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Biliary Atresia

  1. The term biliary atresia is imprecise because the anatomy of abnormal bile ducts in affected patients varies markedly.
  2. The most common form of biliary atresia, accounting for =85% of the cases, however, is obliteration of the entire extrahepatic biliary tree at or above the porta hepatis. 

Differentiation of idiopathic neonatal hepaiitis from biliary atresia

  1. It may be difficult to clearly differentiate infants with biliary atresia, who require surgical correction, from those with intrahepatic disease (neonatal hepatitis) and patent bile ducts.
  2. No single biochemical test or imaging procedure is entirely satisfactory.
  3. Diagnostic schemas incorporate clinical, historical, biochemical, and radiologic features.
  4. Idiopathic neonatal hepatitis has a familial incidence of =20% whereas biliary atresia is unlikely to recur within the same family.
  5. A few infants with fetal onset of biliary atresia have an increased incidence of other abnormalities, such as the polysplenia syndrome with abdominal heterotaxia, malrotation, levocardia, and intra-abdominal vascular anomalies.
  6. Neonatal hepatitis appears to be more common in premature or small for gestational age infants.
  7. Persistently acholic stools suggest biliary obstruction (biliary atresia), but patients with severe idiopathic neonatal heparins may have a transient severe impairment of bile excretion.
  8. Consistently pigmented stools rule against billiary atresia Q.
  9. The finding of bile-stained fluid on Q duodenal intubation also excludes biliary atresia.
  10. Palpation of the liver may find an abnormal size or consistency in patients with extrahepatic biliary atresia; this is less common with neonatal hepatitis.
  11. Abdominal ultrasound is a helpful diagnostic tool in the evaluation of neonatal cholestasis because it will identify choledocholithiasis, perforation of rhe bile duct, or other structural abnormalities of the biliary tree such as a choledochal cyst.
  12. Hepatobiliary scintigraphy with technetium-labeled iminodiacetic acid derivatives Q is used to differentiate biliary atresia from nonobstructive causes of cholestasis. 

Management of Patients with Suspected Biliary Atresia


For patients in whom no correctable lesion in found, the hepatoportoenterostomy (Kasai)Q procedure should be performed.

Conjugated bilirubin >2 mg/ dL or 20 % of total bilirubin if total bilirubin is below 10 mg/ dL

  1. Intrahepatic cholestasis (Neonatal hepatitis syndrome)- herpes simplex, enteroviruses, cytomegalovirus or intrahepatic bile duct paucity -{Alagille syndrome. Byler disease, Zollinger syndrome}
  2. Extrahepatic biliary atresia-Persistent embolic stool ,hepatosplenomegaly

Liver biopsy is most reliable Q -for differentiating intrahepatic and extrahepatic


Hepatobiliary scintigraphy - (HIDA)*

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