Abstract

Caroli disease is a cystic congenital malformation of the intrahepatic biliary tract, frequently associated with lithiasis. Commonly, cystic dilatations are bilateral and infrequently they affect only one hepatic lobule or segment. We report six patients with localized Caroli disease, three in the right and three in the left hepatic lobule, that were subjected to a hepatic resection. There were no postoperative complications or mortality. After 28 months of follow up, patients are asymptomatic and with normal hepatic function and ultrasonography. The importance of diagnosing localized Caroli disease, in which hepatic resection can be curative, is emphasized.