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

Ophthalmology. 2000 Apr;107(4):737-41. doi: 10.1016/s0161-6420(99)00147-5.

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.

Publication types

  • Case Reports

MeSH terms

  • Blood Pressure
  • Child
  • Ciliary Arteries*
  • Female
  • Fibromuscular Dysplasia / complications*
  • Fibromuscular Dysplasia / diagnosis
  • Fibromuscular Dysplasia / surgery
  • Fluorescein Angiography
  • Humans
  • Hypertension, Renovascular / diagnosis
  • Hypertension, Renovascular / etiology*
  • Intraocular Pressure
  • Magnetic Resonance Angiography
  • Renal Artery Obstruction / complications*
  • Renal Artery Obstruction / diagnosis
  • Renal Artery Obstruction / surgery
  • Retinal Artery Occlusion / diagnosis
  • Retinal Artery Occlusion / etiology*
  • Visual Acuity