Perifoveal Exudative Vascular Anomalous Complex Research Articles

Exudative vascular anomalous complex-like lesion of the peripapillary area.

Perifoveal exudative vascular anomalous complex is an uncommon retinal lesion of idiopathic origin characteristically described as an isolated, aneurysmal lesion of the perifoveal region. We report an unusual presentation of an exudative vascular anomalous complex-like lesion affecting the peripapillary area. A 69-year-old diabetic woman presented with blurred vision in her left eye for two months. Fundus examination of the left eye showed a small, reddish-orange lesion just supratemporal to the optic disc with perifoveal hard exudates and retinal thickening. Fundus fluorescein angiography demonstrated a peripapillary hyperfluorescent lesion with minimal leakage. Ocular coherence tomography showed an oval structure extending throughout the outer and inner plexiform and nuclear layers with a hyper-reflective wall accompanied by subfoveal and intraretinal fluid suggestive of an eVAC-like lesion in the peripapillary area. Intravitreal anti-vascular endothelial growth factor injection was given, and the lesion persisted even three weeks after the injection. Later, focal laser photocoagulation of the aneurysmal lesion was done. At 6 weeks follow-up, a complete resolution of the aneurysmal lesion with a marked decrease in retinal edema and an improvement of the visual acuity was observed. Exudative intraretinal aneurysmal lesions can occur in areas other than perifoveal area. OCT is an useful investigation for knowing their characteristics and the response to treatment. These aneurysmal lesions irrespective of their location can be called by the name eVAC-like or more aptly retinal capillary macro aneurysms (RCM).

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

To report the use of intravitreal triamcinolone to treat macular edema associated with isolated perifoveal exudative vascular anomalous complex (PVAC) and resembling lesion (PVAC-RL). In this case series, three diabetic patients (3 eyes) with PVAC-RLs and one healthy patient (1 eye) with PVAC lesion associated with cystic spaces underwent three intravitreal injections of aflibercept before switching to one intravitreal triamcinolone injection in each patient. Macular edema improved from 297.5 ± 8.10 μm, at baseline, to 269.2 ± 8.89 μm, after triamcinolone; whereas visual acuity improved from 20/38 to 20/26 (ETDRS). PVAC and PVAC-RL are rare and often misdiagnosed lesions that may be associated with decreased vision. Our outcomes suggest that intravitreal injection of triamcinolone may be an effective and affordable treatment for PVAC and PVAC-RL with intraretinal fluid.

First report of perifoveal exudative vascular anomalous complex in a healthy Chinese individual: a case description

First report of perifoveal exudative vascular anomalous complex in a healthy Chinese individual: a case description

Laser photocoagulation of a hypofluorescent perifoveal exudative vascular anomalous complex/capillary macroaneurysm.

To report successful treatment of a hypofluorescent perifoveal exudative vascular anomalous complex (PEVAC)/capillary macroaneurysm. A 63 year old healthy gentleman had a perifoveal isolated aneurysmal lesion with white rim. Optical coherence tomography (OCT) showed cystoid macular edema (CME) with neurosensory detachment. The aneurysmal lesion was seen in the inner retina as a hyperreflective intraretinal structure with a heterogenous lumen suggestive of PEVAC/capillary macroaneurysm. OCT angiography showed a capillary loop and a slightly hyperreflective lesion at the tip of the loop in the superficial capillary plexus slab. Minimal reduction in edema was noted following one dose of intravitreal triamcinolone (2 mg). Fundus fluorescein angiography performed at 6 weeks demonstrated the capillary loop, but the aneurysmal lesion remained hypofluorescent with no definite leak in the late phase. Few perifoveal microaneurysms were seen in both the eyes. Six weeks later, focal laser photocoagulation of the aneurysmal lesion was performed, which resulted in complete resolution of macular edema at 1 month. There was no recurrence of macular edema till his recent follow up, which is 4 month post laser. PEVAC is typically described as unifocal lesion and is not associated with other retinal vascular abnormalities. But in this case, in addition to the lesion, perifoveal microaneurysms were seen in both the eyes. Despite the absence of leak on fundus fluorescein angiography, targeted focal laser photocoagulation resulted in complete resolution of macular edema at 1 month. Laser photocoagulation would be helpful even in hypofluorescent PEVAC/capillary macroaneurysms.

