眼底自发荧光联合频域 OCT 对 Stargardt 病病理改变的临床观察

摘要

AIM:To analyze clinical features of Stargardt disease by fundus autofluorescence ( FAF ) and observe microstructural changes of the retina by frequency -domain optical coherence tomography ( Spectralis OCT ) to explore the clinical value of combined FAF and OCT in diagnosis of Stargardt's disease. METHODS: Non -interventional observation study. Confocal scanning laser ophthalmoscope ( Heidelberg, Germany Company) was applied on 9 patients (18 eyes) with Stargardt disease for blue autofluorescence ( BL-FAF, excitation 488nm, filter >500nm), Spectralis OCT examination, fundus photography, mfERG examination and fundus fluorescein angiography ( FFA) examination. Seven cases also received indocyanine green angiography ( ICGA ) inspection. The study analyzed distribution characteristics of Stargardt's disease under FAF examination and microstructural changes of the retina under Spectralis OCT. RESULTS:The BL-FAF of the oval macular lesions presented low fluorescence and visible edge demonstrated varying thickness and strong fluorescence. OCT showed retinal pigment epithelial lesions ( RPE ) , photoreceptors ( PR) atrophy and neurosensory meager. Under FFA examination, scattered point-like fluorescence could be seen in the early stage, and macular atrophy tumor size appears consistent with the bull's -eye fluorescence could be seen in the late stage.The disease demonstrated choriocapillaris atrophy in the late stage under ICGA examination.New vessels could be seen in rare cases. CONCLUSION:FAF with Spectralis OCT and mf-ERG is an effective non -invasive examination methods for diagnosis of Stargardt disease. Changes of its clinical characteristics might suggest degeneration of both RPE and PR and various pathological and physiological changes of lipofuscin.%目的：分析Stargardt 病中眼底自发荧光（ FAF ）的临床特征，应用频域光学相干断层扫描（ Spectralis OCT ）观察相应的视网膜微结构改变，进一步探讨两者联合应用在Stargardt病诊断中的价值。　　方法：非干预性、观察性研究。应用激光共焦扫描检眼镜（德国Heidelberg公司）对Stargardt病患者9例18眼进行蓝光自发荧光（BL-FAF，激发光488nm，滤光片＞500nm）、Spectralis OCT检查、眼底照相、mfERG检查及荧光素眼底血管造影（ fundus fluorescein angiography ，FFA）检查，其中7例同时接受了吲哚青绿血管造影（ indocyanine green angiography ，ICGA）检查；分析Stargardt病FAF分布特征及Spectralis OCT所示视网膜微结构的对应改变。　　结果：黄斑部椭圆形病变区域呈现BL-FAF均为低荧光，活动期病例可见边缘厚薄不一的强荧光。 OCT显示病变区视网膜色素上皮（ retinal pigment epithelial ，RPE）和光感受器（photoreceptors，PR）萎缩，神经上皮层菲薄。 FFA早期可出现散在的点状透见荧光，晚期黄斑部出现与萎缩灶大小相符的牛眼状透见荧光；病变晚期ICGA表现为脉络膜毛细血管萎缩，个别病例可见新生血管。　　结论：眼底自发荧光联合Spectralis OCT 及mf-ERG是诊断Stargardt病十分有用的非侵入性检查手段，其特征性改变提示病变存在RPE及PR的共同退化，同时也揭示脂褐素这种荧光性物质不同的病理生理变化。