Long term follow-up after surgical treatment of intrahepatic biliary cysts (Types V - Caroli's Disease) in Argentina

Abstract

Background: Caroli's Disease (CD) is a rare congenital disorder characterized by cystic dilatation of the intrahepatic bile ducts. Objetive: Analyze the results of surgical treatment and long term outcome of CD in a multicenter study. Methods: Between 1991 and 2017, 55 patients with CD were operated on at 13 HPB centers. Diagnosis was performed by US, CT, ERCP and MRI. In all patients the diagnosis of CD was confirmed by histology. Demographic, clinical, surgical and pathological variables were analyzed. Results: 32 patients were female, average age was 49,22 years. 85,5% symptomatic. Chronic cholangitis was the main presented symptom (38,2%). The left hemiliver was involved in 67,3%. Mean interval time from the onset of symptoms was 527,1 days. Surgical procedures performed were: A) Hemiliver disease: left lateral sectionectomy 19, left hepatectomy (LH) 18 and right hepatectomy (RH) 6 B) Bilateral disease: RH 1, hepaticojejunostomy 5, LH 3 and liver transplant 3 . Vascular control was by ischemic preconditioning 5 (21,7%) and Pringle maneuver 18 (78,3%). Blood transfusion was required in 16,4%. Average hospitalization was 10,4 days. Morbidity was 32,7% (Dindo-Clavien type 1=4; 2=4; 3a=9 and 3b=1). Mortality was 0 %. A median follow-up of 66,6 months. Cholangiocarcinoma was present in two patients (3,6%). Conclusions: CD in Argentina is more common in females with left hemiliver involment. Surgical resection is the best curative option in unilateral disease with long term survival free of symptoms and complications. In cases of bilateral disease hepaticojejunostomy could be an optional treatment prior to liver transplantation.