Perifoveal exudative vascular anomalous complex (PEVAC) resembling lesion in a patient with multiple myeloma.

Purpose: To report a case of a perifoveal exudative vascular anomalous complex (PEVAC) resembling lesion in a patient with multiple myeloma. Observations: A 56-year-old male with multiple myeloma presented with sudden moderate vision loss in the right eye. Best-corrected visual acuity was 20/25 in his right eye. Fundus examination showed a vascular irregularity in the perifoveal region. Fluorescein angiography (FA) revealed an isolated perifoveal aneurysmal lesion with minimal leakage. On optical coherence tomography (OCT) examination, a large oval structure with a hyperreflective wall and exudation was visualised. Three weeks later, spontaneous improvement of the intraretinal fluid was observed on OCT without treatment. However, 3 months later the macular edema recurred. The appearance of the aneurysmal lesion is similar to a PEVAC lesion, which is an isolated well-defined perifoveal intraretinal vascular abnormality presenting on OCT as a round hyperreflective structure with a dark lumen containing variably reflective material and is commonly associated with cystic intraretinal fluid. Conclusions and Importance: PEVAC was originally described as occurring in healthy patients, but recent observations suggest that it also appears in association with other retinal/choroidal vascular abnormalities or underlying cardiovascular abnormalities. Our case supports this hypothesis by demonstrating a PEVAC resembling lesion in a patient with multiple myeloma.

Complejo anómalo vascular exudativo perifoveal (PEVAC) / Perifoveal exudative vascular anomalous complex (PEVAC)

Se presentan 3 casos de pacientes, con 66, 80 y 23años de edad, que presentaron una pérdida de visión unilateral. La tomografía de coherencia óptica (OCT) mostró edema macular junto con una lesión redondeada de pared hiperreflectiva y la angiografía con fluoresceína (AFG) de dos de ellos, dilataciones aneurismáticas perifoveales hiperfluorescentes con exudación. Ninguno de los casos mostró respuesta al tratamiento tras un año de seguimiento, diagnosticándose finalmente de complejo anómalo vascular exudativo perifoveal (PEVAC) (AU)

We present three cases of patients aged 66, 80 and 23, who presented unilateral vision loss. Optical coherence tomography (OCT) in all of them showed macular oedema and a rounded lesion with hyper-reflective wall, and fluorescein angiography (FAG) in two of them showed hyperfluorescent perifoveal aneurysmal dilations with exudation. None of the cases showed response to treatment after one year of follow-up, finally being diagnosed with perifoveal exudative vascular anomalous complex (PEVAC) (AU)

Perifoveal exudative vascular anomalous complex (PEVAC).

We present three cases of patients aged 66, 80 and 23, who presented unilateral vision loss. Optical coherence tomography (OCT) in all of them showed macular oedema and a rounded lesion with hyperreflective wall, and fluorescein angiography (FAG) in two of them showed hyperfluorescent perifoveal aneurysmal dilations with exudation. None of the cases showed response to treatment after one year of follow-up, finally being diagnosed with Perifoveal Exudative Vascular Anomalous Complex (PEVAC).

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

PURPOSE: To report successful treatment of a hypofluorescent perifoveal exudative vascular anomalous complex (PEVAC)/capillary macroaneurysm. CASE DESCRIPTION: 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. DISCUSSION: 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. CONCLUSION: Laser photocoagulation would be helpful even in hypofluorescent PEVAC/capillary macroaneurysms.

Progress in clinical features and treatment of perifoveal exudative vascular anomalous complex / 中华眼底病杂志

Perifoveal exudative vascular anomalous complex (PEVAC) is a very rare macular vascular disease characterized by an isolated large aneurysmal lesion in the fovea, with accompanied by small retinal hemorrhage and exudation. The main clinical symptoms of the patients are various degree of impaired vision. Clinically, it is often confused with type 1 macular telangiectasia and type 3 macular neovascularization. A thorough understanding of the clinical features of PEVAC is particularly important for its differential diagnosis. Due to the unclear pathogenesis of PEVAC, there is no specific treatment for the cause of disease. Most scholars use intravitreal injection against vascular endothelial growth factor drugs for treatment, but can not improve patients' visual acuity. At present, many attempts have been made to eliminate abnormal exudation of the lesion, maintain visual function and achieve a good prognosis by simple or combined laser photocoagulation. At present, it is still necessary to explore the pathogenesis of PEVAC, improve the understanding of the disease, and find a better treatment plan.

