*Purpose: Kasai hepatoportoenterostomy (HPE) followed by liver transplantation (LT) if needed is the standard of care for children with biliary atresia (BA). Some, however, have advocated for primary liver transplantation (pLT) as a superior treatment approach. The aim of this study was to characterize the rate of pLT in the treatment of BA and compare outcomes of pediatric candidates with BA listed for liver transplantation with and without prior HPE using the national Scientific Registry of Transplant Recipients (SRTR)/Organ Procurement and Transplantation (OPTN) database.

*Methods: The SRTR/OPTN database was retrospectively reviewed for all children with BA listed for primary liver transplant between March 2002 and December 2017. Candidates were categorized as pLT if they had not undergone previous abdominal surgery prior to listing. A multivariable Cox regression was performed to determine risk for waiting list mortality and post-transplant graft survival.

*Results: 3,006 patients with BA were listed for LT during the study period. Only 11% of these candidates had not undergone previous abdominal surgery. Candidates without prior abdominal surgery were younger, smaller, had higher calculated PELD/MELD scores, and were more commonly female, non-White, and without private insurance. Candidates without prior abdominal surgery had higher risk for waiting list mortality (adjusted HR 0.53, 95% CI 0.34 – 0.84, p = 0.007). Among those that successfully underwent LT, there was no significant difference in graft survival by prior surgery status (p = 0.6).

*Conclusions: Liver transplant candidates with BA initially treated with HPE had lower risk of death on the waiting list. HPE should remain the gold standard of care for children with BA, followed by LT if needed for progressive disease.

« Back to 2021 American Transplant Congress