Department of Ophthalmology and Visual Sciences, University of Louisville, Louisville, Kentucky.
JAMA Ophthalmol. 2015 Apr;133(4):398-405. doi: 10.1001/jamaophthalmol.2014.5604.
The presence of choroidal hyperreflective foci in Stargardt disease is, to our knowledge, a potentially new finding. Evaluation of these foci may aid in better understanding of the disease process.
To report the presence of choroidal hyperreflective foci in spectral-domain optical coherence tomography (SD-OCT) images from eyes with Stargardt disease and investigate the relationship between the number of hyperreflective foci and disease severity.
DESIGN, SETTING, AND PARTICIPANTS: Twenty-six eyes of 13 patients with a clinical diagnosis of Stargardt disease were evaluated in a retrospective case series. Patient data were collected between January 1, 2009, and August 31, 2014.
The number of choroidal hyperreflective foci in Stargardt disease as well as correlation with visual acuity, central macular thickness (CMT), and disease duration were the main outcomes. A total of 707 macular SD-OCT scans of 13 patients with Stargardt disease were reviewed and evaluated for the presence and number of retinal/choroidal hyperreflective foci, central macular thickness, visual acuity, and disease duration. Enhanced depth imaging with OCT (EDI-OCT) scans available for 2 patients were compared with SD-OCT scans. A PubMed/Google search was performed to identify SD-OCT images in Stargardt disease; these findings were reviewed for the presence of choroidal hyperreflective foci.
The mean (SD) numbers of hyperreflective foci in each retinal/choroidal layer in decreasing frequency were as follows: Bruch membrane/retinal pigment epithelial (RPE) complex, 78.22 (24.39); choriocapillaris, 25.77 (17.57); Sattler layer, 18.59 (12.89); outer retina, 16.64 (6.96); inner retina, 0.95 (1.58); and Haller layer, 0.73 (0.87). The number of hyperreflective foci in the Bruch membrane/RPE complex increased exponentially with decreasing CMT (R2 = 0.99; P = .008). The number of hyperreflective foci in the Bruch membrane/RPE complex, choriocapillaris, and Sattler layer increased proportionally with decreasing visual acuity (R2 = 0.97, R2 = 0.95, and R2 = 0.99, respectively; and P = .007, P = .006, and P = .008, respectively). Direct correlation existed between the number of hyperreflective foci in the choriocapillaris and the Sattler layer and disease duration (R2 = 0.98 and R2 = 0.99, respectively; and P = .006 and P =.009, respectively). In the 10 studies on Stargardt disease, choroidal hyperreflective foci were present in 51 of 54 SD-OCT images (94%).
Based on the findings of the present study, choroidal hyperreflective foci in Stargardt disease, prominent at the Bruch membrane/RPE complex, choriocapillaris, and Sattler layer, correlate with disease severity in terms of retinal atrophy, decline in vision, and disease duration. Further studies are necessary to assess whether these findings are unique to Stargardt disease.
据我们所知，在斯塔加特病中出现脉络膜高反射灶是一种潜在的新发现。评估这些病灶可能有助于更好地了解疾病过程。
报告在斯塔加特病的光谱域光学相干断层扫描（SD-OCT）图像中存在脉络膜高反射灶，并研究高反射灶数量与疾病严重程度之间的关系。
设计、设置和参与者：回顾性病例系列研究了 13 名临床诊断为斯塔加特病的患者的 26 只眼。患者数据于 2009 年 1 月 1 日至 2014 年 8 月 31 日收集。
斯塔加特病的脉络膜高反射灶数量以及与视力、中央黄斑厚度（CMT）和疾病持续时间的相关性是主要结果。对 13 名斯塔加特病患者的 707 个黄斑 SD-OCT 扫描进行了回顾性评估，以评估视网膜/脉络膜高反射灶、中央黄斑厚度、视力和疾病持续时间的存在和数量。对 2 名患者的增强深度成像与 OCT（EDI-OCT）扫描进行了比较。在 PubMed/Google 上进行了搜索，以确定斯塔加特病的 SD-OCT 图像；审查了这些发现以确定脉络膜高反射灶的存在。
每个视网膜/脉络膜层的高反射灶数量以降序排列如下：Bruch 膜/视网膜色素上皮（RPE）复合物 78.22（24.39）；脉络膜毛细血管 25.77（17.57）；Sattler 层 18.59（12.89）；外视网膜 16.64（6.96）；内视网膜 0.95（1.58）；和 Haller 层 0.73（0.87）。Bruch 膜/RPE 复合物中的高反射灶数量随 CMT 的降低呈指数增加（R2=0.99；P=0.008）。Bruch 膜/RPE 复合物、脉络膜毛细血管和 Sattler 层中的高反射灶数量与视力下降呈比例增加（R2=0.97、R2=0.95 和 R2=0.99，分别；P=0.007、P=0.006 和 P=0.008，分别）。脉络膜毛细血管和 Sattler 层中的高反射灶数量与疾病持续时间呈直接相关（R2=0.98 和 R2=0.99，分别；P=0.006 和 P=0.009，分别）。在 10 项斯塔加特病研究中，54 张 SD-OCT 图像中有 51 张（94%）存在脉络膜高反射灶。
根据本研究的结果，在斯塔加特病中，Bruch 膜/RPE 复合物、脉络膜毛细血管和 Sattler 层中明显存在的脉络膜高反射灶与视网膜萎缩、视力下降和疾病持续时间等疾病严重程度相关。需要进一步研究以评估这些发现是否仅存在于斯塔加特病中。