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

Perifoveal exudative vascular anomalous complex (PEVAC) is a perifoveal aneurysmal vascular lesion found in healthy subjects. A 68-year-old woman was diagnosed with a typical unilateral and unifocal PEVAC lesion after extensive multimodal imaging and was treated with three-monthly intravitreal injections of ranibizumab. An immediate and complete resolution of the intraretinal fluid was observed. Visual acuity returned to 20/20 without any recurrence of the exudative signs along the 5 years of follow-up. Therefore, an initial anti-VEGF treatment with three-monthly intravitreal injections may be considered as a first-line treatment in PEVAC lesions and may result in long-term preservation of visual acuity.

Atypical case of perifoveal exudative vascular anomalous complex associated with pachychoroid pigment epitheliopathy.

Purpose: To report an unusual association of a perifoveal exudative vascular anomalous complex (PEVAC) and a bilateral pachychoroid pigment epitheliopathy (PPE), which responded positively to anti-vascular endothelial growth factor (VEGF) intravitreal injections (IVI). Observations: A 44 year-old man with no significant medical or ocular history, complained of unilateral blurred vision in his right eye (RE) over several months. On examination, best corrected visual acuity (BCVA) was 75 letters in the RE and 85 in the left eye (LE). Fundus examination in the RE showed a large perifoveal aneurysmal lesion with a macular thickening, small hemorrhages and linear hard exudates accumulation, associated with multifocal retinal pigment epithelium (RPE) changes in the posterior pole of both eyes. Optical coherence tomography of the RE showed the PEVAC as a large round retinal capillary aneurysm with surrounding intraretinal fluid, associated with serous and drusenoid RPE elevations in both eyes, consistent with PPE. Subfoveal choroidal thickness was more than 500 µm in both eyes, with several dilated choroidal veins. Fluorescein angiography showed, in the RE, the hyperfluorescent aneurysmal lesion with late leakage, associated with scattered hyperfluorescent areas in the posterior pole of both eyes. Indocyanine green angiography showed, in the RE, the same hyperfluorescent lesion but without leakage, associated with areas of choroidal hyperpermeability in both eyes. After 2 anti-VEGF IVI in the RE, good functional and anatomical improvement was observed. After 10 months of follow-up, there was no evidence of new exudation. BCVA remained stable and RPE abnormalities remained unchanged. Conclusion and importance: We describe an atypical case of PEVAC associated with PPE, which responded positively to anti-VEGF therapy. To our knowledge, this is the first report of a patient presenting PEVAC and diseases of the pachychoroid spectrum. Further studies, assessing the choroid in PEVAC, are required to investigate the hypothetical relationship between these 2 entities and the efficiency of anti-VEGF therapy.

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

Purpose: To report a case of progressive resolution of exudation in a patient affected by perifoveal vascular anomalous complex (PVAC) undergoing topical diclofenac therapy. Observations: A 74-year-old man presented to our department with visual decrease in his right eye lasting six months. Lack of clinical history of arterial hypertension, diabetes, or any other systemic or local vasculopathy, together with retinal multimodal imaging, led to the diagnosis of exudative (e)PVAC. Serial spectral-domain optical coherence tomography (SD-OCT) examinations documented a resolution of intraretinal exudation after one-month topical diclofenac therapy. Conclusion and Importance: Initiation of topical diclofenac was associated with resolution of exudation, therefore we hypothesize its potential role in the treatment of ePVAC.

