The Expanded Spectrum of Perifoveal Exudative Vascular Anomalous Complex

R Sacconi, KB Freund, LA Yannuzzi, R Dolz-Marco, E Souied, V Capuano, O Semoun, N Phasukkijwatana, D Sarraf, Adriano Carnevali, L Querques, F Bandello, G Querques

Research output: Contribution to journalArticle

7 Citations (Scopus)

Abstract

Purpose To expand our understanding of the uncommon entity, referred to as perifoveal exudative vascular anomalous complex (PEVAC) by describing multimodal imaging findings, including optical coherence tomography angiography (OCT-A). Design Retrospective cohort study. Methods Patients diagnosed with PEVAC were identified at 4 retina referral centers worldwide and underwent complete ophthalmologic examination including structural OCT, OCT-A, fluorescein angiography (FA), and indocyanine green angiography (ICGA). Demographics and clinical findings were analyzed at baseline and at available follow-ups. Results Fifteen eyes (15 patients, mean age 73 ± 13 years) were included. Six of 15 eyes were diagnosed with coincident age-related macular degeneration (AMD) and 2 with myopic macular degeneration. On fundus examination PEVAC presented as a large perifoveal isolated aneurysm, unifocal in 12 of 15 eyes, associated with small retinal hemorrhages and intraretinal exudation. On structural OCT, PEVAC appeared as a round hyperreflective lesion with hyporeflective lumen, typically surrounded by intraretinal cystic spaces. Dye angiography demonstrated a well-defined hyperfluorescent lesion with variable leakage on FA and without leakage on ICGA. OCT-A showed flow signal correlating with the aneurysmal lesion connecting to retinal capillary plexuses. Seven patients were followed for 13.0 ± 10.5 months with no evidence of functional/anatomic changes. Three patients underwent anti–vascular endothelial growth factor (VEGF) intravitreal injections without improvement. Two eyes were associated with a type 3 neovascularization eccentric to PEVAC. Conclusions PEVAC is an isolated, perifoveal, aneurysmal abnormality, occurring in otherwise healthy patients who may manifest other macular disease including AMD and myopic macular degeneration. PEVAC did not typically respond to anti-VEGF therapy, and may be associated with type 3 neovascularization. © 2017 Elsevier Inc.
Original languageEnglish
Pages (from-to)137-146
Number of pages10
JournalAmerican Journal of Ophthalmology
Volume184
Issue number3
DOIs
Publication statusPublished - 2017

Fingerprint

Blood Vessels
Angiography
Optical Coherence Tomography
Macular Degeneration
Indocyanine Green
Fluorescein Angiography
Vascular Endothelial Growth Factor A
Multimodal Imaging
Endothelial Growth Factors
Retinal Hemorrhage
Intravitreal Injections
Aneurysm
Retina
Cohort Studies
Coloring Agents
Referral and Consultation
Retrospective Studies
Demography

Cite this

Sacconi, R., Freund, KB., Yannuzzi, LA., Dolz-Marco, R., Souied, E., Capuano, V., ... Querques, G. (2017). The Expanded Spectrum of Perifoveal Exudative Vascular Anomalous Complex. American Journal of Ophthalmology, 184(3), 137-146. https://doi.org/10.1016/j.ajo.2017.10.009

The Expanded Spectrum of Perifoveal Exudative Vascular Anomalous Complex. / Sacconi, R; Freund, KB; Yannuzzi, LA; Dolz-Marco, R; Souied, E; Capuano, V; Semoun, O; Phasukkijwatana, N; Sarraf, D; Carnevali, Adriano; Querques, L; Bandello, F; Querques, G.

In: American Journal of Ophthalmology, Vol. 184, No. 3, 2017, p. 137-146.

