Exudative perifoveal vascular anomalous complex (ePVAC) resembling lesion in a patient with adult-onset foveomacular vitelliform dystrophy

Purpose: to report a case of exudative perifoveal vascular anomalous complex (ePVAC) in a patient with adult-onset foveomacular vitelliform dystrophy. Observations: A 71-year-old male presented with moderate vision loss in his left eye. His past medical and ocular history were unremarkable. The best...

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Main Authors: Serena Milan, Marco Rocco Pastore, Andrea Gaggino, Silvia Rinaldi, Daniele Tognetto
Format: Article
Language:English
Published: Elsevier 2024-12-01
Series:American Journal of Ophthalmology Case Reports
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Online Access:http://www.sciencedirect.com/science/article/pii/S2451993624002214
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author Serena Milan
Marco Rocco Pastore
Andrea Gaggino
Silvia Rinaldi
Daniele Tognetto
author_facet Serena Milan
Marco Rocco Pastore
Andrea Gaggino
Silvia Rinaldi
Daniele Tognetto
author_sort Serena Milan
collection DOAJ
description Purpose: to report a case of exudative perifoveal vascular anomalous complex (ePVAC) in a patient with adult-onset foveomacular vitelliform dystrophy. Observations: A 71-year-old male presented with moderate vision loss in his left eye. His past medical and ocular history were unremarkable. The best-corrected visual acuity was 20/32. Fundus examination disclosed the presence of a perifoveal vascular abnormality, associated with a small egg-yolk lesion. Optical coherence tomography (OCT) scan passing through the perifoveal vascular abnormality seen on fundus, revealed a small round hyperreflective lesion located at the outer plexiform layer-inner nuclear layer, surrounded by intraretinal cysts. Fluorescein angiography displayed an isolated perifoveal aneurysmal lesion with minimal leakage. OCT angiography permitted to localize the lesion flow signal at the deep capillary plexus (DCP) and the avascular slab; choriocapillaris structure was unaffected; any retino-choroidal anastomosis was detected. A systemic workup was suggested to rule out any vascular diseases, and any abnormality was found. He was thus diagnosed with ePVAC. A stability of the clinical picture was demonstrated after 3 months of follow up. Conclusion: ePVAC has recently been described to appear in association with other macular abnormalities, such as age-related macular degeneration and myopic macular degeneration. Our case firstly demonstrates an ePVAC lesion in an eye with adult-onset foveomacular vitelliform dystrophy. This observation highlights the importance of discerning between different vascular disorders of the macula, to offer the right treatment to the patient.
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spelling doaj-art-d26a3f040e604ff29e88cd01c16d32cc2025-08-20T02:48:58ZengElsevierAmerican Journal of Ophthalmology Case Reports2451-99362024-12-013610221110.1016/j.ajoc.2024.102211Exudative perifoveal vascular anomalous complex (ePVAC) resembling lesion in a patient with adult-onset foveomacular vitelliform dystrophySerena Milan0Marco Rocco Pastore1Andrea Gaggino2Silvia Rinaldi3Daniele Tognetto4Eye Clinic, Department of Medical Surgical Sciences and Health, University of Trieste, 34129, Trieste, ItalyCorresponding author. Eye Clinic, University of Trieste, Ospedale Maggiore, Piazza dell’Ospitale 1, 34129, Trieste, Italy.; Eye Clinic, Department of Medical Surgical Sciences and Health, University of Trieste, 34129, Trieste, ItalyEye Clinic, Department of Medical Surgical Sciences and Health, University of Trieste, 34129, Trieste, ItalyEye Clinic, Department of Medical Surgical Sciences and Health, University of Trieste, 34129, Trieste, ItalyEye Clinic, Department of Medical Surgical Sciences and Health, University of Trieste, 34129, Trieste, ItalyPurpose: to report a case of exudative perifoveal vascular anomalous complex (ePVAC) in a patient with adult-onset foveomacular vitelliform dystrophy. Observations: A 71-year-old male presented with moderate vision loss in his left eye. His past medical and ocular history were unremarkable. The best-corrected visual acuity was 20/32. Fundus examination disclosed the presence of a perifoveal vascular abnormality, associated with a small egg-yolk lesion. Optical coherence tomography (OCT) scan passing through the perifoveal vascular abnormality seen on fundus, revealed a small round hyperreflective lesion located at the outer plexiform layer-inner nuclear layer, surrounded by intraretinal cysts. Fluorescein angiography displayed an isolated perifoveal aneurysmal lesion with minimal leakage. OCT angiography permitted to localize the lesion flow signal at the deep capillary plexus (DCP) and the avascular slab; choriocapillaris structure was unaffected; any retino-choroidal anastomosis was detected. A systemic workup was suggested to rule out any vascular diseases, and any abnormality was found. He was thus diagnosed with ePVAC. A stability of the clinical picture was demonstrated after 3 months of follow up. Conclusion: ePVAC has recently been described to appear in association with other macular abnormalities, such as age-related macular degeneration and myopic macular degeneration. Our case firstly demonstrates an ePVAC lesion in an eye with adult-onset foveomacular vitelliform dystrophy. This observation highlights the importance of discerning between different vascular disorders of the macula, to offer the right treatment to the patient.http://www.sciencedirect.com/science/article/pii/S2451993624002214Adult-onset foveomacular vitelliform dystrophyPEVAC
spellingShingle Serena Milan
Marco Rocco Pastore
Andrea Gaggino
Silvia Rinaldi
Daniele Tognetto
Exudative perifoveal vascular anomalous complex (ePVAC) resembling lesion in a patient with adult-onset foveomacular vitelliform dystrophy
American Journal of Ophthalmology Case Reports
Adult-onset foveomacular vitelliform dystrophy
PEVAC
title Exudative perifoveal vascular anomalous complex (ePVAC) resembling lesion in a patient with adult-onset foveomacular vitelliform dystrophy
title_full Exudative perifoveal vascular anomalous complex (ePVAC) resembling lesion in a patient with adult-onset foveomacular vitelliform dystrophy
title_fullStr Exudative perifoveal vascular anomalous complex (ePVAC) resembling lesion in a patient with adult-onset foveomacular vitelliform dystrophy
title_full_unstemmed Exudative perifoveal vascular anomalous complex (ePVAC) resembling lesion in a patient with adult-onset foveomacular vitelliform dystrophy
title_short Exudative perifoveal vascular anomalous complex (ePVAC) resembling lesion in a patient with adult-onset foveomacular vitelliform dystrophy
title_sort exudative perifoveal vascular anomalous complex epvac resembling lesion in a patient with adult onset foveomacular vitelliform dystrophy
topic Adult-onset foveomacular vitelliform dystrophy
PEVAC
url http://www.sciencedirect.com/science/article/pii/S2451993624002214
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