目的:使用谱域(SD)光学相干断层扫描(OCT)和OCT血管造影(OCT-A)确定最佳疾病患者中中央凹发育不全(也称为中央凹平面)的患病率和特征。
方法:一项回顾性观察研究,包括诊断为Best疾病的患者。
方法:32例患者59只眼(女性15例(46.9%),男性17例(53.1%),p=0.9)诊断为最佳疾病。患者的眼睛分为两组:有中央凹平面外观的眼睛(“FP组”)和无中央凹平面外观的眼睛(“无FP组”),基于B扫描SD-OCT的中央凹外观。
方法:评估横截面OCT图像中视网膜内层(IRL)的持久性,并分析OCT-A中是否存在中央凹无血管区(FAZ),其大小在适用时确定。
结果:总体而言,9例患者中有16眼(27.1%)出现中央凹平面外观(“FP组”),并伴有IRL的持久性,23例患者中有43只眼(72.9%)没有中央凹平面外观(\'无FP组\')。在FP的眼睛中,在13只眼睛中进行的OCT-A显示,使用OCT-A的眼睛中有100%通过FAZ的桥接血管。使用托马斯分类,16只眼中有14只(87.5%)患有不典型的中央凹发育不全,另外2人(12.5%)有1b级中央凹平面。
结论:在我们的系列中,27.1%的Best病患者存在中央凹发育不全。OCT-A显示所有眼睛中通过FAZ的桥接血管。这些发现突出了与最佳疾病相关的微血管变化,这可能是有家族史的患者患病的早期征兆。
OBJECTIVE: 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).
METHODS: A retrospective observational study including patients diagnosed with Best disease.
METHODS: 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: 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.
CONCLUSIONS: 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.