Significance and indications for reoperative portoenterostomy in biliary atresia in light of long‐term outcome

Background Among biliary atresia (BA) patients undergoing revision following failed portoenterostomy, long‐term native liver survival (NLS), physical condition, and indications for revision were explored. Methods From 1977–2015, 33 of 95 BA patients (35%) at our institution underwent revision. Ten‐ and 20‐year NLS rates (NLSRs) and biochemical statuses of 20‐year‐old native liver survivors were compared between post‐portoenterostomy BA patients with and without history of revision. Factors associated with 10‐year NLS following revision and optimal cut‐offs for potential predictors were analyzed. Results Overall 10‐ and 20‐year NLSRs were 57% and 54%, respectively. Ten‐ and 20‐year NLSRs were 49% and 45% in the revision group and 63% and 61% in the non‐revision group, respectively. Among 20‐year‐old native liver survivors, differences in admission rates between ages 10–20 years were not significant for cholangitis (revision, 46%; non‐revision, 40%; P = 0.30) or portal hypertension (revision, 38%; non‐revision, 15%; P = 0.21). Differences in hepatobiliary function‐associated blood tests between these groups at 20 years old were only significant for aspartate aminotransferase (P = 0.02) and gamma‐glutamyl transpeptidase (P = 0.047). Among potential predictors of 10‐year NLS that we investigated, rate of change in total bilirubin (TB) over the first month post‐portoenterostomy was best (P = 0.0019), and the receiver operating characteristic curve revealed an optimal cut‐off for this first‐month change in TB of −3.7 mg/dl (area under the curve, 0.85; sensitivity, 0.79; specificity, 0.83). Conclusions In approximately half of the patients, revision provided 10‐ and 20‐year NLS, and biochemical status at 20 years old was comparable across revision and non‐revision patients. Rate of change in TB during the first month post‐portoenterostomy offers a sensitive predictor of revision. Highlight Saito and colleagues report on the long‐term native liver survival and biochemical status of biliary atresia patients who underwent revision following failed portoenterostomy. They also introduce the indications for reoperative surgery by statistically comparing various clinical parameters between patients with successful and those with unsuccessful revision following failed initial surgery.