Clinical characteristics and multimodal imaging of perifoveal exudative vascular anomalous complex_Chinese Journal of Ocular Fundus Diseases

Authors：

Tang Jiyang , Han Xinyao , Tang Ran , Zhang Linqi , Qi Huijun , Zhao Mingwei ,  Qu Jinfeng

Department of Ophthalmology, Peking University People's Hospital, Eye Diseases and Optometry Institute, Beijing Key Laboratory of Diagnosis and Therapy of Retinal and Choroid Diseases, College of Optometry, Peking University Health Science Center, Beijing 100044, China;

Corresponding author：

Qu Jinfeng, Email: drbari@163.com

Keywords：

Perifoveal exudative vascular anomalous complex; Multimodal imaging characteristics; Tomography, optical coherence

DOI：

10.3760/cma.j.cn511434-20211206-00685

Video：

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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.

Citation： Tang Jiyang, Han Xinyao, Tang Ran, Zhang Linqi, Qi Huijun, Zhao Mingwei, Qu Jinfeng. Clinical characteristics and multimodal imaging of perifoveal exudative vascular anomalous complex. Chinese Journal of Ocular Fundus Diseases, 2022, 38(11): 885-890. doi: 10.3760/cma.j.cn511434-20211206-00685 Copy

1.	Querques G, Kuhn D, Massamba N, et al. Perifoveal exudative vascular anomalous complex[J]. J Fr Ophtalmol, 2011, 34(8): 559. DOI: 10.1016/j.jfo.2011.03.002.
2.	魏文文, 于伟泓, 李略. 黄斑中心凹旁渗出性血管异常复合体一例[J]. 中华眼科杂志, 2019, 55(6): 458-459. DOI: 10.3760/cma.j.issn.0412-4081.2019.06.010.Wei WW, Yu WH, Li L. Parafoveal exudative vascular complex: a case report[J]. Chin J Ophthalmol, 2019, 55(6): 458-459. DOI: 10.3760/cma.j.issn.0412-4081.2019.06.010.
3.	Zhang Z, Xu L, Wu Z, et al. Case report: perifoveal exudative vascular anomalous complex in a Chinese patient with diabetes mellitus[J]. Optom Vis Sci, 2019, 96(7): 531-535. DOI: 10.1097/OPX.0000000000001401.
4.	Sacconi R, Freund KB, Yannuzzi LA, et al. The expanded spectrum of perifoveal exudative vascular anomalous complex[J]. Am J Ophthalmol, 2017, 184: 137-146. DOI: 10.1016/j.ajo.2017.10.009.
5.	Mrejen S, Le HM, Nghiem-Buffet S, et al. Insights into perifoveal exudative vascular anomalous complex[J]. Retina, 2020, 40(1): 80-86. DOI: 10.1097/IAE.0000000000002435.
6.	Kim JH, Kim JW, Kim CG, et al. Characteristics of perifoveal exudative vascular anomalous complex in Korean patients[J]. Semin Ophthalmol, 2019, 34(5): 353-358. DOI: 10.1080/08820538.2019.1626450.
7.	Verhoekx JSN, Smid LM, Vermeer KA, et al. Anatomical changes on sequential multimodal imaging in perifoveal exudative vascular anomalous complex[J]. Retina, 2021, 41(1): 162-169. DOI: 10.1097/IAE.0000000000002809.
8.	Henriques J, Pinto F, Rosa PC, et al. Continuous wave milipulse yellow laser treatment for perifoveal exudative vascular anomalous complex-like lesion: a case report[J]. Eur J Ophthalmol, 2022, 32(1): 119-124. DOI: 10.1177/1120672120966564.
9.	Sacconi R, Borrelli E, Sadda S, et al. Nonexudative perifoveal vascular anomalous complex: the subclinical stage of perifoveal exudative vascular anomalous complex?[J]. Am J Ophthalmol, 2020, 218: 59-67. DOI: 10.1016/j.ajo.2020.04.025.
10.	Smid LM, Verhoekx JSN, Martinez Ciriano JP, et al. Multimodal imaging comparison of perifoveal exudative vascular anomalous complex and resembling lesions[J]. Acta Ophthalmol, 2021, 99(5): 553-558. DOI: 10.1111/aos.14650.
11.	Fernández-Vigo JI, Burgos-Blasco B, Dolz-Marco R, et al. Atypical perifoveal exudative vascular anomalous complex (PEVAC) with multifocal and bilateral presentation[J/OL]. Am J Ophthalmol Case Rep, 2020, 18: 100717[2020-04-21]. https://pubmed.ncbi.nlm.nih.gov/32368691/. DOI: 10.1016/j.ajoc.2020.100717.
12.	Bourhis A, Girmens JF, Boni S, et al. Imaging of macroaneurysms occurring during retinal vein occlusion and diabetic retinopathy by indocyanine green angiography and high resolution optical coherence tomography[J]. Graefe's Arch Clin Exp Ophthalmol, 2010, 248(2): 161-166. DOI: 10.1007/s00417-009-1175-6.
13.	Sacconi R, Sarraf D, Garrity S, et al. Nascent type 3 neovascularization in age-related macular degeneration[J]. Ophthalmol Retina, 2018, 2(11): 1097-1106. DOI: 10.1016/j.oret.2018.04.016.
14.	Takayama K, Ooto S, Tamura H, et al. Retinal structural alterations and macular sensitivity in idiopathic macular telangiectasia type 1[J]. Retina, 2012, 32(9): 1973-1980. DOI: 10.1097/IAE.0b013e318251a38b.

