Pan Fei, Yao Yu-feng, Nie Xin, Zhang Bei
Department of Ophthalmology, Sir Run Run Shaw Hospital, Zhejiang University, Hangzhou 310016, China.
Zhejiang Da Xue Xue Bao Yi Xue Ban. 2011 May;40(3):321-6. doi: 10.3785/j.issn.1008-9292.2011.03.016.
To investigate the clinical characteristics and the corneal morphological abnormalities in Fleck corneal dystrophy.
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.
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 hyper reflective 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 hyper reflective 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.
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.
探讨斑点状角膜营养不良的临床特征及角膜形态异常。
对来自两个无血缘关系家庭的9例患者的18只患斑点状角膜营养不良的眼睛进行裂隙灯显微镜检查、Cochet-Bonnet眼压计检查及活体共聚焦显微镜检查。使用NAVIS软件对角膜细胞、神经和基质沉积物进行定量分析。
斑点状角膜营养不良的裂隙灯显微镜检查显示,双侧灰白色小斑点状或环状混浊从角膜基质中央到周边散在于各层,病变之间的基质清晰。活体共聚焦显微镜检查发现,混浊表现为大小约70.6μm×110.3μm的甜甜圈样或肾形沉积物,密度为(1.6±0.4)/帧,累及角膜基质约(438.4±22.0)μm,伴有直径2至18μm的高反射点状细胞内颗粒。1例患者的2只眼睛伴有角膜感觉减退,共聚焦图像显示异常高反射沉积物累及Bowman层,基底膜下神经分支和密度降低。其他角膜感觉正常的眼睛角膜神经形态和密度正常。
斑点状角膜营养不良具有典型的临床特征。活体共聚焦显微镜检查能够在细胞水平研究角膜的形态变化,对角膜营养不良的临床诊断和评估具有重要价值。斑点状角膜营养不良中角膜敏感性降低可能是由累及Bowman层的角膜基质沉积物所致。