Autosomal dominant polycystic kidney disease associated with Caroli disease and pancreatic disease

Case contributed by Professor Ali Hekmatnia

Presentation

Presented with abdominal pain and fever and jaundice.

Patient Data

Age: 50 years
Gender: Female

MRI of the abdomen (cronal reformats) demonstrates both kidneys markedly enlarged by multiple renal cysts ranging in size with saccular dilation of the intrahepatic billiary tree. There are also small cysts at pancreatic body.

MRCP demonstrates continuity with the biliary tree.

Case Discussion

Caroli disease is associated with ADPKD and pancreatic cysts. Pancreatic cysts are more common in Von Hippel Lindau disease (VHL).

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