Tuberous sclerosis with renal angiomyolipomas

Case contributed by Dr Jake Ball

Presentation

Reduced GCS with abdominal pain.

Patient Data

Age: 60 years
Gender: Male

Multiple cortical tubers and subependymal nodules

Extensive white matter hypodensity.

Left eye prosthesis.

CT Angiogram - Abdominal

ct

The background left and right kidneys are enlarged with heterogenous fat-containing lesions throughout. 

Large hematoma in the right kidney with no evidence of active bleed. Abrupt cut-off of a right renal artery is seen in keeping with prior embolization history. No pseudoaneurysms are noted. 

No retroperitoneal cysts. Remainder of solid viscera are normal with no hepatic AMLs.

Sclerotic bone lesions throughout. 

Case Discussion

This case demonstrates multiple facets of tuberous sclerosis with bilateral angiomyolipomas and sclerotic bony lesions. Interestingly left eye prosthesis in keeping with previous retinal phakoma. 

There has been interim removal of the right kidney due to ongoing hemorrhage, a complication of enlarging renal angiomyolipoma.

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