Review began 02/11/2022 Review ended 03/07/2022 Published 03/10/2022 © Copyright 2022 Mohd Azmi et al. This is an open access article distributed under the terms of the Creative Commons Attribution License CC- BY 4.0., which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Idiopathic Retinitis, Vasculitis, Aneurysms, and Neuroretinitis (IRVAN): Early Treatment Saves Sight Aini Mohd Azmi , Wan Haslina Wan Abdul Halim , Maizan Yaakub , Rosiah Muda 1. Ophthalmology, Hospital Canselor Tuanku Muhriz UKM (Universiti Kebangsaan Malaysia), Kuala Lumpur, MYS 2. Ophthalmology, Hospital Sultanah Nur Zahirah, Kuala Terengganu, MYS Corresponding author: Aini Mohd Azmi, ainimaz@gmail.com Abstract 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. Categories: Ophthalmology Keywords: oral prednisolone, retinal photocoagulation, macula edema, irvan, occlusive vasculitis, arteriolar aneurysm, neuroretinitis Introduction Idiopathic Retinitis, Vasculitis, Aneurysms, and Neuroretinitis (IRVAN) is a rare but well-recognized clinical entity. It typically affects young, healthy individuals, has a female predominance, and is not associated with systemic abnormalities [1]. The acronym IRVAN highlights the most prominent clinical features of this syndrome [1]. If left untreated, it may lead to severe bilateral visual loss [2-3]. This case highlights the importance of early treatment initiation to preserve good vision in patients with IRVAN. This case report was presented as a poster at the 10th MSO Annual Scientific Meeting in conjunction with 34th Malaysia-Singapore Joint Ophthalmic Congress 2019, Malaysia, March 22-24, 2019. Case Presentation 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 arteriolar aneurysm on the disc (Figure 1), 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 regions (Figure 2), peripheral vasculitis, neovascularization, and vitreous hemorrhage. 1 1 2 2 Open Access Case Report DOI: 10.7759/cureus.23049 How to cite this article Mohd Azmi A, Wan Abdul Halim W, Yaakub M, et al. (March 10, 2022) Idiopathic Retinitis, Vasculitis, Aneurysms, and Neuroretinitis (IRVAN): Early Treatment Saves Sight. Cureus 14(3): e23049. DOI 10.7759/cureus.23049