两种波长自发荧光联合频域光学相干断层扫描对视网膜色素变性微结构与功能的再认识

Two.wavelength fundus autofluorescence combined with spectral-domain optical coherence tomography for recognition of retinitis pigmentosa in microstructure and function

目的分析视网膜色素变性（RP）中两种波长眼底自发荧光（FAF）和多焦视网膜电陶（mf-ERG）的临床特征，应用频域光学相干断层扫描（Spectralis OCT）观察相应的视网膜做结构改变，进一步探讨两者联合应用在RP诊断中的价值。方法非十预性、观察性研究。应用激光共焦扫描检眼镜（德国Heidelberg公司）对8例（15眼）RP患者进行蓝光白发荧光（BL-FAF，激发光488nm，滤光片〉500nm）、近红外波长自发荧光fNIR-FAF，激发光787nm，滤光片〉800nm）及Speetralis OCT检查。5例进行了荧光素眼底血管造影（FFA）检查，其中3例同时接受了吲哚青绿血管造影（ICGA）检查；7例进行了眼底照相：4例患者接受了mf-ERG检查。分析RP病例FAF分布特征及Spectralis OCT所示视网膜微结构与mf-ERG中反应振幅密度的对应改变。结果在视网膜色素上皮（RPE）和光感受器（PR）萎缩及骨细胞沉着部位，BL-FAF与NIR-FAF均为低荧光，mf-ERG反应强度降低．中心反应区正常尖峰消失。正常FAF区域内Spectralis OCT提示视网膜PR、RPE保存完整，外界膜（OLM）部分断裂。部分病例NIR-FAF高荧光区域小于BL-FAF。FFA早期m现典型的“窗样缺损”现象，提示RPE受损：早期ICGA表现为脉络膜毛细血管萎缩。结论两种波长FAF联合Spectralis OCT及mf-ERG是诊断RP十分有用的非侵入性T具，其特征性改变提示PR和RPE细胞拥有共同的退化途径．而BL-FAF和NIR-FAF提示该途径所累及的脂褐素和非脂褐素的荧光性物质分别存在不同的病理生理变化。

Objective To combine two-wavelength fundus autofluorescence （FAF） with multifocal electroretinography （nff-ERG） in retinitis pigmentosa （RP） and discuss the microstructural abnormalities as seen with spectral-domain optical conherence tomography （Spectralis OCT）. Methods This was a noninterventional, observational study, based on the observation and analysis of a series of cases. Fifteen eyes of 8 patients with RP were evaluated with Spectralis OCT, blue light fundus autofluorescence （BL-FAF, excitation 488 nm, emission 〉500 nm） and near-infrared fundus autofluoreseence （NIR-FAF, excitation 787 rim, emission 〉800 nm）. BL-FAF and NIR-FAF imaging were performed with a confocal scanning laser ophthalmoscope （Heidelberg Retina Angiograph 2）. Five of 8 patients received fnndus fluorescein angiography （FFA） while 3 also had indocyanine green angiography （ICGA） simultaneously. Seven had fundus photography and mf-ERG was performed on 4 cases. The FAF distribution in RP was observed and the correlated changes of microstructural abnormalities were analyzed with Spectralis OCT and the response （Amp. P1） in mf-ERG. Results BL-FAF and NIR-FAF showed a hypoautofluorescent pattern in the atrophied area of the RPE and photoreceptor and bone spiculepigmentation, where mf-ERG showed that the peak in the fovea had disappeared and responses had markedly decreased throughout the maeula. Spectralis OCT demonstrated that normal FAF demarcated the border within an area of preserved PR and RPE, While outer limiting membrane （OLM） showed disturbances. In several cases, the hyperautofluorescent pattern of NIR-FAF was less obvious than that of BL-FAF in these areas. In FFA images showing early stages, a window defect was seen at the posterior pole, suggesting RPE malfunction and in ICGA images of early stages, choroidal capillary atrophy was observed. Conclusion The two-wavelength FAF, Spectralis OCT and mf-ERG are useful noninvasive diagnostic tools to identify retinitis pigmentosa. The change in these measures indicate a common pathway for photoreeeptor and RPE degeneration involving lipofuscin and non-lipofusein fluorophores such as melanin （oxidation） with different pathophysiologic processes by BL-FAF and NIR-FAF.