Scheie Eye Institute, Department of Ophthalmology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA.
Invest Ophthalmol Vis Sci. 2012 Feb 21;53(2):841-52. doi: 10.1167/iovs.11-8415. Print 2012 Feb.
To measure macular visual function in patients with unstable fixation, to define the photoreceptor source of this function, and to estimate its test-retest repeatability as a prerequisite to clinical trials.
Patients (n = 38) with ABCA4-associated retinal degeneration (RD) or with retinitis pigmentosa (RP) were studied with retina-tracking microperimetry along the foveo-papillary profile between the fovea and the optic nerve head, and point-by-point test-retest repeatability was estimated. A subset with foveal fixation was also studied with dark-adapted projection perimetry using monochromatic blue and red stimuli along the horizontal meridian.
Macular function in ABCA4-RD patients transitioned from lower sensitivity at the parafovea to higher sensitivity in the perifovea. RP patients had the inverse pattern. Red-on-red microperimetric sensitivities successfully avoided ceiling effects and were highly correlated with absolute sensitivities. Point-by-point test-retest limits (95% confidence intervals) were ±4.2 dB; repeatability was not related to mean sensitivity, eccentricity from the fovea, age, fixation location, or instability. Repeatability was also not related to the local slope of sensitivity and was unchanged in the parapapillary retina.
Microperimetry allows reliable testing of macular function in RD patients without foveal fixation in longitudinal studies evaluating natural disease progression or efficacy of therapeutic trials. A single estimate of test-retest repeatability can be used to determine significant changes in visual function at individual retinal loci within diseased regions that are homogeneous and those that are heterogeneous and also in transition zones at high risk for disease progression.
测量不稳定固视患者的黄斑视觉功能，确定该功能的光感受器来源，并评估其测试-重测重复性，以为临床试验做准备。
对 ABCA4 相关性视网膜变性（RD）或色素性视网膜炎（RP）患者进行视网膜跟踪微视野检查，在黄斑与视神经头之间的黄斑-乳头轮廓上进行检测，并评估点与点之间的测试-重测重复性。对一小部分具有中心凹固视的患者，还使用暗适应投影视野计，用单色蓝色和红色刺激物在水平子午线方向上进行检测。
ABCA4-RD 患者的黄斑功能从中等敏感的旁中心区过渡到高敏感的中心凹周围区。RP 患者则相反。红对红微视野测量敏感性成功避免了天花板效应，与绝对敏感性高度相关。点与点测试-重测界限（95%置信区间）为±4.2dB；重复性与平均敏感性、与黄斑中心凹的偏心度、年龄、固视位置或不稳定性无关。重复性也与局部敏感性斜率无关，在旁中心凹视网膜中没有改变。
微视野测量法可在不进行黄斑固视的情况下，可靠地测试 RD 患者的黄斑功能，适用于评估自然疾病进展或治疗试验疗效的纵向研究。可单次估计测试-重测重复性，以确定病变区域内同质和异质的个体视网膜部位以及疾病进展高风险的过渡区的视觉功能的显著变化。
