Management of biliary atresia: Experience in a single institute

Background: The management of biliary atresia (BA) has evolved with time. The outcome of BA treatment in the Chang Gung Memorial Hospital-Kaohsiung Medical Center had not been recorded and we addressed this issue in this study. Methods: Medical records of the 93 patients with BA who underwent Kasai portoenterostomy (KP) in the Chang Gung Memorial Hospital-Kaohsiung Medical Center from 1986 to 2005 were reviewed retrospectively. There were 46 male and 47 female patients included in this study. Sixty patients received KP before 60 days of age and 33 patients received KP after 60 days of age. Results: Jaundice-free survival with native liver was accomplished in 29 of 60 patients who received KP before 60 days of age but in only 9 of 33 after that age (p = 0.048). Cholangitis developed in 56 patients (60.2%). Incidence of cholangitis was not significantly different between the patients free of jaundice (22/38) and those with persistent jaundice (34/55) (p = 0.704). Seven patients had single cysts and 1 had multiple cysts, with 4 being jaundice-free. Five out of 9 were free of jaundice after re-do KP. Among the 62 patients followed for more than 5 years, 32 (51.6%) survived with native liver. Conclusion: This study revealed that half of the patients with BA survived with their native liver for more than 5 years. Age at operation, not post-KP cholangitis or liver cysts, was the most determinant factor of BA outcome.