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Atypical Foveal Hypoplasia in Best Disease

Purpose: To determine the prevalence and characteristics of foveal hypoplasia (also called fovea plana) in patients with Best disease using spectral-domain (SD) optical coherence tomography (OCT) and OCT-angiography (OCT-A). Design: A retrospective observational study including patients diagnosed wi...

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Autores principales: Moret, Emmanuelle, Lejoyeux, Raphaël, Bonnin, Sophie, Azar, Georges, Guillaume, Jessica, Le Cossec, Chloé, Lafolie, Justine, Alonso, Anne-Sophie, Favard, Catherine, Meunier, Isabelle, Vasseur, Vivien, Mauget-Faÿsse, Martine
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9959407/
https://www.ncbi.nlm.nih.gov/pubmed/36836571
http://dx.doi.org/10.3390/jpm13020337
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author Moret, Emmanuelle
Lejoyeux, Raphaël
Bonnin, Sophie
Azar, Georges
Guillaume, Jessica
Le Cossec, Chloé
Lafolie, Justine
Alonso, Anne-Sophie
Favard, Catherine
Meunier, Isabelle
Vasseur, Vivien
Mauget-Faÿsse, Martine
author_facet Moret, Emmanuelle
Lejoyeux, Raphaël
Bonnin, Sophie
Azar, Georges
Guillaume, Jessica
Le Cossec, Chloé
Lafolie, Justine
Alonso, Anne-Sophie
Favard, Catherine
Meunier, Isabelle
Vasseur, Vivien
Mauget-Faÿsse, Martine
author_sort Moret, Emmanuelle
collection PubMed
description Purpose: To determine the prevalence and characteristics of foveal hypoplasia (also called fovea plana) in patients with Best disease using spectral-domain (SD) optical coherence tomography (OCT) and OCT-angiography (OCT-A). Design: A retrospective observational study including patients diagnosed with Best disease. Subjects and Participants: Fifty-nine eyes of thirty-two patients (fifteen females (46.9%) and seventeen males (53.1%), p = 0.9) diagnosed with Best disease were included. Patients’ eyes were categorized into two groups: Eyes with a fovea plana appearance (‘FP group’) and eyes without fovea plana appearance (‘no FP group’), based on the foveal appearance on B-scan SD-OCT. Methods and Main Outcome Measures: Cross-sectional OCT images were assessed for the persistence of inner retinal layers (IRL) and OCT-A was analyzed for the presence of a foveal avascular zone (FAZ), the size of which was determined when applicable. Results: Overall, 16 eyes (27.1%) of 9 patients had a fovea plana appearance (‘FP group’) with the persistence of IRL, and 43 eyes (72.9%) of 23 patients did not have fovea plana appearance (‘no FP group’). Among FP eyes, OCT-A performed in 13 eyes showed bridging vessels through the FAZ in 100% of eyes with OCT-A. Using Thomas classification, 14 out of the 16 eyes with fovea plana (87.5%) had atypical foveal hypoplasia, and the 2 others (12.5%) had a grade 1b fovea plana. Conclusion: In our series, foveal hypoplasia was present in 27.1% of patients with Best disease. OCT-A showed bridging vessels through the FAZ in all eyes. These findings highlight the microvascular changes associated with Best disease, which can be an early sign of the disease in patients with a family history.
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spelling pubmed-99594072023-02-26 Atypical Foveal Hypoplasia in Best Disease Moret, Emmanuelle Lejoyeux, Raphaël Bonnin, Sophie Azar, Georges Guillaume, Jessica Le Cossec, Chloé Lafolie, Justine Alonso, Anne-Sophie Favard, Catherine Meunier, Isabelle Vasseur, Vivien Mauget-Faÿsse, Martine J Pers Med Article Purpose: To determine the prevalence and characteristics of foveal hypoplasia (also called fovea plana) in patients with Best disease using spectral-domain (SD) optical coherence tomography (OCT) and OCT-angiography (OCT-A). Design: A retrospective observational study including patients diagnosed with Best disease. Subjects and Participants: Fifty-nine eyes of thirty-two patients (fifteen females (46.9%) and seventeen males (53.1%), p = 0.9) diagnosed with Best disease were included. Patients’ eyes were categorized into two groups: Eyes with a fovea plana appearance (‘FP group’) and eyes without fovea plana appearance (‘no FP group’), based on the foveal appearance on B-scan SD-OCT. Methods and Main Outcome Measures: Cross-sectional OCT images were assessed for the persistence of inner retinal layers (IRL) and OCT-A was analyzed for the presence of a foveal avascular zone (FAZ), the size of which was determined when applicable. Results: Overall, 16 eyes (27.1%) of 9 patients had a fovea plana appearance (‘FP group’) with the persistence of IRL, and 43 eyes (72.9%) of 23 patients did not have fovea plana appearance (‘no FP group’). Among FP eyes, OCT-A performed in 13 eyes showed bridging vessels through the FAZ in 100% of eyes with OCT-A. Using Thomas classification, 14 out of the 16 eyes with fovea plana (87.5%) had atypical foveal hypoplasia, and the 2 others (12.5%) had a grade 1b fovea plana. Conclusion: In our series, foveal hypoplasia was present in 27.1% of patients with Best disease. OCT-A showed bridging vessels through the FAZ in all eyes. These findings highlight the microvascular changes associated with Best disease, which can be an early sign of the disease in patients with a family history. MDPI 2023-02-15 /pmc/articles/PMC9959407/ /pubmed/36836571 http://dx.doi.org/10.3390/jpm13020337 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Moret, Emmanuelle
Lejoyeux, Raphaël
Bonnin, Sophie
Azar, Georges
Guillaume, Jessica
Le Cossec, Chloé
Lafolie, Justine
Alonso, Anne-Sophie
Favard, Catherine
Meunier, Isabelle
Vasseur, Vivien
Mauget-Faÿsse, Martine
Atypical Foveal Hypoplasia in Best Disease
title Atypical Foveal Hypoplasia in Best Disease
title_full Atypical Foveal Hypoplasia in Best Disease
title_fullStr Atypical Foveal Hypoplasia in Best Disease
title_full_unstemmed Atypical Foveal Hypoplasia in Best Disease
title_short Atypical Foveal Hypoplasia in Best Disease
title_sort atypical foveal hypoplasia in best disease
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9959407/
https://www.ncbi.nlm.nih.gov/pubmed/36836571
http://dx.doi.org/10.3390/jpm13020337
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