Schönbach Etienne M, Wolfson Yulia, Strauss Rupert W, Ibrahim Mohamed A, Kong Xiangrong, Muñoz Beatriz, Birch David G, Cideciyan Artur V, Hahn Gesa-Astrid, Nittala Muneeswar, Sunness Janet S, Sadda SriniVas R, West Sheila K, Scholl Hendrik P N
Wilmer Eye Institute, Johns Hopkins University, Baltimore, Maryland.
Wilmer Eye Institute, Johns Hopkins University, Baltimore, Maryland2Moorfields Eye Hospital, London, United Kingdom3Department of Ophthalmology, Johannes Kepler University, Linz, Austria4Department of Ophthalmology, Medical University, Graz, Austria5Department of Ophthalmology, University of Basel, Switzerland.
JAMA Ophthalmol. 2017 Jul 1;135(7):696-703. doi: 10.1001/jamaophthalmol.2017.1162.
New outcome measures for treatment trials for Stargardt disease type 1 (STGD1) and other macular diseases are needed. Microperimetry allows mapping of light sensitivity of the macula and provides topographic information on visual function beyond visual acuity.
To measure and analyze retinal light sensitivity of the macula in STGD1 using fundus-controlled perimetry (microperimetry).
DESIGN, SETTING, AND PARTICIPANTS: This was a multicenter prospective cohort study. A total of 199 patients and 326 eyes with molecularly confirmed (ABCA4) STGD1 underwent testing with the Nidek MP-1 microperimeter as part of the multicenter, prospective Natural History of the Progression of Atrophy Secondary to Stargardt Disease (ProgStar) study. Sensitivity of 68 retinal loci was tested, and the mean sensitivity (MS) was determined; each point was categorized as "normal," "relative," or "deep" scotoma.
Mean sensitivity and the number of points with normal sensitivity, relative, or deep scotomas.
Mean (SD) patient age was 34.2 (14.7) years, mean (SD) best-corrected visual acuity of all eyes was 47.8 (16.9) Early Treatment Diabetic Retinopathy Study letter score (approximately 20/100 Snellen equivalent), and mean MS of all eyes of all 68 points was 11.0 (5.0) dB. The median number of normal points per eye was 49 (mean [SD], 41.3 [20.8]; range, 0-68); abnormal sensitivity and deep scotomas were more prevalent in the central macula. Mean sensitivity was lower in the fovea (mean [SD], 2.7 [4.4] dB) than in the inner (mean [SD], 6.8 [5.8] dB) and outer ring (mean [SD], 12.7 [5.3] dB). Overall MS per eye was 0.086 dB lower per year of additional age (95% CI, -0.13 to -0.041; P < .001) and 0.21 dB lower per additional year of duration of STGD1 (95% CI, -0.28 to -0.14; P < .001). Longer duration of STGD1 was associated with worse MS (β = -0.18; P < .001), with a lower number of normal test points (β = -0.71; P < .001), and with a higher number of deep scotoma points (β = -0.70; P < .001). We found 11 eyes with low MS (<6 dB) but very good best-corrected visual acuity of at least 72 Early Treatment Diabetic Retinopathy Study letter score (20/40 Snellen equivalent).
We provide an extensive analysis of macular sensitivity parameters in STGD1 and demonstrate their association with demographic characteristics and vision. These data suggest microperimetry testing provides a more comprehensive assessment of retinal function and will be an important outcome measure in future clinical trials.
对于1型斯塔加特病(STGD1)和其他黄斑疾病的治疗试验,需要新的疗效指标。微视野计可绘制黄斑的光敏感度图,并提供超出视力的视觉功能的地形图信息。
使用眼底控制视野计(微视野计)测量和分析STGD1患者黄斑的视网膜光敏感度。
设计、地点和参与者:这是一项多中心前瞻性队列研究。作为多中心前瞻性斯塔加特病继发萎缩进展的自然史(ProgStar)研究的一部分,共有199例患者和326只经分子确诊(ABCA4)的STGD1眼睛接受了尼德克MP-1微视野计测试。测试了68个视网膜位点的敏感度,并确定了平均敏感度(MS);每个点被分类为“正常”、“相对”或“深度”暗点。
平均敏感度以及具有正常敏感度、相对或深度暗点的点数。
患者平均(标准差)年龄为34.2(14.7)岁,所有眼睛的平均(标准差)最佳矫正视力为47.8(16.9)早期糖尿病性视网膜病变研究字母评分(约相当于20/100 Snellen视力),所有68个点的所有眼睛的平均MS为11.0(5.0)dB。每只眼睛正常点的中位数为49个(平均[标准差],41.3[20.8];范围,0 - 68);异常敏感度和深度暗点在黄斑中心更常见。中央凹的平均敏感度(平均[标准差],2.7[4.4]dB)低于内环(平均[标准差],6.8[5.8]dB)和外环(平均[标准差],12.7[5.3]dB)。每只眼睛的总体MS每增加一岁降低0.086 dB(95%置信区间,-0.13至-0.0 .041;P < .001),每增加一年STGD1病程降低0.21 dB(95%置信区间,-0.28至-0.14;P < .001)。STGD1病程较长与较差的MS相关(β = -0.18;P < .001),正常测试点数量较少(β = -0.71;P < .001),深度暗点点数较多(β = -0.70;P < .001)。我们发现11只眼睛的MS较低(<6 dB),但最佳矫正视力非常好,至少为72早期糖尿病性视网膜病变研究字母评分(20/40 Snellen视力相当)。
我们对STGD1患者的黄斑敏感度参数进行了广泛分析,并证明了它们与人口统计学特征和视力的关联。这些数据表明微视野计测试可提供对视网膜功能更全面的评估,并将成为未来临床试验中的一项重要疗效指标。