Salomon Y. Cohen

Optical Coherence Tomography Angiography Findings in Perifoveal Exudative Vascular Anomalous Complex


Salomon Y Cohen, Sylvia Nghiem-Buffet, Sarah Mrejen


Background: Perifoveal exudative vascular anomalous complex (PEVAC) has been initially described in 2 patients as a large isolated perifoveal aneurismal change, with no relevant personal or familial medical history. To date, only one series of 15 eyes weas published. We report a second series of 8 cases of PEVAC imaged with OCTA.


Methods: 8 patients were examined with PEVAC in the Centre d’Imagerie et de Laser, a tertiary care center located in Paris, France. All cases were imaged with color pictures, OCT B scan and OCTA, and most cases with fluorescein angiography. Demographic data and imaging were retrospectively analyzed.


Results: Age at onset ranged from 45 to 84 years. Five cases were initially misdiagnosed as Mactel 1, pseudophakic edema, or exudative AMD with type 3 neovascularization. Associated findings were myopic fundus changes (2 cases), subretinal deposits (1 case) and pigment epithelial changes (2 cases) in the affected or the contralateral eye. Vitreoretinal adhesion, and thin epiretinal membrane were observed in 4 eyes. The PEVAC lesion was unique in 7 eyes, and consisted in 2 aneurismal lesions in 1 eye. In OCTA, the aneurismal lesion was located strictly in the superficial capillary plexus (SCP) in 1 eye, strictly in the deep capillary plexus (DCP) in 2 eyes, but was frequently observed between the plexus (4 eyes). One patient presented one lesion in the SCP and one in the DCP. Capillary rarefaction was observed around the lesion in 6 eyes. On OCT B-scan, associated findings were intreretinal hemorrhages (3 eyes), lipid exudates (5 eyes), and intraretinal cysts (8 eyes). Two cases evolved towards sustainable resolution of exudation, one after 2 sessions of focal argon laser photocoagulation, and one after 12 intravitreal injection of ranibizumab.


Conclusion: The present series confirms that PEVAC corresponds to a new entity that should be known. We report associated findings such as anomalies of the vitreo-retinal interface, and the possibility of treatment with focal laser or intravitreal anti-VEGF agents.