Nonexudative Perifoveal Vascular Anomalous Complex: The Subclinical Stage of Perifoveal Exudative Vascular Anomalous Complex?

doi:10.1016/j.ajo.2020.04.025

American Journal of Ophthalmology

Volume 218, October 2020, Pages 59-67

https://doi.org/10.1016/j.ajo.2020.04.025 Get rights and content

Purpose

To describe the pre-exudative stage of exudative perifoveal vascular anomalous complex (ePVAC), referred to as nonexudative PVAC (nePVAC).

Design

Retrospective noncomparative case series.

Methods

Patients diagnosed with nePVAC were identified at 4 retina referral centers worldwide. Multimodal retinal imaging, including structural optical coherence tomography (OCT) and OCT-angiography (OCT-A), were performed at baseline and follow-up visits.

Results

Six eyes (6 patients, mean 75 ± 10 years of age) were included. Unrelated chorioretinal diseases were diagnosed in the affected eyes in 5 of 6 cases. At baseline, nePVAC is characterized by microvascular abnormalities featuring an isolated, perifoveal, large intraretinal aneurysm surrounded by capillary rarefaction at OCT-A examination, without any sign of exudation with structural OCT, and without visual impairment. Four patients were followed for a mean of 21 ± 14 months. During the follow-up, 3 of 4 eyes (75%) developed signs of exudation after a mean of 15 ± 9 months, associated with metamorphopsia and visual decline at the time of exudation. Best-corrected visual acuity decreased from 20/25 to 20/40 Snellen equivalent (P = .035) and central macular thickness increased from 268 ± 27 μm to 339 ± 65 μm (P = .145). Three patients were treated with 2.3 ± 0.6 intravitreal injections of anti–vascular endothelial growth factor without significant improvement of best-corrected visual acuity or macular edema.

Conclusions

nePVAC may represent the subclinical pre-exudative stage of ePVAC, notable for an absence of exudation or visual impairment. nePVAC and ePVAC should be considered as part of the same spectrum, namely PVAC. Typically, nePVAC develops signs of exudation over time, causing metamorphopsia and visual decline and therefore these lesions warrant continued close monitoring with multimodal retinal imaging.

Section snippets

Methods

This was a retrospective noncomparative case series comprised of 4 retina referral centers (Medical Retina and Imaging Unit of the Department of Ophthalmology of University Vita-Salute San Raffaele in Milan, Italy; Vitreous Retina Macula Consultants of New York, in New York, New York, USA; Department of Ophthalmology of University Paris Est, in Creteil, France; and the Doheny Eye Institute, David Geffen School of Medicine, University of California, Los Angeles, Los Angeles, California, USA).

Results

Six eyes of 6 white patients fulfilled the inclusion criteria and were included in the current study. One patient was included from the Department of Ophthalmology of University Vita-Salute San Raffaele, 3 from the Vitreous Retina Macula Consultants of New York, 1 from the Department of Ophthalmology of University Paris Est, and 1 from the Doheny Eye Centers in Los Angeles. The mean age of the cohort was 75 ± 10 years (median 75.5 years; range 60-88 years) and 2 patients were female and 4 were

Discussion

In this study, we have described a novel lesion that may represent a subclinical pre-exudative stage of PEVAC, which we propose renaming nePVAC. Importantly, if we have demonstrated the presence of a nonexudative stage, we also proposed to rename the exudative lesion previously known as PEVAC as ePVAC. The latter slight change allows to better distinguish the nonexudative and exudative forms and it reduces possible confusion in distinguishing these 2 lesions.

