Effect of corticosteroid therapy on outcomes in biliary atresia after Kasai portoenterostomy.

PURPOSE

This study tests the hypothesis that steroid administration improves the outcome of biliary atresia (BA) by evaluating the efficacy of postoperative steroid use on surgical outcomes in infants with BA.

METHODS

Steroid use and outcomes in patients with BA were retrospectively analyzed at a tertiary pediatric hospital. Institutional review board approval was obtained.

RESULTS

Kasai portoenterostomy (PE) was performed in 43 patients with BA treated from 1992 to 2004 (16 boys and 27 girls). Twenty-one PE patients received steroids and 22 did not. Portoenterostomy was successful in 24 patients (55.8%) with consistent serum bilirubin less than 2 mg/dL. Sixteen (66%) received postoperative steroids. A normal postoperative bilirubin was achieved at 6 months in 16 (76%) of 21 patients with steroids compared with 8 (37%) of 22 in untreated controls (Fisher's Exact test, P = .01). Of the 43 patients, 19 (44%) required liver transplantation, including 7 (37%) of 19 with steroids vs 12 (63%) of 19 without (P = .2). Twenty-eight infants developed cholangitis (fever with and without changes in hepatic function): 25 after PE and 3 after transplant. Of the 25, 12 (48%) received steroids. Seven died (16%) (range, 7 months to 4 years): 2 while awaiting transplantation (received steroids) and 5 after transplantation (1 received steroids and 4 were untreated). Survival was 86% (18/21) in patients with steroids and 82% (18/22) in those without. Transplant survival (74%) was comparable to previously reported historical controls (82%).

CONCLUSIONS

The Kasai PE continues to be the procedure of choice in infants with BA younger than 3 months. A significantly improved clearance of postoperative jaundice and lower serum bilirubin levels were observed in patients receiving steroids. However, steroids had no effect on the incidence of cholangitis, need for liver transplantation, and overall survival. A prospective study with standardized dose and length of steroid administration and longer period of follow-up is necessary to more accurately assess the effectiveness of steroids after PE.