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World J Transplant. Sep 18, 2021; 11(9): 400-409
Published online Sep 18, 2021. doi: 10.5500/wjt.v11.i9.400
Management of biliary atresia: To transplant or not to transplant
Christos Dimitrios Kakos, Ioannis A Ziogas, Sophoclis P Alexopoulos, Georgios Tsoulfas
Christos Dimitrios Kakos, Ioannis A Ziogas, Surgery Working Group, Society of Junior Doctors, Athens 15123, Greece
Ioannis A Ziogas, Sophoclis P Alexopoulos, Department of Surgery, Division of Hepatobiliary Surgery and Liver Transplantation, Vanderbilt University Medical Center, Nashville, TN 37212, United States
Georgios Tsoulfas, Department of Transplant Surgery, Aristotle University School of Medicine, Thessaloniki 54622, Greece
Author contributions: Kakos CD, Ziogas IA, Alexopoulos SP and Tsoulfas G conceived and designed the study, acquired, analyzed, and interpreted the data, drafted, and critically revised the manuscript, and approved the final version of the manuscript.
Conflict-of-interest statement: The authors declare no conflict of interests for this article.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Georgios Tsoulfas, FACS, FICS, MD, PhD, Associate Professor, Department of Transplant Surgery, Aristotle University School of Medicine, 66 Tsimiski Street, Thessaloniki 54622, Greece. tsoulfasg@gmail.com
Received: May 20, 2021
Peer-review started: May 20, 2021
First decision: June 17, 2021
Revised: June 26, 2021
Accepted: August 18, 2021
Article in press: August 18, 2021
Published online: September 18, 2021
Processing time: 117 Days and 8.4 Hours
Abstract

Kasai procedure (KP) and liver transplantation (LT) represent the only therapeutic options for patients with biliary atresia (BA), the most common indication for LT in the pediatric population. However, KP represents by no means a radical option but rather a bridging one, as nearly all patients will finally require a liver graft. More and more experts in the field of transplant surgery propose that maybe it is time for a paradigm change in BA treatment and abandon KP as transplantation seems inevitable. Inadequacy of organs yet makes this option currently not feasible, so it seems useful to find ways to maximize the efficacy of KP. In previous decades, multiple studies tried to identify these factors which opt for better results, but in general, outcomes of KP have not improved to the level that was anticipated. This review provides the framework of conditions which favor native liver survival after KP and the ones which optimize a positive LT outcome. Strategies of transition of care at the right time are also presented, as transplantation plays a key role in the surgical treatment of BA. Future studies and further organization in the transplant field will allow for greater organ availability and better outcomes to be achieved for BA patients.

Keywords: Biliary atresia; Kasai procedure; Portoenterostomy; Native liver survival; Liver transplantation

Core Tip: Timely diagnosis of biliary atresia (BA) is critical to optimizing the outcomes of Kasai procedure (KP), which should be performed as early as possible. Children with a delayed diagnosis of BA at high risk of early KP failure or those presenting with clear evidence of decompensated cirrhosis should be considered for primary liver transplantation (LT). Early KP failure requiring salvage LT within the first 2-3 years of life occurs in nearly half of all children with BA but even those with a successful KP need life-long monitoring for progression of liver disease that may require salvage LT.