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Ophthalmic Images
November 17, 2021

Central Retinal Artery Occlusion With 2 Cilioretinal Arteries

Author Affiliations
  • 1Department of Ophthalmology and Visual Sciences, Kellogg Eye Center, University of Michigan, Ann Arbor
  • 2Rogel Cancer Center, University of Michigan, Ann Arbor
  • 3Department of Pathology, University of Michigan, Ann Arbor
  • 4Department of Computational Medicine and Bioinformatics, University of Michigan, Ann Arbor
  • 5A. Alfred Taubman Medical Research Institute, University of Michigan, Ann Arbor
  • 6Section of Ophthalmology, Surgery Service, Veterans Administration Ann Arbor Health System, Ann Arbor, Michigan
JAMA Ophthalmol. 2021;139(11):e214059. doi:10.1001/jamaophthalmol.2021.4059

A patient with cutaneous lupus, hyperlipidemia, and 46–pack-year smoking history presented 5 hours after experiencing left-eye vision loss that spared a small horizontal portion of the visual field, with no other associated systemic or neurologic symptoms. Visual acuity was 20/25 OS eccentrically, and there was a +1 relative afferent pupillary defect. A dilated fundus examination (Figure, A) and fluorescein angiography (Figure, B) demonstrated diffuse retinal whitening and severely delayed arterial transit sparing the fovea and nasal retina, consistent with a central retinal artery occlusion with 2 perfusing cilioretinal arteries, a very rare anatomical variant that appears to have only been reported in single-case reports (ie, prevalence in the population is unknown). No intra-arterial embolus was visualized on examination. A systemic evaluation with computed tomography angiography imaging of the head, neck, chest, abdomen, and pelvis revealed extensive atherosclerotic disease (in carotid, coronary, and aortic arteries), and a transesophageal echocardiogram revealed a mildly dilated ascending aorta but normal cardiac valves and normal ventricular systolic function.

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