Biliary atresia is the leading indication for liver transplantation in children.
Dr Benjamin Schneider and colleagues determined the prognostic factors and optimal management of biliary atresia.
The team performed a retrospective study of all children who underwent hepatoportoenterostomy for biliary atresia between 1997 and 2000.
The children underwent the procedures at 9 centers in the United States.
The research team correlated outcomes at age 24 months with demographic and clinical parameters.
The researchers reported that a total of 104 children underwent hepatoportoenterostomy.
|The post-hepatoportoenterostomy survival rate with bilirubin less than 2 mg/dL was 84%|
|Journal of Pediatrics|
Of these, 25% had congenital anomalies, and outcome was worse in those with biliary atresia splenic malformation syndrome.
Diagnostic and clinical approaches varied, although specific approaches did not appear to correlate with outcome.
The team noted that the average age at referral was 53 days, and the average age at hepatoportoenterostomy was 61 days.
At age 24 months, 58 children were alive with their native liver, and 42 had undergone liver transplantation.
The researchers also observed that 4 had died without undergoing transplantation.
The survival rate in children with total bilirubin less than 2 mg/dL at 3 months after hepatoportoenterostomy was 84% vs 16% in those with higher levels.
Dr Schneider's team concluded, “Outcomes in the study centers was equivalent to that reported in other countries.”
“Total bilirubin in early follow-up after hepatoportoenterostomy was highly predictive of outcome.”
“Efforts to improve bile flow after hepatoportoenterostomy may lead to improved outcome in children with biliary atresia.”