Renal Artery Fibromuscular Dysplasia

Pediatric renal artery fibromuscular dysplasia (FMD) is a systemic noninflammatory, non-atherosclerotic vascular disease, presenting primarily in young children with hypertension. The renal, mesenteric, extracranial carotid, intracranial carotid, and aorta are the most common sites of lesions. Common radiologic presentations include focal segmental stenosis and a string of beads appearance, although less common presentations such as dissection, tortuosity, and aneurysm should raise suspicion of renal artery FMD. Due to its vague clinical and radiologic presentation, diagnosis of renal artery FMD in children is often delayed and thus should be considered in the differential diagnosis of renovascular hypertension.