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
Plateforme d'Investigation Clinique, Hôpital Fondation Adolphe de Rothschild, 75019 Paris, France.
Unité de Recherche Clinique, Hôpital Fondation Adolphe de Rothschild, 75019 Paris, France.
J Pers Med. 2023 Feb 15;13(2):337. doi: 10.3390/jpm13020337.
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).
A retrospective observational study including patients diagnosed with Best disease.
Fifty-nine eyes of thirty-two patients (fifteen females (46.9%) and seventeen males (53.1%), = 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.
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.
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.
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.
使用频域（SD）光学相干断层扫描（OCT）和OCT血管造影（OCT-A）确定Best病患者中黄斑发育不全（也称为扁平黄斑）的患病率和特征。
一项回顾性观察研究，纳入被诊断为Best病的患者。
纳入32例被诊断为Best病的患者的59只眼（15名女性（46.9%）和17名男性（53.1%），平均年龄0.9岁）。根据B超SD-OCT上的黄斑外观，将患者的眼睛分为两组：有扁平黄斑外观的眼睛（“FP组”）和无扁平黄斑外观的眼睛（“无FP组”）。
评估横断面OCT图像中视网膜内层（IRL）的持续性，并分析OCT-A中是否存在黄斑无血管区（FAZ），并在适用时确定其大小。
总体而言，9例患者的16只眼（27.1%）有扁平黄斑外观（“FP组”），IRL持续存在，23例患者的43只眼（72.9%）无扁平黄斑外观（“无FP组”）。在FP眼中，对13只眼进行的OCT-A显示，100%有OCT-A的眼中有穿过FAZ的桥接血管。使用Thomas分类法，16只扁平黄斑眼中有14只（87.5%）有非典型黄斑发育不全，另外2只（12.5%）有1b级扁平黄斑。
在我们的系列研究中，2
