Abstract
Background: Caroli’s disease is a rare entity of congenital bile duct disease with cystic or fusiform dilated intrahepatic bile ducts with consecutive cholestasis and recurrent episodes of bacterial induced cholangitis finally leading to liver cirrhosis and potentially CCC.Therapeutic options are based on the treatment of cholestasis finally leading to liver resection or transplantation. Methods: A female pat. was referred to our department with recurrent abdominal pain with cramp-like character. Family history showed frequent occurrence of PSC. Ultrasound and MRI screening showed inhomogeneous structures in the central bile ducts of S II and III with additional choledocholithiasis. Slight elevation of the lab parameters was observed.Additional SpyGlas ERCP revealed a diffuse choledocholithiasis of the sub segmental bile duct S II and III as wellwith completely inconspicuous right-sided bile duct system. Results: We performed a left hemihepatectomy,as PSC was evident within the family. The histological examination revealed an incidental Caroli’s syndrom in the left lateral liver segments with absence of recurrent cholangitis, history of chronic inflammatory bowl disease and intra hepatic bile duct dilation. Conclusion: In conclusion we present an unusual case of left lateral Caroli’s syndrom. Nevertheless recurrence of cholangitis and intrahepatic choledocholithiais as well as the significant elevated risk for a CCC contribute to the curative decision of left sided hemihepatectomy.
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