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Year : 2021  |  Volume : 1  |  Issue : 2  |  Page : 323-325

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

Smt Kanuri Santhamma Center for Vitreo-Retinal Diseases, L V Prasad Eye Institute, Hyderabad, Telangana, India

Date of Submission28-May-2020
Date of Acceptance15-Dec-2020
Date of Web Publication01-Apr-2021

Correspondence Address:
Dr. Komal Agarwal
Smt. Kanuri Santhamma Centre for Vitreo-Retinal Diseases, L V Prasad Eye Institute, Banjara Hills, Hyderabad - 500 034, Telangana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_1706_20

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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.

Keywords: Focal laser, perifoveal exudative vascular anomalous complex (PEVAC)

How to cite this article:
Reddy S, Agrawal H, Agarwal K. Focal laser photocoagulation in the management of perifoveal exudative vascular anomalous complex. Indian J Ophthalmol Case Rep 2021;1:323-5

How to cite this URL:
Reddy S, Agrawal H, Agarwal K. Focal laser photocoagulation in the management of perifoveal exudative vascular anomalous complex. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2022 Dec 5];1:323-5. Available from: https://www.ijoreports.in/text.asp?2021/1/2/323/312338

Perifoveal exudative vascular abnormalities can occur in association with a variety of retinal disorders such as retinal vascular occlusion, type 1 macular telangiectasia's, retinal vascular diseases like diabetic retinopathy and hypertensive retinopathy.[1] Querques et al. first described perifoveal exudative vascular anomalous complex (PEVAC) in two otherwise healthy patients without any other associated retinal diseases, defining these as unilateral, isolated, perifoveal aneurysm with adjacent exudative maculopathy.[2]

Existing literature reports poor response of PEVAC to anti-VEGF injections.[2],[3],[4] Role of focal laser photocoagulation in PEVAC remains unexplored due to the perifoveal nature of the lesion. Herein, we report a case of PEVAC and its course of resolution with focal laser photocoagulation.

  Case Report Top

A 33-year-young woman presented to us with gradual diminution of vision in her right eye since 1 year. Systemic examination was essentially normal without any vascular disorders. The best-corrected visual acuity (BCVA) was 20/50, N12 in the right eye and 20/20, N6 in the left eye. Anterior segment and intraocular pressure (IOP) was normal in both eyes. Fundus examination of her right eye showed hard exudates around the fovea [Figure 1]a on the nasal half without any vascular anomolies. Left eye fundus was essentially normal. Optical coherence tomography (OCT) of the right eye showed well-defined cystic space, 800 μ away from the center of the fovea, extending from ganglion cell layer to inner nuclear layer with hyperreflective wall with heterogenous reflectivity in the central lumen, surrounding cystic spaces and hard exudates [Figure 1]b and [Figure 1]c. Fundus fluorescein angiography (FFA) showed well defined early hyperfluorescent lesion which did not leak profusely in the late phase [Figure 1]d and [Figure 1]e. A similar well-defined hyperfluorescent lesion was noted on ICG angiography [Figure 1]g. OCT angiography (OCTA) showed singular high flow signal in deep capillary plexus [Figure 1]f. No other vascular anomalies were noted.
Figure 1: Fundus photograph of the right eye showing hard exudates around the fovea. (a). OCT passing through the lesion showing a well defined hyperreflective cystic space with heterogenous reflectivity in the central lumen (yellow arrow) and surrounding intraretinal cystic spaces. (b). B scan of OCTA showing high flow signals through the PEVAC lesion (yellow arrow) (c). Fundus fluorescein angiogram showing a well-defined hyperfluorescence (yellow arrow) in the early phase with mild leakage in the later phase (d and e). Deep capillary plexus segment on OCTA showing single hyper-reflective round lesion (yellow arrow) (f). Single hyperfluorecent lesion seen on late phase of ICG angiography (g)

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The patient was diagnosed with PEVAC and FFA guided focal laser was done with 577 nm laser to PEVAC lesion (3 spots, grade 2 burn, 100 μm spot size, 60 mW power, 40 ms duration). At the 1 month follow-up, presence of a small neurosensory detachment (NSD) and collapsed cystic space corresponding to the PEVAC [Figure 2]a and [Figure 2]b was noted. No intervention was done at this visit. Three months later, BCVA in the right eye improved to 20/20P N6. Fundus showed hard exudates similar to that on presentation [Figure 2]c. The OCT depicted collapsed PEVAC lesion, resolved cystic spaces, and NSD [Figure 2]d. She did not complain of any scotoma. The patient was subsequently asked to follow up after 3 months.
Figure 2: Fundus photo and corresponding OCT 1 month post focal laser showing collapsed PEVAC lesion, few intraretinal cysts and a small neurosensory detachment. (a and b). Fundus photo and corresponding OCT 3 months post focal laser showing collapsed PEVAC and resolved intra and subretinal fluid (c and d)

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  Discussion Top

PEVAC is defined as unilateral, isolated, perifoveal aneurysm in otherwise healthy patients.[2] Although the pathogenesis of such localized aneurysms is not clear, it is hypothesized to be due to progressive retinal endothelial cell degeneration.[2] Spaide et al. introduced the term Retinal capillary macroaneurysms for solitary exudative lesions of size >200 μ for lesions looking similar to what was originally proposed as PEVAC.[5] The description of our case, where the lesion size was 158 μ, tends to corroborate with the original description of PEVAC.

