It is controversial whether adjuvant steroid treatment should be given to biliary atresia (BA) patients following a Kasai portoenterostomy (KPE). This study aimed to quantitatively and systematically evaluate the effect of adjuvant steroid therapy post-KPE in relation to major clinical outcomes of BA patients.

We systematically reviewed the literature in PubMed, Embase, the Cochrane Library, China Knowledge Resource Integrated Database, Wanfang Database, Scholarly and Academic Information Navigator and manually searched for relevant papers published before August, 2015. We extracted data on the effects of steroid treatment following KPE on clinical outcome, including jaundice free rate and native liver survival rate at 6 months, 1 or 2 years after KPE. The weighted overall relative risk (RR) and 95% confidence intervals (CIs) were calculated by using a random-effects model.

Eight cohort studies and two randomized controlled trials (RCTs) were identified (n=998). Of them, 6 cohort studies and 2 trials investigated the effect of steroid treatment as compared to non-users or placebo (n=566), and 2 cohort studies compared the effects of high-dose to low-dose steroid treatment (n=432). Steroid usage increased the clearance rates of jaundice at 6 months (pooled RR: 1.32; 95% CI: 0.995-1.76; I²=72.6%) and 1 year (pooled RR: 1.35; 95% CI: 1.12-1.61; I²=0.0%), but not 2 years (pooled RR: 0.82; 95% CI: 0.55-1.22; I²=0.0%) after KPE. There was no solid evidence supporting that steroid treatment would improve native liver survival rate at 6 months (pooled RR: 1.02; 95% CI: 0.90-1.15; I²=0.0%), 1 year (pooled RR: 1.10; 95% CI: 0.91-1.34; I²=35.2%) or 2 years (pooled RR: 1.00; 95% CI: 0.73-1.35; I²=57.4%) after KPE.

Adjuvant steroid treatment following KPE may improve short-term (≤1 year) clearance rate of jaundice, but no significant effects on long-term (≥2 years) clearance rate of jaundice and native liver survival rate. Studies on doses and duration of steroids, and long-term follow-up studies are warranted.