At baseline, nePVAC lesions

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Histology of Type 3 Macular Neovascularization and Microvascular Anomalies in Treated Age-Related Macular Degeneration: A Case Study
2023, Ophthalmology Science
To investigate intraretinal neovascularization and microvascular anomalies by correlating in vivo multimodal imaging with corresponding ex vivo histology in a single patient.
A case study comprising clinical imaging from a community-based practice, and histologic analysis at a university-based research laboratory (clinicopathologic correlation).
A White woman in her 90s treated with numerous intravitreal anti-VEGF injections for bilateral type 3 macular neovascularization (MNV) secondary to age-related macular degeneration (AMD).
Clinical imaging comprised serial infrared reflectance, eye-tracked spectral-domain OCT, OCT angiography, and fluorescein angiography. Eye tracking, applied to the 2 preserved donor eyes, enabled the correlation of clinical imaging signatures with high-resolution histology and transmission electron microscopy.
Histologic/ultrastructural descriptions and diameters of vessels seen in clinical imaging.
Six vascular lesions were histologically confirmed (type 3 MNV, n = 3; deep retinal age-related microvascular anomalies [DRAMAs], n = 3). Pyramidal (n = 2) or tangled (n = 1) morphologies of type 3 MNV originated at the deep capillary plexus (DCP) and extended posteriorly to approach without penetrating persistent basal laminar deposit. They did not enter the subretinal pigment epithelium (RPE)–basal laminar space or cross the Bruch membrane. Choroidal contributions were not found. The neovascular complexes included pericytes and nonfenestrated endothelial cells, within a collagenous sheath covered by dysmorphic RPE cells. Deep retinal age-related microvascular anomaly lesions extended posteriorly from the DCP into the Henle fiber and the outer nuclear layers without evidence of atrophy, exudation, or anti-VEGF responsiveness. Two DRAMAs lacked collagenous sheaths. External and internal diameters of type 3 MNV and DRAMA vessels were larger than comparison vessels in the index eyes and in aged normal and intermediate AMD eyes.
Type 3 MNV vessels reflect specializations of source capillaries and persist during anti-VEGF therapy. The collagenous sheath of type 3 MNV lesions may provide structural stabilization. If so, vascular characteristics may be useful in disease monitoring in addition to fluid and flow signal detection. Further investigation with longitudinal imaging before exudation onset will help determine if DRAMAs are part of the type 3 MNV progression sequence.
Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.
Perifoveal exudative vascular anomalous complex (PEVAC)
2023, Archivos de la Sociedad Espanola de Oftalmologia
Se presentan 3 casos de pacientes, con 66, 80 y 23 años 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).
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).
Nascent Geographic Atrophy as a Predictor of Type 3 Macular Neovascularization Development
2023, Ophthalmology Retina
To investigate the association of nascent geographic atrophy (GA) preceding the development of exudative type 3 macular neovascularization (MNV) in patients with age-related macular degeneration (AMD).
Retrospective longitudinal study.
Patients with AMD diagnosed with treatment-naive exudative type 3 MNV in 1 or both eyes were evaluated. Inclusion criteria included serial tracked structural OCT examinations for ≥ 2 years before the detection of exudative type 3 MNV.
Clinical characteristics and retinal imaging, including structural OCT at baseline and at each follow-up examination, were analyzed. Eyes showing the presence of nascent GA during the follow-up were selected for analysis of prevalence, and clinical characteristics at the site of subsequent type 3 MNV development.
Description of the prevalence and clinical characteristics of nascent GA at the site of subsequent type 3 MNV development.
Overall, 97 eyes affected by type 3 MNV meeting inclusion criteria were analyzed. Of 97 eyes (71 patients), 22 eyes of 21 patients (mean age 82 ± 9 years) showed nascent GA preceding exudative type 3 MNV. The observed prevalence of nascent GA preceding exudative type 3 MNV was 22.7% (95% confidence interval, 14.4%–31.0%). Exudative type 3 MNV developed a mean of 9 ± 6 months after detection of nascent GA. The presence of reticular pseudodrusen in the study eye did not significantly influence the timing of exudative type 3 MNV development after the observation of nascent GA (P > 0.1 in all analyses). Reduced best-corrected visual acuity was recorded at the exudative type 3 stage in comparison with the nascent GA stage (P = 0.003).
