Multimodal imaging features of perifoveal exudative vascular anomalous complex_Chinese Journal of Ocular Fundus Diseases

Authors：

Duan Hongtao , Li Ying , Xie Shiyong , Li Li , Cong Chunxia , Wang Jian ,  Han Mei

Tianjin Eye Hospital, Nankai University Affiliated Eye Hospital, Clinical College of Ophthalmology, Tianjin Medical University, Tianjin Key Laboratory of Ophthalmology and Visual Science, Tianjin Eye Institute, Tianjin 300020, China;

Corresponding author：

Han Mei, Email: hanmay69@sina.com

Keywords：

Parafoveal exudative vascular anomaly complex; Multimodal imaging features; Tomography, optical coherence

DOI：

10.3760/cma.j.cn511434-20220211-00079

Video：

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Objective To observe the multimodal imaging features and explore the treatment of parafoveal exudative vascular anomaly complex (PEVAC). Methods A retrospective study. Six patients (6 eyes) with PEVAC diagnosed in Tianjin Eye Hospital were included in this study from July 2018 to December 2021. All patients were female with monocular disease. The age was (61.1±9.3) years. All patients showed a sudden painless decline in monocular vision with metamorphopsia. All patients underwent best corrected visual acuity (BCVA), color fundus photography, fundus fluorescein angiography (FFA), optical coherence tomography (OCT) and OCT angiography (OCTA). Indocyanine green angiography (ICGA) was performed in 4 eyes. In 6 eyes, 3 eyes were treated with intravitreal injection of anti-vascular endothelial growth factor drug; 5 eyes were treated with micropulse laser photocoagulation and/or local thermal laser photocoagulation; 1 eye was treated with photodynamic therapy. Five patients were followed up for (9.2±7.4) months, and 1 patient was lost. At follow-up, the same equipment and methods were used as at the initial diagnosis. The clinical manifestations, multimodal image features and treatment response were observed. Results Baseline BCVA of affected eyes were ranged from 0.1 to 0.5. PEVAC was isolated in 6 eyes, and the fundus showed isolated hemangioma-like leision, accompanied by small bleeding and hard exudation. There were 2 isolated hemangiomatous lesions adjacent to each other in 2 eyes. In the early stage of FFA, punctate high fluorescence lesions near the macular fovea were seen, and the leakage was enhanced in the late stage. There was no leakage in the early stage of ICGA, or slight leakage with late scouring. OCT showed an oval lesion with high reflection wall and uneven low reflection. The central macular thickness (CMT) was (431±76) μm. OCTA showed blood flow signals in PEVAC, 2 eyes in the superficial capillary plexus (SCP), and it was also observed in the deep capillary plexus (DCP), but the intensity of blood flow signal was slightly weaker than that in the SCP. The blood flow signal was visible only in DCP in 2 eyes. SCP and DCP showed similar intensity of blood flow signals in 2 eyes. After treatment, the bleeding was absorbed basically in 4 eyes, the hard exudation partially subsided, the CMT decreased, the intercortical cystic cavity of the fovea nerve decreased, the hemangiomatous lesions narrowed, and BCVA increased. In 1 eye, the macular sac was reduced and partially absorbed by hard exudation, which was later relapsed due to blood pressure fluctuation.Conclusions The majority of PEVAC patients had monocular onset. The fundus is characterized by solitary or structure with strong reflex walls, with or without retinal cysts, hard exudates, and subretinal fluid, and visible blood flow signals inside.

Citation： Duan Hongtao, Li Ying, Xie Shiyong, Li Li, Cong Chunxia, Wang Jian, Han Mei. Multimodal imaging features of perifoveal exudative vascular anomalous complex. Chinese Journal of Ocular Fundus Diseases, 2023, 39(5): 394-400. doi: 10.3760/cma.j.cn511434-20220211-00079 Copy

