Caroli's disease: 1977-1995 experiences.

OBJECTIVE: Until recently, congenital cystic dilatation of the intrahepatic biliary ducts (Caroli's disease) has been recognized infrequently. This report aimed to analyse and discuss our observations on 21 adult patients with the disease.

PATIENTS: From 1977 to 1995, 21 patients (eight women and 13 men), aged 17 to 68 years, were diagnosed and treated for Caroli's disease at Yuksek Ihtisas Hospital. The mean duration of the disease was 6.5 years.

RESULTS: The commonest presenting symptom was abdominal pain, a feature in 18 cases. The distribution of the biliary lesions was bilobar in 12 patients and monolobar in nine. Two of them were congenital hepatic fibrosis. Twenty-one patients had coexisting hepatobiliary disease, associated with Caroli's disease. Approximately 95% of our patients had cystolithiasis, cholelithiasis or both. Surgical treatment was used in 18 patients, a partial hepatectomy being carried out in seven of them, an internal biliary drainage by choledocystojejunostomy in five cases, and by choledochotomy in four cases. Three cases with Caroli's disease were treated with endoscopic sphincterotomy and stone extraction. In the follow-up period, four of our patients died.

CONCLUSION: Caroli's disease is being diagnosed more frequently as a result of improved diagnostic capabilities. The aim of the treatment is to obtain sufficient biliary drainage and to relieve the symptoms.