Vascular Dysplasia in a Child with Tuberous Sclerosis

Abstract

Tuberous sclerosis is characterized by the development of hamartomas in many organs, particularly the brain, skin, heart, and kidney. We report a child with typical features of this disease who also had a severe generalized vascular dysplasia of both muscular arteries and veins and an abdominal aortic aneurysm in association with obliterative dysplasia of the aortic vasa vasorum. This is the first report of such a widespread vascular abnormality in association with tuberous sclerosis. This case along with several reports of more isolated vascular abnormalities in these patients indicates that vascular dysplasia represents another manifestation of the generalized tendency to hamartoma formation seen in this disorder.

Key Words:

• tuberous sclerosis
• vascular dysplasia
• aortic aneurysm
• hamartoma