Biliary atresia — The current management

Abstract

Despite extensive research, controversies still exist regarding the etiology, pathology and management of biliary atresia. It is now thought to be a progressive panductal inflammatory obliterative process and not a developmental anomaly. The histologic changes are indistinguishable from neonatal hepatitis but some changes have prognostic significance. The clinical presentation is that of infantile obstructive cholangiopathy-waxing and waning icterus, clay coloured stools and high coloured urine from early neonatal period. The diagnosis is suggested by the absence of intestinal excretion on HIDA scan and confirmed on operative cholangiogram. Of utmost importance towards the final prognosis is early detection, prompt confirmation and surgical treatment before 2 months of age. Even with early treatment the result of bilioenteric drainage procedures have been discouraging in the long term. Portoenterostomy (PE) done in older children has been largely unsuccessful all over the world. The poor results of PE prompted the search for alternative treatment and liver transplantation (LT) has emerged as a viable treatment option both as a primary procedure and after failed PE. Although the technical know-how and infrastructure are available, LT in children has still not been done in India because of various economic and social constraints. It is hoped that all physicians and surgeons dealing with such patients would also consider this treatment modality.