Abstract: Biliary atresia (BA) is the leading cause of end-stage liver disease and pediatric liver transplantation worldwide.Although a timely Kasai portoenterostomy can significantly improve the natural history of the disease,achieving long-term complication-free native liver survival remains rare.Native liver survivors continually confront complex and lifelong challenges throughout the disease course,including cholangitis,progressive liver fibrosis,and portal hypertension.This article systematically reviews the latest advances in the diagnosis,treatment,and postoperative complication management of BA,with a particular focus on the challenges and future perspectives in the comprehensive,wholecourse management of this disease.

Key words: biliary atresia, full-course management, Kasai portoenterostomy, cholangitis, liver transplantation