Images in Clinical Medicine

Tuberous Sclerosis

Alan D. Salama, M.D., and Charles D. Pusey, M.D.

N Engl J Med 1998; 338:1886June 25, 1998

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Figure 1 A 16-year-old girl presented with right-sided loin pain, anemia, and severe hypotension. Ultrasonographic scanning revealed multiple highly reflective lesions of various sizes consistent with the presence of angiomyolipomas (arrows in Panel A) in the left kidney and a large hematoma in the right kidney. Magnetic resonance imaging demonstrated multiple bilateral lesions with high signal intensity in the renal cortex, consistent with fat in appearance (solid arrows in Panel B). In the right hilum there was a space-occupying lesion consisting of fat and vessels, with a spherical area of mixed signal intensity consistent with the occurrence of a recent hemorrhage from the angiomyolipoma (open arrows in Panel B). A further area with mixed vessels and fat was evident in the right upper pole. Computed tomographic scanning of the brain revealed multiple subependymal tubers that were not enhanced after the addition of contrast medium (Panel C). Screening of other family members disclosed no similar findings. The patient underwent a right nephrectomy. Three years after discharge, she remains well and is returning for follow-up renal ultrasonography every six months.

Alan D. Salama, M.D.
Charles D. Pusey, M.D.
Hammersmith Hospital, London W12 0NN, United Kingdom

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