Fibromuscular Dysplasia

• a heterogenous group of disorders defined pathologically by their site of involvement in the arterial wall (intima, media, adventitial)
• it may be a primary condition (can be autosomal dominant) or secondary to such diseases as NF-1 or cutis laxa
• most commonly seen in 20-30 y/o women; ~ accounts for 75-90 of renovascular disease in children
• renal arteries are involved most frequently, though aorta, celiac and carotid arteries also may be affected
• classic angiographic appearance is beads on a string
• ultimately need biopsy to absolutely prove dx (although this may not be necessary)

Pediatric Annals 34(1) Jan 2005