PDF(Pubmed)
Abstract：
UNASSIGNED: To characterize retinal structural biomarkers for progression in adult-onset Stargardt disease from multimodal retinal imaging in-vivo maps.
UNASSIGNED: Seven adult patients (29-69 years; 3 males) with genetically-confirmed and clinically diagnosed adult-onset Stargardt disease and age-matched healthy controls were imaged with confocal and non-confocal Adaptive Optics Scanning Light Ophthalmoscopy (AOSLO), optical coherence tomography (OCT), fundus infrared (FIR), short wavelength-autofluorescence (FAF) and color fundus photography (CFP). Images from each modality were scaled for differences in lateral magnification before montages of AOSLO images were aligned with en-face FIR, FAF and OCT scans to explore changes in retinal structure across imaging modalities. Photoreceptors, retinal pigment epithelium (RPE) cells, flecks, and other retinal alterations in macular regions were identified, delineated, and correlated across imaging modalities. Retinal layer-thicknesses were extracted from segmented OCT images in areas of normal appearance on clinical imaging and intact outer retinal structure on OCT. Eccentricity dependency in cell density was compared with retinal thickness and outer retinal layer thickness, evaluated across patients, and compared with data from healthy controls.
UNASSIGNED: In patients with Stargardt disease, alterations in retinal structure were visible in different image modalities depending on layer location and structural properties. The patients had highly variable foveal structure, associated with equally variable visual acuity (-0.02 to 0.98 logMAR). Cone and rod photoreceptors, as well as RPE-like structures in some areas, could be quantified on non-confocal split-detection AOSLO images. RPE cells were also visible on dark field AOSLO images close to the foveal center. Hypo-reflective gaps of non-waveguiding cones (dark cones) were seen on confocal AOSLO in regions with clinically normal CFP, FIR, FAF and OCT appearance and an intact cone inner segment mosaic in three patients.
UNASSIGNED: Dark cones were identified as a possible first sign of retinal disease progression in adult-onset Stargardt disease as these are observed in retinal locations with otherwise normal appearance and outer retinal thickness. This corroborates a previous report where dark cones were proposed as a first sign of progression in childhood-onset Stargardt disease. This also supports the hypothesis that, in Stargardt disease, photoreceptor degeneration occurs before RPE cell death.
UNASSIGNED: Seven adult patients (29-69 years; 3 males) with genetically-confirmed and clinically diagnosed adult-onset Stargardt disease and age-matched healthy controls were imaged with confocal and non-confocal Adaptive Optics Scanning Light Ophthalmoscopy (AOSLO), optical coherence tomography (OCT), fundus infrared (FIR), short wavelength-autofluorescence (FAF) and color fundus photography (CFP). Images from each modality were scaled for differences in lateral magnification before montages of AOSLO images were aligned with en-face FIR, FAF and OCT scans to explore changes in retinal structure across imaging modalities. Photoreceptors, retinal pigment epithelium (RPE) cells, flecks, and other retinal alterations in macular regions were identified, delineated, and correlated across imaging modalities. Retinal layer-thicknesses were extracted from segmented OCT images in areas of normal appearance on clinical imaging and intact outer retinal structure on OCT. Eccentricity dependency in cell density was compared with retinal thickness and outer retinal layer thickness, evaluated across patients, and compared with data from healthy controls.
UNASSIGNED: In patients with Stargardt disease, alterations in retinal structure were visible in different image modalities depending on layer location and structural properties. The patients had highly variable foveal structure, associated with equally variable visual acuity (-0.02 to 0.98 logMAR). Cone and rod photoreceptors, as well as RPE-like structures in some areas, could be quantified on non-confocal split-detection AOSLO images. RPE cells were also visible on dark field AOSLO images close to the foveal center. Hypo-reflective gaps of non-waveguiding cones (dark cones) were seen on confocal AOSLO in regions with clinically normal CFP, FIR, FAF and OCT appearance and an intact cone inner segment mosaic in three patients.
UNASSIGNED: Dark cones were identified as a possible first sign of retinal disease progression in adult-onset Stargardt disease as these are observed in retinal locations with otherwise normal appearance and outer retinal thickness. This corroborates a previous report where dark cones were proposed as a first sign of progression in childhood-onset Stargardt disease. This also supports the hypothesis that, in Stargardt disease, photoreceptor degeneration occurs before RPE cell death.
