Department of Clinical and Experimental Medicine, Faculty of Health Sciences, Linköping University, Linköping, Sweden.
Invest Ophthalmol Vis Sci. 2013 Aug 7;54(8):5333-42. doi: 10.1167/iovs.13-11780.
To investigate morphologic alterations in the limbal palisades of Vogt in a progressive form of limbal stem cell deficiency.
Twenty Norwegian subjects (40 eyes) with congenital aniridia and 9 healthy family members (18 eyes) without aniridia were examined. Clinical grade of aniridia-related keratopathy (ARK) was assessed by slit-lamp biomicroscopy, and tear production and quality, corneal thickness, and sensitivity were additionally measured. The superior and inferior limbal palisades of Vogt and central cornea were examined by laser scanning in vivo confocal microscopy (IVCM).
In an aniridia patient with grade 0 ARK, a transparent cornea and normal limbal palisade morphology were found. In grade 1 ARK, 5 of 12 eyes had degraded palisade structures. In the remaining grade 1 eyes and in all 20 eyes with stage 2, 3, and 4 ARK, palisade structures were absent by IVCM. Increasing ARK grade significantly correlated with reduced visual acuity and corneal sensitivity, increased corneal thickness, degree of degradation of superior and inferior palisade structures, reduced peripheral nerves, increased inflammatory cell invasion, and reduced density of basal epithelial cells and central subbasal nerves. Moreover, limbal basal epithelial cell density and central corneal subbasal nerve density were both significantly reduced in aniridia compared to healthy corneas (P = 0.002 and 0.003, respectively).
Progression of limbal stem cell deficiency in aniridia correlates with degradation of palisade structures, gradual transformation of epithelial phenotype, onset of inflammation, and a corneal nerve deficit. IVCM can be useful in monitoring early- to late-stage degenerative changes in stem cell-deficient patients.
研究进行性边缘干细胞缺乏症中 Vogt 角膜缘栅栏状结构的形态改变。
对 20 名挪威先天性无虹膜患者（40 只眼）和 9 名无虹膜的健康家族成员（18 只眼）进行检查。通过裂隙灯生物显微镜评估无虹膜相关角膜病变（ARK）的临床分级，并额外测量泪液产生和质量、角膜厚度和敏感度。通过激光共聚焦显微镜（IVCM）活体检查上、下 Vogt 角膜缘栅栏状结构和中央角膜。
在 ARK 0 级的无虹膜患者中，发现角膜透明，角膜缘栅栏状结构正常。在 ARK 1 级中，12 只眼中有 5 只出现退化的栅栏状结构。在其余的 ARK 1 级和所有 20 只处于 2、3 和 4 级 ARK 的眼中，IVCM 显示不存在栅栏状结构。ARK 分级的增加与视力下降和角膜敏感度降低、角膜厚度增加、上、下栅栏状结构退化程度、周边神经减少、炎症细胞浸润增加以及基底上皮细胞和中央亚基底神经密度降低显著相关。此外，与健康角膜相比，无虹膜的角膜缘基底上皮细胞密度和中央角膜亚基底神经密度均显著降低（分别为 P = 0.002 和 0.003）。
无虹膜患者的边缘干细胞缺乏症进展与栅栏状结构退化、上皮表型逐渐转化、炎症发作和角膜神经缺失相关。IVCM 可用于监测干细胞缺乏症患者早期至晚期的退行性变化。
