Central retinal artery occlusion as initial presentation of Moyamoya disease in a middle-aged woman

Alekya P. Rajanala, Hong Gam T. Le, Manjot K. Gill*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

5 Scopus citations


Purpose: To present a case of central retinal artery occlusion as the first symptomatic manifestation of Moyamoya disease in a middle-aged patient. Observations: Case report of a 48-year-old female Chinese-American patient who presented with sudden onset painless unilateral vision loss. Fundus photos, optical coherence tomography, fluorescein angiography, magnetic resonance angiography, computed tomography angiography, and catheter cerebral angiogram were performed. The patient's dilated fundus examination showed classic findings of a central retinal artery occlusion. Diagnostic brain imaging demonstrated extensive stenosis of the cerebrovascular network, with almost complete unilateral occlusion of the internal carotid artery along with compensatory collateral vessels. This led to a new diagnosis of Moyamoya disease. The patient was treated with extracranial-intracranial bypass surgery. Conclusions and importance: Arterial abnormalities in patients with Moyamoya disease are uncommon and have previously only been reported in younger patients in their teens and 20s. Young and middle-aged patients presenting with central retinal artery occlusions should undergo complete neurologic workup including stroke evaluation; in this case, revealing Moyamoya disease, a rare yet life-threatening condition, as the underlying etiology.

Original languageEnglish (US)
Article number100705
JournalAmerican Journal of Ophthalmology Case Reports
StatePublished - Jun 2020


  • Central retinal artery occlusion
  • Cerebrovascular disease
  • Moyamoya disease
  • Optical coherence tomography
  • Retina
  • Vascular occlusion

ASJC Scopus subject areas

  • Ophthalmology


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