First report of perifoveal exudative vascular anomalous complex in a healthy Chinese individual: a case description
Introduction
Perifoveal exudative vascular anomalous complex (PEVAC) was first reported in 2011 by Querques et al. (1) and was defined as a perifoveal isolated aneurysm, similar to a microaneurysm. It may be accompanied by retinal hemorrhage and exudation without retinal vascular or inflammatory disease. Since it was first discovered, few PEVAC cases have been reported, and there is scant knowledge concerning this disease and its treatment. To our knowledge, we here report the first case of PEVAC in a healthy Chinese individual who had no general disease or eye problems. We hope this case report can serve as a reminder to ophthalmologists of this fundus oculi disease and stimulate further research into PEVAC.
Case presentation
All procedures performed in this study were in accordance with the ethical standards of Zhejiang Hospital Ethics Committee and with the Helsinki Declaration (as revised in 2013). Written informed consent was obtained from the patient for publication of this article and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.
A healthy 53-year-old Chinese woman complained of blurry vision for almost half a year in her left eye. Therefore, she was referred to see a doctor in March 2020. The patient was healthy without hepatitis, tuberculosis, hypertension, diabetes, heart disease, hemopathy, or any other hereditary disease. She denied any remarkable ophthalmological treatment, such as laser therapy, drug injection history in vitreous cavity, or photodynamic therapy, and denied any other ocular surgery. The patient underwent a detailed ophthalmic examination at the department of ophthalmology in Zhejiang Hospital. The best-corrected visual acuity (BCVA) of the left eye (20/25) was weaker than that of the right eye (20/20). Intraocular pressure was normal (14.3 mmHg for the right eye and 15.7 mmHg for the left eye). Slit-lamp microscope examination of the cornea, iris, lens, pupil, anterior chamber, etc., were normal for both eyes. The fundus of the right eye was unremarkable. However, the left eye showed an isolated neovascular lesion temporal to the fovea surrounded by small intraretinal hard exudate (Figure 1). Optical coherence tomography (OCT) revealed an oval lesion with a hyperreflective wall surrounding a dark lumen containing variable internal reflectivity and hard exudate (Figure 2). The lesion was consistent with the perifoveal vascular alteration according to the characteristic fundus changes and was located between the outer nuclear layer (ONL) and the inner plexiform layer (IPL). Optical coherence tomography angiography (OCTA) showed an isolated dilation temporal to the fovea with detectable blood flow in both the superficial capillary plexus (SCP) and deep capillary plexus (DCP) correlating with the lesion (Figures 3,4). Fluorescein fundus angiography (FFA) showed a sharply marginated hyperfluorescent lesion in the early phase characterized by isolated aneurysmal dilation, with a small degree of leakage in the late phase. Indocyanine green angiography (ICGA) showed a hyperfluorescent lesion but without leakage in the late phase (Figure 5). However, none of these examinations, including OCTA, OCT, FFA, and ICGA, indicated choroidal neovascularization (CNV).
The patient provided an OCT result from 1 year prior when she was underdoing regular physical exams. This revealed that the lesion was present 1 year prior but was smaller, and the BCVA of left eye was normal (20/20). The patient had not seen a doctor for an extended period, and thus this issue received little medical attention.
On the basis of these above mentioned characteristics, we diagnosed the patient’s disease as PEVAC. The lesion in this patient developed slowly and did little to reduce the function of the involved eye. Considering the potential for damage to the near adjacent fovea, we did not believe laser coagulation to be appropriate. As previous studies have reported uncertainty regarding the efficacy of anti-vascular endothelial growth factor (anti-VEGF) therapy (1,2), we also did not attempt anti-VEGF therapy, and decided upon a follow-up visit instead.
