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Caroli’s disease: Identification and treatment strategy

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Abstract

Caroli’s disease is a rare congenital disease of the liver characterized by cystic dilation of the intrahepatic bile duct. Classic Caroli’s disease involves malformations of the biliary tract alone, whereas Caroli’s syndrome refers to the presence of associated congenital hepatic fibrosis. Caroli’s disease usually presents during childhood and early adulthood. The clinical features of Caroli’s disease include jaundice, right upper abdominal pain, and fever due to the associated complications of hepatolithiasis or bacterial cholangitis. Endoscopic or percutaneous cholangiography is the traditional method of diagnosis, but magnetic resonance cholangiopancreatography is emerging as the diagnostic modality of choice. The treatment for Caroli’s disease includes supportive care with antibiotics for cholangitis and ursodeoxycholic acid for hepatolithiasis. Surgical resection has been used successfully in patients with monolobar disease. For patients with diffuse involvement, the treatment of choice is orthotopic liver transplantation.

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Authors and Affiliations

  1. Division of Gastroenterology and Hepatology, Medical College of Wisconsin, 9200 W. Wisconsin Avenue, Milwaukee, WI, 53226, USA

    Kia Saeian MD, MSc

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  1. Ashwin N. Ananthakrishnan MD, MPH
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  2. Kia Saeian MD, MSc
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Correspondence to Kia Saeian MD, MSc.

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Ananthakrishnan, A.N., Saeian, K. Caroli’s disease: Identification and treatment strategy. Curr Gastroenterol Rep 9, 151–155 (2007). https://doi.org/10.1007/s11894-007-0010-7

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  • DOI: https://doi.org/10.1007/s11894-007-0010-7

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