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. 2021 Apr;259(4):873-882.
doi: 10.1007/s00417-020-04979-z. Epub 2020 Oct 19.

Non-exudative macular neovascularization in pseudoxanthoma elasticum

João Pedro Marques #  1   2   3   4 , João Bernardes #  5 , Sara Geada  5 , Mário Soares  5 , Dora Teixeira  5 , Cláudia Farinha  5   6   7   8 , Isabel Pires  5   6   7   8 , Maria Luz Cachulo  5   6   7   8 , Rufino Silva  5   6
Affiliations

Non-exudative macular neovascularization in pseudoxanthoma elasticum

João Pedro Marques et al. Graefes Arch Clin Exp Ophthalmol. 2021 Apr.

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.

Keywords: Angioid streaks; Non-exudative macular neovascularization; Optical coherence tomography; Optical coherence tomography angiography; Pseudoxanthoma elasticum; Quiescent.

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