Biliary atresia is a progressive inflammatory sclerosing disease of the bile ducts in the neonatal liver. Without surgical intervention these patients are destined to succumb to the disease. The development of the hepatoportoenterostomy in 1959 and liver transplantation in 1963 ushered a new era of success treating these patients. While many surgical modifications and adjuncts to treatment have been attempted over the last 50 years, the mainstay of treatment to give the child the best chance at prolonged survival with the native liver is a properly performed operation, in a timely fashion, with minimal post-operative complications. This review presents the authors current practice guideline to achieve these goals.
Keywords: Biliary atresia; Hepatoportoenterostomy; Kasai operation; Pediatric liver failure; Pediatric liver transplant.
Copyright © 2019. Published by Elsevier Inc.