Glomerulocystic kidney disease

Case contributed by Yaïr Glick
Diagnosis almost certain

Presentation

Right upper abdominal pain.

Patient Data

Age: 65 years
Gender: Female

CT abdomen after intake of oral contrast, without injected contrast due to renal failure.

Retained contrast material in the esophagus. Substantial focal wall thickening in the distal esophagus.
Status post Roux-en-Y gastric bypass.
Status post cholecystectomy.
Large diverticulum in 2nd duodenal segment.
Innumerable small cortical renal cysts envelop the kidneys, seen more clearly around the right kidney. Most cysts are subcentimeter in diameter. Several high-density cysts in the right kidney.
Lumbar scoliosis convex to the right.

Non-contrast MRI done 3.5 years later - suspected "complex renal cyst".

Only non-contrast scans done due to renal failure.

Normal-sized kidneys without hydronephrosis. Each kidney contains innumerable small subcapsular cortical cysts surrounding the periphery, some filled with or containing a sediment of proteinaceous material.

Hemangiomas in several vertebrae.

Case Discussion

Features pathognomonic of glomerulocystic kidney disease.

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