Non-exudative macular neovascularization in pseudoxanthoma elasticum
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RETINAL DISORDERS
Non-exudative macular neovascularization in pseudoxanthoma elasticum João Pedro Marques 1,2,3,4 & João Bernardes 1 & Sara Geada 1 & Mário Soares 1 & Dora Teixeira 1 & Cláudia Farinha 1,2,3,4 & Isabel Pires 1,2,3,4 & Maria Luz Cachulo 1,2,3,4 & Rufino Silva 1,2 Received: 25 August 2020 / Revised: 24 September 2020 / Accepted: 9 October 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
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 crosssectional examination comprising color fundus photography, spectral-domain optical coherence tomography (SD-OCT), OCTAngiography (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. Keywords Pseudoxanthoma elasticum . Angioid streaks . Non-exudative macular neovascularization . Optical coherence tomography . Optical coherence tomography angiography . Quiescent
João Pedro Marques and João Bernardes contributed equally to this work. * João Pedro Marques [email protected] 1
Ophthalmology Unit, Centro de Responsabilidade Integrado em Oftalmologia (CRIO), Centro Hospitalar e Universitário de Coimbra (CHUC), Praceta Prof. Mota Pinto, 3000-075 Coimbra, Portugal
2
University Clinic of Ophthalmology, Faculty of Medicine, University of Coimbra (FMUC), Coimbra, Portugal
3
Clinical Academic Center of Coimbra (CACC), Coimbra, Portugal
4
Coimbra Institute for Clinical and Biomedical Research (iCBR), Faculty of Medicine, University of Coimbra (FMUC), University of Coimbra, Coimbra, Portugal
Graefes Arch Clin Exp Ophthalmol
KEY MESSAGES WHAT WAS KNOWN BEFORE Macular neovasculariza
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