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Eric Weinlander, Thomas Wubben, Merina Thomas, Katherine Davis, Wayne Cornblath, Mark W Johnson; Cilioretinal Artery Occlusion Associated with Optic Disc Edema. Invest. Ophthalmol. Vis. Sci. 2018;59(9):5444.
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The mechanism of non-arteritic cilioretinal artery occlusion (CLRAO) is controversial. In the setting of central retinal vein occlusion (CRVO), failure of cilioretinal artery autoregulation and choroidal steal are proposed mechanisms of CLRAO/stasis and are driven by increased central retinal venous pressure. Other pathology causing increased retinal venous pressure and/or decreased cilioretinal arterial pressure might therefore predispose to CLRAO. We investigate the clinical features of CLRAO associated with optic disc edema without concomitant CRVO or arteritis.
Retrospective chart review of three patients with CLRAO associated with optic disc edema without concomitant CRVO or arteritis.
Three patients with unilateral CLRAO and optic disc edema, with no evidence of frank CRVO or retinal arteritis, were identified. The patients were otherwise healthy women with a median age of 32 years. Presenting visual acuities were 20/500, 20/1000, and 20/70 in the affected eyes. All patients had optic disc edema accompanied by mild retinal venous dilation/tortuosity and ischemic retinal whitening in the cilioretinal artery distribution. Each patient was diagnosed with atypical optic neuritis based on neuro-ophthalmologic evaluation and/or MRI. In all cases, clinical examination and fluorescein angiography revealed no frank CRVO or evidence of retinal arteritis, and OCT imaging exhibited inner retinal thickening/hyperreflectivity followed by thinning in the cilioretinal artery distribution. No active infection, hypercoagulable state, or systemic inflammatory disease was diagnosed in any of the three cases. Two patients were treated with high-dose corticosteroids with final visual acuities of 20/80 (from 20/1000) at six months, and 20/60 (from 20/70) at seven weeks. One patient was lost to follow up.
CLRAO associated with optic papillitis in the absence of frank CRVO or retinal arteritis is a rare but visually-devastating entity. The findings in our cases suggest that CLRAO may develop when optic disc edema compresses the central retinal vein and cilioretinal artery, resulting in increased retinal venous pressure and possibly reduced cilioretinal artery pressure, which in turn leads to cilioretinal arterial stasis and retinal infarction.
This is an abstract that was submitted for the 2018 ARVO Annual Meeting, held in Honolulu, Hawaii, April 29 - May 3, 2018.
Initial fundus appearance and fluorescein angiography in Case 1 (A, B) and Case 2 (C, D).
OCT-A of Case 3 with capillary dropout (arrowhead) underlying retinal thinning (arrow).
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