https://pubmed.ncbi.nlm.nih.gov/35393118/ Biliary atresia

J Pediatr Surg. 2022 Mar 15;S0022-3468(22)00216-0.
doi: 10.1016/j.jpedsurg.2022.03.009.Online ahead of print.

Progress in living donor liver transplantation for biliary atresia and challenges faced: A thirty-year single institutional experience

Tatsuya Okamoto 1, Hideaki Okajima 2, Eri Ogawa 3, Mari Sonoda 3, Elena Yukie Uebayashi 3, Takashi Ito 3, Satoru Seo 3, Koichiro Hata 3, Toshihiko Masui 3, Kojiro Taura 3, Shinji Uemoto 4, Etsurou Hatano 3

Abstract
Background: Biliary atresia (BA) is the most common indication for liver transplantation in the pediatric population, and living donor liver transplantation (LDLT) and deceased donor liver transplantation (DDLT) have been established as a radical treatment for BA .The aim of this study was to clarify the long term outcomes and risk factors affecting the LDLT outcomes, as well as the challenges faced.

Methods: Between 1990 and 2019, 666 BA patients underwent LDLT in our institution and were enrolled in this study. Data regarding the recipient's age, anatomic findings of the biliary tree at Kasai's portoenterostomy, basic characteristics at transplantation, transplant profiles, donor characteristics, and outcomes of LDLT were analyzed.

Results: The 1-, 5-, 10-, 15-, 20-, and 25-year graft survival rates of BA patients who underwent LDLT were 88.1%, 85.4%, 81.5%, 78.9%, 76.6%, and 75.5%, respectively. The transplant era, age at transplantation, ABO incompatible transplant, and presence of pulmonary vascular complications were identified as significant risk factors for overall graft survival. When the study period was divided into the first (1990-1999) and second (2000-2019) phases and re analyzed, the outcomes of ABO-incompatible transplants and LDLT for adult BA patients remained inferior to others in the second phase. The 20-year graft survival rate in patients who underwent re transplantation in the second phase was 54.2%.

Conclusions: The outcomes of LDLT in children are generally good, but the immunosuppression procedures need to be further improved for ABO-incompatible cases in the future. Further improvements in LDLT results for adult patients and re transplantation remain challenges to be addressed in this field, and future attempts, including revision to the organ allocation system of deceased donors, are necessary.