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Article

Atypical Foveal Hypoplasia in Best Disease

1
Plateforme d’Investigation Clinique, Hôpital Fondation Adolphe de Rothschild, 75019 Paris, France
2
Unité de Recherche Clinique, Hôpital Fondation Adolphe de Rothschild, 75019 Paris, France
3
Centre Ophtalmologique de l’Odéon, 113 Boulevard Saint Germain, 75006 Paris, France
4
CHU Gui de Chauliac, 80 Avenue Augustin Fliche, 34090 Montpellier, France
*
Author to whom correspondence should be addressed.
J. Pers. Med. 2023, 13(2), 337; https://doi.org/10.3390/jpm13020337
Submission received: 22 November 2022 / Revised: 6 February 2023 / Accepted: 7 February 2023 / Published: 15 February 2023
(This article belongs to the Special Issue Precision Medicine for Retinal Disease)

Abstract

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.
Keywords: Best disease; fovea plana; foveal hypoplasia; foveal avascular zone; OCT-A Best disease; fovea plana; foveal hypoplasia; foveal avascular zone; OCT-A

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MDPI and ACS Style

Moret, E.; Lejoyeux, R.; Bonnin, S.; Azar, G.; Guillaume, J.; Le Cossec, C.; Lafolie, J.; Alonso, A.-S.; Favard, C.; Meunier, I.; et al. Atypical Foveal Hypoplasia in Best Disease. J. Pers. Med. 2023, 13, 337. https://doi.org/10.3390/jpm13020337

AMA Style

Moret E, Lejoyeux R, Bonnin S, Azar G, Guillaume J, Le Cossec C, Lafolie J, Alonso A-S, Favard C, Meunier I, et al. Atypical Foveal Hypoplasia in Best Disease. Journal of Personalized Medicine. 2023; 13(2):337. https://doi.org/10.3390/jpm13020337

Chicago/Turabian Style

Moret, Emmanuelle, Raphaël Lejoyeux, Sophie Bonnin, Georges Azar, Jessica Guillaume, Chloé Le Cossec, Justine Lafolie, Anne-Sophie Alonso, Catherine Favard, Isabelle Meunier, and et al. 2023. "Atypical Foveal Hypoplasia in Best Disease" Journal of Personalized Medicine 13, no. 2: 337. https://doi.org/10.3390/jpm13020337

APA Style

Moret, E., Lejoyeux, R., Bonnin, S., Azar, G., Guillaume, J., Le Cossec, C., Lafolie, J., Alonso, A.-S., Favard, C., Meunier, I., Vasseur, V., & Mauget-Faÿsse, M. (2023). Atypical Foveal Hypoplasia in Best Disease. Journal of Personalized Medicine, 13(2), 337. https://doi.org/10.3390/jpm13020337

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