Objective: To retrospectively investigate the diagnosis and the outcome of Caroli's disease treated by surgical procedures.
Methods: The clinical data of 68 patients with Caroli's disease treated by surgical procedures between 1996 and 2002 were reviewed, retrospectively.
Results: The patients, with a M/F ratio of 1:1.35 and a mean age of 46, presented mainly with recurrent cholangitis. Of all the patients, 26 had a history of operation for cholelithiasis or cholangitis. On admission, the image investigations suggested that the lesions located at left lobe in 44 patients, right lobe in 9 patients, and whole liver in 15 patients. The coexisting cyst in common bile duct was found in 20 patients. The malignant transformation was found in 5 patients (8.8%). Hepatectomy was performed in 82.4% of patients, with a morbidity rate of 15.0% and mortality rate of 0 after the surgery. The long-term outcome of symptom-free in hepatectomy group was 90.2%, significantly higher than the 33.3% in non-hepatectomy group (P < 0.01) after a 3 to 10 years of follow-up.
Conclusions: Hepatectomy offers a curative procedure for local Caroli's disease, and liver transplantation is a good option for diffuse sufferers.