic anaemia4)Portal hypertension
  18. 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. 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. 20. CAUSES OF HEPATOSPLENOMEGALY BY AGE A. NEONATE B. CHILD COMMON UNCOMMON COMMON UNCOMMONCongestive heart Hemangiomatosis Hemolytic anaemias Budd-Chiari syndromefailure Histiocytosis Biliary obstruction Constrictive pericarditisMaternal diabetes Isoimmunization Congestive heart Gauchers diseaseMetabolic failure Hemangiomas Neuroblastomadisorders Leukemia/lymphoma Immune deficienciesSepsis Parasitic infections Metastaic tumorsStorage disease Sepsis Neiman-Picks diseaseTORCH infection Systemic infections Collagen vascular diseases Veno-oclusive disease
  21. 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. 22. CLINICAL EXAMINATIONCAUSES 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. 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. 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. 25. INVESTIGATIONSComplete haemogram - Infections, anaemiaPeripheral 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 diseasesReticulocyte count - High in haemolytic anaemia
  26. 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. 27. Miscellaneous testsRaised alpha foeto protein- HepatoblastomaHbs Ag - Hepatitis BHigh prothrombin time - Liver parenchymal dysfunctionHigh sweat chlorides - Cystic fibrosisWilsons disease - Low ceruloplasminLiver scan - To differentiate biliary atresia from neonatal hepatitisUrine and stool examination - In case of jaundice
  28. 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. 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. 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. 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. 32. Approach in children with Hepatosplenomegaly To summarize
  33. 33. Approach in neonates with Hepatosplenomegaly
  34. 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. 35. Thank you

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