DOI: 10.36347/sjmcr.2022.v10i05.016

Pages: 472-475

Caroli’s disease is a rare congenital hepatobiliary disease characterized by multifocal segmental dilatation of intrahepatic bile ducts affecting all or parts of the liver. Which predisposes to biliary stasis and consequent lithiasis, cholangitis, abscesses, and septicemia. Most often diffuse; it is much rarely localized to one lobe of the liver, mainly on the left. We report three cases of monolobar Caroli's disease revealed by cholestatic jaundice in 2 cases and recurrent cholangitis in 1 case. All patients were men with an average age of 41 years old. The average duration between first symptoms and diagnosis was 3 months. In all cases the diagnosis was suggested by radiology and confirmed by histology. The Caroli's disease was located on the left liver lobe with the presence of intrahepatic lithiasis in 2 cases. Regarding treatment, it consisted of a left hepatectomy in 2 cases and a biliary-digestive diversion in 1 case. The outcome was favorable in two cases while the third case was lost.