Quantitative Fundus Autofluorescence and Optical Coherence Tomography in Best Vitelliform Macular Dystrophy
Quantitative Fundus Autofluorescence and Optical Coherence Tomography in Best Vitelliform Macular Dystrophy
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DOI：
10.1167/iovs.13-13834
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发表时间：
2014-03-01
影响因子：
4.4
通讯作者：
Sparrow, Janet R.
中科院分区：
文献类型：
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作者：
Duncker, Tobias;Greenberg, Jonathan P.;Sparrow, Janet R.
目的。采用定量眼底自身荧光（qAF）、光谱域光学相干断层扫描（SD-OCT）分割、多模态成像等方法，探讨最佳黄斑营养不良（BVMD）的发病机制，并鉴别病变与非病变眼底区域的异常。本文对16例临床诊断为BVMD的患者进行了研究。使用共聚焦扫描激光检眼镜获得自荧光图像（30度，488 nm激发），该检眼镜配有内部荧光参考，以考虑可变激光功率和检测器灵敏度。每个图像的灰度级（GLs）被校准到参考，零GL，放大倍率和标准光学介质密度，以产生qAF。获得水平SD-OCT扫描并手动分割视网膜层。此外，彩色和近红外反射率（NIR-R）图像被配准到AF图像。对所有患者进行BEST1基因突变筛查。在另外三名BVMD患者中，在卵黄样病变内进行了体内荧光光谱测量。平均非损伤性qAF在正常年龄范围内。与对照组相比，病灶内最大qAF明显增加。SD-OCT分割显示病灶外节等效厚度增加，外核层厚度减少。病变边界以外的过渡区也有变化。在亚临床患者中，未发现视网膜层厚度异常。卵黄样病变记录的荧光光谱与视网膜色素上皮细胞脂褐素的荧光光谱一致。基于qAF， BEST1突变不会导致非病变眼底区域脂褐素水平升高。
PURPOSE. Quantitative fundus autofluorescence (qAF), spectral domain optical coherence tomography (SD-OCT) segmentation, and multimodal imaging were performed to elucidate the pathogenesis of Best vitelliform macular dystrophy (BVMD) and to identify abnormalities in lesion versus nonlesion fundus areas.METHODS. Sixteen patients with a clinical diagnosis of BVMD were studied. Autofluorescence images (30 degrees, 488-nm excitation) were acquired with a confocal scanning laser ophthalmoscope equipped with an internal fluorescent reference to account for variable laser power and detector sensitivity. The grey levels (GLs) of each image were calibrated to the reference, zero GL, magnification, and normative optical media density, to yield qAF. Horizontal SD-OCT scans were obtained and retinal layers manually segmented. Additionally, color and near-infrared reflectance (NIR-R) images were registered to AF images. All patients were screened for mutations in BEST1. In three additional BVMD patients, in vivo spectrofluorometric measurements were obtained within the vitelliform lesion.RESULTS. Mean nonlesion qAF was within normal limits for age. Maximum qAF within the lesion was markedly increased compared with controls. By SD-OCT segmentation, outer segment equivalent thickness was increased and outer nuclear layer thickness decreased in the lesion. Changes were also present in a transition zone beyond the lesion border. In subclinical patients, no abnormalities in retinal layer thickness were identified. Fluorescence spectra recorded from the vitelliform lesion were consistent with those of retinal pigment epithelial cell lipofuscin.CONCLUSIONS. Based on qAF, mutations in BEST1 do not cause increased lipofuscin levels in nonlesion fundus areas.