Multimodal imaging in IRVAN syndrome presenting with Branch Retinal Artery Occlusion

To describe multimodal imaging findings in a patient with idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome presenting with branch retinal artery occlusion (BRAO).

A 33-year-old woman presented with acute BRAO in the right eye. A diagnosis of underlying IRVAN syndrome was made based on the presence of arteriolar aneurysms on the optic disc and along major arterioles and faint retinal hard exudates in both eyes. Eight months later, best-corrected visual acuity was 20/25 in the right eye and 20/20 in the left eye. The hard exudates had increased, and there were extensive areas of peripheral retinal capillary nonperfusion without new vessels. Optical coherence tomography (OCT) showed a localized retinal thinning corresponding to the prior BRAO. Fundus autofluorescence showed nodulo-linear periarterial hypoautofluorescence. OCT angiography (OCTA) showed localized ischemic changes, mainly involving the deep capillary plexus, corresponding to the area of resolved BRAO. It also clearly delineated the optic disc aneurysms. The patient received bilateral scatter laser photocoagulation directed to areas of peripheral capillary nonperfusion. Over a 6-month follow-up period, visual acuity remained unchanged, and there was no evidence of disease progression.

Multimodal imaging, including fundus autofluorescence, OCT, and OCTA can provide additional valuable information in the evaluation of IRVAN syndrome complicated with BRAO.