Wilmer Eye Institute, Johns Hopkins University, Baltimore, Maryland, USA; Department of Ophthalmology and Visual Sciences, Case Western Reserve University, Cleveland, Ohio, USA.
Wilmer Eye Institute, Johns Hopkins University, Baltimore, Maryland, USA; Department of Ophthalmology, Medical University, Graz, Austria; Moorfields Eye Hospital, London, UK; Department of Ophthalmology, Johannes Kepler University, Linz, Austria.
Am J Ophthalmol. 2018 Sep;193:54-61. doi: 10.1016/j.ajo.2018.06.003. Epub 2018 Jun 8.
To investigate the natural history of Stargardt disease (STGD1) using fixation location and fixation stability.
Multicenter, international, prospective cohort study.
Fixation testing was performed using the Nidek MP-1 microperimeter as part of the prospective, multicenter, natural history study on the Progression of Stargardt disease (ProgStar). A total of 238 patients with ABCA4-related STGD1 were enrolled at baseline (bilateral enrollment in 86.6%) and underwent repeat testing at months 6 and 12.
Outcome measures included the distance of the preferred retinal locus from the fovea (PRL) and the bivariate contour ellipse area (BCEA). After 12 months of follow-up, the change in the eccentricity of the PRL from the anatomic fovea was -0.0014 degrees (95% confidence interval [CI], -0.27 degrees, 0.27 degrees; P = .99). The deterioration in the stability of fixation as expressed by a larger BCEA encompassing 1 standard deviation of all fixation points was 1.21 degrees squared (deg) (95% CI, -1.23 deg, 3.65 deg; P = .33). Eyes with increases and decreases in PRL eccentricity and/or BCEA values were observed.
Our observations point to the complexity of fixation parameters. The association of increasingly eccentric and unstable fixation with longer disease duration that is typically found in cross-sectional studies may be countered within individual patients by poorly understood processes like neuronal adaptation. Nevertheless, fixation parameters may serve as useful secondary outcome parameters in selected cases and for counseling patients to explain changes to their visual functionality.
通过固视位置和固视稳定性研究斯塔加特病（STGD1）的自然史。
多中心、国际、前瞻性队列研究。
固视测试使用 Nidek MP-1 微视野计进行，作为前瞻性、多中心斯塔加特病进展（ProgStar）自然史研究的一部分。共有 238 名 ABCA4 相关 STGD1 患者在基线时入组（86.6%为双侧入组），并在 6 个月和 12 个月时进行重复测试。
主要观察指标包括视网膜优势位置与黄斑中心凹的距离（PRL）和双变量轮廓椭圆面积（BCEA）。在 12 个月的随访后，PRL 从解剖性黄斑中心凹的偏心率变化为-0.0014 度（95%置信区间[CI]，-0.27 度，0.27 度；P=.99）。固视稳定性的恶化表现为 BCEA 增大，包含所有固视点的 1 个标准差，为 1.21 度平方（deg）（95%CI，-1.23 deg，3.65 deg；P=.33）。观察到 PRL 偏心率和/或 BCEA 值增加和减少的眼睛。
我们的观察结果表明固视参数的复杂性。在横断面研究中，通常发现与疾病持续时间较长相关的越来越偏心和不稳定的固视与在个体患者中由神经元适应等尚未充分了解的过程相抵消。然而，固视参数在某些情况下可能作为有用的次要结局参数，并为解释患者视觉功能变化提供依据。
