CAROLIS-DISEASE - 1977-1995 EXPERIENCES

Objective Until recently, congenital cystic dilatation of the intrahep atic biliary ducts (Caroli's disease) has been recognized infrequently . This report aimed to analyse and discuss our observations on 21 adul t patients with the disease. Patients From 1977 to 1995, 21 patients ( eight women and 13 men), aged 17 to 68 years, were diagnosed and treat ed 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 bi liary lesions was bilobar in 12 patients and monolobar in nine. Two of them were congenital hepatic fibrosis. Twenty-one patients had coexis ting hepatobiliary disease, associated with Caroli's disease. Approxim ately 95% of our patients had cystolithiasis, cholelithiasis or both. Surgical treatment was used in 18 patients, a partial hepatectomy bein g carried out in seven of them, an internal biliary drainage by choled ocystojejunostomy in five cases, and by choledochotomy in four cases. Three cases with Caroli's disease were treated with endoscopic sphinct erotomy and stone extraction. In the follow-up period, four of our pat ients died. Conclusion Caroli's disease is being diagnosed more freque ntly as a result of improved diagnostic capabilities. The aim of the t reatment is to obtain sufficient biliary drainage and to relieve the s ymptoms. (C) 1998 Rapid Science Ltd.