Prediction of survival in extrahepatic biliary atresia by hepatic duplex sonography

Background: The clinical course of biliary atresia patients is extremely va riable. To optimize conservative treatment and correctly schedule liver tra nsplantation, noninvasive investigations that are predictive of individual survival and that can be performed regularly an needed. In this study, the prognostic value of Doppler sonography was investigated in these patients. Methods: Thirty biliary atresia patients (age range, 1 month to 15.2 years; mean, 4.0 years) and 38 control subjects underwent standardized Doppler so nography of liver and spleen. Biochemical rests of liver function and of fi brogenesis were performed in parallel. Individual clinical outcome was regi stered 1 and 2 years later. Results: In control subjects, maximum portal flow velocity (V-max) was more than 16 cm/sec, and the hepatic vein flow pattern was triphasic. Among chi ldren with biliary atresia, those with diminished portal V-max a flattened hepatic vein flow curve, or a hepatic artery resistance index of 0.8 or mor e had significantly lower indices of hepatic protein synthesis (albumin, ch olinesterase), higher bilirubin levels, and higher concentrations of marker s of connective tissue turnover (procollagen peptides, laminin P1) than did those with normal Doppler sonography measurements. The rate of survival wi thout transplantation during the following 2 years was significantly lower in children with abnormal Doppler findings. From portal and hepatic vein fl ow measurements, patient survival 2 years later could be predicted with an accuracy of 93%. Conclusions: In children with extrahepatic biliary atresia, Doppler sonogra phy of the hepatic blood flow is a noninvasive indicator of disease severit y, Moreover, it allows a highly accurate prediction of patient survival for the following 2 years.