Anti-VEGF agents have been tried for exudation associated with PEVAC, with limited success. Sacconi et al. in their series reported no significant difference in BCVA and central macular thickness in patients receiving anti-VEGF treatment versus those who were observed.[2] Similar poor response was noted by multiple authors in their series of patients with PEVAC.[4],[6],[7],[8] Based on such a poor response to anti-VEGF agents, it has been hypothesized that the focal vasculopathy in PEVAC is due to mechanisms not necessarily dependent on VEGF. It has been hypothesized that breakdown of basement membrane proteins due to increased expression of matrix metalloproteinase -9 leads to pericyte loss and subsequent aneursymal changes.[5] Such a mechanism is not dependent on VEGF and hence explaining poor response to anti-VEGF. We did not treat our patient with anti-VEGF's.

Focal laser photocoagulation to the PEVAC lesion has been tried by some authors with good and stable results.[4],[9] Laser photocoagulation acts by the coagulation necrosis of the lesion, similar to microaneurysms/macroaneurysms in other retinal vascular diseases, and the surrounding edema slowly resolves. Our patient showed a good response to focal laser photocoagulation. The initial appearance of NSD might be a transient response to the laser which resolved spontaneously over 3 months. However, the exact reason cannot be established. Concerns have been raised due to the perifoveal location of the lesion, which might lead to paracentral scotomas. Such a complaint was not noted in our case.

In a recent article, three cases of spontaneous resolution of PEVAC lesion was reported.[10] It can hence, be argued that the whether the resolution seen in our case was a part of the natural history of the disease. It is, therefore, important to note that resolution of PEVAC lesion in our case was seen in a period of 4 months which is much less as compared to the existing literature (ranging from 11-22 months). This points toward a role of laser in hastening the resolution. We also noted improvement in BCVA.

  Conclusion Top

In conclusion, thermal focal laser seems to be an effective treatment in the management of PEVAC. Although special caution should be taken to avoid inadvertent complications.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Support provided by Hyderabad Eye Research Foundation, Hyderabad, India. The funders had no role in the preparation, review or approval of the manuscript.

Conflicts of interest

There are no conflicts of interest.

  References Top

Bourhis A, Girmens JF, Boni S, Pecha F, Favard C, Sahel JA, et al. Imaging of macroaneurysms occurring during retinal vein occlusion and diabetic retinopathy by indocyanine green angiography and high resolution optical coherence tomography. Graefes Arch Clin Exp Ophthalmol 2010;248:161-6.  Back to cited text no. 1
Querques G, Kuhn D, Massamba N, Leveziel N, Querques L, Souied EH. Perifoveal exudative vascular anomalous complex. J Fr Ophtalmol 2011;34:559.e1-4.  Back to cited text no. 2
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;184:137-46.  Back to cited text no. 3
Mrejen-Uretsky S, Le HM, Nghiem-Buffet S, Tabary S, Quentel G, Cohen SY. Insights into perifoveal exudative vascular anomalous complex. Retina 2018;40:80-6.  Back to cited text no. 4
Spaide RF, Barquet LA. Retinal capillary macroaneurysms. Retina 2019;39:1889-95.  Back to cited text no. 5
Kim JH, Kim JW, Kim CG, Lee DW. Characteristics of perifoveal exudative vascular anomalous complex in Korean patients. Semin Ophthalmol 2019;34:353-8.  Back to cited text no. 6
Zhang Z, Xu L, Wu Z, Zhang J. Case report: Perifoveal exudative vascular anomalous complex in a Chinese patient with diabetes mellitus. Optom Vis Sci 2019;96:531-5.  Back to cited text no. 7
Fernández-Vigo JI, Burgos-Blasco B, Dolz-Marco R, Jiménez-Santos M, López-Guajardo L, Donate-López J. Atypical perifoveal exudative vascular anomalous complex (PEVAC) with multifocal and bilateral presentation. Am J Ophthalmol Case Rep 2020;18:100717.  Back to cited text no. 8
Ayachit AG, Sacconi R, Ayachit GS. Perifoveal exudative vascular anomalous complex-like lesion as a complication of prepapillary arterial loops. Ophthalmic Surg Lasers Imaging Retina 2018;49:974-8.  Back to cited text no. 9
Verhoekx JSN, Smid LM, Vermeer KA, Martinez Ciriano JP, Yzer S. Anatomical changes on sequential multimodal imaging in perifoveal exudative vascular anomalous complex. Retina 2020;41:162-9.  Back to cited text no. 10


  [Figure 1], [Figure 2]

This article has been cited by
1 A Review of Perifoveal Exudative Vascular Anomalous Complex
Kevin Zhang, Aleksandra Rachitskaya
Current Ophthalmology Reports. 2022;
[Pubmed] | [DOI]


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