Biliary atresia

Abstract

Biliary atresia (BA) is a congenital obliterative cholangiopathy of unknown aetiology, affecting both the intra- and extrahepatic bile ducts. Although relatively rare, BA must be excluded in any infant with conjugated hyperbilirubinaemia since the prognosis is improved by early diagnosis and prompt surgery. At least two phenotypes of BA are currently recognized; the syndromic variety is associated with other congenital anomalies and a poorer outcome. The results of treatment have steadily improved and, with a combination of timely expert surgery (Kasai portoenterostomy) and liver transplantation in specialist centres, good quality long-term survival is now possible in more than 90% of affected patients. A better understanding of the aetiology of BA and the pathogenesis of hepatic fibrosis is needed in order to develop new therapeutic strategies.