Biliary atresia, if left untreated, leads to chronic cholestasis, progressive liver failure and biliary cirrhosis and is the most frequently seen indication for liver transplantation in childhood. Idiopathic progressive fibrosis and obliteration in the bile ducts are typical for this rare disorder. Biliary atresia is also the major cause of neonatal cholestasis and in the presence of prolonged jaundice together with acholic stools and dark urine, biliary atresia is the most important diagnosis to consider. Current recommended treatment is surgery. Kasai hepatoportoenterostomy (HPE) when performed in the very first months of life, in order to minimize hepatocyte injury and to restore bile flow, can be life saving. In case of surgery failure and/or presence chronic complications liver transplantation is indicated. Throughout the last two decades, with increasing success rates of Kasai HPE and liver transplantation, prognoses of biliary atresia patients have improved.

Keywords: biliary atresia, Kasai hepatoportoenterostomy