The role of endoscopic retrograde cholangiopancreatography in cholestatic infants.

Conjugated hyperbilirubinemia in infants is a serious problem, and one that requires further investigation. Early differentiation between extrahepatic and intrahepatic causes of cholestasis is essential for the proper management of cholestasis in infancy. A successful outcome in patients with extrahepatic biliary atresia is significantly influenced by the age at which the Kasai procedure (hepatoportoenterostomy) is performed. However, extensive diagnostic workup may fail to distinguish definitively between intrahepatic and extrahepatic disease, and exploratory laparotomy and intraoperative cholangiography are sometimes necessary to exclude extrahepatic disease. In the last four years, 20 cholestatic infants were referred to our center in whom no definite diagnosis was possible, despite thorough diagnostic evaluation. In an attempt to increase the accuracy of our diagnostic work-up, we performed ERCP during the anesthetic period planned for liver biopsy. We succeeded in performing ERCP in 18 children (90%); in five of them (28%), patent bile ducts were demonstrated, and unnecessary laparotomy could be avoided. In four patients (22.2%), a stricture of the common bile duct was seen, in three combined with a choledochal cyst. In two patients (11.1%), only the common bile duct and gallbladder were opacified. Five of seven patients in whom only the pancreatic duct was visualized had complete occlusion of the extrahepatic biliary ducts at surgery. The other two patients did not undergo surgery because initially no signs of extrahepatic obstruction were found in their biopsy. One of these patients deteriorated and needed a liver transplant one year later. This patient proved to have extrahepatic biliary atresia. No severe complications of ERCP were observed; only sporadically, a slight elevation of pancreatic enzymes was noted. We advocate this procedure in the workup of infants with unexplained conjugated hyperbilirubinemia. Anomalies in the biliary tree can be demonstrated, and when patent bile ducts are found, laparotomy can be avoided.