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Caroli´s Syndrome

Caroli´s syndrome is a congenital condition, where the intrahepatic (and sometimes the extrahepatic) bile ducts are severely dilatated. These MRI-pictures shows a typical finding in this rare disease.
This disease is also known as "Non-obstructive intrahepatic biliary dilatation". There are five types of biliary cystic dilatation according to the Todani classification. Caroli´s syndrome is the same as type V cystic dilatations of the bile ducts.

The saccular dilatation of the intrahepatic bile ducts are clearly seen in this MRI-scan.

Caroli´s syndrome may have a benign course for a very long time. This patient was completely free of symtoms and had normal liver enzymes and normal liver function. However, recurrent cholangitis will probably occur.

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