Quantification of Peripapillary Sparing and Macular Involvement in Stargardt Disease (STGD1)
Quantification of Peripapillary Sparing and Macular Involvement in Stargardt Disease (STGD1)
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DOI：
10.1167/iovs.11-7693
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发表时间：
2011-10-01
影响因子：
4.4
通讯作者：
Greenstein, Vivienne C.
中科院分区：
文献类型：
--
作者：
Burke, Tomas R.;Rhee, David W.;Greenstein, Vivienne C.
目的.对Stargardt病（STGD 1）黄斑区和视乳头周围区的结构和功能进行量化和比较。27例患者（27只眼）和12例年龄相似的对照组（12只眼）进行了研究。根据全视野视网膜电图（ERG）结果对患者进行分类。眼底自体荧光（FAF）和光谱域光学相干断层扫描（SD-OCT）水平线扫描通过中央凹和视乳头周围区域获得。采用计算机辅助、人工分割技术，测量从1 ~ 4颞侧至视盘边缘的中央凹和视乳头周围区的外核层+外丛状层（ONL+）、外段（OS）和视网膜色素上皮（RPE）的厚度。中央10视敏度采用微视野法进行评估，并与视网膜层厚度相关。与中央黄斑相比，对照组和患者之间的ONL+、OS和RPE层厚度的差异在鼻和颞黄斑中较小。在鼻中检测到ONL+和/或OS层的相对保留（即，13例黄斑外病变患者中，8例在FAF检查中发现了视乳头周围黄斑;在该亚组中也检测到了相对的功能保留。所有14例局限于中央黄斑的患者，如FAF所检测到的，在正常RPE厚度区域显示ONL+和OS层变薄。在FAF上检测到患有黄斑外疾病的STGD 1患者的相对视乳头周围保留。STGD 1中，光感受器变薄可能先于RPE变性。（Invest Ophthalmol维斯科学。2011;52：8006-8015）DOI：10.1167/iovs.11-7693
PURPOSE. To quantify and compare structure and function across the macula and peripapillary area in Stargardt disease (STGD1).METHODS. Twenty-seven patients (27 eyes) and 12 age-similar controls (12 eyes) were studied. Patients were classified on the basis of full-field electroretinogram (ERG) results. Fundus autofluorescence (FAF) and spectral domain-optical coherence tomography (SD-OCT) horizontal line scans were obtained through the fovea and peripapillary area. The thicknesses of the outer nuclear layer plus outer plexiform layer (ONL+), outer segment (OS), and retinal pigment epithelium (RPE) were measured through the fovea, and peripapillary areas from 1 to 4 temporal to the optic disc edge using a computeraided, manual segmentation technique. Visual sensitivities in the central 10 were assessed using microperimetry and related to retinal layer thicknesses.RESULTS. Compared to the central macula, the differences between controls and patients in ONL+, OS, and RPE layer thicknesses were less in the nasal and temporal macula. Relative sparing of the ONL+ and/or OS layers was detected in the nasal (i.e., peripapillary) macula in 8 of 13 patients with extramacular disease on FAF; relative functional sparing was also detected in this subgroup. All 14 patients with disease confined to the central macula, as detected on FAF, showed ONL+ and OS layer thinning in regions of normal RPE thickness.CONCLUSIONS. Relative peripapillary sparing was detected in STGD1 patients with extramacular disease on FAF. Photoreceptor thinning may precede RPE degeneration in STGD1. (Invest Ophthalmol Vis Sci. 2011;52:8006-8015) DOI:10.1167/iovs.11-7693