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