Abstract:

Caroli's disease, with congenital, cystic dilatations of the intrahepatic bile ducts, is a rare clinical entity. The patients are frequently misdiagnosed and operated for other associated biliary tract diseases. Three cases of Caroli's disease, a case limited to left lobe and two cases of diffuse from are presented. All of the cases experienced previous biliary tract operations and admitted with recurrent episodes of chills and fever complaints. Computed tomographies of the patients have demonstrated the intrahepatic lesions with pathonomonic signs. As far as the surgical treatments are considered partial hepatectomy was sufficient in the case of limited form, but a two stage operation-first external drainage of cavities and than Roux-en-Y hepaticojejunostomy over the drained cavities was performed for the case of diffuse involvement. In the third case, for the stricture on the hepatic bifurcation, central hepatic resection + Roux-en-Y hepaticojejunostomy was performed. Surgical treatment of the disease directed to extrahepatic biliary tree has been performed with limited success. We suggest that the drainage of pseudocystic intrahepatic bile ducts should be over the cystic dilatations like hepaticojejunostomy. This is the most effective method for the surgical management of Caroli's disease.

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