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Approach to a child with Hepatosplenomegaly

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Approach to a child with Hepatosplenomegaly

  1. 1. Dr. Sunil Agrawal 1st year MD Resident Department 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 - 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. 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, toxoplasmosis b)Bacterial - Typhoid, sepsis c)Spirochaetal - Leptospirosis d)Viral -Infectious mononucleosis, cytomegalo virus
  11. 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. 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. 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. 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. 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. 16. Miscellaneous causes i) Serum sickness ii) Connective tissue disorders a)Juvenile rheumatoid arthritis b)SLE iii) Sarcoidosis
  17. 17. CAUSES OF SPLENOHEPATOMEGALY 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 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. 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 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. 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 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. 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 Wilson's 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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