Riccardo Sacconi1, Enrico Borrelli1, SriniVas Sadda2, Giulia Corradetti2, K Bailey Freund3, Lawrence A Yannuzzi3, Eric Souied4, Vittorio Capuano4, David Sarraf5, Lea Querques1, Francesco Bandello1, Giuseppe Querques6. 1. Division of Head and Neck, Ophthalmology Unit, IRCCS San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan, Italy. 2. Doheny Eye Institute, David Geffen School of Medicine, University of California, Los Angeles, Los Angeles, California, USA. 3. Vitreous Retina Macula Consultants of New York, New York, New York, USA. 4. Department of Ophthalmology, Hospital Intercommunal de Creteil, University Paris Est Creteil, Creteil, France. 5. Retinal Disorders and Ophthalmic Genetics Division, Stein Eye Institute, University of California, Los Angeles, Los Angeles, California, USA. 6. Division of Head and Neck, Ophthalmology Unit, IRCCS San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan, Italy. Electronic address: giuseppe.querques@hotmail.it.
Abstract
PURPOSE: To describe the pre-exudative stage of exudative perifoveal vascular anomalous complex (ePVAC), referred to as nonexudative PVAC (nePVAC). DESIGN: Retrospective noncomparative case series. METHODS: Patients diagnosed with nePVAC were identified at 4 retina referral centers worldwide. Multimodal retinal imaging, including structural optical coherence tomography (OCT) and OCT-angiography (OCT-A), were performed at baseline and follow-up visits. RESULTS: Six eyes (6 patients, mean 75 ± 10 years of age) were included. Unrelated chorioretinal diseases were diagnosed in the affected eyes in 5 of 6 cases. At baseline, nePVAC is characterized by microvascular abnormalities featuring an isolated, perifoveal, large intraretinal aneurysm surrounded by capillary rarefaction at OCT-A examination, without any sign of exudation with structural OCT, and without visual impairment. Four patients were followed for a mean of 21 ± 14 months. During the follow-up, 3 of 4 eyes (75%) developed signs of exudation after a mean of 15 ± 9 months, associated with metamorphopsia and visual decline at the time of exudation. Best-corrected visual acuity decreased from 20/25 to 20/40 Snellen equivalent (P = .035) and central macular thickness increased from 268 ± 27 μm to 339 ± 65 μm (P = .145). Three patients were treated with 2.3 ± 0.6 intravitreal injections of anti-vascular endothelial growth factor without significant improvement of best-corrected visual acuity or macular edema. CONCLUSIONS: nePVAC may represent the subclinical pre-exudative stage of ePVAC, notable for an absence of exudation or visual impairment. nePVAC and ePVAC should be considered as part of the same spectrum, namely PVAC. Typically, nePVAC develops signs of exudation over time, causing metamorphopsia and visual decline and therefore these lesions warrant continued close monitoring with multimodal retinal imaging.
PURPOSE: To describe the pre-exudative stage of exudative perifoveal vascular anomalous complex (ePVAC), referred to as nonexudative PVAC (nePVAC). DESIGN: Retrospective noncomparative case series. METHODS:Patients diagnosed with nePVAC were identified at 4 retina referral centers worldwide. Multimodal retinal imaging, including structural optical coherence tomography (OCT) and OCT-angiography (OCT-A), were performed at baseline and follow-up visits. RESULTS: Six eyes (6 patients, mean 75 ± 10 years of age) were included. Unrelated chorioretinal diseases were diagnosed in the affected eyes in 5 of 6 cases. At baseline, nePVAC is characterized by microvascular abnormalities featuring an isolated, perifoveal, large intraretinal aneurysm surrounded by capillary rarefaction at OCT-A examination, without any sign of exudation with structural OCT, and without visual impairment. Four patients were followed for a mean of 21 ± 14 months. During the follow-up, 3 of 4 eyes (75%) developed signs of exudation after a mean of 15 ± 9 months, associated with metamorphopsia and visual decline at the time of exudation. Best-corrected visual acuity decreased from 20/25 to 20/40 Snellen equivalent (P = .035) and central macular thickness increased from 268 ± 27 μm to 339 ± 65 μm (P = .145). Three patients were treated with 2.3 ± 0.6 intravitreal injections of anti-vascular endothelial growth factor without significant improvement of best-corrected visual acuity or macular edema. CONCLUSIONS: nePVAC may represent the subclinical pre-exudative stage of ePVAC, notable for an absence of exudation or visual impairment. nePVAC and ePVAC should be considered as part of the same spectrum, namely PVAC. Typically, nePVAC develops signs of exudation over time, causing metamorphopsia and visual decline and therefore these lesions warrant continued close monitoring with multimodal retinal imaging.