Current concept of the treatment of biliary atresia

Abstract

Hepatic portoenterostomy (Kasai operation) for the patient with biliary atresia (BA) can restore the bile flow in approximately 80% of children 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 10 years' survival, and only 157 patients (7.8%) remained jaundice-free with normal liver function [2]. About 20% of BA cases without jaundice are generally able to survive for long periods; and most of those patients have portal hypertension or abnormal liver function [3-5]. As the results of liver transplantation have improved, controversy has arisen over the optimal care of these children [4, 6, 7]. Some investigators have claimed that transplantation is the favored primary therapy for most patients with BA [8]. We are thus at a turning point concerning the primary therapy of BA, which makes it necessary to determine the exact indications for the Kasai portoenterostomy and the timing of liver transplantation. This paper describes our strategy for the optimal treatment of BA patients based on our 117 patients who have had various form of portoenterostomy.

Improved Outcome of Biliary Atresia with Postoperative High-Dose Steroid

“…Miyano and colleagues [22] showed that although about 70% of their patients had evidence of bile secretion immediately after surgery, only 32% maintained bile secretion after 5 years. However, children successfully treated with adjuvant steroid in Dillon's study [3] seem to maintain their bile flow for 5 years.…”

A scoring system to predict the need for liver transplantation for biliary atresia after Kasai portoenterostomy

“…In untreated infants the mean expected survival is about 11 months [8]. The Kasai portoenterostomy, introduced in 1959, can restore bile flow in approximately 62% to 80% of infants operated on before 60 days of life [4,6]. The long-term prognosis for 30% to 50% of those undergoing the procedure is good [9].…”

Digestive System

“…Without correction of bile fl ow obstruction within 2-3 months of life, irreversible hepatic damage and complete obliteration of the extrahepatic biliary tree will result. This process could be progressive even after surgical correction of the obstruction [ 114 ]. The neonatal hepatitis syndrome includes various kinds of diseases such as idiopathic neonatal hepatitis, infectious hepatitis, and hepatitis from metabolic or genetic causes.…”