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Évolution sur deux ans d'un syndrome d'IRVAN: à propos d'un cas

Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome is a rare clinical entity, that requires urgent management and long-term follow-up. We aimed through a case report to describe the clinical course of this syndrome.

A 40-year-old female with no medical history presented for bilateral blurred vision. The best corrected visual acuity was 8/10 in both eyes. Biomicroscopy showed no rubeosis iridis, a mild vitritis in both eyes and fundus examination revealed blurred disc margins with papillary aneurysm, perivascular and peripapillary hard exudates, arterial sheathing with large areas of capillary dropout and retinal hemorrhages. Fluorescein angiography showed early staining of the right optic disc increasing in the late frames suggestive of neovascularization, staining of the left optic disc with a punctate hyperfluorescence of the temporo-superior artery corresponding to an aneurysm and large areas of capillary non-perfusion and staining of peripheral vessels bilaterally. Optical coherence tomography (OCT) of the macula was normal in both eyes. OCT-Angiography confirmed the pre-papillary neovascularization in the right eye, showed a papillary aneurysm in the left eye with areas of capillary non-perfusion in both eyes. Our patient underwent a course of oral corticosteroids with panretinal laser photocoagulation (PRP) in both eyes and intravitreal injection of bevacizumab in the right eye.

At one-month follow-up, visual acuity remained stable, vitritis and neovascularization disappeared. At 6 months follow-up, we noticed a decreasing of visual acuity to 4/10 in both eyes. Biomicroscopic examination showed recurrence of vitritis with increasing of hard exudates which reached the macular area. We performed intravitreal injections of bevacizumab in both eyes and prescribed a second course of oral corticosteroids. At 8 months follow-up, visual acuity increased to 6/10 with disappearance of vitritis and decreasing of exudates. The patient remained stable at 24 months follow-up.

IRVAN syndrome is a chronic disease that typically affects asymptomatic young women.  It includes three major criteria: retinal vasculitis, aneurysmal dilations at arterial bifurcations, and neuroretinitis. The minor criteria are peripheral capillary nonperfusion, retinal neovascularization, and macular exudation. It Management options include PRP, intra-vitreous injections of anti-VEGF and the use of oral corticosteroids which remains controversial.

Management of IRVAN remains challenging for ophthalmologist. Its prognosis depends on early detection of neovascular complications and macular exudation. A long and close follow up is required.