Multiple intracranial and systemic aneurysms associated with infantile-onset arterial fibromuscular dysplasia

Fibromuscular dysplasia (FMD) is usually an adult onset, segmental, nonarteriosclerotic, and noninflammatory vasculopathy involving predominantly systemic medium and small arteries. The renal and extracranial portion of the internal carotid arteries are most commonly affected, followed by visceral and other systemic arteries. According to collective experience, renovascular disease occurs in 60 to 75% of patients with FMD, cerebrovascular disease in 25 to 30%, and multivessel involvement in up to 24%.1 We report a patient with infantile-onset, biopsy-proven renal and mesenteric FMD, who at age 23 years presented with sudden blindness caused by ophthalmic artery occlusion from FMD and visceral hemorrhages caused by ruptured FMD aneurysms. Case report. Current episode. The patient, a 23-year-old right-handed man with hypertension, was hospitalized for sudden blindness and retroorbital pressure of the right eye, both preceded 3 weeks earlier by tunnel vision of the right eye. On admission, pulse rate was 76 beats per minute and blood pressure 111/87 mm Hg. Multiple, small pulsating protrusions of the scalp were evident. Neurologic examination revealed poor light perception in the right eye, a right afferent pupillary defect, and pallor of the right fundus with swollen optic disc and collapsed peripheral arterioles. Results of funduscopic examination of the left eye were normal. Treatment with acetazolamide, oxygen, and timolol maleate eye drops improved light perception. Admission laboratory …