Idiopathic Retinitis, Vasculitis, Aneurysms, and Neuroretinitis (IRVAN). New Observations and a Proposed Staging System

Purpose To review the clinical features, disease progression, and effects of treatment on idiopathic retinitis, vasculitis, aneurysms, and neuroretinitis (IRVAN). Design Retrospective interventional case series. Participants Ten patients with IRVAN originally reported in 1995 and 12 additional patients identified since the original series. Intervention Patients in the series had testing that may have included fluorescein angiography, indocyanine green angiography, and systemic evaluation. Treatments included panretinal laser photocoagulation, cryotherapy, vitrectomy surgery, and injection of periocular or intravitreal steroids. Main Outcome Measures Initial visual acuity (VA), initial stage at diagnosis, clinical course, surgical intervention, final VA, and complications of disease. Results A total of 44 eyes of 22 patients were studied; 9 eyes had reached stage 1 or 2 disease at last follow-up, 17 had reached stage 3, and 12 had reached stage 4 or 5. At the time of last follow-up, 14 eyes had maintained 20/20 vision, 15 had between 20/40 and 20/200 vision, and 9 had 20/300 vision or worse. Later stages of retinal ischemia are associated with worse VA. Thirty-two of 38 followed eyes were treated. Twenty-five were treated initially with panretinal laser photocoagulation. The clinical course of each eye after initiation of panretinal laser photocoagulation was evaluated with respect to the final VA and stage of ischemic retinopathy at the initiation of treatment. Panretinal laser photocoagulation was initiated in 3 eyes at stage 2, 16 at stage 3, 5 at stage 4, and 1 at stage 5. Seven eyes underwent grid laser retinal photocoagulation of the macula for macular edema. Conclusions Idiopathic retinitis, vasculitis, aneurysms, and neuroretinitis is an isolated retinal vascular disease that can progress rapidly to severe vision loss due to ischemic sequelae despite treatment with panretinal laser photocoagulation. Based on our review of the largest cohort of IRVAN patients, early panretinal laser photocoagulation should be considered when angiographic evidence of widespread retinal nonperfusion is present, and before (or shortly after) the development of neovascularization. A functional staging system is proposed to improve treatment paradigms.