Fleck角膜营养不良的临床和活体共焦显微镜特征分析  

Clinical characteristics and in vivo confocal microscopic imaging of Fleck corneal dystrophy

作　　者：潘飞[1] 姚玉峰[1] 聂欣[1] 张蓓[1] 

机构地区：[1]浙江大学医学院附属邵逸夫医院眼科浙江省生物治疗重点实验室,浙江杭州310016

出　　处：《浙江大学学报（医学版）》2011年第3期321-326,共6页Journal of Zhejiang University(Medical Sciences)

摘　　要：目的:探讨Fleck角膜营养不良的临床特征和共焦显微镜下角膜各层组织的形态学改变。方法:对来自2个不同家系的9例(18只眼)Fleck角膜营养不良患者进行裂隙灯显微镜、角膜知觉及共焦显微镜检查,应用NAVIS软件测量和分析角膜各层细胞、神经纤维及角膜内沉积物的大小、密度和累及厚度。结果:Fleck角膜营养不良患者经裂隙灯显微镜检查发现,角膜基质全层呈散在、灰白色斑点状或花冠状混浊,累及角膜中央和周边部,混浊间的角膜透明。共焦显微镜图像表现为角膜基质层内油炸圈样或肾形异常沉积物,平均大小为70.6μm×110.3μm,密度为(1.6±0.4)个/幅图像,累及基质层厚度为(438.4±22.0)μm。异常沉积物内充满直径约2~18μm的点状高反光物质。9例患者有1例双眼角膜知觉减退,共焦显微镜下角膜上皮下神经纤维的分支和密度减少,前弹力层处发现异常高反光的沉积物。其余病例角膜知觉和神经纤维的形态和密度均正常。结论:Fleck角膜营养不良有其特征性临床表现,结合活体共焦显微镜角膜内沉积物的形态学特点,有助于对疾病的临床诊断和病情评估。Fleck角膜营养不良患者的角膜知觉减退可能与角膜基质层内的异常沉积物累及到前弹力...Objective:To investigate the clinical characteristics and the corneal morphological abnormalities in Fleck corneal dystrophy.Methods:Eighteen eyes with Fleck corneal dystrophy of 9 patients from two unrelated families were examined by slit-lamp biomicroscopy,Cochet-Bonnet esthesiometer and in vivo confocal microscopy.The corneal cells,nerves and stromal deposits were analyzed quantitatively with NAVIS software.Results:Slit-lamp biomicroscopy of the Fleck corneal dystrophy revealed that bilateral small grey-white fleck-like or wreath-like opacities were scattered in all layer of the corneal stroma from the center to the periphery,the intervening stroma between the lesions was clear.In vivo confocal microscopy identified that the opacities appeared as doughnut-like or nephroid-like deposits approximately 70.6 μm×110.3 μm in size,(1.6± 0.4)/frame in density and involved about(438.4±22.0) μm of the corneal stroma,with hyperreflective dot-like intracellular particles,measuring 2 to 18 μm in diameter.In two eyes of 1 patient associated with decreased corneal sensation,confocal images showed that abnormal hyperreflective deposits involved the Bowman′s layer and the branch and density of the subbasal nerve was reduced.The other eyes with normal corneal sensation appeared normal in the morphology and the density of the corneal nerves.Conclusions:Fleck corneal dystrophy has typical clinical characteristics.In vivo confocal microscopy allows to study the morphological changes of the cornea at cellular level,which is valuable for the clinical diagnosis and evaluation of the corneal dystrophy.Decreased corneal sensitivity in Fleck corneal dystrophy is probably caused by corneal stromal deposits involved in the Bowman′s layer.

关 键 词：角膜营养不良 遗传性/病理学 Fleck角膜营养不良/病理学 角膜/细胞学 显微镜检查 共焦 

分 类 号：R772.23[医药卫生—眼科]