The role of therapeutic endoscopy associated with extracorporeal shock-wave lithotripsy and bile acid treatment in the management of Caroli's disease.

Background And Study Aims: Caroli's disease causes relapsing episodes of cholangitis due to the presence of intrahepatic lithiasis. Strategies for cholangitis prevention are still widely debated. Ursodeoxycholic acid, hepaticojejunostomy, partial hepatectomy, or transplantation, have all been proposed as therapeutic options. The aim of this study was to evaluate the role of therapeutic endoscopy, and especially endoscopic sphincterotomy (ES), in the management of Caroli's disease.

Patients And Methods: Between 1983 and 1995, six patients with Caroli's disease (mean age 52, range 17-75) underwent endoscopic retrograde cholangiopancreatography (ERCP) for acute cholangitis. Sphincterotomy was performed if common bile duct stones were present. Extracorporeal shock-wave lithotripsy, (ESWL) or intraductal electrohydraulic lithotripsy (IEL) were performed if necessary.

Results: The mean number of endoscopic sessions per patient was four (range three to seven). Sphincterotomy was performed in five patients and cholangioscopy in three. ESWL was performed twice in each of four patients. A Strecker expandable metal stent was placed in one patient to maintain sphincterotomy patency. In one patient, two sessions of IEL and pulsed laser were carried out. Complete clearance of intrahepatic stones was achieved in four of the six subjects (66.6%) and partial clearance in two patients. No morbidity or mortality was observed. During the follow-up (mean 6.2 years; range: 2.1-16.3), only two patients had acute cholangitis at nine months and three years, respectively, after the endoscopic treatment. Both had residual intrahepatic stones left after the initial endoscopic attempt at clearance.

Conclusion: ERCP is a necessary diagnostic procedure which should always be carried out in patients with Caroli's disease. Our experience shows that ES does not result in an increased incidence of cholangitis and that therapeutic endoscopy allows complete clearance of intrahepatic stones in the majority of patients with unresectable symptomatic Caroli's disease. Nevertheless, the oncological risk in these patients remains unchanged, and they still have an increased risk of cholangiocarcinoma.