Rare, autosomal recessive disease. Patients often present aged 10-30 years with recurrent cholangitis. Predisposes to infection, inflammatio...
Rare, autosomal recessive disease. Patients often present aged 10-30 years with recurrent cholangitis.
Predisposes to infection, inflammation, stones and cholangiocarcinoma.
Associated with medullary sponge kidney and renal cysts.
• Multiple cystic structures converging towards the porta hepatis and communicating with the bile ducts
• No strictures
CT
• Multiple cystic structures with a central enhancing ‘dot’, representing the portal vein radicles surrounded by dilated ducts—the central dot sign.
• The central dot sign and communication with the bile ducts help distinguish Caroli disease from multiple hepatic cysts.
MRCP
• Ectatic intrahepatic ducts extending to the periphery
• Dilated common bile duct
• No strictures
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