Introduction

Idiopathic retinal vasculitis, aneurysms and neuro- retinitis (IRVAN) syndrome is a rare clinical entity of unknown etiology with vision threatening potentials.

Case presentation

In this article, we present a case of a 38-year-old woman complaining of decreased visual acuity OU (oculi utriusque) for a duration of 3 months. On admission, the best-corrected visual acuity (BCVA) was 5/50 in the right eye (RE) and 2/50 in the left eye (LE). Mild rubeosis iridis in the LE was noted. Posterior segment examination revealed hemorrhage floaters in the vitreous chamber, macular edema, hemorrhages and aneurysms in OU. Fundus fluorescein angiography (FFA) showed the presence of numerous microaneurysms, enhancement of vascular contrast, leakage on the optic nerve disc, as well as extensive areas of non-perfusion in the middle peripheral retina in OU. Based on constellation of clinical and angiographic features as well as the negative extensive workup, a diagnosis of IRVAN was established. Anti-inflammatory, immunosuppressive and hemostatic treatments were applied, and panretinal photocoagulation was performed in OU. In the next stage, due to visual impairment, persistent vascular leak, macular edema in OU, anti-vascular endothelial growth factor (VEGF) intravitreal injections were given. As a result, BCVA increased to 5/12 in the RE and 5/50 in the LE. The patient remains under regular follow-up.

Conclusions

Late diagnosis and lack of appropriate treatment of IRVAN syndrome may lead to complete loss of vision due to complications secondary to lack of perfusion. It is important for ophthalmologists to improve understanding of this disease and manage the symptoms as soon as possible.