Long-Term Results of Biliary Atresia in the Era of Liver Transplantation

Abstract

Introduction: Management of biliary atresia (BA) was begun with Kasai portoenterostomy and then has drastically changed with the recent adaptation of liver transplantation (LT) to the treatment scheme. We have reviewed our experience of BA treatment in the era of LT. The purpose of this study was to analyze the survival of patients with BA after Kasai operation and LT and to analyze the factors affecting survival. Methods: Seventy-two patients diagnosed with BA and operated on between April 1995 and December 2009 were retrospectively analyzed. Results: Out of the 72 patients, 59 received Kasai operation and 13 received de novo LT. Twenty-seven patients received LT after Kasai operation. Survival with native liver was 46.4% at 10 years. Overall 10 year survival for patients with biliary atresia was 94.9%. There was no difference in survival between patients receiving liver transplantation (LT) after failure of Kasai operation and patients receiving de novo LT. Age at Kasai operation (60 days) and postoperative normalization of bilirubin were independent risk factors affecting survival in multivariate analysis (HR 2.87, P = .035 & HR 11.84, P = .001). Among the 40 patients who received LT, operation earlier than 1 year of age (83.3% vs. 100%) and growth retardation at the time of LT (80.0% vs. 100%) were related to poor survival in univariate analysis. Conclusions: Survival of biliary atresia patients has greatly increased in with the timely application of the 2 surgical interventions, Kasai operation and LT. Age at Kasai operation and normalization of jaundice after surgery are independent risk factors for survival after Kasai operation.