Successful treatments of idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome-related macular edema and exudation by intravitreal aflibercept injection in a senile patient: A case report.

Abstract

The prevalence of idiopathic retinitis, vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome is <1/1,000,000, and it tends to occur in young women. Panretinal photocoagulation, focal laser photocoagulation, peripheral cryopexy, vitrectomy, and corticosteroid therapy are the traditional treatments. We reported a case of a senile patient with IRVAN syndrome who presented with severe exudation in both eyes and macular edema in the left eye, successfully treated by serial intravitreal aflibercept injections. A 77-year-old Taiwanese woman complained of progressive blurred vision in the left eye and ocular examinations revealed a visual acuity of 20/125 in the left eye. Indirect fundoscopy and fluorescein angiography showed bilateral multiple aneurysms, vasculitis, optic nerve staining, and neovascularizations. In addition, optical coherence tomography demonstrated macular edema with subretinal fluid and exudations in the left eye. Monthly intravitreal injections of antivascular endothelial growth factor with aflibercept 2.0 mg were administered in the left eye. The visual acuity in the left eye improved to 20/50 after 18 months of treatment. Macular edema and subretinal fluid regressed. Furthermore, vessel leakage and optic disc staining also improved. This case is the first to demonstrate successful treatment of IRVAN syndrome-related central macular edema and exudations, in the absence of neovascularization, using a series of intravitreal aflibercept injections as monotherapy.