Primary vs. salvage liver transplantation for biliary atresia: A retrospective cohort study

J Pediatr Surg. 2022 Oct;57(10):407-413. doi: 10.1016/j.jpedsurg.2021.12.027. Epub 2022 Jan 10.


Introduction: Kasai hepatoportoenterostomy is the standard of care for children with biliary atresia, but a majority of patients progress to end-stage liver disease and require a salvage liver transplant. Given the high failure rates of the hepatoportoenterostomy operation, some have advocated for primary liver transplantation as a superior treatment approach. The aim of this study was to compare outcomes of pediatric candidates with biliary atresia listed for primary vs. salvage liver transplantation.

Methods: The SRTR/OPTN database was retrospectively reviewed for all children with biliary atresia listed for liver transplant between March 2002 and February 2021. Candidates were categorized as primary liver transplant if they had not undergone previous abdominal surgery prior to listing and salvage liver transplant if they had. Salvage transplants were further categorized as early failure if listed within the first year of life or late failure if listed at an older age.

Results: 3438 children with biliary atresia were listed for transplant during the study period, with 15% of them listed for a primary transplant, 17% for salvage transplant after early failure, and 67% after late failure. Recipients of salvage liver transplant with late failure had lower bilirubin levels and were less critically ill as demonstrated by MELD/PELD scores and hospitalization status. Correspondingly, these recipients had higher waiting list and graft survival, though this did not remain statistically significant after adjustment in multivariable models. There were no differences in waiting list, recipient, or graft survival with primary vs. salvage liver transplant after early failure.

Conclusion: Kasai hepatoportoenterostomy should remain the standard of care in biliary atresia as it may delay need for transplant beyond the first year of life in a subset of recipients and does not jeopardize subsequent transplant outcomes, even with early failure.

Levels of evidence: Retrospective cohort study (Level III).

Keywords: Biliary atresia; Disparities; Infant; Liver transplantation; Pediatric; Waiting list.

MeSH terms

  • Biliary Atresia* / surgery
  • Child
  • Graft Survival
  • Humans
  • Infant
  • Liver Transplantation*
  • Portoenterostomy, Hepatic
  • Retrospective Studies