Real-world Multi-institutional Data From the Midwest Pediatric Surgery Consortium (MWPSC) to Assess the Effect of Delayed Kasai Procedure on Biliary Drainage in Patients With Biliary Atresia

PURPOSE: Early Kasai portoenterostomy (KPE) for infants with biliary atresia (BA) increases the chance of transplant-free survival (TFS). However, early timing of KPE is not consistently achieved in the United States. Clearance of jaundice at three months is predictive of TFS. Among a cohort of patients with BA, we investigated institutional variability in the initiation of hyperbilirubinemia evaluation and operative timing to identify factors associated with successful jaundice clearance.

METHODS: A multi-institutional, retrospective study was performed at eleven U.S. tertiary children's hospitals. Infants diagnosed with BA between 10/1/2015-10/1/2020 were identified. Age at initiation of diagnostic workup and age at KPE were collected. Adjusted multivariable logistic regression was used to determine factors associated with direct bilirubin normalization at three months following KPE.

RESULTS: In 161 infants, the median age at initiation of jaundice evaluation was 35 days (IQR 8-60). Among 148 patients who underwent KPE, median age at surgery was 53 days (IQR 37.3-67.5). Each 10-day increase in age at KPE was associated with a 18.8 % decrease in odds of normalizing bilirubin at three months (OR 0.81, 95 % CI 0.66-0.99), with infants who underwent KPE ≤50 days significantly more likely to normalize bilirubin (OR 2.6, CI 1.1-6.1) compared to KPE >50 days. There was significant variation among institutions in the time from initiation of workup to KPE (range 0-24.5 days, p = 0.02) and the odds of patients normalizing direct bilirubin at three months (range 0.04-0.89, p = 0.044).

CONCLUSION: Our results confirmed that increasing age at KPE decreases the odds of clearing bilirubin at three months post-KPE. We identified significant institutional variability in the time from workup to KPE that may have impacted the likelihood of successful biliary drainage.

LEVEL OF EVIDENCE: IV (Well-designed case-control or cohort study).