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定量分析圆锥-杆状细胞营养不良患者的剩余椭圆带及其与视觉功能的相关性。

Quantification of residual ellipsoid zone and its correlation with visual functions in patients with cone-rod dystrophy.

机构信息

Department of Ophthalmology, The University of Tokyo, Tokyo, Japan.

Department of Ophthalmology and Micro-Technology, Yokohama City University School of Medicine, Yokohama, Japan.

出版信息

Eur J Ophthalmol. 2021 Nov;31(6):3117-3123. doi: 10.1177/1120672121990561. Epub 2021 Jan 26.

DOI:10.1177/1120672121990561
PMID:33499660
Abstract

Cone-rod dystrophy (CORD) is one of the inherited retinal diseases that result in central visual field deterioration and decreased visual acuity (VA). In CORD patients, impaired photoreceptor cells are observed as the disruption of ellipsoid zone (EZ) on optical coherence tomography (OCT) images. In the present study, we calculated the index of residual EZ (rEZ) to quantify the function of photoreceptor cells and investigated the correlation between rEZ index and visual functions. Twenty-six eyes of 13 patients with clinical suspicion of CORD were examined. Visual field was tested with the Humphrey Visual Field Analyzer (HFA 10-2 program). We simultaneously obtained OCT images and calculated the area of decreased EZ intensity (EZa). Using the binarized OCT images, the percentage of the rEZ in a 3 × 3 mm area surrounding the macula was analyzed. To clarify interrator reproducibility, intraclass correlation coefficient (ICC) was calculated. Moreover, we investigated the association between OCT parameters and VA as well as the mean deviation (MD) value measured with HFA. The mean age of the patients was 48.5 ± 16.9 years. The mean central retinal thickness was 122.7 ± 73.2 μm. The mean EZa and rEZ were 22.2 ± 23.6 μm and 0.35 ± 0.31, respectively. The ICC of each rEZ index was 0.91 (95% CI: 0.89 < ICC < 0.93). Multivariate analysis indicated rEZ was significantly related to logMAR VA ( = 0.05) and rEZ and EZa were associated with the MD value ( = 0.014 and  = 0.009, linear mixed model). Furthermore, rEZ was also associated with photopic a- and b-wave amplitudes ( = 0.027 and  = 0.0024, respectively, linear mixed model). Taken together, the current results suggested the usefulness of rEZ quantification for predicting visual functions in CORD patients.

摘要

Cone-rod 营养不良 (CORD) 是一种遗传性视网膜疾病,可导致中央视野恶化和视力下降。在 CORD 患者中,光相干断层扫描 (OCT) 图像上观察到椭圆体带 (EZ) 中断,表明光感受器细胞受损。在本研究中,我们计算了剩余 EZ 指数 (rEZ) 以量化光感受器细胞的功能,并研究了 rEZ 指数与视觉功能之间的相关性。对 13 例临床疑似 CORD 的患者的 26 只眼进行了检查。使用 Humphrey 视野分析仪 (HFA 10-2 程序) 进行视野测试。我们同时获得 OCT 图像并计算 EZ 强度降低区 (EZa) 的面积。使用二值化 OCT 图像,分析黄斑周围 3×3mm 区域内 rEZ 的百分比。为了明确评估者间的可重复性,计算了组内相关系数 (ICC)。此外,我们还研究了 OCT 参数与视力 (VA) 以及 HFA 测量的平均偏差 (MD) 值之间的相关性。患者的平均年龄为 48.5±16.9 岁。平均中央视网膜厚度为 122.7±73.2μm。平均 EZa 和 rEZ 分别为 22.2±23.6μm 和 0.35±0.31。每个 rEZ 指数的 ICC 均为 0.91(95%CI: 0.89<ICC<0.93)。多变量分析表明 rEZ 与 logMAR VA 显著相关 ( = 0.05),rEZ 和 EZa 与 MD 值相关 ( = 0.014 和  = 0.009,线性混合模型)。此外,rEZ 还与明视 a-和 b-波幅度相关 ( = 0.027 和  = 0.0024,分别为线性混合模型)。综上所述,本研究结果表明 rEZ 定量分析在预测 CORD 患者的视觉功能方面具有一定的应用价值。

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