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Bilateral, Multiple, Episodic Retinal Vein Occlusions Associated With Common Variable Immunodeficiency

Ian C. Han, MD1; Lawrence J. Singerman, MD2; Alvin Schmaier, MD3; Neil M. Bressler, MD1,4
[+] Author Affiliations
1Retina Division, Wilmer Eye Institute, Johns Hopkins School of Medicine, Baltimore, Maryland
2Retina Associates of Cleveland, Cleveland, Ohio
3Case Western Reserve University, University Hospitals Case Medical Center, Cleveland, Ohio
4Editor, JAMA Ophthalmology
JAMA Ophthalmol. 2015;133(10):1216-1218. doi:10.1001/jamaophthalmol.2015.2414.
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This case report describes a patient with common variable immunodeficiency who developed bilateral, multiple, episodic retinal vein occlusions.

Common variable immunodeficiency (CVID) is a heterogeneous family of primary immunodeficiencies characterized by reduced serum levels of immunoglobulins with reduced immunity.1 Reported ocular involvement with CVID includes retinal vasculitis,2 placoid choroidopathy,3 and retinal pigmentary alterations.4 In one report, 3 patients with CVID had retinal vasculitis and macular edema.2 Autoimmune disorders are found in 20% of these patients,5 and an association with uveitis has been suggested.6 We describe a patient with CVID who developed bilateral, multiple, episodic retinal vein occlusions, with peripheral retinal neovascularization at the boundary of areas of retinal capillary nonperfusion treated with scatter photocoagulation, and macular edema treated with bevacizumab.

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Figure 1.
Fundus Photographs and Fluorescein Angiographic (FA) Frames on Presentation

A and D, Fundus photographs show venous tortuosity, greater in the left eye (A) than in the right eye (D). B, C, E, and F, Fluorescein angiography demonstrates delayed venous filling (B) with no leakage in the midframe (E) or the late frame (C and F). Abnormal hyperfluorescence is seen within a venous branch (arrowhead in C) and larger vein (arrowheads in F).

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Figure 2.
Fluorescein Angiographic Images Depicting Multiple Retinal Venous Occlusive Episodes in the Right Eye

A, Superotemporal branch vein occlusion (black arrowhead) and delayed filling at venous bifurcation (white arrowheads), 5 months after initial presentation. B, Hemorrhages resolved, 13 months after initial presentation. C, Exacerbation with new nonperfusion temporally, 17 months after initial presentation. D, Hemorrhages resolved, 37 months after initial presentation. E, New inferotemporal vein occlusion, 42 months after initial presentation. F, Improvement, 45 months after initial presentation.

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