The role of surgery in Caroli's disease

Aims: Caroli's disease is a rare congenital disorder, which is characterized by multifocal segmental dilatation of the intrahepatic bile ducts. Whether conservative or surgical strategies should be preferred is still a matter of debate. Aim of this study was to evaluate the role of surgery in the managment of Caroli's disease.

Methods: From April, 1998– 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 7 men and 5 women with a median age of 39 years (range 7 months –70 years). Eight patients had a monolobar, 4 a bilobar liver affection. All patients had a history of recurrent cholangitis, with up to 16 unsuccessful conservative treatment attempts. Nine patients (75%) underwent liver resection and 2 (17%) liver transplantation. Intraoperatively, 3 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 follow up 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 long term results.