Biliary atresia

Abstract

Biliary atresia is a dynamic process ultimately resulting in complete extrahepatic biliary obstruction. The cause is unknown, but if left untreated, progressive hepatic failure leads to death. Treatment, including timely diagnosis and early biliary reconstruction by Kasai's hepatic portoenterostomy, results in sustained postoperative bile flow in 90% of patients. Nearly 50% of those treated survive to 10 years and beyond. Liver transplantation rescues many patients who develop liver failure. Advances in patient selection, surgical technique, postoperative management, and liver transplantation have improved the outlook for these patients. Quality of life is excellent in many long-term survivors, and they will benefit from advances in our understanding of the specific nutritional, hormonal, and immunologic aspects of this disease.