Discussion
PEVAC was first reported in 2011 by Querques et al. (1). Although it usually occurs in patients with diseases of the fundus oculi, such as diabetic retinopathy, macular degeneration, and high myopia, experts generally agree that it is a separate disease due to its lack of retinal ischemia and inflammation (2). Several reports consider PEVAC to be a milder form of macular telangiectasia type 1 (Mac Tel 1) (3). Besides aneurysmal lesion, it is usually accompanied by hemorrhage, hard exudate, and macular edema. Regarding the location on OCT, this varies across the published reports. In the 15 cases reported by Sacconi et al. (2), the lesion was located between inner nuclear layer (INL) and the outer plexiform layer (OPL), and in a small portion of cases, the location was between the IPL and the ganglion cells. Kim et al. (4) reported 8 Korean cases, with the majority of locations being between the IPL and ONL or between the INL and ONL and a small portion being between the IPL and the ganglion cells. Consistent with Kim et al.’s study, in our case, the lesion was located between the IPL and ONL. We thought the location was possibly not unique, however, the common characteristic was that the lesion always did not break through the retinal pigment epithelium (RPE). And PEVAC usually exhibits characteristic fundus changes, especially on OCT. PEVAC appears as an oval lesion with a hyperreflective wall and relatively dark lumen. We could see a perifoveal isolated aneurismal change in the fundus and absence of any choroidal vascular abnormality. On OCTA, we observed blood flow in both the DCP and SCP, in line with reports by Sacconi et al. (2) and Mrejen et al. (3).
Without sufficient knowledge of PEVAC, it may be easy for clinicians to misdiagnose this disease. Indeed, most of the cases reported by Mrejen et al. (3) and Sacconi et al. (2) were initially misdiagnosed, and Kim et al. (4) reported 8 cases that were misdiagnosed. Macular neovascularization (MNV) type 3 and Mac Tel 1 were the most common misdiagnoses, and some speculate that PEVAC may be an early stage of MNV type 3 (5).
In our case, the OCT images were critically informative. PEVAC showed an oval lesion with a hyper reflective wall surrounding a dark lumen containing variable internal reflectivity. Type 3 MNV typically shows a hyperreflective lesion without a hyper reflective wall, progressing to the RPE and showing more exudation. In our case, the PEVAC lesion was limited within the retinal layers, while the lesion in type 3 MNV typically progresses to disrupt the outer retinal and disruption, even proliferating through the RPE and causing pigment epithelial detachment (PED). Different from Mac Tel 1, which is more prevalent among young people, PEVAC more often occurs in older adults. The characteristic changes on OCT are also highly distinct. Mac Tel 1 typically features not only capillary aneurysms but also arteriolar, venular, and multiple capillary aneurysms. However, PEVAC is only associated with capillary aneurysms, and in this way, may be thought of as a minor manifestation of MacTel 1.
In our case and similar reports, PEVAC developed very slowly (2,6). Most cases were stable both in anatomy and function; therefore, given the lack of consensus regarding effective measures, treatment was not considered to be urgent. When a retinal fluid sac or macular edema is present, anti-VEGF or thermal laser photocoagulation should be considered. However, thermal laser photocoagulation is limited by location. Mrejen et al. (3) reported one case in which thermal laser photocoagulation was effective for improving macular edema and one case in which 13 sessions of anti-VEGF therapy provided stable vision and reduced macular edema. However, most cases showed no response to anti-VEGF treatment (1,2,6,7), which also distinguishes PEVAC from MNV type 3 and Mac Tel 1. In seven reported cases of PEVAC, the retinal fluid sac resolved without any treatment and disappeared in 3 cases. In our study, the patient’s vision was slightly impaired, but there was no retinal fluid sac or macular edema. Considering the stable progression, we chose follow-up instead of active treatment.
The pathogenesis of PEVAC is not clear, with some speculating (2) that it is a vascular anomaly of the macular retina and others suggesting that it is a reaction to the slow retinal blood flow between the ramus communicans and interarterial loops (8). Overall, greater attention should be paid to this disease to avoid misdiagnosis and to clarify its pathogenesis and treatment.
Acknowledgments
Funding: This work was supported by
Footnote
Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://qims.amegroups.com/article/view/10.21037/qims-23-1655/coif). The authors have no conflicts of interest to declare.
Ethical Statement: The authors are responsible for ensuring that all aspects of the work are investigated and resolved concerning questions regarding accuracy or integrity. All procedures performed in this study were in accordance with the ethical standards of Zhejiang Hospital Ethics Committee and with the Declaration of Helsinki (as revised in 2013). Written informed consent was obtained from the patient for publication of this article and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.
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