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Purpose: Since its first description by Chang et al. in 1995, the diagnosis of Idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) syndrome has been based on the findings of Fundus Fluorescein Angiography (FFA). Our purpose was to describe the utility of optical coherence tomography angiography (OCT-A) in its diagnosis and management. Case description: A 40-year-old female presented with bilateral blurred vision. Her best corrected visual acuity was at 8/10. Fundus examination revealed blurred disc margins, perivascular exudates, arterial sheaths and retinal hemorrhages bilaterally. FFA showed staining of the optic disc with dye leakage in the right eye, a punctuate hyperfluorescence of the temporosuperior artery in the left eye, bilateral vascular sheathing and capillary dropout. OCT-A showed simultaneous presence of papillary aneurysm and neovascularization of the optic nerve head in the right eye, a papillary aneurysm in the left eye and bilateral capillary non-perfusion. Our patient was diagnosed with IRVAN syndrome. Oral steroids associated with panretinal laser photocoagulation and intravitreal injection of bevacizumab in the right eye resulted in vanishing of the papillary neovascularization with no recurrence on OCT-A at 10-month follow-up. Conclusions: OCT-A is an additional tool to FFA for visualization of arterial macroaneurysms and retinal neovascularization without the interference of dye leakage. It well demarcates nonperfused areas and ensures follow-up of retinal neovascularization. Its limitations are the limited field of view and the low sensitivity in detecting arteriolar dilations. Thus, OCT-A is unable to outplace FFA but should be considered alternately with it for non-invasive follow-up of IRVAN syndrome.

Purpose: To analyze the macular microvascular network and the correlations between visual acuity and quantitative parameters using optical coherence tomography angiography (OCTA) in eyes with retinal vein occlusion (RVO). Methods: We conducted a prospective cross-sectional study including patients with unilateral RVO. We performed 4.5 mm × 4.5 mm macular OCTA angiograms for assessment of quantitative parameters in both superficial and deep capillary plexuses (SCP, DCP). Area of foveal avascular zone (FAZ), vascular density (VD), skeleton density (SD), fractal dimension (FD), vessel diameter index (VDI), and lacunarity (LAC) were analyzed. Results: Seventy eyes of 35 patients were enrolled. As compared to fellow eyes, OCTA analysis in eyes with RVO showed larger FAZ, lower VD, lower SD, lower FD, higher VDI, and increased LAC in both plexuses (All P < 0.05). The enlargement of FAZ in the SCP was associated with visual loss ( P = 0.025, r = 0.378). In the DCP, visual acuity was negatively correlated with parafoveal VD, SD, and FD ( P = 0.004, r = −0.472; P = 0.003, r = −0.482 and P = 0.036, r = −0.308, respectively). Stepwise multivariate regression analysis showed that lower SD and lower FD in the DCP remained correlated with poorer visual acuity ( P = 0.04, r = −0.261 and P = 0.032, r = −0.264, respectively). Conclusions: OCTA provides quantitative parameters to analyze retinal microvasculature in eyes with RVO. These OCTA biomarkers could be used to predict the impact of macular ischemia and capillary dropout on visual acuity in RVO.

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