KEY FACTS

Terminology

  • Caroli disease : Congenital, multifocal, segmental, saccular dilation of large intrahepatic bile ducts (IHBDs)

    • Type V choledochal cyst (Todani classification)

  • Caroli syndrome : (More common variant): Cystic bile duct dilation plus hepatic fibrosis (± portal hypertension)

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