Primary Liver Transplantation vs. Transplant after Kasai Portoenterostomy for Infants with Biliary Atresia

Introduction: Primary liver transplants (pLT) in patients with biliary atresia (BA) are infrequent, since most babies with BA undergo a prior Kasai portoenterostomy (KPE). This study compared transplant outcomes in children with BA with or without a prior KPE. We hypothesized that pLT have less morbidity and better outcomes compared to those done after a failed KPE. Methods: A retrospective review of patients with BA transplanted at our institution was performed. Patients were included if they received a pLT or if they were transplanted less than 2 years from KPE. Outcomes were compared between those groups. Comparisons were also made based on era (early: 1997-2008 vs. modern: 2009-2020). p < 0.05 was considered significant. Results: Patients who received a pLT were older at diagnosis (141.5 +/- 46.0 vs. KPE 67.1 +/- 25.5 days, p < 0.001). The time between diagnosis and listing for transplant was shorter in the pLT group (44.5 +/- 44.7 vs. KPE 140.8 +/- 102.8 days, p < 0.001). In the modern era, the calculated PELD score for the pLT was significantly higher (23 +/- 8 vs. KPE 16 +/- 8, p = 0.022). Two waitlist deaths occurred in the KPE group (none in pLT, p = 0.14). Both the duration of transplant surgery and transfusion requirements were similar in both groups. There was a significant improvement in graft survival in transplants after KPE between eras (early era 84.3% vs. modern era 97.8%, p = 0.025). The 1-year patient and graft survival after pLT was 100%. Conclusions: Patient and graft survival after pLT are comparable to transplants after a failed KPE but pLT avoids a prior intervention. There was no significant difference in pre- or peri-transplant morbidity between groups other than wait list mortality. A multicenter collaboration with more patients may help demonstrate the potential benefits of pLT in patients predicted to have early failure of KPE.

Automated summary

This study compared transplant outcomes in children with biliary atresia who underwent primary liver transplant (pLT) with or without a prior Kasai portoenterostomy (KPE). The results showed comparable patient and graft survival between the pLT and failed KPE groups. The study suggests that pLT may be a beneficial option for patients predicted to have early failure of KPE.