University of California, San Francisco, CA, USA.
Jaeb Center for Health Research, Tampa, FL, USA.
Am J Ophthalmol. 2023 Aug;252:77-93. doi: 10.1016/j.ajo.2023.03.006. Epub 2023 Mar 21.
To describe cone structure changes using adaptive optics scanning laser ophthalmoscopy (AOSLO) in the Rate of Progression of USH2A-related Retinal Degeneration (RUSH2A) study.
Multicenter, longitudinal natural history study.
AOSLO images were acquired at 4 centers, twice at baseline and annually for 24 months in this natural history study. For each eye, at least 10 regions of interest (ROIs) with ≥50 contiguous cones were analyzed by masked, independent graders. Cone spacing Z-scores, standard deviations from the normal mean at the measured location, were compared between graders and tests at baseline. The association of cone spacing with clinical characteristics was assessed using linear mixed effects regression models weighted by image quality score. Annual rates of change were calculated based on differences between visits.
Fourteen eyes of 14 participants were imaged, with 192 ROIs selected at baseline. There was variability among graders, which was greater in images with lower image quality score (P < .001). Cone spacing was significantly correlated with eccentricity, quality score, and disease duration (P < .02). On average, the cone spacing Z-score increased 0.14 annually (about 9%, P < .001). We observed no significant differences in rate of change between disease type (Usher syndrome or retinitis pigmentosa), imaging site, or grader.
Using current methods, the analysis of quantitative measures of cone structure showed some challenges, yet showed promise that AOSLO images can be used to characterize progressive change over 24 months. Additional multicenter studies using AOSLO are needed to advance cone mosaic metrics as sensitive outcome measures for clinical trials. NOTE: Publication of this article is sponsored by the American Ophthalmological Society.
使用自适应光学扫描激光检眼镜（AOSLO）描述 USH2A 相关视网膜变性（RUSH2A）研究中的锥体结构变化。
多中心、纵向的自然史研究。
在这项自然史研究中，AOSLO 图像在 4 个中心采集，基线时采集 2 次，随后每年采集 1 次，共 24 个月。对每只眼，至少分析 10 个具有≥50 个连续锥体的感兴趣区（ROI），由盲法、独立评分者进行分析。在基线时，比较评分者和测试之间的锥体间距 Z 分数（在测量位置与正态平均值的标准偏差）。使用线性混合效应回归模型，根据图像质量评分加权，评估锥体间距与临床特征的相关性。根据随访之间的差异计算年度变化率。
对 14 名参与者的 14 只眼进行成像，在基线时选择了 192 个 ROI。评分者之间存在差异，图像质量评分较低时差异更大（P <.001）。锥体间距与偏心度、质量评分和疾病持续时间显著相关（P <.02）。平均而言，锥体间距 Z 分数每年增加 0.14（约 9%，P <.001）。我们没有观察到疾病类型（先天性聋-视网膜色素变性综合征或色素性视网膜炎）、成像部位或评分者之间变化率的显著差异。
使用当前的方法，对锥体结构的定量测量进行分析，虽然存在一些挑战，但具有一定的应用前景，即 AOSLO 图像可用于在 24 个月内描述进行性变化。需要开展更多的多中心研究，以推进作为临床试验敏感终点的锥体镶嵌度量标准。
本文的发表由美国眼科学会赞助。
