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: 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.