CURRENT CONCEPT OF THE TREATMENT OF BILIARY ATRESIA

Hepatic portoenterostomy (Kasai operation) for the patient with biliar y atresia (BA) can restore the bile flow in approximately 80% of child ren operated on before 60 days of life [1]. However, in terms of long- term survival, according to a recent nationwide survey among the major pediatric centers in Japan, only 325 of 2013 patients had more than 1 0 years' survival, and only 157 patients (7.8%) remained jaundice-free with normal liver function [2]. About 20% of BA cases without jaundic e are generally able to survive for long periods; and most of those pa tients have portal hypertension or abnormal liver function [3-5]. As t he results of liver transplantation have improved, controversy has ari sen over the optimal care of these children [4, 6, 7]. Some investigat ors have claimed that transplantation is the favored primary therapy f or most patients with BA [8]. We are thus at a turning point concernin g the primary therapy of BA, which makes it necessary to determine the exact indications for the Kasai portoenterostomy and the timing of li ver transplantation. This paper describes our strategy for the optimal treatment of BA patients based on our 117 patients who have had vario us form of portoenterostomy.