Quantifying the Rate of Ellipsoid Zone Loss in Stargardt Disease
Quantifying the Rate of Ellipsoid Zone Loss in Stargardt Disease
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DOI：
10.1016/j.ajo.2017.10.032
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发表时间：
2018-02-01
影响因子：
4.2
通讯作者：
Handa, James T.
中科院分区：
文献类型：
--
作者：
Cai, Cindy X.;Light, Jacob G.;Handa, James T.
目得：为了确定一个可靠的方法，使用椭圆体区（EZ）的光学相干断层扫描（OCT）跟踪疾病进展Stardgardt disease（STGD）.DESIGN：Retrospective reliability study.METHODS：STGD患者与遗传学证实ABCA 4基因突变，在威尔默眼科研究所与后续访问分开至少12个月确定。获得以中央凹为中心的光谱域光学相干断层扫描（SD-OCT）黄斑体积扫描和眼底自发荧光（FAF）图像。根据SD-OCT计算EZ损失面积，根据FAF计算视网膜色素上皮（RPE）损失面积。扫描重新分析的主要分级机，以评估intragrader的可靠性，并重新分析的第二个等级，以评估intergrader reliability.RESULTS：16 STGD患者（共31只眼），平均随访2年（范围1-4.7年）。EZ的平均丢失率为0.31 +/- 0.31 mm（2）/年，与RPE的平均丢失率0.33 +/- 0.38 mm（2）/年相似。在初次检查时，EZ损失的平均面积为4.18 +/- 1.91 mm（2），大于RPE损失的初始面积2.25 +/- 1.66 mm（2）（P <0.01）。一年级学生在重测时EZ损失面积的绝对差异为0.12 ± 0.10 mm（2），两年级学生之间为0.21 ± 0.21 mm（2）。EZ丢失的分级器内和分级器间可靠性的组内相关性（ICC）为0.99。结论：纵向跟踪SD-OCT黄斑体积扫描的EZ丢失面积是监测STGD疾病进展的可靠方法。这可用作STGD相关临床试验中敏感的解剖结局指标。(C)2017爱思唯尔公司All rights reserved.
PURPOSE: To determine a reliable method of using the ellipsoid zone (EZ) on optical coherence tomography (OCT) to track disease progression in Stardgardt disease (STGD).DESIGN: Retrospective reliability study.METHODS: STGD patients with genetically confirmed ABCA4 gene mutations seen at the Wilmer Eye Institute with follow-up visits separated by at least 12 months were identified. Spectral-domain optical coherence tomography (SD-OCT) macula volume scans centered at the fovea and fundus autofluorescence (FAF) images were obtained. The area of EZ loss was calculated from the SD-OCT and the area of retinal pigment epithelium (RPE) loss from the FAF. Scans were reanalyzed by the primary grader to assess intragrader reliability, and reanalyzed by a second grader to assess intergrader reliability.RESULTS: Sixteen STGD patients (total of 31 eyes) were followed for a mean of 2 years (range 1-4.7 years). The mean rate of EZ loss, 0.31 +/- 0.31 mm(2)/year, was similar to the average rate of RPE loss, 0.33 +/- 0.38 mm(2)/year. The average area of EZ loss at the initial examination, 4.18 +/- 1.91 mm(2), was larger than the initial area of RPE loss, 2.25 +/- 1.66 mm(2) (P < .01). The absolute difference of the area of EZ loss on test-retest for the first grader was 0.12 0.10 mm(2), and between graders 0.21 +/- 0.21 mm(2). The intraclass correlation (ICC) of both intragrader and intergrader reliability for EZ loss was excellent at 0.99.CONCLUSIONS: Tracking the area of EZ loss on SD-OCT macular volume scans longitudinally is a reliable way of monitoring disease progression in STGD. This could be used as a sensitive anatomic outcome measure in clinical trials related to STGD. (C) 2017 Elsevier Inc. All rights reserved.