Spontaneous branch artery occlusion in idiopathic retinitis, vasculitis, aneurysms, and neuroretinitis syndrome despite panretinal laser photocoagulation of widespread retina nonperfusion

Abstract

Editor, I diopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome is an exceptional form of arteries disease. The diagnosis is based on three major criteria (retinal vasculitis, aneurysmal dilatations at arterial bifurcations, and neuroretinitis) and three minor criteria (peripheral capillary nonperfusion, retinal neovascularization, and macular exsudation) (Chang et al. 1995). In most cases, visual loss is because of peripheral neovascular retinopathy with vitreous haemorrhage secondary to peripheral nonperfusion or to exsudative maculopathy with retro-foveal exsudate. One case of branch artery occlusion has been reported after direct laser of the aneurysm. To our knowledge, spontaneous occlusion of first-order artery has not been described. Here, we report a spontaneous branch artery occlusion that occurred after prior peripheral laser photocoagulation as recommended before or shortly after the development of neovascularization. We present a case report describing a young patient affected with idiopathic retinitis, vasculitis, aneurysms, and neuroretinitis syndrome (IRVAN) who developed spontaneously branch artery occlusion after the photocoagulation of the no-perfuse peripheral retinal surface. A 29-year-old woman presented at our department for scotoma and decreased vision in her left eye (LE) (20 ⁄ 50). She presented with a history of sudden visual decrease in the LE 4 days ago. She had no known systemic illnesses. Infectious, autoinflammatory, and vascular causes were eliminated. Preliminary aetiological investigations were negatives. The patient signed a comprehensive consent form, according to Good Clinical Practice guidelines and French legislation, before proceeding any examinations or treatments. Slit-lamp biomicroscopy did not reveal any abnormality. On fundus examination and photographs of both eyes, we observed an extensive peripapillary exudation, arterial narrowing, and aneurysmal dilatations along the arteries. In the LE, the inferotemporal area showed a yellow-white ischemic retina (Fig. 1A). The spectral domain optical coherence tomography (SD-OCT) (SD-OCT, Cirrus; Carl Zeiss Meditec, Dublin, CA, USA) confirmed the presence of exudation translated as focal hyperreflectivity in the plexiform outer layer with any modification of the retinal pigment epithelium (RPE) or cystoid macular oedema (Fig. 1B). Fluorescein angiography of the LE illustrated the macroaneurysmal dilatations along the arterial tree leakage. The left inferotemporal arterial flow stopped after a macroaneurysm and delayed filling of the corresponding vein (Fig. 1C). A retrograde arterial staining was observed along the FA sequence (Fig. 1D). Photocoagulation peripheral retinal scars were also seen in the both eyes. After discussing treatment options, both antiaggregants and steroids were introduced. Two months later, arterial flow was partially restored without extensive photocoagulation on fluorescein angiography (Fig. 2A early stage FA, Fig. 2B late stage FA), but the patient had no visual acuity improve-