Novel grading system for quantification of cystic macular lesions in Usher syndrome.
Novel grading system for quantification of cystic macular lesions in Usher syndrome.
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DOI：
10.1186/s13023-015-0372-0
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发表时间：
2015-12-10
影响因子：
3.7
通讯作者：
Sahel JA
中科院分区：
文献类型：
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作者：
Sliesoraityte I;Peto T;Mohand-Said S;Sahel JA
评估用于量化Usher综合征（USH）患者囊性黄斑病变（CML）的光学相干断层扫描（OCT）的新型分级系统，重点关注MOY7A和USH2A突变的CML相关改变。2位读者对Eurush数据库中前瞻性上传的76例患者（平均年龄42±14岁）数据进行了评估。OCT通过中央凹获得高质量的横断面图像。根据以下特征，将CML分为无、轻度、中度或重度：视网膜下积液，无明显CML边界；中央黄斑厚度；CML最大直径；所有可检测CML的计算平均值；CML总检出数；受CML影响的视网膜层。评价了年级内和年级间的再现性。37%的USH、45%的MYO7A、29%的USH2A眼存在CML。在CML患者中：52%为轻度，22%为中度，26%为重度。视网膜内核层占50%，外核层占44%，视网膜神经节细胞层占6%。对于跨年级的重复性分析，CML的符合率为97%，kappa统计量为0.91 （95% CI 0.83-0.99）。对于评分者分析，CML的一致性率为98%，而kappa统计值为0.96 （95% CI 0.92-0.99）。新的分级系统是一种可重复的工具，用于对USH合并CML的OCT图像进行分级，并有可能在临床治疗试验中用于客观跟踪黄斑病理。
To evaluate novel grading system used to quantify optical coherence tomography (OCT) scans for cystic macular lesions (CML) in Usher syndrome (USH) patients, focusing on CML associated alterations in MOY7A and USH2A mutations. Two readers evaluated 76 patients’ (mean age 42 ± 14 years) data prospectively uploaded on Eurush database. OCT was used to obtain high quality cross-sectional images through the fovea. The CML was graded as none, mild, moderate or severe, depending on the following features set: subretinal fluid without clearly detectable CML boundaries; central macular thickness; largest diameter of CML; calculated mean of all detectable CML; total number of detectable CML; retinal layers affected by CML. Intra-and inter-grader reproducibility was evaluated. CML were observed in 37 % of USH eyes, while 45 % were observed in MYO7A and 29 % in USH2A cases. Of those with CML: 52 % had mild, 22 % had moderate and 26 % had severe changes, respectively. CML were found in following retinal layers: 50 % inner nuclear layer, 44 % outer nuclear layer, 6 % retinal ganglion cell layer. For the inter-grader repeatability analysis, agreements rates for CML were 97 % and kappa statistics was 0.91 (95 % CI 0.83-0.99). For the intra-grader analysis, agreement rates for CML were 98 %, while kappa statistics was 0.96 (95 % CI 0.92-0.99). The novel grading system is a reproducible tool for grading OCT images in USH complicated by CML, and potentially could be used for objective tracking of macular pathology in clinical therapy trials.