Caroli disease with medullary sponge kidney and nephrocalcinosis

Discussion:

MRI reveals intrahepatic and extrahepatic cystic dilatations of the biliary tract with multiple large calculi. Incidental detection of medullary sponge kidney. Renal USG performed subsequently also revealed nephrocalcinosis.

Isolated case reports of association of Caroli disease with renal cystic disease are scattered in the English literature. There is also an association of oro-facial-digital syndrome with cystic disease of the kidneys and the biliary tree.

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