Adjuvant steroids following Kasai portoenterostomy for biliary atresia: a single-center 18-year experience comparing different steroid dose regimens.

PURPOSE

In 2017, our hospital transitioned to a standardized post-KPE high-dose steroid protocol. We sought to compare outcomes for biliary atresia (BA) for this protocol against historical treatment with no or low-dose steroids.

METHODS

Between 2006 and 2024, 50 children underwent KPE for BA. Patients were stratified into three groups: no steroids, low-dose steroids (defined by a starting dose of 2-4 mg/kg/day), and high-dose steroids (starting dose of 10 mg/kg/day). After the initial taper, patients in both steroid groups continued with 2 mg/kg/day for 4-6 weeks.

RESULTS

8 patients received no steroids, 21 received low-dose steroids, and 21 received high-dose steroids. Patients treated with high-dose steroids had significantly greater readmission rates compared to the no or low-dose steroid cohorts. There was an overall trend towards improved native liver survival for the high-dose steroid cohort at 1 and 5 years after KPE. There was no significant difference in rates of cholangitis, though the high-dose cohort tended to present with cholangitis within 30 days of discharge.

CONCLUSIONS

We describe the 18-year experience of adjuvant steroid use in BA patients at a single institution. We show improved early postoperative biliary drainage with high-dose steroid use and identify a trend towards improved native liver survival with high-dose steroids.