Predicting Cirrhosis and Poor Outcomes of Bile Drainage Surgery for Biliary Atresia

Objective: To identify patients with biliary atresia (BA) with extremely poor outcomes of bile drainage surgery using the infant BA liver fibrosis (iBALF) score, a liver fibrosis marker based on standard blood analysis. Summary Background Data: Although primary liver transplantation (LTx) is beginning to be considered as an alternative to bile drainage surgery in patients with BA, those most likely to benefit from this procedure have not yet been identified. Methods: The medical records of 380 patients with BA with bile drainage surgery between 2015 and 2019 were collected for retrospective analysis from 60 participating hospitals. To predict native liver survival at age 1 year, a receiver-operating characteristic curve was drawn for the iBALF score. The cutoff value was determined as the point indicating >99% sensitivity. Results: The median age at surgery was 56 days (range: 4–183 d), and native liver survival at age 1 year was achieved in 258 (67.9%) patients. An iBALF score of 5.27 was chosen as the cutoff, and 18 patients (4.7%) were found to have an iBALF score >5.27; of these, only two (95% confidence interval [CI]: 1.4–34.7%) had native liver survival at age 1 year, indicating a significantly poorer outcome than in the other patients (95% CI: 65.7–75.4%). Moreover, patients with an iBALF score >5.27 had significantly higher mortality and younger age at salvage LTx. Conclusions: Patients with BA having a preoperative iBALF score >5.27 had extremely poor outcomes of bile drainage surgery and may be considered as candidates for primary LTx.