Idiopathic Retinitis, Vasculitis, Aneurysms, and Neuroretinitis (IRVAN): Early Treatment Saves Sight

We report a rare case of bilateral Idiopathic Retinitis, Vasculitis, Aneurysms, and Neuroretinitis (IRVAN) with occlusive vasculitis. A 28-year-old female presented with sudden decreased vision in her left eye for three days. Visual acuity in the right eye was 6/6, whereas it was 6/9 in the left eye. The anterior segment was examined and found to be normal. A fundus examination of the right eye showed an arteriolar aneurysm on the optic disc, vascular sheathing, and generalized retinal pigment epithelial atrophy. The left eye was in worse condition, with a swollen optic disc, disc hemorrhage, multiple arteriolar aneurysms, hard exudates at the peripapillary and macular region, peripheral vasculitis, neovascularization, and vitreous hemorrhage. Optical coherence tomography revealed mild cystoid macula edema (CME) in both eyes. Fluorescein angiography of both eyes demonstrated arteriolar aneurysms, vascular leakage, and peripheral ischemia. There was additional leakage from new vessels and masking secondary to vitreous hemorrhage in the left eye. The results of the systemic evaluation and extensive laboratory testing were negative. She had bilateral retinal photocoagulation and was administered oral prednisolone later with slow tapering due to increasing CME. Her eye condition did not worsen, and she maintained good vision in both eyes. IRVAN, even though rare, should be suspected in patients with occlusive vasculitis, arteriolar aneurysm, and macula exudation. Since the nature of the disease is more aggressive than other ischemic retinopathies, early detection, intervention, and close follow-up are crucial to prevent rapid visual loss.