Research output: Contribution to journalArticle

Sacconi, R, Freund, KB, Yannuzzi, LA, Dolz-Marco, R, Souied, E, Capuano, V, Semoun, O, Phasukkijwatana, N, Sarraf, D, Carnevali, A, Querques, L, Bandello, F & Querques, G 2017, 'The Expanded Spectrum of Perifoveal Exudative Vascular Anomalous Complex', American Journal of Ophthalmology, vol. 184, no. 3, pp. 137-146. https://doi.org/10.1016/j.ajo.2017.10.009
Sacconi R, Freund KB, Yannuzzi LA, Dolz-Marco R, Souied E, Capuano V et al. The Expanded Spectrum of Perifoveal Exudative Vascular Anomalous Complex. American Journal of Ophthalmology. 2017;184(3):137-146. https://doi.org/10.1016/j.ajo.2017.10.009
Sacconi, R ; Freund, KB ; Yannuzzi, LA ; Dolz-Marco, R ; Souied, E ; Capuano, V ; Semoun, O ; Phasukkijwatana, N ; Sarraf, D ; Carnevali, Adriano ; Querques, L ; Bandello, F ; Querques, G. / The Expanded Spectrum of Perifoveal Exudative Vascular Anomalous Complex. In: American Journal of Ophthalmology. 2017 ; Vol. 184, No. 3. pp. 137-146.
@article{f09eec45fe0e49d184cee2b83a735472,
title = "The Expanded Spectrum of Perifoveal Exudative Vascular Anomalous Complex",
abstract = "Purpose To expand our understanding of the uncommon entity, referred to as perifoveal exudative vascular anomalous complex (PEVAC) by describing multimodal imaging findings, including optical coherence tomography angiography (OCT-A). Design Retrospective cohort study. Methods Patients diagnosed with PEVAC were identified at 4 retina referral centers worldwide and underwent complete ophthalmologic examination including structural OCT, OCT-A, fluorescein angiography (FA), and indocyanine green angiography (ICGA). Demographics and clinical findings were analyzed at baseline and at available follow-ups. Results Fifteen eyes (15 patients, mean age 73 ± 13 years) were included. Six of 15 eyes were diagnosed with coincident age-related macular degeneration (AMD) and 2 with myopic macular degeneration. On fundus examination PEVAC presented as a large perifoveal isolated aneurysm, unifocal in 12 of 15 eyes, associated with small retinal hemorrhages and intraretinal exudation. On structural OCT, PEVAC appeared as a round hyperreflective lesion with hyporeflective lumen, typically surrounded by intraretinal cystic spaces. Dye angiography demonstrated a well-defined hyperfluorescent lesion with variable leakage on FA and without leakage on ICGA. OCT-A showed flow signal correlating with the aneurysmal lesion connecting to retinal capillary plexuses. Seven patients were followed for 13.0 ± 10.5 months with no evidence of functional/anatomic changes. Three patients underwent anti–vascular endothelial growth factor (VEGF) intravitreal injections without improvement. Two eyes were associated with a type 3 neovascularization eccentric to PEVAC. Conclusions PEVAC is an isolated, perifoveal, aneurysmal abnormality, occurring in otherwise healthy patients who may manifest other macular disease including AMD and myopic macular degeneration. PEVAC did not typically respond to anti-VEGF therapy, and may be associated with type 3 neovascularization. {\circledC} 2017 Elsevier Inc.",
author = "R Sacconi and KB Freund and LA Yannuzzi and R Dolz-Marco and E Souied and V Capuano and O Semoun and N Phasukkijwatana and D Sarraf and Adriano Carnevali and L Querques and F Bandello and G Querques",
year = "2017",
doi = "10.1016/j.ajo.2017.10.009",
language = "English",
volume = "184",
pages = "137--146",
journal = "American Journal of Ophthalmology",
issn = "0002-9394",
publisher = "Elsevier USA",
number = "3",