1.	Querques G, Kuhn D, Massamba N, et al. Perifoveal exudative vascular anomalous complex[J]. J Fr Ophtalmol, 2011, 34(8): 551-554. DOI:10.1016/j.jfo.2011.03.002.
2.	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.
3.	Venkatesh R, Yadav NK, Bavaharan B, et al. Multimodal imaging in perifoveal exudative vascular anomalous complex with co-existent diabetic retinopathy[J]. Clin Exp Optom, 2019, 102(5): 528-532. DOI: 10.1111/cxo.12868.
4.	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.
5.	Yannuzzi LA, Bardal AM, Freund KB, et al. Idiopathic macular telangiectasia[J]. Arch Ophthalmol, 2006, 124(4): 450-460. DOI: 10.1001/archopht.124.4.450.
6.	Yannuzzi LA, Negrao S, Iida T, et al. Retinal angiomatous proliferation in age-related macular degeneration[J]. Retina, 2001, 21(5): 416-434. DOI: 10.1097/00006982-200110000-00003.
7.	Fernandez-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://linkinghub.elsevier.com/retrieve/pii/S2451-9936(20)30071-2. DOI:10.1016/j.ajoc.2020.100717.
8.	Siedlecki J, Vounotrypidis E, Vogt D, et al. Lamellar hole-associated epiretinal proliferation presenting with perifoveal exudative vascular anomalous complex[J]. Am J Ophthalmol Case Rep, 2019, 14: 112-116. DOI: 10.1016/j.ajoc.2019.03.008.
9.	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.
10.	Corvi F, Corradetti G, Juhn A, et al. Long-term follow-up of perifoveal exudative vascular anomalous complex treated with intravitreal injections of anti-vascular endothelial growth factor and thermal laser photocoagulation[J/OL]. Am J Ophthalmol Case Rep, 2020, 20: 100883[2020-08-20]. https://linkinghub.elsevier.com/retrieve/pii/S2451-9936(20)30198-5. DOI:10.1016/j.ajoc.2020.100883.
11.	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.
12.	Verhoekx JSN, Smid LM, Vermeer KA, et al. Comment on: nonexudative perifoveal vascular anomalous complex: the subclinical stage of perifoveal exudative vascular anomalous complex?[J]. Am J Ophthalmol, 2021, 223: 450-451. DOI: 10.1016/j.ajo.2020.10.010.
13.	Sacconi R, Borrelli E, Querques L, et al. Reply to: Non-exudative perifoveal vascular anomalous complex: the subclinical stage of perifoveal exudative vascular anomalous complex?[J]. Am J Ophthalmol, 2021, 223: 159. DOI: 10.1016/j.ajo.2020.10.009.

1. Querques G, Kuhn D, Massamba N, et al. Perifoveal exudative vascular anomalous complex[J]. J Fr Ophtalmol, 2011, 34(8): 551-554. DOI:10.1016/j.jfo.2011.03.002.
2. 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.
3. Venkatesh R, Yadav NK, Bavaharan B, et al. Multimodal imaging in perifoveal exudative vascular anomalous complex with co-existent diabetic retinopathy[J]. Clin Exp Optom, 2019, 102(5): 528-532. DOI: 10.1111/cxo.12868.
4. 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.
5. Yannuzzi LA, Bardal AM, Freund KB, et al. Idiopathic macular telangiectasia[J]. Arch Ophthalmol, 2006, 124(4): 450-460. DOI: 10.1001/archopht.124.4.450.
6. Yannuzzi LA, Negrao S, Iida T, et al. Retinal angiomatous proliferation in age-related macular degeneration[J]. Retina, 2001, 21(5): 416-434. DOI: 10.1097/00006982-200110000-00003.
7. Fernandez-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://linkinghub.elsevier.com/retrieve/pii/S2451-9936(20)30071-2. DOI:10.1016/j.ajoc.2020.100717.
8. Siedlecki J, Vounotrypidis E, Vogt D, et al. Lamellar hole-associated epiretinal proliferation presenting with perifoveal exudative vascular anomalous complex[J]. Am J Ophthalmol Case Rep, 2019, 14: 112-116. DOI: 10.1016/j.ajoc.2019.03.008.
9. 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.
10. Corvi F, Corradetti G, Juhn A, et al. Long-term follow-up of perifoveal exudative vascular anomalous complex treated with intravitreal injections of anti-vascular endothelial growth factor and thermal laser photocoagulation[J/OL]. Am J Ophthalmol Case Rep, 2020, 20: 100883[2020-08-20]. https://linkinghub.elsevier.com/retrieve/pii/S2451-9936(20)30198-5. DOI:10.1016/j.ajoc.2020.100883.
11. 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.
12. Verhoekx JSN, Smid LM, Vermeer KA, et al. Comment on: nonexudative perifoveal vascular anomalous complex: the subclinical stage of perifoveal exudative vascular anomalous complex?[J]. Am J Ophthalmol, 2021, 223: 450-451. DOI: 10.1016/j.ajo.2020.10.010.
13. Sacconi R, Borrelli E, Querques L, et al. Reply to: Non-exudative perifoveal vascular anomalous complex: the subclinical stage of perifoveal exudative vascular anomalous complex?[J]. Am J Ophthalmol, 2021, 223: 159. DOI: 10.1016/j.ajo.2020.10.009.

Chinese Journal of Ocular Fundus Diseases

Multimodal imaging features of perifoveal exudative vascular anomalous complex

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