Fibromuscular dysplasia: A rare cause of cilioretinal artery occlusion in childhood

Abstract Objective To report a case of cilioretinal artery occlusion with angiographic findings characteristic of the "string of beads" associated with renovascular hypertension secondary to fibromuscular dysplasia of the renal artery in a child. Design Case report. Intervention The patient underwent ex vivo renal artery reconstruction with saphenous vein graft and reimplantation. Main outcome measures The main clinical outcomes were control of severe hypertension, reversible hypertensive retinopathy, and improvement of vision. Results Revascularization of the kidney improved renal function, and renovascular hypertension was clearly improved. Visual acuity improved to 20/200. Conclusions A child with hypertensive retinopathy and arterial occlusion in the retina should undergo investigation to rule out a surgically curable hypertension. Magnetic resonance angiography of extrarenal vessels may reveal other sites of involvement of fibromuscular dysplasia. Evaluation and early diagnosis of renovascular hypertension will prevent severe end-organ damage.