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Caroli's disease with medullary sponge kidney & nephrocalcinosis

Case contributed by: Dr Paresh K Desai

Presentation:

Obstructive jaundice

Patient Data:

Age: 35
Gender: Male
From the case: Caroli's disease with medullary sponge kidney & nephrocalcinosis
Modality: MRI

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  • <p>MRCP</p>

T2

MRCP

From the case: Caroli's disease with medullary sponge kidney & nephrocalcinosis

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  • <p>MRCP</p>

MRCP

Case Discussion:

MR reveals - intrahepatic and extrhepatic cystic dilatations of billiary tract with multiple large calculi.

Incidental detection of Medullary sponge kidney. (There are no reported cases of Medullary sponge kidney on MRI)

Isolated case reports of association of Caroli's 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

Renal USG perfomed subsequently also revealed nephrocalcinosis. 

References:

  1. 1. Mrowka C, Adam G, Sieberth HG et-al. Caroli's syndrome associated with medullary sponge kidney and nephrocalcinosis. Nephrol. Dial. Transplant. 1996;11 (6): 1142-5. Nephrol. Dial. Transplant. (citation) - Pubmed citation
  2. 2. Toprak O, Uzum A, Cirit M et-al. Oral-facial-digital syndrome type 1, Caroli's disease and cystic renal disease. Nephrol. Dial. Transplant. 2006;21 (6): 1705-9. doi:10.1093/ndt/gfk013 - Pubmed citation

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