Purpose: To describe a multimodal imaging and a 5-year follow-up of a case of cilioretinal artery occlusion (CLRAO) in a 40-year-old woman with idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome.
Methods: Fundus photography, indocyanine green and fluorescein angiography, optical coherence tomography (OCT), OCT angiography (OCTA), and a literature review of retinal artery occlusions associated with IRVAN syndrome were performed.
Results: Fundus examination revealed bilateral blurred margins of the discs and peri-vascular hard exudates. Fluorescein angiography (FA) demonstrated peripheral capillary nonperfusion areas in both eyes (OU). It also revealed an aberrant cilioretinal artery in the right eye (OD). OCT showed attenuation of the inner retinal layers and disruption of the outer retinal layers secondary to CLRAO in OD. OCTA allowed a clear identification of macular hypoperfusion in OU. Laser photocoagulation, along with anti-VEGF and corticosteroid intravitreal injections, was administered in OU. Best-corrected visual acuity remained stable, with no evidence of progression or development of neovascularization after a 5-year follow-up.
Conclusion: IRVAN syndrome may be complicated by CLRAO, resulting in poor final visual acuity despite reperfusion of the occluded artery. In such cases, multimodal imaging allows the identification of complications such as macular atrophy on OCT and hypoperfusion areas that may not be detected on FA on OCTA.