}

TY - JOUR

T1 - The Expanded Spectrum of Perifoveal Exudative Vascular Anomalous Complex

AU - Sacconi, R

AU - Freund, KB

AU - Yannuzzi, LA

AU - Dolz-Marco, R

AU - Souied, E

AU - Capuano, V

AU - Semoun, O

AU - Phasukkijwatana, N

AU - Sarraf, D

AU - Carnevali, Adriano

AU - Querques, L

AU - Bandello, F

AU - Querques, G

PY - 2017

Y1 - 2017

N2 - Purpose To expand our understanding of the uncommon entity, referred to as perifoveal exudative vascular anomalous complex (PEVAC) by describing multimodal imaging findings, including optical coherence tomography angiography (OCT-A). Design Retrospective cohort study. Methods Patients diagnosed with PEVAC were identified at 4 retina referral centers worldwide and underwent complete ophthalmologic examination including structural OCT, OCT-A, fluorescein angiography (FA), and indocyanine green angiography (ICGA). Demographics and clinical findings were analyzed at baseline and at available follow-ups. Results Fifteen eyes (15 patients, mean age 73 ± 13 years) were included. Six of 15 eyes were diagnosed with coincident age-related macular degeneration (AMD) and 2 with myopic macular degeneration. On fundus examination PEVAC presented as a large perifoveal isolated aneurysm, unifocal in 12 of 15 eyes, associated with small retinal hemorrhages and intraretinal exudation. On structural OCT, PEVAC appeared as a round hyperreflective lesion with hyporeflective lumen, typically surrounded by intraretinal cystic spaces. Dye angiography demonstrated a well-defined hyperfluorescent lesion with variable leakage on FA and without leakage on ICGA. OCT-A showed flow signal correlating with the aneurysmal lesion connecting to retinal capillary plexuses. Seven patients were followed for 13.0 ± 10.5 months with no evidence of functional/anatomic changes. Three patients underwent anti–vascular endothelial growth factor (VEGF) intravitreal injections without improvement. Two eyes were associated with a type 3 neovascularization eccentric to PEVAC. Conclusions PEVAC is an isolated, perifoveal, aneurysmal abnormality, occurring in otherwise healthy patients who may manifest other macular disease including AMD and myopic macular degeneration. PEVAC did not typically respond to anti-VEGF therapy, and may be associated with type 3 neovascularization. © 2017 Elsevier Inc.

AB - Purpose To expand our understanding of the uncommon entity, referred to as perifoveal exudative vascular anomalous complex (PEVAC) by describing multimodal imaging findings, including optical coherence tomography angiography (OCT-A). Design Retrospective cohort study. Methods Patients diagnosed with PEVAC were identified at 4 retina referral centers worldwide and underwent complete ophthalmologic examination including structural OCT, OCT-A, fluorescein angiography (FA), and indocyanine green angiography (ICGA). Demographics and clinical findings were analyzed at baseline and at available follow-ups. Results Fifteen eyes (15 patients, mean age 73 ± 13 years) were included. Six of 15 eyes were diagnosed with coincident age-related macular degeneration (AMD) and 2 with myopic macular degeneration. On fundus examination PEVAC presented as a large perifoveal isolated aneurysm, unifocal in 12 of 15 eyes, associated with small retinal hemorrhages and intraretinal exudation. On structural OCT, PEVAC appeared as a round hyperreflective lesion with hyporeflective lumen, typically surrounded by intraretinal cystic spaces. Dye angiography demonstrated a well-defined hyperfluorescent lesion with variable leakage on FA and without leakage on ICGA. OCT-A showed flow signal correlating with the aneurysmal lesion connecting to retinal capillary plexuses. Seven patients were followed for 13.0 ± 10.5 months with no evidence of functional/anatomic changes. Three patients underwent anti–vascular endothelial growth factor (VEGF) intravitreal injections without improvement. Two eyes were associated with a type 3 neovascularization eccentric to PEVAC. Conclusions PEVAC is an isolated, perifoveal, aneurysmal abnormality, occurring in otherwise healthy patients who may manifest other macular disease including AMD and myopic macular degeneration. PEVAC did not typically respond to anti-VEGF therapy, and may be associated with type 3 neovascularization. © 2017 Elsevier Inc.

U2 - 10.1016/j.ajo.2017.10.009

DO - 10.1016/j.ajo.2017.10.009

M3 - Article

VL - 184

SP - 137

EP - 146

JO - American Journal of Ophthalmology

JF - American Journal of Ophthalmology

SN - 0002-9394

IS - 3

ER -