Optical Coherence Tomography Angiography in Perifoveal Exudative Vascular Anomalous Complex

Abstract

Purpose : Perifoveal Exudative Vascular Anomalous Complex (PEVAC) is an uncommon disease recently described by our group as a unilateral, isolated, single, perifoveal, large aneurismal change. It generally affects otherwise healthy patients that do not show evidence of arterial hypertension, diabetes or any other vasculopathy. We reviewed the charts of patients with diagnosis of PEVAC to report their imaging features on optical coherence tomography angiography (OCT-A).

Methods : All patients affected by PEVAC were identified from a pool of patients at 4 retina referral centers. The main exclusion criteria were the presence of retinal vascular diseases (e.g. diabetic retinopathy, hypertension retinopathy, retinal vein occlusion and retinal inflammatory diseases). All patients underwent a complete ophthalmologic examination including structural optical coherence tomography (OCT) and OCT-A (3x3 area). All OCT-A imagines were analyzed by two trained examiners (GQ and RS) to investigate the qualitative features of PEVAC.

Results : A total of 12 eyes of 12 patients were included in the analysis. In 11 out of 12 cases, PEVAC was located inside 500 μm from the center of the fovea. In all cases, OCT-A showed an isolated large dilation in the retinal capillary plexuses, with a detectable flow inside the complex. Typically, PEVAC was characterized by a rarefaction of retinal capillaries surrounding the lesion. Three out of 12 cases were detected only in superficial retinal plexus, 2 out of 12 only in deep retinal plexus and 7 out of 12 in both superficial and deep retinal plexuses. In all cases there was no sign of flow in the avascular plexus and no sign of anastomosis between the retinal capillary plexuses and the choriocapillaris; a shadow effect was constantly present in the choriocapillaris segmentation. No other macular abnormalities were present on OCTA outside the area of the lesion.

Conclusions : Optical coherence tomography angiography of PEVAC showed almost constantly a high-flow, isolated large dilation in the retinal vascularization associated with a surrounding rarefaction of normal retinal capillaries.

This is an abstract that was submitted for the 2017 ARVO Annual Meeting, held in Baltimore, MD, May 7-11, 2017.