Features
- hypertension
- recurrent UTIs
- abdominal pain
- renal stones
- haematuria
- chronic kidney disease
Extra-renal manifestations
- liver cysts (70%)
- berry aneurysms (8%)
- cardiovascular system: mitral valve prolapse, mitral/tricuspid incompetence, aortic root dilation, aortic dissection
- cysts in other organs: pancreas, spleen; very rarely: thyroid, oesophagus, ovary
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Extensive cysts are seen in an enlarged kidney
CT of the abdomen demonstrates both kidneys to be markedly enlarged by innumerable cysts ranging in size from a few millimeters to multiple centimeters with cysts also present in the liver
CT showing multiple cysts of varying sizes in the liver, and bilateral kidneys with little remaining normal renal parenchyma.
