Biliary atresia: A transplant perspective

Abstract

Biliary atresia is the most common single pediatric liver disease leading to liver transplantation (LT) during childhood. Between 1995 and 2002, 42% of children undergoing their first liver transplant while enrolled in the Studies of Pediatric Liver Transplantation (SPLIT) did so for a primary diagnosis of biliary atresia. There have been a number of recent reviews and advances related to the detection, diagnosis, and pathogenesis of biliary atresia. The purpose of this review is to focus on aspects of biliary atresia that are directly relevant to LT as a modality for its treatment. In particular, this review will concentrate on (1) specific indications for LT in the setting of biliary atresia, (2) management issues in children awaiting LT, (3) special surgical approaches in transplantation for biliary atresia, and (4) outcomes after LT. Biliary atresia is a unique disease with a relatively well-characterized clinical course. Careful analysis of the clinical course permits identification of indications for and timing of LT (Table 1). Transplantation decisions should take into consideration therapeutic approaches to the specific indications and complications that have developed. Thus, the following sections will examine both indications for transplantation and the management of those clinical problems.