Clinical features of patients with PEVAC in University Hospital of Canarias

AbstractPurposeTo investigate the clinical characteristics of Perifoveal Exudative Vascular Anomalous Complex (PEVAC) patients diagnosed in the University Hospital of Canarias.MethodsThis retrospective study included 7 eyes (of 7 patients) diagnosed with PEVAC. We reviewed the demographic characteristics. All of our patients had undergone a complete ophtalmological examination including color retinography, autofluorescence, Optical Coherence Tomography (OCT), fluorescein angiography(FA) and OCT‐Angiography (OCTA). We also reviewed the follow‐up information.ResultsOf our 7 patients, 3 were women and 4 were men. The average age and standard deviation were 71.0 +‐12.8 years. Average visual acuity was 0.54 + −0.24.Of our 7 patients, 4 had no remarkable comorbidities, 2 had age‐related macular degeneration and the resting patient had myopic degeneration. PEVAC presented as a big isolated perifoveal aneurysm. On OCT PEVAC appeared as a round hyperreflective lesion, typically surrounded by intraretinal cystic spaces. FA showed a well defined hyperfluorescent lesion with variable leakage. OCTA revealed that the isolated aneurysmatic lesion was located in the superficial capillary plexus in 2 cases; in the deep capillary plexus in 4 cases and in one cases it seemed to be in the intermediate plexus. In 5 cases, there was only subtle exudation that did not require treatment, remaining stable during follow‐up. In two cases, exudation was greater, so we treated them with anti‐VEGF, with partial response.ConclusionsPEVAC is an aneurysmatic isolated perifoveal abnormality that can occur in patients with or without retinal disease. PEVAC did not respond to anti‐VEGF therapy.Bibliography Querques G, Kuhn D, Massamba N, Leveziel N, Querques L, Souied EH. Perifoveal Exudative Vascular Anomalous Complex. J Fr Ophtalmol 2011; 34(8): 559.e1–4. Sacconi R, Freund KB, Yannuzzi LA, Dolz‐Marco R, Souied E, Capuano V, et al. The Expanded Spectrum of Perifoveal Exudative Vascular Anomalous Complex. Am J Ophthalmol 2017; 184:137–146. Mrejen S,Le HM,Nghiem‐Buffet S,Tabary S,Quentel G,Cohen SY. Insights into Perifoveal Exudative Vascular Anomalous Complex. Retina 2020;40(1):80–86.

Refractory perifoveal exudative vascular anomalous complex like lesion responding to intravitreal dexamethasone implant: A therapeutic challenge

<br>A 66-year-old male patient presented with the right eye (OD) decreased vision for 1 month duration. His best-corrected visual acuity (BCVA) was 6/36 in OD and 6/6 in the left-eye (OS). He had bilateral non-proliferative diabetic retinopathy (NPDR) changes with the presence of a large perifoveal aneurysmal lesion, which was diagnosed as perifoveal exudative vascular anomalous complex (PEVAC)-like lesion based on clinical evaluation, spectral domain optical coherence tomography (SD-OCT), and fundus fluorescein angiography (FFA). The patient underwent three intravitreal ranibizumab injections with minimal response. However, he showed excellent improvement in BCVA to 6/6 with complete resolution of fluid on SD-OCT after switching to intravitreal Ozurdex implant. This case highlights that an intravitreal dexamethasone implant can be considered as a viable option for the optimal management of PEVAC-like lesions, with good visual acuity outcomes and morphologic response on SD-OCT. Further studies are warranted to gain better insight into the pathogenesis of PEVAC-like lesions and the potential role and mechanism of action of intravitreal dexamethasone implant in its management.<br>

Perifoveal Exudative Vascular Anomalous Complex-Like Lesion: A Case Report

Perifoveal exudative vascular anomalous complex PEVAC and its expanded spectrum have been recently elucidated by multimodal imaging. Although there is some clarity about its characteristic features and natural history the cause remains undetermined. We describe an unknown association with peripapillary arterial loops and suggest a possible hypothesis for the development of a PEVAC-like lesion and its response to treatment with thermal laser.

This issue of ACTA

This issue of ACTA

Focal laser photocoagulation in the management of perifoveal exudative vascular anomalous complex

<br>Perifoveal exudative vascular anomalous complex is an unilateral, isolated, perifoveal aneurysm with adjacent exudative maculopathy. It has been hypothesized to develop due to progressive endothelial injury leading to aneurysmal changes. Usually, it is unresponsive to routinely used anti-VEGF agents. We present a case of a 33-year-old woman presented with PEVAC who was treated with a thermal focal laser. Complete resolution of PEVAC and improvement in BCVA was noted.<br>

ANATOMICAL CHANGES ON SEQUENTIAL MULTIMODAL IMAGING IN PERIFOVEAL EXUDATIVE VASCULAR ANOMALOUS COMPLEX.

To report a series of 21 patients with perifoveal exudative vascular anomalous complex (PEVAC) and to investigate the anatomical changes over time. We conducted a retrospective study. Clinical data of consecutive patients, presenting at the Rotterdam Eye Hospital between 2014 and 2019, were analyzed. The data collected included best-corrected visual acuity, fundus photography, optical coherence tomography (OCT), OCT-angiography, fluorescence angiography, and indocyanine green angiography. We included 21 patients with a PEVAC lesion with a mean follow-up of 24.3 ± 13.8 months (range, 9-46 months). Patients with PEVAC were on average 75.3 ± 11.1 years (range, 53-90 years). The large perifoveal vascular aneurysmal abnormality was associated with small retinal hemorrhages in six patients and hard exudates in three patients. The PEVAC lesion was associated with intraretinal cystic spaces on OCT in 15 patients. Twelve of 21 patients showed no changes in cystic spaces on OCT during follow-up: 9 patients had stable cystic spaces and 3 patients had no cystic spaces. In contrast, in 9 of 21 patients, we observed changes in cystic spaces on OCT during follow-up. In two patients, cystic spaces appeared during follow-up, and in seven patients, there was a spontaneous resolution of cystic spaces. In three of these seven patients, the PEVAC lesion completely disappeared. Two patients, with stable intraretinal cystic spaces on OCT, were treated with intravitreal injections of anti-vascular endothelial growth factor without improvement. Perifoveal exudative vascular anomalous complex is an idiopathic perifoveal retinal vascular abnormality that is associated with intraretinal cystic spaces. These intraretinal cystic spaces associated with a PEVAC lesion, and even the PEVAC lesion itself, can have a spontaneous resolution over time.

