Multimodal imaging comparison of perifoveal exudative vascular anomalous complex and resembling lesions

Abstract

Purpose Perifoveal exudative vascular anomalous complex (PEVAC) was initially described as an isolated aneurysmal lesion in healthy eyes. Similar aneurysmal abnormalities may occur in association with retinal vascular diseases such as diabetic retinopathy or retinal vein occlusions (PEVAC‐resembling). The aim of this study was to compare several imaging characteristics of PEVAC and PEVAC‐resembling lesions. Methods Ten eyes with a PEVAC and 27 eyes with a PEVAC‐resembling lesion were included in this cross‐sectional study. They were all imaged with optical coherence tomography (OCT), OCT angiography (OCT‐A) and colour fundus photography (CFP). Several clinical, morphological and vascular characteristics were assessed and compared between both PEVAC types. Results All PEVAC lesions were unilateral, while PEVAC‐resembling lesions appeared bilateral in 23% of patients (p > 0.05). Unilateral multifocal PEVAC‐resembling lesions were more frequently observed (56%) than unilateral multifocal PEVAC lesions (10%, p < 0.01). Furthermore, 90% of the PEVAC lesions were located within 500 µm from the centre of the fovea, while this was only true for 56% of the PEVAC‐resembling lesions (p > 0.05). No notable differences were observed in other studied characteristics. Conclusions The clinical, morphological and vascular features of PEVAC and PEVAC‐resembling lesions are similar based on multimodal imaging. Given the bilaterality and multifocality seen in PEVAC‐resembling lesions, an underlying retinal vascular disease may stimulate the quantity of aneurysmal abnormalities. Due to the similarities with PEVAC‐resembling lesions, PEVAC may also be considered a microangiopathy but with an unknown origin.

Long-Term Resolution of Perifoveal Exudative Vascular Anomalous Complex after Intravitreal Injections of Anti-Vascular Endothelial Growth Factor

“…It was suggested that their apoptosis could trigger the endothelium to proliferate into microaneurysms. Smid et al [ 2 ] hypothesized that aging is a critical factor in the development of PEVAC lesions, given that an association between microvascular rarefaction and aging has already been established. Thus, Smid et al [ 2 ] proposed that PEVAC could be considered a local microangiopathy emerging associated with aging and/or past or ongoing cardiovascular problems.…”

“…Smid et al [ 2 ] hypothesized that aging is a critical factor in the development of PEVAC lesions, given that an association between microvascular rarefaction and aging has already been established. Thus, Smid et al [ 2 ] proposed that PEVAC could be considered a local microangiopathy emerging associated with aging and/or past or ongoing cardiovascular problems. Indeed, 40% of patients categorized as PEVAC had suffered from a stroke or ischemic attack, and 70% presented controlled hypertension.…”

“…Perifoveal exudative vascular anomalous complex (PEVAC) is a rare condition of uncertain etiology described for the first time in 2011 by Querques and associates as a perifoveal, unilateral, usually unifocal, and aneurysmal vascular lesion in otherwise healthy subjects [ 1 ]. It is usually associated with intraretinal cystic spaces which are the cause of vision loss, and it can also be associated with hemorrhages or hard exudates [ 2 , 3 ]. Recently, the term PVAC has been proposed to name this entity rather than PEVAC, considering that it can be classified into two different forms: an exudative form (ePVAC) and a non-exudative one.…”

Progressive resolution of exudation from perifoveal vascular anomalous complex: A possible role of diclofenac therapy?

“… 3 Both perifoveal aneurysm and perilesional capillaries rarefaction seem to result from pericytes loss, in particular from breakdown of pericytes' basement membrane by matrix metalloproteinase-9. 6 Thus, PVAC genesis would be not regulated by VEGF, but by the imbalance between a reduced wall strength and an increased wall tension, in accordance with Laplace's law. 7 Notably, perilesional microvascular rarefaction has been associated to an impaired angiogenesis 8 and local ageing.…”

Perifoveal exudative vascular anomalous complex and resembling lesions treated with intravitreal triamcinolone

“…[1][2][3]5 However, this lesion has been reported in subjects with dry age-related macular degeneration (AMD) and myopic macular degeneration, and in patients with diabetes or hypertension (PVAC-resembling lesions). 7 We present here a case series involving 4 patients (4 eyes), where one patient had isolated PVAC lesion, and the other three patients had PVAC-resembling lesion with macular edema. To date, there are few cases reporting the findings, course, outcomes, and treatment of this condition.…”