As nascent GA may precede the development of exudative type 3 MNV, the detection of nascent GA in eyes with AMD may warrant closer surveillance to identify early exudative type 3 MNV warranting treatment.
Proprietary or commercial disclosure may be found after the references.
Reply
2023, Ophthalmology Retina
Re: Cabral et al: Volume rendering of deep retinal age-related microvascular anomalies. Ophthalmol Retina (2022;6:1185-1193)
2023, Ophthalmology Retina
Volume Rendering of Deep Retinal Age-Related Microvascular Anomalies
2022, Ophthalmology Retina
Citation Excerpt :
As detected using OCTA, the DRAMA lesions invariably originated at the DCP, whereas PVAC lesions typically exhibit flow signal in both the SCP and DCP. Moreover, perilesional capillary rarefaction, detected using OCTA, which is considered a hallmark of the PVAC spectrum, was not observed in our series.30,32 We believe that the specific features of these non-neovascular lesions merit a descriptive terminology and propose the term “DRAMA” to describe them.
To characterize and distinguish non-neovascular deep retinal age-related microvascular anomalies (DRAMA) from type 3 macular neovascularization (MNV) using volume rendering of OCT and OCT angiography (OCTA).
Retrospective, consecutive case series.
Consecutive patients with age-related macular degeneration (AMD) exhibiting de novo non-neovascular abnormalities within the deep vascular plexus (DCP), as detected using high-resolution (High-Res) spectral-domain (SD) and swept-source (SS) OCT or OCTA. Patients with retinal vascular alterations attributable to other disease entities were excluded.
Complete ophthalmic examination and multimodal imaging, including confocal fundus photography (CFP), SD-OCT, High-Res SD-OCT and OCTA, and volume-averaged SS-OCTA. The volume renderings of High-Res OCTA and averaged SS-OCTA were used to analyze capillary abnormalities and inflow or outflow connectivity pathways.
The primary outcomes were the characteristics of capillary abnormalities (number, size, shape, reflectivity, and location) and inflow or outflow connectivity pathways. The secondary outcomes were nearby changes in CFP and structural OCT (hyperreflective foci [HRF], outer retinal atrophy, and retinal pigment epithelium [RPE] atrophy).
From 8 eyes of 8 patients, 2 subtypes of DRAMA were identified: small-diameter perifoveal capillary dilations with hyperreflective walls within the inner nuclear layer (type 1, n = 4) and vascular outpouchings, typically multiple, extending posteriorly into the Henle fiber layer, with reflectivity similar to adjacent normal retinal capillaries (type 2, n = 10). Four eyes had both subtypes of DRAMA. The 3-dimensional visualization of OCTA data demonstrated DRAMA corresponding to the dilations of DCP capillaries without direct inflow or outflow connections to the superficial plexus. Fundus photographs showed circular red dots in 3 eyes, all corresponding to type 1 DRAMA. In all the cases, DRAMA colocalized with HRF. No lesions were found anterior to the areas of the RPE or outer retina atrophy. Asymptomatic intraretinal exudation varied through a follow-up duration of up to 6 years, with no lesions progressing to type 3 MNV.
In eyes with non-neovascular AMD, DRAMA include 2 types of capillary dilations occurring without the remodeling of the surrounding vascular network. Deep retinal age-related microvascular anomalies can resemble microvascular changes due to other causes and can masquerade as type 3 MNV. Mild intraretinal exudation can vary during follow-up, without progression to type 3 MNV.

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Original ArticleNonexudative Perifoveal Vascular Anomalous Complex: The Subclinical Stage of Perifoveal Exudative Vascular Anomalous Complex?

Purpose

Design

Methods

Results

Conclusions

Section snippets

Methods

Results

Discussion

J Fr Ophtalmol

Am J Ophthalmol

Am J Ophthalmol Case Rep

Ophthalmol Retina

Am J Ophthalmol

Ophthalmology

Ophthalmol Retina

Insights into perifoveal exudative vascular anomalous complex

Retina

Characteristics of perifoveal exudative vascular anomalous complex in Korean patients

Semin Ophthalmol

Perifoveal exudative vascular anomalous complex-like lesion as a complication of prepapillary arterial loops

Ophthalmic Surg Lasers Imaging Retina

Original Article
Nonexudative Perifoveal Vascular Anomalous Complex: The Subclinical Stage of Perifoveal Exudative Vascular Anomalous Complex?