Translations:Biliary atresia/2/en

Intraoperative view of complete extrahepatic biliary atresia.
  • Rare, but leading indication for liver transplantation in children[1]
  • Exact etiology unclear, but inflammation leads to destruction of intra/extrahepatic bile ducts
  • Prognosis significantly improved by early diagnosis[2]
  • Two forms:
    • Peri/postnatal (90% of cases), possibly related to infection
    • Fetal/embryonic (10%)
      • Often with other associated malformations (e.g congenital heart disease, polysplenia or asplenia, malrotation, situs inversus, portal vein or hepatic artery malformations)
  1. ↑ Mieli-Vergani G1, Vergani D. Biliary atresia. Semin Immunopathol. 2009 Sep;31(3):371-81. PMID: 19533128
  2. ↑ Wadhwani SI1, Turmelle YP, Nagy R, Lowell J, Dillon P, Shepherd RW. Prolonged neonatal jaundice and the diagnosis of biliary atresia: a single-center analysis of trends in age at diagnosis and outcomes. Pediatrics. 2008 May;121(5):e1438-40. PMID: 18443020
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