Abstract
A case of biliary atresia with preduodenal portal vein was reported. Laparotomy revealed a symmetric liver in association with the fibrosis (F_1) but the patient had not polysplenia, malrotation and other anomalies. At present, two years after hepatic portoenterostomy, the patient is free of jaundice. Pathoembryologically, it is supposed that biliary atresia with preduodenal portal vein is caused by a developmental anomaly in contrast to that of the usual type of congenital biliary atresia. The prognosis of this association has been poor because anatomical variations at the porta hepatis made the portoenterostomy difficult in addition to the presence of severe congenital hepatic fibrosis. Embryology of preduodenal portal vein and anatomy of the porta hepatis in congenital biliary atresia with preduodenal portal vein were briefly discussed.