1. Querques G, Kuhn D, Massamba N, et al. Perifoveal exudative vascular anomalous complex[J]. J Fr Ophtalmol, 2011, 34(8): 559. DOI: 10.1016/j.jfo.2011.03.002.
2. 魏文文, 于伟泓, 李略. 黄斑中心凹旁渗出性血管异常复合体一例[J]. 中华眼科杂志, 2019, 55(6): 458-459. DOI: 10.3760/cma.j.issn.0412-4081.2019.06.010.Wei WW, Yu WH, Li L. Parafoveal exudative vascular complex: a case report[J]. Chin J Ophthalmol, 2019, 55(6): 458-459. DOI: 10.3760/cma.j.issn.0412-4081.2019.06.010.
3. Zhang Z, Xu L, Wu Z, et al. Case report: perifoveal exudative vascular anomalous complex in a Chinese patient with diabetes mellitus[J]. Optom Vis Sci, 2019, 96(7): 531-535. DOI: 10.1097/OPX.0000000000001401.
4. Sacconi R, Freund KB, Yannuzzi LA, et al. The expanded spectrum of perifoveal exudative vascular anomalous complex[J]. Am J Ophthalmol, 2017, 184: 137-146. DOI: 10.1016/j.ajo.2017.10.009.
5. Mrejen S, Le HM, Nghiem-Buffet S, et al. Insights into perifoveal exudative vascular anomalous complex[J]. Retina, 2020, 40(1): 80-86. DOI: 10.1097/IAE.0000000000002435.
6. Kim JH, Kim JW, Kim CG, et al. Characteristics of perifoveal exudative vascular anomalous complex in Korean patients[J]. Semin Ophthalmol, 2019, 34(5): 353-358. DOI: 10.1080/08820538.2019.1626450.
7. Verhoekx JSN, Smid LM, Vermeer KA, et al. Anatomical changes on sequential multimodal imaging in perifoveal exudative vascular anomalous complex[J]. Retina, 2021, 41(1): 162-169. DOI: 10.1097/IAE.0000000000002809.
8. Henriques J, Pinto F, Rosa PC, et al. Continuous wave milipulse yellow laser treatment for perifoveal exudative vascular anomalous complex-like lesion: a case report[J]. Eur J Ophthalmol, 2022, 32(1): 119-124. DOI: 10.1177/1120672120966564.
9. Sacconi R, Borrelli E, Sadda S, et al. Nonexudative perifoveal vascular anomalous complex: the subclinical stage of perifoveal exudative vascular anomalous complex?[J]. Am J Ophthalmol, 2020, 218: 59-67. DOI: 10.1016/j.ajo.2020.04.025.
10. Smid LM, Verhoekx JSN, Martinez Ciriano JP, et al. Multimodal imaging comparison of perifoveal exudative vascular anomalous complex and resembling lesions[J]. Acta Ophthalmol, 2021, 99(5): 553-558. DOI: 10.1111/aos.14650.
11. Fernández-Vigo JI, Burgos-Blasco B, Dolz-Marco R, et al. Atypical perifoveal exudative vascular anomalous complex (PEVAC) with multifocal and bilateral presentation[J/OL]. Am J Ophthalmol Case Rep, 2020, 18: 100717[2020-04-21]. https://pubmed.ncbi.nlm.nih.gov/32368691/. DOI: 10.1016/j.ajoc.2020.100717.
12. Bourhis A, Girmens JF, Boni S, et al. Imaging of macroaneurysms occurring during retinal vein occlusion and diabetic retinopathy by indocyanine green angiography and high resolution optical coherence tomography[J]. Graefe's Arch Clin Exp Ophthalmol, 2010, 248(2): 161-166. DOI: 10.1007/s00417-009-1175-6.
13. Sacconi R, Sarraf D, Garrity S, et al. Nascent type 3 neovascularization in age-related macular degeneration[J]. Ophthalmol Retina, 2018, 2(11): 1097-1106. DOI: 10.1016/j.oret.2018.04.016.
14. Takayama K, Ooto S, Tamura H, et al. Retinal structural alterations and macular sensitivity in idiopathic macular telangiectasia type 1[J]. Retina, 2012, 32(9): 1973-1980. DOI: 10.1097/IAE.0b013e318251a38b.

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