Decision Analysis in the Differential Diagnosis of Extrahepatic Billiary Atresia from Other Causes of Neonatal Liver Diseases. 500

Abstract

Extrahepatic biliary atresia (EHBA) is a devastating disorder in which there is complete obstruction to bile flow and progressive liver injury during infancy. Because hepatic portoenterostomy only alleviates biliary obstruction enough to improve function 30% of the time, biliary atresia is the most common indication for pediatric liver transplantation. Early diagnosis is thought to improve the response to portoenterostomy. The clinical manifestations of EHBA are difficult to distinguish from other causes of liver injury; physicians must make a timely decision about the need for definitive intraoperative diagnosis on the basis of a complex, often expensive, diagnostic evaluation. In this study, we applied decision analysis methodology to previously published data on cholestatic infants to provide quantitative information about the contribution of liver biopsy to this decision. The results show that evaluation including percutaneous liver biopsy had higher expected utility than evaluation not including biopsy. Since the literature contains a wide range of probability values for correct diagnosis via hepatobiliary scan and liver biopsy, sensitivity analysis was performed; the decision was robust unless the probability of incorrect pathologic diagnosis was greater than 0.2. The results show that decision analysis methodology can be used to give the clinician quantitative information regarding effective evaluation of infants with neonatal liver disease.