Clinical characteristics and multimodal imaging of perifoveal exudative vascular anomalous complex / 中华眼底病杂志

Objective:To investigate the clinical features and multimodal imaging features of eyes with perifoveal exudative vascular anomalous complex (PEVAC).Methods:A retrospective case study. From February 2014 to November 2020, 7 eyes of 7 patients with PEVAC diagnosed by ophthalmology examination in Department of Ophthalmologyof Peking University People's Hospital were included in this study. There were 6 males and 1 female. The age was 60.1±9.1 years. All were monocular. The chief complaints of visual deformation and vision loss were 3 and 1 cases, respectively. All patients underwent best corrected visual acuity (BCVA), fundus color photography, optical coherence tomography (OCT), fundus fluorescein angiography (FFA). BCVA examination was performed using the standard logarithmic visual acuity chart, which was converted to logarithm of the minimum angle of resolution (logMAR) visual acuity. OCT angiography (OCTA) and indocyanine green angiography (ICGA) were performed in 4 and 2 eyes, respectively. Three eyes were treated with intravitreal injection of anti-vascular endothelial growth factor (VEGF) combined with local laser photocoagulation. Two eyes were treated with laser photocoagulation alone. The follow-up time was 16.7±19.1 months. During follow-up, relevant examinations were performed with the same equipment and methods as at the initial diagnosis. The multimodal imaging characteristics and treatment response of the affected eyes were observed.Results:The baseline logMAR BCVA was 0.33±0.19 (0.20-0.80). All eyes showed isolated hemangiomatous lesions in the macular fovea with rigid retinal exudation, and 2 adjacent isolated hemangiomatous lesions were observed in 1 eye. FFA and ICGA examination showed that all eyes with macular hemangiomatous lesions showed clear boundary and strong fluorescence in the early stage. No other retinal or choroidal vascular abnormalities were observed. On OCT examination, circular lumen-like structures with strong reflective wall near the fovea were observed in the macular region of all eyes, accompanied by intraretinal cystic lumen. The macular central retinal thickness (CMT) was 326±125 (207-479) μm. In the four eyes examined by OCTA, blood flow signals were observed in the circular lumenoid structures with strong reflective walls adjacent to the fovea. Blood flow signals were observed in the superficial capillary layer (SCP) and deep capillary layer (DCP) of the retina in 3 eyes. SCP showed blood flow signal in 1 eye. In 4 eyes treated with intravitreal injection of anti-VEGF drugs, there was no significant improvement in the intraretinal capsule space after treatment. Subretinal fluid absorption, retinal cystoid edema persisted, and rigid exudation decreased in 1 eye. CMT decreased and BCVA increased in 5 eyes treated with laser photocoagulation or laser photocoagulation alone. At last follow-up, logMAR BCVA was 0.16±0.06 (0.10-0.20) and CMT was 212±34 (154-252) μm. Compared with baseline, the difference of BCVA was statistically significant ( t=2.661, P=0.037). Conclusions:The fundus of PEVAC patients is characterized by solitary or multiple solitary hemangiomatous lesions in the macular fovea. Round lumenoid structures with strong reflective walls, with or without intraretinal cystic lumen, rigid exudate, and subretinal fluid, in which blood flow signals can be seen in OCT.

Advances in the study of perifoveal exudative vascular anomalous complex / 中华眼底病杂志

Perifoveal exudative vascular anomalous complex (PEVAC) are class of diseases characterized by isolated aneurysmal lesions of the perifovea with hemorrhage and hard exudates, which are found in recent years. Its pathogenesis and pathological process are not yet fully elucidated. The current consensus through multimodal imaging studies is that PEVAC is an idiopathic perifoveal retinal vascular abnormality with cystoid macular edema (CME) and hard exudate. It is not easily distinguished clinically from retinal microaneurysm due to diabetic retinopathy or retinal vein occlusion, aneurysmal telangiectasia, and type 3 macular neovascularization (stage Ⅰ). PEVAC is a partially self-healing property, and some aneurysms and CME may resolve on their own during follow-up period. Laser photocoagulation is an effective measure for the treatment of PEVAC. In the future, with the rapid development of imaging technology, the progress of research on pathogenesis and treatment strategies and the updating of theories are expected to provide more effective treatments for PEVAC.

