O3289: Adjuvant Steroids Following Kasai Portoenterostomy for Biliary Atresia: A Single Center Eighteen-Year Experience

Background: The role of postoperative steroids following Kasai portoenterostomy (KPE) in patients with biliary atresia (BA) is controversial. While current evidence does not support routine adjuvant steroid use, the use of high-dose steroids has not been well interrogated. In 2017, our quaternary academic medical center developed a post-KPE treatment protocol whereby all BA patients received 6-8 weeks of postoperative steroids, starting at a dose of 10 milligrams (mg) per kilogram (kg) per day. Previously, the use of steroids at our institution varied between surgeons.

Methods: Medical records of 50 children who underwent KPE for BA between 2006 and 2024 were reviewed. Patients were stratified into 3 groups: no steroids, low-dose steroids (defined by a starting dose of 2-4 mg/kg/day), and high-dose steroids (starting dose 10 mg/kg/day). After initial taper, patients in both steroid groups continued 2 mg/kg/day of steroids for 4-6 weeks. Baseline demographics and postoperative outcomes including cholangitis rates, native liver survival, postoperative weight and direct bilirubin laboratory values were collected.

Results: 8 patients received no steroids, with a median age at surgery of 57.0 days. 21 patients received low-dose steroids (median age 70.0 days) and 21 patients received high-dose steroids (median age 57.0 days; P=0.41). Patients who received high-dose steroids had significantly greater median length of hospital stay and readmission rates, compared to no or low-dose steroid cohorts (Table 1). Of the 11 patients in the high-dose cohort who were readmitted within 30 days, 8 were readmitted for cholangitis, 1 for biloma, 1 for bile leak, and 1 for wound dehiscence. Of the 5 in the low-dose cohort, 2 were readmitted for cholangitis, 1 for wound dehiscence, 1 for hepatic abscess and 1 for bowel obstruction. There was no significant difference in cholangitis rates between the three groups, though the high-dose cohort tended to present with cholangitis earlier. Median follow up was 1223 days in the no steroid cohort, 2851 days in the low-dose cohort, and 656 days in the high-dose cohort (P=0.14). Notably, direct bilirubin was significantly lower in the high-dose cohort by 6 weeks after KPE [0.5 milligrams (mg) per deciliter (dL)], compared to the no steroid cohort (1.7 mg/dL) and the low-dose cohort (5.4 mg/dL; *P=0.01). There was a trend towards improved native liver survival overall and at 1-year post-KPE between the three steroid groups (Fig. 1A-C), though this effect was less evident by 2 years post-KPE.

Conclusion: We describe the 18-year experience of adjuvant steroid use in BA patients at a single institution. Though limited by sample size, our results demonstrate improved early postoperative biliary drainage with high-dose steroid use, an effect that abrogates with time. Our data also identify a trend towards improved native liver survival with high-dose steroids, most notable at 1 year after KPE.