Idiopathic retinal vasculitis, aneurysms and neuroretinitis case report

PURPOSE: To report a case of idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) in a young woman. OBSERVATIONS: A 21-year-old white female patient was referred to retina clinic with decreased vision in the left eye. On examination, best corrected visual acuity was 20/20 in the right eye and counting fingers in the left eye. Fundus examination revealed bilateral optic disc edema, peripapillary and macular exudates, retinal arterial aneurysms, and venous beading. In the left eye, there was a large focus of exudative material in the central macula. Examination of the peripheral retina was unremarkable, bilaterally. Fluorescein angiography demonstrated arterial aneurysms and venous beading in both eyes, with optic nerve activity and vascular sheathing noted on late frames in both eyes. In the left eye, there was a large central area of blockage corresponding to hard exudates surrounding a more central area of hyperfluorescence with leakage, representing retinal neovascularization. Review of systems and extensive laboratory workup were negative. The patient was diagnosed with IRVAN. She was observed, and her exam at 6-month follow-up revealed low grade inflammation for which the patient was started on oral prednisone. CONCLUSIONS AND IMPORTANCE: Management of IRVAN remains challenging because of its idiopathic nature and the lack of controlled clinical trials for such a rare entity. Bilateral involvement in IRVAN is variable and close follow up is crucial.