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Peripheral Retinal Ischemia, Neovascularization, and Choroidal Infarction in Wyburn-Mason Syndrome

Prethy Rao, MD, MPH1; Benjamin J. Thomas, MD1,2; Yoshihiro Yonekawa, MD1,2; Joshua Robinson, MD3; Antonio Capone Jr, MD1,2
[+] Author Affiliations
1Department of Ophthalmology, Beaumont Eye Institute, Royal Oak, Michigan
2Associated Retinal Consultants, Royal Oak, Michigan
3Emory Eye Center, Atlanta, Georgia
JAMA Ophthalmol. 2015;133(7):852-854. doi:10.1001/jamaophthalmol.2015.0716.
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Wyburn-Mason syndrome is a unilateral, sporadic disorder characterized by arteriovenous malformations (AVMs) of the retina, face, and central nervous system. Clinically, these lesions often manifest as dilated, tortuous retinal vessels.1 We report a complication of Wyburn-Mason syndrome, peripheral retinal neovascularization, that has not yet been described to our knowledge. Additionally, we present an iatrogenic consequence consisting of extensive retinal and choroidal ischemia after intra-arterial embolization therapy for posterior intracranial AVM extension.

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Figure 1.
Findings on Fluorescein Angiography

A, Right eye with an optic nerve arteriovenous malformation, retinal ischemia, and sclerotic vessels (arrowhead). B, Late phase with capillary dropout (yellow arrowheads) and leakage (red arrowhead). C, Regression of retinal neovascularization after sectoral laser photocoagulation. D, Late phase without leakage.

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Figure 2.
Fundus Photograph and Ultra–Wide-Field Angiographic Findings

A, Fundus photograph of the right eye demonstrates regression of the arteriovenous malformation and diffuse sclerosis of the retinal vasculature after treatment with intra-arterial embolization. B, Late-phase ultra–wide-field angiography shows near-complete retinal and choroidal infarction.

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