Characterizing the Natural History of Visual Function in Choroideremia Using Microperimetry and Multimodal Retinal Imaging.
Characterizing the Natural History of Visual Function in Choroideremia Using Microperimetry and Multimodal Retinal Imaging.
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DOI：
10.1167/iovs.17-22486
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发表时间：
2017-10-01
影响因子：
4.4
通讯作者：
MacLaren RE
中科院分区：
文献类型：
--
作者：
Jolly JK;Xue K;Edwards TL;Groppe M;MacLaren RE
脉络膜病视网膜向心性变性可导致早期视野受限和晚期中心视力丧失。后者标志着生活质量的急剧下降，但视觉预测仍然具有挑战性。我们通过相关的最佳矫正视力(BCVA)、微视野和多模式成像来研究CHM的视觉功能。对连续56例在牛津眼科医院就诊的CHM患者进行了BCVA、10-2微视野、光学相干断层扫描和眼底自发荧光(AF)检查。对21只眼进行重复微视野检查，并用Bland-Altman法进行统计分析。建立保留20/20BCVA的Kaplan-Meier生存图。评估眼间对称性。微视野重复性系数为1.45分贝。生存分析显示，两眼之间的模式难以区分(中位生存期39年)。黄斑敏感度在右眼和左眼表现出类似的下降，半衰期为13.6年。分区分析显示鼻侧到中心凹的下降速度更快。眼间对称性对微视野敏感度的一致性(r=0.95P&t；0.001)高于最佳矫正视力(r=0.42P=0.0006)。当中心凹距离房颤前缘至少2500μm时，黄斑中心凹的敏感度维持在接近正常的水平。BCVA是中央变性的标志，可以提供关于剩余视网膜位置的有价值的信息，以及对日常生活影响的衡量标准。微视野检查代表全球黄斑区域。两种视觉功能都表现出高度的眼间对称性，特别是在早期阶段，这表明对侧眼可以为评估对一只眼的干预提供合适的对照。这些发现可能有助于定制视觉预后并解释试验结果。
Centripetal retinal degeneration in choroideremia (CHM) leads to early visual field restriction and late central vision loss. The latter marks an acute decline in quality of life but visual prognostication remains challenging. We investigated visual function in CHM by correlating best-corrected visual acuity (BCVA), microperimetry and multimodal imaging. Fifty-six consecutive CHM patients attending Oxford Eye Hospital were examined with BCVA, 10–2 microperimetry, optical coherence tomography, and fundus autofluorescence (AF). Microperimetry was repeated in 21 eyes and analyzed with Bland-Altman. Kaplan-Meier survival plots of eyes retaining 20/20 BCVA were created. Intereye symmetry was assessed. Microperimetry coefficient of repeatability was 1.45 dB. Survival analysis showed an indistinguishable pattern between eyes (median survival 39 years). Macular sensitivity showed a similar decline in right and left eyes, with half-lives of 13.6 years. Zonal analysis showed faster decline nasal to the fovea. Intereye symmetry was more consistent for microperimetry sensitivity (r = 0.95, P < 0.001) than BCVA (r = 0.42, P = 0.0006). Near normal foveal sensitivity was maintained when the fovea was at least 2500 μm from the advancing edge of AF. BCVA is a marker of central degeneration and can provide valuable information about the position of the remaining retina as well as a measure of the impact on daily living. Microperimetry represents the global macular region. Both visual functions showed a high degree of intereye symmetry, particularly in early stages, indicating the fellow eye can provide a suitable control for assessing interventions to one eye. The findings may help to tailor visual prognosis and interpret outcomes of trials.
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影响因子：
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通讯作者：
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