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

  • Dor Yoeli
    Correspondence
    Corresponding author.
    Affiliations
    Division of Transplant Surgery, Department of Surgery, Children's Hospital Colorado, University of Colorado School of Medicine, 13123 East 16th Avenue, Aurora, CO 80045, USA
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  • Rashikh A Choudhury
    Affiliations
    Division of Transplant Surgery, Department of Surgery, Children's Hospital Colorado, University of Colorado School of Medicine, 13123 East 16th Avenue, Aurora, CO 80045, USA
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  • Shikha S Sundaram
    Affiliations
    Department of Pediatrics, Section of Gastroenterology, Hepatology and Nutrition, The Digestive Health Institute, Children's Hospital Colorado, University of Colorado School of Medicine, 13123 East 16th Avenue, Aurora, CO 80045, USA
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  • Cara L Mack
    Affiliations
    Department of Pediatrics, Section of Gastroenterology, Hepatology and Nutrition, The Digestive Health Institute, Children's Hospital Colorado, University of Colorado School of Medicine, 13123 East 16th Avenue, Aurora, CO 80045, USA
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  • Jonathan P Roach
    Affiliations
    Division of Pediatric Surgery, Department of Surgery, Children's Hospital Colorado, University of Colorado School of Medicine, 13123 East 16th Avenue, Aurora, CO 80045, USA
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  • Frederick M Karrer
    Affiliations
    Division of Pediatric Surgery, Department of Surgery, Children's Hospital Colorado, University of Colorado School of Medicine, 13123 East 16th Avenue, Aurora, CO 80045, USA
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  • Michael E Wachs
    Affiliations
    Division of Transplant Surgery, Department of Surgery, Children's Hospital Colorado, University of Colorado School of Medicine, 13123 East 16th Avenue, Aurora, CO 80045, USA
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  • Megan A Adams
    Affiliations
    Division of Transplant Surgery, Department of Surgery, Children's Hospital Colorado, University of Colorado School of Medicine, 13123 East 16th Avenue, Aurora, CO 80045, USA
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      Highlights

      • The impact of hepatoportoenterostomy on liver transplant waiting list survival among children with biliary atresia is not well known, and its influence on post-transplant outcomes is controversial.
      • Out of 3,438 transplant candidates with biliary atresia listed for liver transplantation, 15% were listed for primary transplantation, 17% for salvage transplant after early Kasai failure, and 67% after late Kasai failure.
      • Candidates listed after late Kasai failure had lower disease severity as demonstrated by lower bilirubin levels, lower MELD/PELD scores, and lower incidence of hospitalization at time of transplant in comparison to candidates listed for primary transplantation.
      • Children with late Kasai failure had significantly superior waiting list and post-transplant graft survival in comparison to those that did not undergo Kasai hepatoportoenterostomy and were listed for primary liver transplantation.
      • Candidates listed for primary liver transplantation and for salvage transplantation after early Kasai failure had equivalent waiting list and post-transplant survival outcomes.

      Abstract

      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

      Abbreviations:

      BA (biliary atresia), CI (confidence interval), ESLD (end stage liver disease), HPE (hepatoportoenterostomy), HR (hazard ratio), LT (liver transplant), MELD (model for end-stage liver disease), OPTN (organ procurement and transplantation), PELD (pediatric end-stage liver disease), SD (standard deviation), SRTR (scientific registry of transplant recipients)
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