Vol.:(0123456789) 1 3 Pediatric Surgery International https://doi.org/10.1007/s00383-019-04553-7 ORIGINAL ARTICLE A multicenter study of primary liver transplantation for biliary atresia in Japan Keiichi Uto 1  · Yukihiro Inomata 1  · Seisuke Sakamoto 1  · Taizo Hibi 1  · Hideyuki Sasaki 2  · Masaki Nio 2 Accepted: 9 September 2019 © Springer-Verlag GmbH Germany, part of Springer Nature 2019 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 frst 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 fbrous 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 · Kasai portoenterostomy · Liver transplantation · Primary liver transplantation · Exploratory laparotomy Introduction Kasai portoenterostomy (KP) has been accepted as the primary procedure for biliary atresia (BA) worldwide. In Japan, where KP originated, KP is the preferred procedure and is performed more often than in Western countries. In fact, the outcome of KP tends to be good in terms of native liver survival [1, 2]. Limited access to deceased donor liver transplantation (LT) was another reason for the priority of KP in Japan, where living donor LT (LDLT) account for more than 95% of all LTs. There have been reports that some factors, such as older age at KP, have a negative impact on KP outcomes [3]. If these factors are identifed at the time of diagnosis, they may infuence the decision for the selec- tion of primary LT rather than KP, as reported previously [4]. In contrast, even relatively late KP has been reported to have good outcomes in some cases [5]. KP at 132 days of age has been reported to have a satisfactory result [6]. These reports suggest that even a late diagnosis cannot exclude the possibility of KP as the primary procedure. With the establishment of LT in Japan, especially with LDLT, the hurdles for LT in Japan are lower than ever before. So far, however, there is no consensus regarding the timeframe or under what conditions LT should be considered over KP as the primary procedure in Japan. As the frst step toward defning the appropriate indications for LT without KP, the present status of primary LT in Japan should be clarifed. The aim of this study was to identify the indications of pri- mary LT for BA in Japan. * Yukihiro Inomata yino@kuh.kumamoto-u.ac.jp 1 Department of Pediatric Surgery and Transplantation, Graduate School of Medical Sciences, Kumamoto University, 1-1-1 Honjo, Kumamoto 860-8556, Japan 2 Department of Pediatric Surgery, Tohoku University Graduate School of Medicine, 1-1 Seiryomachi, Aobaku, Sendai 980-8574, Japan