Caroli's disease: role of endoscopic retrograde cholangiopancreatography

Several modalities have been used in the diagnosis of congenital dilatation of the intrahepatic bile ducts (Caroli's disease) with varying degrees of reliability and morbidity. We report two patients with Caroli's disease where endoscopic retrograde cholangiopancreatography was used to define the anatomy of the extra and intrahepatic biliary trees. In one of the patients (BT) esophageal varices were easily visualized on endoscopy which had been previously demonstrated by a more invasive procedure (splenoportography). There were no complications of retrograde cholangiopancreatography in these two patients. We believe that retrograde cholangiopancreatography is a safe and effective method of evaluating the biliary tree and status of portal hypertension in patients with Caroli's disease as well as providing a reliable means of following the progression of their disease.