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...
| Main Authors: | , , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
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MDPI AG
2023-02-01
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| Series: | Journal of Personalized Medicine |
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| Online Access: | https://www.mdpi.com/2075-4426/13/2/337 |
| _version_ | 1797619945195962368 |
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| author | Emmanuelle Moret Raphaël Lejoyeux Sophie Bonnin Georges Azar Jessica Guillaume Chloé Le Cossec Justine Lafolie Anne-Sophie Alonso Catherine Favard Isabelle Meunier Vivien Vasseur Martine Mauget-Faÿsse |
| author_facet | Emmanuelle Moret Raphaël Lejoyeux Sophie Bonnin Georges Azar Jessica Guillaume Chloé Le Cossec Justine Lafolie Anne-Sophie Alonso Catherine Favard Isabelle Meunier Vivien Vasseur Martine Mauget-Faÿsse |
| author_sort | Emmanuelle Moret |
| collection | DOAJ |
| 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%), <i>p</i> = 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. |
| first_indexed | 2024-03-11T08:34:14Z |
| format | Article |
| id | doaj.art-13fde559535a426cad7e320dba171d44 |
| institution | Directory Open Access Journal |
| issn | 2075-4426 |
| language | English |
| last_indexed | 2024-03-11T08:34:14Z |
| publishDate | 2023-02-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Journal of Personalized Medicine |
| spelling | doaj.art-13fde559535a426cad7e320dba171d442023-11-16T21:34:01ZengMDPI AGJournal of Personalized Medicine2075-44262023-02-0113233710.3390/jpm13020337Atypical Foveal Hypoplasia in Best DiseaseEmmanuelle Moret0Raphaël Lejoyeux1Sophie Bonnin2Georges Azar3Jessica Guillaume4Chloé Le Cossec5Justine Lafolie6Anne-Sophie Alonso7Catherine Favard8Isabelle Meunier9Vivien Vasseur10Martine Mauget-Faÿsse11Plateforme d’Investigation Clinique, Hôpital Fondation Adolphe de Rothschild, 75019 Paris, FrancePlateforme d’Investigation Clinique, Hôpital Fondation Adolphe de Rothschild, 75019 Paris, FrancePlateforme d’Investigation Clinique, Hôpital Fondation Adolphe de Rothschild, 75019 Paris, FrancePlateforme d’Investigation Clinique, Hôpital Fondation Adolphe de Rothschild, 75019 Paris, FranceUnité de Recherche Clinique, Hôpital Fondation Adolphe de Rothschild, 75019 Paris, FranceUnité de Recherche Clinique, Hôpital Fondation Adolphe de Rothschild, 75019 Paris, FrancePlateforme d’Investigation Clinique, Hôpital Fondation Adolphe de Rothschild, 75019 Paris, FrancePlateforme d’Investigation Clinique, Hôpital Fondation Adolphe de Rothschild, 75019 Paris, FranceCentre Ophtalmologique de l’Odéon, 113 Boulevard Saint Germain, 75006 Paris, FranceCHU Gui de Chauliac, 80 Avenue Augustin Fliche, 34090 Montpellier, FrancePlateforme d’Investigation Clinique, Hôpital Fondation Adolphe de Rothschild, 75019 Paris, FrancePlateforme d’Investigation Clinique, Hôpital Fondation Adolphe de Rothschild, 75019 Paris, FrancePurpose: 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%), <i>p</i> = 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.https://www.mdpi.com/2075-4426/13/2/337Best diseasefovea planafoveal hypoplasiafoveal avascular zoneOCT-A |
| spellingShingle | Emmanuelle Moret Raphaël Lejoyeux Sophie Bonnin Georges Azar Jessica Guillaume Chloé Le Cossec Justine Lafolie Anne-Sophie Alonso Catherine Favard Isabelle Meunier Vivien Vasseur Martine Mauget-Faÿsse Atypical Foveal Hypoplasia in Best Disease Journal of Personalized Medicine Best disease fovea plana foveal hypoplasia foveal avascular zone OCT-A |
| 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 | Best disease fovea plana foveal hypoplasia foveal avascular zone OCT-A |
| url | https://www.mdpi.com/2075-4426/13/2/337 |
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