Atypical Foveal Hypoplasia in Best Disease

被引:0
作者
Moret, Emmanuelle [1 ]
Lejoyeux, Raphael [1 ]
Bonnin, Sophie [1 ]
Azar, Georges [1 ]
Guillaume, Jessica [2 ]
Le Cossec, Chloe [2 ]
Lafolie, Justine [1 ]
Alonso, Anne-Sophie [1 ]
Favard, Catherine [3 ]
Meunier, Isabelle [4 ]
Vasseur, Vivien [1 ]
Mauget-Faysse, Martine [1 ]
机构
[1] Hop Fdn Adolphe De Rothschild, Plateforme Invest Clin, F-75019 Paris, France
[2] Hop Fdn Adolphe De Rothschild, Unite Rech Clin, F-75019 Paris, France
[3] Ctr Ophtalmol Odeon, 113 Blvd St Germain, F-75006 Paris, France
[4] CHU Gui Chauliac, 80 Ave Augustin Fliche, F-34090 Montpellier, France
关键词
Best disease; fovea plana; foveal hypoplasia; foveal avascular zone; OCT-A; DYSTROPHY; PLANA; MUTATIONS; MEMBRANE;
D O I
10.3390/jpm13020337
中图分类号
R19 [保健组织与事业(卫生事业管理)];
学科分类号
摘要
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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页数:12
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