Related Experiment Videos
Current concept of the treatment of biliary atresia
1Department of Pediatric Surgery, Juntendo University, School of Medicine, Tokyo, Japan.
Insights
Hepatic portoenterostomy (Kasai operation) offers improved bile flow for biliary atresia (BA) patients. However, long-term survival and normal liver function remain challenges, necessitating a re-evaluation of treatment strategies.
Area of Science:
- Pediatric Surgery
- Hepatology
- Gastroenterology
Background:
- Biliary atresia (BA) affects bile flow, with Kasai operation restoring it in ~80% of early-treated children.
- Long-term survival post-Kasai operation is limited, with only 7.8% remaining jaundice-free with normal liver function.
- Improved liver transplantation outcomes raise questions about optimal primary therapy for BA.
Purpose of the Study:
- To determine optimal treatment strategies for biliary atresia (BA).
- To define exact indications for Kasai portoenterostomy.
- To establish optimal timing for liver transplantation in BA patients.
Main Methods:
- Retrospective analysis of 117 biliary atresia patients.
- Review of various portoenterostomy techniques.
- Evaluation of long-term outcomes including survival, jaundice, and liver function.
Main Results:
- Kasai operation restores bile flow in a majority of infants operated on before 60 days.
- Long-term survival and normal liver function post-Kasai operation are achieved in a minority of patients.
- Many long-term survivors without jaundice still experience portal hypertension or abnormal liver function.
Conclusions:
- A critical juncture exists in determining primary therapy for biliary atresia.
- Defining precise indications for Kasai portoenterostomy and timing of liver transplantation is crucial.
- This study outlines a treatment strategy based on extensive clinical experience.
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.