The role of surgery in Caroli's disease

BACKGROUND: Caroli's disease is a rare congenital disorder characterized by multifocal segmental dilation of the intrahepatic bile ducts. Whether conservative or surgical strategies should be preferred is still a matter of debate. The aim of this study was to evaluate the role of surgery in the management of Caroli's disease.

STUDY DESIGN: From April 1998 until August 2005, 12 consecutive patients with Caroli's disease were treated in the Department of General, Visceral, and Transplantation Surgery, University Hospital Essen, Germany. All patients were intended to receive liver resections or liver transplantations.

RESULTS: There were seven men and five women, with a median age of 39 years (range 7 months to 70 years). Eight patients had monolobar and four patients had bilobar liver involvement. All patients had a history of recurrent cholangitis, with up to 16 unsuccessful conservative treatment attempts. Nine patients (75%) underwent liver resection and two (17%) had liver transplantation. Intraoperatively, three patients (25%) were found to have cholangiocarcinoma, of which one was unresectable. There was no mortality and only low morbidity (16%) postoperatively. After a median followup of 31 months, 11 patients are well with no recurrent symptoms.

CONCLUSIONS: Surgery can offer a definite therapy, with an acceptable morbidity and virtually no mortality in localized Caroli's disease. In diffuse disease, the use of extended resections or liver transplantation can provide good longterm results.