Insights Into Perifoveal Exudative Vascular Anomalous Complex

Abstract

Purpose: To report a series of eight patients with perifoveal exudative vascular anomalous complex imaged with optical coherence tomography angiography and the results of anti–vascular endothelial growth factor therapy or laser photocoagulation. Methods: Retrospective analysis of demographic data, imaging including color pictures, spectral domain optical coherence tomography, and optical coherence tomography angiography, and fluorescein angiography, course, and outcome. Results: Age at onset ranged from 45 to 84 years (mean ± SD: 68.6 ± 13.7). Five cases were initially misdiagnosed. The perifoveal exudative vascular anomalous complex lesion was unique in seven eyes and located predominantly in the superficial capillary plexus in two eyes, strictly in the deep capillary plexus in two eyes, but observed at the level of both plexi (3 eyes). One patient presented two lesions, one in the superficial capillary plexus and one in the deep capillary plexus. Capillary rarefaction was observed around the lesion in six eyes. Sustainable resolution of exudation could be achieved in 2 patients, one after 2 sessions of focal thermal laser photocoagulation and one after 13 intravitreal injections of anti–vascular endothelial growth factor. Conclusion: The present series confirms that perifoveal exudative vascular anomalous complex corresponds to a new entity that differs from other conditions associated with capillary aneurysmal lesions. Visual improvement could be obtained after treatment with focal laser or intravitreal anti–vascular endothelial growth factor agents.

Optical Coherence Tomography Findings (SD-OCT and OCTA) in Early-Stage Type 3 Neovascularization

“…Type 3 neovascularization may be confused with PEVAC that usually appears as an isolated perifoveal aneurysmal abnormality with flow typically located between superficial and deep retinal plexi on OCTA, typically with no other vascular changes associated. PEVAC features on SD-OCT are distinguishing as it appears as unilateral, round, hyperreflective isolated lesion, often accompanied by intraretinal cystic spaces without any sign of neovascularization or progression into the RPE [17,18].…”

OCT Angiographic Findings in Retinal Angiomatous Proliferation

“…SD-OCT shows a hyperreflective lesion located between the outer plexiform and ganglion cell layers, which is often surrounded by cystoid macular oedema (exudation). Blood flow within the aneurysm can be registered using OCT-A in both the deep and superficial plexus [26,27]. To date, only one case was reported in which complete resolution of the macular oedema was apparent after 13 injections of a VEGF inhibitor [27].…”

“…Blood flow within the aneurysm can be registered using OCT-A in both the deep and superficial plexus [26,27]. To date, only one case was reported in which complete resolution of the macular oedema was apparent after 13 injections of a VEGF inhibitor [27]. In a normal case, the macular oedema does not re-act to treatment with VEGF inhibitors [24 -26].…”

“…In a normal case, the macular oedema does not re-act to treatment with VEGF inhibitors [24 -26]. Successful treatment by focal laser coagulation was also observed in a patient by Mrejen et al [27]. The pathogenesis of the disease is still unclear.…”

“…Im SD-OCT zeigt sich eine zwischen der äußeren plexiformen Schicht und der Ganglienzellschicht lokalisierte hyperreflektive Läsion, die häufig von einem zystoiden Makulaödem umgeben ist (Exsudation). Mittels OCT-A kann sowohl im tiefen als auch superfiziellen Plexus innerhalb des Aneurysmas Blutfluss registriert werden [26,27]. Bisher wurde nur 1 Fall berichtet, bei dem sich nach 13-maliger Injektion eines VEGF-Hemmers die komplette Rückbildung des Makulaödems gezeigt hat [27].…”