[Arterial fibromuscular dysplasia and Bourneville's tuberous sclerosis].
Tuberous sclerosis is an autosomal dominant disease characterized by the development of multiple hamartomas which involve mainly the skin, the brain, the kidneys, and the heart. Vascular involvement is uncommon, with essentially several reports of aortic aneurysm. The present case concerns a two and a half year-old girl with known tuberous sclerosis, who underwent a graft replacement of an abdominal aortic aneurysm revealed by hypertension, associated with a right nephrectomy for multiple renal angiomyolipomas and cysts. Microscopic examination of the aortic aneurysm and the right renal artery showed fibromuscular dysplasia. This case and the rare previous reports emphasize the possible vascular involvement in tuberous sclerosis, and suggest that arterial fibromuscular dysplasia may represent an additional manifestation of the disease.
Paraf
F
F
Service d'Anatomie Pathologique, Hôpital Broussais, Paris.
Bruneval
P
P
D001014:Aortic Aneurysm / Q000150:complications* / Q000473:pathology; D002675:Child, Preschool; D005260:Female; D005352:Fibromuscular Dysplasia / Q000150:complications* / Q000473:pathology; D006801:Humans; D012077:Renal Artery; D014402:Tuberous Sclerosis / Q000150:complications* / Q000473:pathology
Dysplasie fibromusculaire artérielle et sclérose tubéreuse de Bourneville.