ELLIPSOID ZONE MAPPING AND OUTER RETINAL ASSESSMENT IN STARGARDT DISEASE.
ELLIPSOID ZONE MAPPING AND OUTER RETINAL ASSESSMENT IN STARGARDT DISEASE.
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DOI：
10.1097/iae.0000000000001716
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发表时间：
2018-07
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影响因子：
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通讯作者：
Ehlers JP
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作者：
Arepalli S;Traboulsi EI;Ehlers JP
目的：量化并关联Stargardt病患者椭球区及光感受器外段变化与视力的关系。一项经irb批准的研究对32只患有Stargardt病的眼睛进行了研究。在光谱域光学相干断层扫描后，将黄斑立方体导出到一种新的分析工具中，并对从椭球区到视网膜色素上皮（RPE）的体积进行评估。利用这些信息，完成了对椭球区和RPE之间高度的正面表示。该分析提供了椭球区和光感受器外段的定量，包括萎缩（椭球区至RPE厚度= 0微米）和衰减（椭球区至RPE厚度< 20微米）。将这些参数与视力和对照组（n = 12只眼）进行比较。视力范围为20/30至20/250。椭球和感光器外节中央中央凹b扫描面积显著小于对照组（分别为0.13 +/ - 0.05mm2和0.17 +/ - 0.03 mm2, p=0.0074）。Stargardt中央中央凹b扫描的平均厚度为22.52 +/ - 9.0 um，而30.0 +/ - 5.08 um （p=0.0096）。Stargardt患者的萎缩和衰减明显更高（22%对1%，p=0.005, 43%对1%，p=0.0002）。视力与椭球区/外段体积呈正相关（R= 0.57, p < 0.005），与衰减和萎缩呈负相关（R= - 0.53和- 0.57，p均< 0.005）。患有Stargardt病的眼睛通常有明显的椭球区和外节的破坏。这种退行性变化通过一种新的评估平台成功地量化，并确定与视觉功能相关。该软件为定量评估和可能的纵向监测提供了机会。
To quantify and correlate ellipsoid zone and photoreceptor outer segment changes with visual acuity in Stargardt disease. An IRB-approved study of 32 eyes with Stargardt disease was performed. Following spectral domain optical coherence tomography, the macular cube was exported into a novel analysis tool and volumetric assessment from the ellipsoid zone to the retinal pigment epithelium (RPE) was performed. Utilizing this information, mapping was completed with en face representation of the height between the ellipsoid zone and RPE. This analysis provided quantification of ellipsoid zone and photoreceptor outer segments, including atrophy (ellipsoid zone to RPE thickness = 0 microns) and attenuation (ellipsoid zone to RPE thickness < 20 microns). These parameters were compared to visual acuity and to controls (n = 12 eyes). Visual acuity ranged from 20/30 to 20/250. Central foveal B-scan area of ellipsoid and photoreceptor outer segments was significantly less than controls (0.13 +/− 0.05mm2 vs 0.17 +/− 0.03 mm2, respectively p=0.0074). Central foveal B-scan mean thickness measured 22.52 +/− 9.0 um in Stargardt versus 30.0 +/− 5.08 um (p=0.0096). Atrophy and attenuation were significantly higher in Stargardt patients (22% vs. 1%, p=0.005 and 43% vs 1%, p=0.0002). Visual acuity directly correlated with ellipsoid zone/outer segment volume (R = 0.57, p < 0.005), and inversely correlated with attenuation and atrophy (R= −0.53 and −0.57; p < 0.005 for all). Eyes with Stargardt disease frequently have significant disruption of the ellipsoid zone and outer segments. This degenerative change was successfully quantified with a novel assessment platform and identified correlates with visual function. This software provides the opportunity for quantitative assessment and possible longitudinal surveillance.