Parafoveal exudative vascular anomalous complex and its characteristics of multi-modal imaging in 11 cases / 国际眼科杂志(Guoji Yanke Zazhi)

AIM: To observe the imaging characteristics of multi-modal imaging in perifoveal exudative vascular anomalous complex(PEVAC).METHODS: A retrospective case analysis. There were 11 patients(11 eyes)diagnosed as PEVAC by multi-modal imaging in our hospital included in this study from February 2021 to February 2022. All patients underwent the examinations of best corrected visual acuity(BCVA), fundus photography, fluorescein fundus angiography(FFA), spectral-domain optical coherence tomography(SD-OCT)and optical coherence tomography angiography(OCTA).RESULTS: On fundus examination, six out of eleven eyes presented as a unilateral and isolated lesion with diameter ≥150μm, five out of eleven eyes presented as multiple lesions(ranging from 2 to 4)and the diameter of each lesion <150μm, seven eyes with retinal edema and/or hard exudation. FFA revealed an oval-like hyper-fluorescent lesion of parafoveal in the late phase with enhancement but no leakage. Late peripheral telangiectasia leakage is seen with oedema. OCTA examination revealed a parafoveal small mass hyper-reflex showed aneurysmal dilation in the superficial and deep capillary, and capillary rarefaction was observed around the lesion. B-scan showed strong blood flow signal inside. Anti-vascular endothelial growth factor(VEGF)therapy was performed in 4 patients with macular edema. However, there was no notable improvement after treatment.CONCLUSION: Multi-modal imaging can help confirm the diagnosis and differential diagnosis of PEVAC, there is a poorly response to anti-VEGF therapy.

Case Report: A Case of Perifoveal Exudative Vascular Anomalous Complex With a Good Prognosis.

Significance: Perifoveal exudative vascular anomalous complex (PEVAC) is a unique clinical lesion. It manifests as isolated lesions and is easily misdiagnosed. Thus far, few PEVAC case reports have been published. PEVAC is typically inconsistent with other reported macular lesions. Purpose: To report our 24-month follow-up experience on the treatment of PEVAC with a micropulse laser (MPL). Case Report: A 56-year-old Chinese woman with no history of other diseases complained of decreased vision in her left eye that had persisted for more than 1 year. Comprehensive ophthalmic examinations were performed, including a vision test, slit lamp fundus exam, optical coherence tomography (OCT), optical coherence tomography angiography (OCT-A), fluorescein fundus angiography (FFA) and indocyanine green angiography (ICGA). Intravitreal injection of ranibizumab was ineffective, and bleeding, exudation and visual acuity were not improved. After two rounds of micropulse laser (MPL) treatment, the patient was followed up, and the prognosis was good. Conclusion: PEVAC is very rare, and early diagnosis is important, as the lesions readily cause irreversible damage. Our results indicate that an MPL can be used as an alternative treatment for PEVAC patients.

Exudative non-neovascular age-related macular degeneration.

PURPOSE: To describe the clinical and multimodal imaging (MMI) features of age-related macular degeneration (AMD) eyes presenting with intraretinal exudation and no evidence of neovascularization or structural alterations of native retinal vessels. METHODS: This was a retrospective review of the MMI and electronic health records for 3 consecutive patients presenting with unilateral exudative non-neovascular age-related macular degeneration. MMI included confocal color fundus photography (CFP), fundus autofluorescence (FAF), fluorescein angiography (FA), spectral domain optical coherence tomography (SD-OCT), swept-source optical coherence tomography angiography (SS-OCTA), and spectral domain optical coherence tomography angiography (SD-OCTA). Dense B-scan OCTA (DB-OCTA) patterns and implemented image post-processing were used to improve spatial resolution in the OCTA analysis and remove projection artifacts. RESULTS: Three eyes of 3 patients (1 male and 2 females, ages 72-87) developed intraretinal fluid (IRF) producing retinal edema during regular follow-up for non-neovascular AMD. FA, SS-OCTA, and DB-OCTA demonstrated no evidence of macular neovascularization or discrete retinal vascular abnormalities that could explain the IRF accumulation. Two eyes received intravitreal anti-VEGF therapy and demonstrated prompt resolution of IRF with periodic recurrences over time. CONCLUSION: Exudative non-neovascular AMD is a novel clinical phenotype characterized by the presence of non-neovascular intraretinal exudation producing macular edema. Differentiating this condition from other manifestations of AMD requires appropriate use of MMI. Further study is needed to assess the clinical impact and optimal management of exudative non-neovascular AMD.

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

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 follow-up of perifoveal exudative vascular anomalous complex treated with intravitreal injections of anti-vascular endothelial growth factor and thermal laser photocoagulation.

