Idiopathic retinal vasculitis, arteriolar macroaneurysms and neuroretinitis ( IRVAN ): case series of three patients with multimodal imaging

Purpose To describe the clinical presentation, disease progression, treatment and complications of IRVAN . Methods Retrospective review of three patients with a diagnosis of IRVAN seen at the Birmingham and Midland Eye Centre between 2010 and 2017. Multimodal imaging investigations included wide‐field fluorescein angiography, optical coherence tomography, and indocyanine green angiography. Results A total of 6 eyes from 3 patients with bilateral disease were included. All six eyes were treated with panretinal photocoagulation ( PRP ). One eye received a dexamethasone implant for refractory macular oedema and exudation. One eye was treated with Avastin injections for persistent optic disc neovascularization. All 3 patients were treated with oral steroids at some point in the course of their disease and one was subsequently immunosuppressed with mycophenolate mofetil. Visual outcomes varied. One patient maintained excellent visual acuity of 6/6 in both eyes at 72‐months follow‐up. The second patient experienced progressive visual loss from 6/9 right eye, 6/6 left eye at presentation; to 6/18 right eye, 6/60 left eye at 12 months follow‐up due to macular exudation despite aggressive PRP and intravitreal Ozurdex. The third patient deteriorated from 6/5 right eye, 6/4 left eye at presentation to 6/60 right eye at 6 months, despite aggressive PRP . He had recurrent vitreous haemorrhages in the right eye secondary to NVD despite further interventions with Avastin injections; seven years after presentation, vision remained at 6/60 right eye and 6/18 left eye. Conclusions The cadence of progression of IRVAN can vary greatly, despite aggressive treatment with PRP , intravitreal steroids, and anti‐ VEGF agents. We observed variation in disease progression both among patients as well as within the same individual, supporting an individualized approach to therapy.