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Graefe's Archive for Clinical and Experimental Ophthalmology

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01-04-2021 | Angiography | Retinal Disorders

Non-exudative macular neovascularization in pseudoxanthoma elasticum

Authors: João Pedro Marques, João Bernardes, Sara Geada, Mário Soares, Dora Teixeira, Cláudia Farinha, Isabel Pires, Maria Luz Cachulo, Rufino Silva

Published in: Graefe's Archive for Clinical and Experimental Ophthalmology | Issue 4/2021

Abstract

Purpose

To characterize morphological changes in the retina and to report the frequency and natural history of non-exudative macular neovascularization (MNV) in a cohort of pseudoxanthoma elasticum (PXE).

Methods

A single-center, retrospective study was complemented by a cross-sectional examination. Consecutive patients with a definitive genetic and/or clinical diagnosis of PXE, visiting our department between January 2019 and December 2019, and with a minimum follow-up of 6 months were recruited. Baseline data were retrieved from each patient file. Additionally, a cross-sectional examination comprising color fundus photography, spectral-domain optical coherence tomography (SD-OCT), OCT-Angiography (OCT-A), and fundus autofluorescence was performed. The presence of typical PXE-related findings, as well as related complications, was multimodally evaluated. The prevalence and natural history of non-exudative MNV were assessed. All images were graded by two independent graders.

Results

Forty-eight eyes from 24 patients (mean age 59.11 ± 18.14) with a median follow-up of 53.00 months were included. Angioid streaks and peau d’orange were observed in 46/48 and 42/48 eyes, while MNV was present in 75.00% of the cohort. The prevalence of non-exudative MNV was 33.33% (6/18). In the 2 eyes that developed exudation, time to conversion was 9.50 ± 4.95 months. No significant difference in visual acuity was found between eyes with non-exudative MNV and those with no signs of MNV.

Conclusion

We have shown that non-exudative MNV is a frequent finding in PXE but the majority of eyes did not develop exudation during follow-up. Our results are a clear evidence of the utility of OCT-A in the management of PXE.

The Consensus Nomenclature for Reporting Neovascular Age-Related Macular Degeneration (AMD) Data (2020) recently established that the term macular neovascularization should be used in AMD-related neovascularization. Even though neovascularization in PXE-associated retinopathy is pathophysiologically different from AMD, the authors chose to use the same term for PXE-related neovascularization.

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Title: Non-exudative macular neovascularization in pseudoxanthoma elasticum
Authors: João Pedro Marques
João Bernardes
Sara Geada
Mário Soares
Dora Teixeira
Cláudia Farinha
Isabel Pires
Maria Luz Cachulo
Rufino Silva
Publication date: 01-04-2021
Publisher: Springer Berlin Heidelberg
Keyword: Angiography
Published in: Graefe's Archive for Clinical and Experimental Ophthalmology / Issue 4/2021
Print ISSN: 0721-832X
Electronic ISSN: 1435-702X
DOI: https://doi.org/10.1007/s00417-020-04979-z

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Non-exudative macular neovascularization in pseudoxanthoma elasticum

Abstract

Purpose

Methods

Results

Conclusion

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Purpose

Methods

Results

Conclusion

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