Abstract

Introduction: Long term outcomes in patients with biliary atresia (BA) undergoing liver transplantation (LT) have improved but there remains significant perioperative morbidity. Acute-on-chronic liver failure (ACLF) is characterized by decompensated liver disease, multi-organ failure, and high mortality. Though well described in adults, pediatric ACLF and its impact on postoperative outcomes in children with BA undergoing LT is relatively understudied. Methods: A single-institutional retrospective analysis was performed. Patients aged < 18 years with BA who underwent LT between 1/2011 – 12/2021 were included. Patients who died awaiting LT, and those who received LT with incomplete documentation were excluded. ACLF was defined using the North American Consortium for the Study of End-Stage Liver Disease (NACSELD)-ACLF criteria: presence of 2 or more extrahepatic organ failures, and was characterized during the index admission for LT. Mann Whitney U test for continuous variables and Fischer Exact for categorical variables was used. Results are expressed as medians with interquartile ranges. Results: Of 132 patients, 10 (7.5%) who died awaiting LT and 15 (11.4%) who had incomplete pre-LT documentation were excluded. 107 (65% female; median age 14 [9-31] months) were included in this study. Based on the NACSELD-ACLF criteria, 11 (10.3%) patients had ACLF during index admission. Five of 11 (46%) patients were listed status 1B prior to LT. Patient with ACLF were younger at time of LT (9 [7-11] vs. 15 [10-37] months, p < 0.001), had a higher PELD score (34 [24-35] vs. 28 [24-33]; p < 0.001), higher total length of stay (178 [119-224] vs. 21 [9-48] days, p < 0.001), and had a higher rate of return to the operating room (36% vs. 18%; p < 0.01) compared to the non-ACLF group (n=96). The 1-year readmission (64 % vs. 38 %; p = 0.09) and overall 1-year survival (100% vs. 91%; p=0.28) rates were comparable between ACLF and non ACLF groups. Conclusions: In children with BA undergoing LT, survival outcomes post-LT between ACLF and non ACLF patients are comparable despite increased perioperative morbidity seen in those with ACLF. This reiterates the need for further studies to understand the impact of ACLF and prioritization of these critically ill patients for LT.

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