Riccardo Sacconi1, K Bailey Freund2, Lawrence A Yannuzzi2, Rosa Dolz-Marco2, Eric Souied3, Vittorio Capuano3, Oudy Semoun3, Nopasak Phasukkijwatana4, David Sarraf4, Adriano Carnevali1, Lea Querques1, Francesco Bandello1, Giuseppe Querques5. 1. Department of Ophthalmology, University Vita-Salute, IRCCS Ospedale San Raffaele, Milan, Italy. 2. Vitreous Retina Macula Consultants of New York, New York, New York. 3. Department of Ophthalmology, Hospital Intercommunal de Creteil, University Paris Est Creteil, Creteil, France. 4. Retinal Disorders and Ophthalmic Genetics Division, Stein Eye Institute, University of California, Los Angeles, Los Angeles, California. 5. Department of Ophthalmology, University Vita-Salute, IRCCS Ospedale San Raffaele, Milan, Italy. Electronic address: giuseppe.querques@hotmail.it.
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.
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.
Authors: Jakob Siedlecki; Efstathios Vounotrypidis; Denise Vogt; Armin Wolf; Siegfried G Priglinger; Ricarda G Schumann Journal: Am J Ophthalmol Case Rep Date: 2019-04-04
Authors: José Ignacio Fernández-Vigo; Bárbara Burgos-Blasco; Rosa Dolz-Marco; María Jiménez-Santos; Lorenzo López-Guajardo; Juan Donate-López Journal: Am J Ophthalmol Case Rep Date: 2020-04-21