peri-operative high-dose v post-operative low dose steroid therapy in the management of biliary atresia: a preliminary report

background: the use of high-dose steroid therapy peri portoenterostomy may have a positive impact on the frequency of cholangitis and survival rate.   methods: a prospective study was conducted on two groups of patients (less than three months of age) suffering from biliary atresia from 1999 to 2005. the patients in group i (g i) were managed peri-operatively by high-dose methylprednisolone while the other group (g ii) received low dose methylprednisolone only post-operatively (2mg/k/day for 1 month). infants in gi (n=30) received methylprednisolone for 3 successive days before operation (10-8-6mg/kg/day), and 10 mg/k at the day of operation respectively. thereafter the dose was tapered in the next successive 6 days by 8, 6, 5, 4, 3, and 2 mg/kg/day and continued for one month.   results: seventy two infants with biliary atresia were operated (39 girls and 33 boys). twenty-six of the 30 patients (86%) in g i became jaundice-free within 90 days after portoenterostomy while only seven (15%) of the 42 patients in g ii had normal bilirubin (p<0.0001). episodes of postoperative cholangitis in g i were 20% (6 of 30), and 53% (24 of 42) in g ii (p<0.005). the difference in 3-year survival rate between the two groups is also remarkable: eighty seven percent (26 of 30) in gi versus 29% (13 of 45) in g ii (p<0.005). death related to biliary atresia occurred in 1 (3.3%) patient in gi compared with 12 (29%) patients in g ii (p<0.005).   conclusion: these results provide strong evidence that peri-operative high dose steroid therapy is not only safe in this patients population, but because of its anti-inflammatory and cholerrhetic effects has a positive impact on preventing recurrent cholangitis, and ultimately survival