PURPOSE: To describe the long-term follow-up of a patient with perifoveal exudative vascular anomalous complex (PEVAC) treated initially with intravitreal injections of anti-vascular endothelial growth factor (VEGF) followed by focal thermal laser photocoagulation. OBSERVATIONS: A 78 years-old man presented with large, soft drusen in both eyes. Optical coherence tomography and fluorescein angiography revealed the presence of PEVAC in the left eye. The patient was in good general health with no history of diabetes and had no signs of other retinal vascular disease. During the follow-up, the intraretinal fluid accumulation progressively increased and the best-corrected visual acuity (BCVA) dropped from 20/20 to 20/30 over a period of 33 months. As the intraretinal fluid continued to increase and BCVA further decreased to 20/50 despite two intravitreal injections of anti-VEGF, the patient underwent focal thermal laser photocoagulation with a reduction in intraretinal fluid observed 1 month later. Two months after laser, the BCVA increased to 20/25 with complete reabsorption of the intraretinal fluid. Ten months after laser, the BCVA remained stable at 20/25 with no recurrence of intraretinal fluid. CONCLUSIONS AND IMPORTANCE: This case illustrates that a PEVAC lesion may remain non-exudative for an extended period of time, but when exudation develops, anti-VEGF therapy may be ineffective requiring the use of thermal laser photocoagulation.

Perifoveal exudative vascular anomalous complex in a highly myopic eye.

'Perifoveal Exudative Vascular Anomalous Complex' (PEVAC) is a perifoveal, unilateral, isolated, perifoveal aneurysm, in otherwise healthy patients. Here, we report a case of PEVAC in a highly myopic eye of a 86-year-old woman affected by a visual decline in the right eye (best-corrected visual acuity of 20/100). She had no other relevant past conditions and/or ocular impairment. Fundus examination in the right eye showed myopic chorioretinal degeneration with a concomitant PEVAC. Structural optical coherence tomography (OCT) showed a round lesion with a hyperreflective wall associated with intraretinal cystic spaces. OCT-angiography nicely disclosed an isolated large aneurysmal retinal dilation featuring the PEVAC with detectable flow in superficial capillary plexus, deep capillary plexus, and avascular slab. This case highlights the importance of discerning between different vascular disorders of the macula, in order to be able to offer the right treatment and/or follow-up to the patient.

Atypical perifoveal exudative vascular anomalous complex (PEVAC) with multifocal and bilateral presentation.

PURPOSE: To report an unusual case of multifocal and bilateral perifoveal exudative vascular anomalous complex (PEVAC) lesions in an otherwise healthy patient. OBSERVATIONS: A 62-year-old male with complaints of long-term progressive blurred vision in both eyes. There was no history of diabetes, arterial hypertension or inflammatory disease. Best-corrected visual acuity was 75 and 80 letters in his right eye (OD) and left eye (OS) respectively. Fundus examination showed three perifoveal isolated large aneurysmal lesions in his OD and an additional one in his OS, associated with smaller microaneurysms, haemorrhages and intraretinal exudation. On optical coherence tomography PEVAC appeared as a round lesions with a hyperreflective wall and lumen containing variably reflective material, typically surrounded by intraretinal fluid. Dye-based angiography demonstrated three and one well-defined large hyperfluorescent aneurysmal lesions in his OD and OS respectively, with no leakage. The optical coherence tomography angiography images showed flow signal within the intraretinal aneurysmal lesions. No anomalous flow signal in the outer retina and choriocapillaris was present. After one bilateral anti-vascular endothelial growth factor intravitreal injection no anatomical or functional improvement was observed, and no changes in the lesions or visual acuity were observed at 6 months follow-up. CONCLUSIONS AND IMPORTANCE: We describe an atypical case of PEVAC, an uncommon and poorly known macular disorder, with bilateral presentation and multifocal lesions in one eye. To our knowledge, this is the first reported case of bilateral PEVAC lesions. Hence, PEVAC should be included in the differential diagnosis of bilateral perifoveal aneurysmal lesions with macular edema.

Characteristics of Perifoveal Exudative Vascular Anomalous Complex in Korean Patients.

Purpose: 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.