A multicenter study of primary liver transplantation for biliary atresia in Japan

Abstract

Purpose: Kasai portoenterostomy (KP) is the primary procedure for biliary atresia (BA). However, due to reports of poor outcomes of KP, primary liver transplantation (LT) is preferred in selected cases. In Japan, primary LT is limited with no study reports details, particularly regarding the indications. We conducted the first nationwide survey to assess the status of primary LT in Japan.

Methods: Questionnaires were sent to institutions where procedures other than KP were performed as the initial procedure for BA and registered to the Japan Biliary Atresia Registry. Parameters related to procedure selection were analyzed.

Results: Of 2895 patients registered (1989-2013), primary LT (n = 15) or exploratory laparotomy (EL) followed by LT (n = 9) without KP was performed in 24 cases (0.8%). The main reason for primary LT was late diagnosis, and for EL followed by LT, lack of fibrous tissue at the porta hepatis (ο-type). The prognoses of LT without KP was good.

Conclusion: Non-KP initial procedures were limited. Clear criteria regarding the timing of diagnosis or patient condition could not be determined. Reasons for not initially selecting KP varied, but late diagnosis was predominant. Further study is needed to create guidelines for the initial treatment of BA patients.

Keywords: Biliary atresia; Exploratory laparotomy; Kasai portoenterostomy; Liver transplantation; Primary liver transplantation.