Surgical treatment of Caroli´s disease: analysis of a multicenter study from Argentina

LENDOIRE J; RAFFIN G; GRONDONA J; BRACCO R; RUSSI R; ARDILES V; GONDOLESI G; DEFELITTO J; DE SANTIBAÑES E; IMVENTARZA O

Congreso; 9th Worfld Congress of the International Hepato-Pancreato-Biliary Association; 2010

Background: Caroli?s Disease (CD) is a rare congenitaldisorder characterized by cystic dilatations of the intrahepaticbile ducts.Objective: Analyze the results of the surgical treatmentand outcome of CD in a multicenter study.Methods: Between 1991 and 2009, 24 patients with CDwere operated on at six HPB centers. Diagnosis wasperformed by US, CT, ERCP and MRI. In all patientsthe diagnosis of CD was confirmed by histology.Demographic, clinical, surgical and pathological variableswere analyzed.Results: 17 (70.8%) patients were female, average agewas 48.7 years (20?71). 95.5% symptomatic. Cholangitiswas the main presenting symptom (96%). The lefthemiliver was involved in 75%. Mean interval time fromthe onset of symptoms was 951 days. According to CDextension surgical procedures performed were: A)Hemiliver disease: left lateral sectionectomy 9, left hepatectomy8 and right hepatectomy 4 B) Bilobar disease:right hepatectomy 1 and hepaticojejunostomy 2. VascularControl was by ischemic preconditioning in 3 (13%)and intermittent Pringle maneuver in 7 (30%). Bloodtransfusion was required in 16.7%. Average hospitalizationwas 7 days (4?21) Morbidity was 27.3% (Dindo-Clavien type 1 and 2, type 2 and 1 and type 3A and 1.Mortality: 0%. After a median follow-up of 166 monthsall patients are alive and free of symptoms. In all patientsthe diagnosis of CD was confirmed by histology (Biliaryhamartomas ? Biliary ramifications ? Granulomatousreaction). Congenital hepatic fibrosis was present in twopatients (8.3%) and cholangiocarcinoma in one patient(4.2%).Conclusion: CD in Argentina is more common in femaleswith left hemiliver involvement. Surgical resection is thebest curative option in unilateral disease with long termsurvival free of symptoms and complications. In cases ofbilateral disease hepaticoyeyunostomy could be an optionaltreatment prior to liver transplantation.