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Case Reports
. 2015 Nov 17;27(1-2):63-6.
doi: 10.1016/j.joco.2015.10.006. eCollection 2015 Mar-Jun.

Unilateral idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome (IRVAN) in a young female

Affiliations
Case Reports

Unilateral idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome (IRVAN) in a young female

MirNaghi Moosavi et al. J Curr Ophthalmol. .

Abstract

Purpose: To report a case of Idiopathic retinal vasculitis, arteriolar macroaneurysms, and neuroretinitis (IRVAN). syndrome in a young female.

Case report: A 21-year-old woman presented with unilateral visual acuity (VA) loss. Ophthalmological examination disclosed unilateral optic disc swelling, star-shaped macular exudation, multiple aneurysms surrounded by perivascular exudation, retinal vasculitis, and mild vitreous reaction. The left eye examination was entirely normal. Clinical and paraclinical findings were compatible with IRVAN syndrome criteria. The patient was treated with a short course of oral steroid, a trans-septal triamcinolone acetonide injection, selective laser photocoagulation in peripheral non-perfusion areas, and intravitreal bevacizumab. In spite of primary good response, after each treatment cessation, VA dropped with increasing central macular thickness (CMT), but response to intravitreal triamcinolone (4 mg/0.1 cc) was permanent and good.

Conclusion: IRVAN syndrome can have unilateral presentation. All treatment options were temporary except intravitreal steroid injection in our case. The effect of steroid in IRVAN will need further evaluation.

Keywords: IRVAN retina; Idiopathic reinal vasculitis, aneurysms, and neuroretinitis syndrome; Unilateral.

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Figures

Fig. 1
Fig. 1
Wide field FAG of the right eye shows hyperfluorescence of the vessel walls in the late phases and macroaneurysm along the arterioles with late staining and significant capillary non-perfusion area with neovascular sheeting nearby in the right eye.
Fig. 2
Fig. 2
Macular OCT of left eye shows increased retinal thickening at the posterior pole, with schisis cavities and cystoid spaces and hyper-reflective excrescences in the outer retina with decay of the outer components.

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