摘要：
■从多模态视网膜成像体内图表征成人发作的Stargardt病进展的视网膜结构生物标志物。
■对7名经过遗传证实和临床诊断的成年Stargardt病的成年患者（29-69岁；3名男性）和年龄匹配的健康对照者进行了共聚焦和非共聚焦自适应光学扫描光学眼镜（AOSLO）成像，光学相干断层扫描(OCT),眼底红外（FIR），短波长自发荧光（FAF）和彩色眼底照相（CFP）。在AOSLO图像的蒙太奇与正面FIR对齐之前，将每种模态的图像按横向放大倍数的差异进行缩放，FAF和OCT扫描以探索不同成像方式的视网膜结构变化。光感受器，视网膜色素上皮（RPE）细胞，斑点,并确定了黄斑区的其他视网膜改变，划定，并在成像模式之间相互关联。从临床成像上正常外观区域和OCT上完整外部视网膜结构的分割OCT图像中提取视网膜层厚度。细胞密度的偏心依赖性与视网膜厚度和视网膜外层厚度进行比较，对患者进行评估，并与健康对照的数据进行比较。
■在Stargardt病患者中，根据层的位置和结构特性，在不同的图像模式中可以看到视网膜结构的改变。患者的中央凹结构高度可变，与同样可变的视力相关(-0.02至0.98logMAR)。锥形和杆状光感受器，以及某些地区的类RPE结构，可以在非共焦分裂检测AOSLO图像上进行量化。RPE细胞在靠近中心凹的暗场AOSLO图像上也可见。在临床上正常CFP的共聚焦AOSLO上观察到非波导锥（暗锥）的低反射间隙，FIR,三名患者的FAF和OCT外观以及完整的圆锥内节段马赛克。
■在成人发作的Stargardt病中,暗锥体被鉴定为视网膜疾病进展的可能的第一迹象,因为这些在具有正常外观和外部视网膜厚度的视网膜位置中观察到。这证实了先前的报告，其中提出了深色视锥细胞作为儿童期发作的Stargardt病进展的第一个迹象。这也支持这样的假设，Stargardt病,光感受器变性发生在RPE细胞死亡之前。
■对7名经过遗传证实和临床诊断的成年Stargardt病的成年患者（29-69岁；3名男性）和年龄匹配的健康对照者进行了共聚焦和非共聚焦自适应光学扫描光学眼镜（AOSLO）成像，光学相干断层扫描(OCT),眼底红外（FIR），短波长自发荧光（FAF）和彩色眼底照相（CFP）。在AOSLO图像的蒙太奇与正面FIR对齐之前，将每种模态的图像按横向放大倍数的差异进行缩放，FAF和OCT扫描以探索不同成像方式的视网膜结构变化。光感受器，视网膜色素上皮（RPE）细胞，斑点,并确定了黄斑区的其他视网膜改变，划定，并在成像模式之间相互关联。从临床成像上正常外观区域和OCT上完整外部视网膜结构的分割OCT图像中提取视网膜层厚度。细胞密度的偏心依赖性与视网膜厚度和视网膜外层厚度进行比较，对患者进行评估，并与健康对照的数据进行比较。
■在Stargardt病患者中，根据层的位置和结构特性，在不同的图像模式中可以看到视网膜结构的改变。患者的中央凹结构高度可变，与同样可变的视力相关(-0.02至0.98logMAR)。锥形和杆状光感受器，以及某些地区的类RPE结构，可以在非共焦分裂检测AOSLO图像上进行量化。RPE细胞在靠近中心凹的暗场AOSLO图像上也可见。在临床上正常CFP的共聚焦AOSLO上观察到非波导锥（暗锥）的低反射间隙，FIR,三名患者的FAF和OCT外观以及完整的圆锥内节段马赛克。
■在成人发作的Stargardt病中,暗锥体被鉴定为视网膜疾病进展的可能的第一迹象,因为这些在具有正常外观和外部视网膜厚度的视网膜位置中观察到。这证实了先前的报告，其中提出了深色视锥细胞作为儿童期发作的Stargardt病进展的第一个迹象。这也支持这样的假设，Stargardt病,光感受器变性发生在RPE细胞死亡之前。