Multimodal imaging in perifoveal exudative vascular anomalous complex with co‐existent diabetic retinopathy

Perifoveal exudative vascular anomalous complex (PEVAC) is a recently described macular entity, which can be overlooked or confused with other retinal pathologies. On the basis of multimodal imagin...

Characteristics of Perifoveal Exudative Vascular Anomalous Complex in Korean Patients

ABSTRACTPurpose: To investigate the clinical characteristics of perifoveal exudative vascular anomalous complex (PEVAC) in Korean patients.Methods: This retrospective study included 8 eyes (8 patients) diagnosed with PEVAC. Demographic characteristics of the patients, fundus photography, optical coherence tomography (OCT), fluorescein angiography, and indocyanine-green angiography (ICGA) findings were analyzed. Available follow-up data were also reviewed.Results: The mean ± standard deviation age was 61.0 ± 11.0 years and mean logarithm of minimal angle of resolution (logMAR) best-corrected visual acuity was 0.15 ± 0.14. PEVAC was noted in 2 eyes with coincident age-related macular degeneration (AMD) and in the fellow eye of 1 eye with unilateral type 3 neovascularization. On fundus photography, PEVAC presented as a perifoveal isolated aneurysm associated with retinal exudation and hemorrhage. Well-defined hyperfluorescent lesions with leakage were noted on fluorescein angiography, but no leakage was observed on ICGA. Anti-vascular endothelial growth factor (VEGF) therapy was performed for 2 patients. However, there was no notable improvement after treatment.Conclusions: The clinical characteristics of PEVAC in Korean patients were similar to those reported in patients of other ethnicities. Further studies are needed to better understand the nature of this newly described clinical entity.

The Expanded Spectrum of Perifoveal Exudative Vascular Anomalous Complex

To expand our understanding of the uncommon entity, referred to as perifoveal exudative vascular anomalous complex (PEVAC) by describing multimodal imaging findings, including optical coherence tomography angiography (OCT-A). Retrospective cohort study. Patients diagnosed with PEVAC were identified at 4 retina referral centers worldwide and underwent complete ophthalmologic examination including structural OCT, OCT-A, fluorescein angiography (FA), and indocyanine green angiography (ICGA). Demographics and clinical findings were analyzed at baseline and at available follow-ups. Fifteen eyes (15 patients, mean age 73 ± 13 years) were included. Six of 15 eyes were diagnosed with coincident age-related macular degeneration (AMD) and 2 with myopic macular degeneration. On fundus examination PEVAC presented as a large perifoveal isolated aneurysm, unifocal in 12 of 15 eyes, associated with small retinal hemorrhages and intraretinal exudation. On structural OCT, PEVAC appeared as a round hyperreflective lesion with hyporeflective lumen, typically surrounded by intraretinal cystic spaces. Dye angiography demonstrated a well-defined hyperfluorescent lesion with variable leakage on FA and without leakage on ICGA. OCT-A showed flow signal correlating with the aneurysmal lesion connecting to retinal capillary plexuses. Seven patients were followed for 13.0 ± 10.5months with no evidence of functional/anatomic changes. Three patients underwent anti-vascular endothelial growth factor (VEGF) intravitreal injections without improvement. Two eyes were associated with a type 3 neovascularization eccentric to PEVAC. PEVAC is an isolated, perifoveal, aneurysmal abnormality, occurring in otherwise healthy patients who may manifest other macular disease including AMD and myopic macular degeneration. PEVAC did not typically respond to anti-VEGF therapy, and may be associated with type 3 neovascularization.

Perifoveal exudative vascular anomalous complex

To report the angiographic and optical coherence tomography (OCT) features of isolated "perifoveal exudative vascular anomalous complex (PEVAC)", a peculiar clinical entity. A complete ophthalmologic examination was performed in two patients (a 82-year old woman [case 1]; a 52-year old man [case 2]) that were referred to our department for unilateral blurred vision. In both cases, fundus examination of the right eye showed a perifoveal isolated large aneurismal change, accompanied by small hemorrhages, intraretinal exudation, and small hard exudates accumulation. Both FA and ICGA revealed the absence of any other retinal or choroidal vascular abnormality associated. OCT showed a round hyperreflective lesion in correspondence of the perifoveal vascular anomalous complex, surrounded by intraretinal cystic spaces. In case 2, the lesion remained unchanged despite 3 monthly intravitreal injections of ranibizumab. PEVAC may develop in absence of capillary ischemia or inflammation, probably due to progressive retinal endothelial cell degeneration. This could explain the unresponsiveness to anti-VEGF treatments.