Preoperative risk factors for the early failure of the Kasai portoenterostomy in patients with biliary atresia.

Abstract

Biliary atresia is managed surgically by the Kasai portoenterostomy (KP). It has been reported by some groups that the outcomes of patients who have an early failed KP requiring a liver transplant (LTx) within the first year of life are worse than the outcomes of patients who undergo a primary LTx. The aim of this study was to identify preoperative parameters that could help predict what patients are at risk for the early failure of the procedure. We conducted a retrospective chart review of all patients who underwent a KP between January 2008 and May 2018. The following preoperative parameters were analyzed: age at KP, anatomical variant of the biliary atresia, degree of liver fibrosis, CMV status, and PELD score. The main outcome of the study was the early failure of the KP (EF-K), which was defined as the need for LTx before 1year of age, or BA-related death before 1year of age. Second, we analyzed the risk factors associated with death without LTx within the first year of life. A total of 58 patients were included in the analysis. The native liver survival (NLS) was 56.5% and 48% at 1 and 5years post KP, respectively. Overall survival (OS) was 79% and 76% at 1 and 5years post KP, respectively. Early failure of KP occurred in 23 (39.7%) patients. OS in this group was 47% and 40% at 1 and 5years, respectively. On the contrary, the OS of the remaining 35 (60.3%) patients was 100% at 1 and 5years (P < 0.0001). When we compared all preoperative parameters, the only predictor of EF-K was the PELD score. When we analyzed the cases in the EF-K group who died without LTx, we found that the significant predictors were the cystic variant, a degree of liver fibrosis >4, and the PELD score. Nevertheless, on multivariate analysis, only PELD score was found as a statistically significant variable. Due to bad prognosis found in EF-K patients, we believe that it could be reasonable to offer them a primary LTx. PELD score was found to be the strongest preoperative parameter that allows predicting which patient will likely have an early failed KP. Further prospective and multicenter studies are needed to reinforce these results.