多模式影像技术在Stargardt病不同病变阶段的临床运用

目的： 观察多模式影像技术在Stargardt病不同病变阶段的影像特征。 方法： 回顾性系列病例研究。 对云南大学附属医院2016 年5 月至2019 年8 月确诊的28 例（ 56 眼） Stargardt患者进行眼底彩色照相、 自发荧光、 眼底荧光血管造影、 光学相干断层扫描（ OCT）、 炫彩眼底成像、 血流成像OCT（ OCTA） 等多模式检查， 总结各类影像检查在疾病不同阶段的特征。 结果： 眼底彩色照相可显示， 随病变进 展黄斑区的萎缩病变逐渐扩大， 伴后极部眼底色泽改变。炫彩成像通过多波长的组合， 可用更好的 对比度和层次感显示出萎缩的细胞和范围。眼底自发荧光通过自发荧光的高低变化提示视网膜色 素上皮（ RPE） 的受损情况， 同时能清楚直观地显示黄斑区萎缩病灶的大小。眼底荧光血管造影在 Stargardt病中的观察可直接反映视网膜色素上皮层、 脉络膜血管的损伤程度。 随着Stargardt病的发展， 黄斑区RPE层萎缩范围扩大， 视网膜及脉络膜毛细血管继发进行性萎缩。 OCT提供了断层眼底的影 像信息， 随病变进展， 中心凹周围神经上皮层变薄， 光感受器不同程度萎缩、 消失， RPE层不同程 度萎缩， 反光减弱， 脉络膜层毛细血管萎缩， 从层次深度和范围的广度都体现了病变的发展。 OCTA 中本研究观察到随着病变的进展， 各层次血管密度呈减少趋势。扫描视网膜深层血管时， 透见了 下方的脉络膜血管， 随着萎缩加重， 透见的脉络膜血管的范围扩大。 结论： 不同的眼科影像技术在 Stargardt病的不同阶段表现有各自特点和优势， 为临床医师提供了更多角度的追踪随访、 病情评估 的手段。

Clinical Application of Multi-Mode Imaging Technology at Different Stages of Stargardt's Disease

Objective: To explore the imaging characteristics of a multi-mode imaging techniques at different stages of Stargardt's disease. Methods: This was a retrospective analysis of 28 cases (56 eyes) of patients with Stargardt's disease diagnosed in Affiliated Hospital of Yunnan University from May 2016 to August 2019. Fundus color photography, autofluorescence, fundus fluorescein angiography, optical coherence tomography (OCT), multicolor fundus imaging, OCT angiography (OCTA), and characteristics of variousimaging examinations at different stages of the disease were summarized. Results: Color photography of the fundus showed that the atrophic lesions in the macular area gradually expand with the progression of the lesion, with color change at the posterior pole. Multicolor imaging combines multiple wavelengths to show the atrophy of cells and range with better contrast. The fundus autofluorescence showed the damage in the retinal pigment epithelium (RPE), and can clearly and visually show the size of the atrophy lesion in the macular area. Fundus fluorescein angiography can directly reflect the degree of damage in the retinal pigment epithelial layer and choroidal blood vessels in Stargardt's disease. With the development of Stargardt's disease, the atrophy of the RPE layer in the macular area was enlarged, and the retina and choroidal capillaries progressively atrophied. OCT provided image information of the fundus in different layers. As the lesion progressed, the peripheral nerve epithelial layer became thinner, the photoreceptors and RPE shrank and disappeared to varying degrees, and the choroidal capillary shrank from the depth and range of the layer. In the OCTA, we observed that as the lesion progressed, the vascular density at each level decreased. When the deep blood vessels of the retina were scanned, the choroidal blood vessels below were observed, and as the atrophy increased, the range of the exposed choroidal vessels increased.