Biliary atresia differential diagnosis

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Biliary atresia must be differentiated from other diseases that present with symptoms typical of cholestasis in neonates, jaundice and acholic pale stools, which are main features of obstructive jaundice, associated with conjugated (direct) hyperbilirubinemia.

Differentiating biliary atresia from other Diseases

Biliary atresia must be differentiated from other diseases that cause cholestatic jaundice and hepatic fibrosis, such as:[1]

  • Alagille Syndrome
    • Presents with cholestatic jaundice, along with a variety of other manifestations such as renal, otic, skeletal, ocular and cardiovascular.
  • Byler disease
    • Progressive Familial Intrahepatic cholestasis (PFIC), an inherited mutation in the ATP8b1 gene that results in the cells of the liver not being able to release bile. This leads to cholestasis and jaundice. Careful history taking can help in differentiating this disease from biliary atresia.
  • Congenital biliary dilatation
  • Choledochal Cysts
  • Cholestasis
  • Galactose-1-Phosphate Uridyltransferase Deficiency (Galactosemia)
  • Idiopathic neonatal hepatitis
  • Inborn errors of bile acid synthesis
  • Neonatal Hemochromatosis
  • Intrahepatic bile duct hypoplasia
  • Lipid Storage Disorders
  • Caroli Disease
    • Caroli disease is the dilatation of larger intrahepatic bile ducts, whereas Caroli syndrome is the combination of dilated small bile ducts and congenital hepatic fibrosis. These present in childhood usually, whereas biliary atresia presents in neonates.
  • Cystic Fibrosis
  • Bile plug syndrome
    • This is a rare form of extrahepatic mechanical obstruction in the major bile duct which is caused by viscous bile (sludge) within its lumen. It presents identically to biliary atresia, diagnosis is confirmed on ultrasound which shows sludge in the lumen of the common bile duct.
  • Toxoplasmosis, other infections, rubella, cytomegalovirus infection, and herpes simplex [TORCH][2]


  1. Schreiber RA, Harpavat S, Hulscher JBF, Wildhaber BE (2022). "Biliary Atresia in 2021: Epidemiology, Screening and Public Policy". J Clin Med. 11 (4). doi:10.3390/jcm11040999. PMC 8876662 Check |pmc= value (help). PMID 35207269 Check |pmid= value (help).
  2. Mack CL, Sokol RJ (2005). "Unraveling the pathogenesis and etiology of biliary atresia". Pediatr Res. 57 (5 Pt 2): 87R–94R. doi:10.1203/01.PDR.0000159569.57354.47. PMID 15817506.