In Vivo Morphology of the Limbal Palisades of Vogt Correlates With Progressive Stem Cell Deficiency in Aniridia-Related Keratopathy
In Vivo Morphology of the Limbal Palisades of Vogt Correlates With Progressive Stem Cell Deficiency in Aniridia-Related Keratopathy
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DOI：
10.1167/iovs.13-11780
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发表时间：
2013-08-01
影响因子：
4.4
通讯作者：
Fagerholm, Per
中科院分区：
文献类型：
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作者：
Lagali, Neil;Eden, Ulla;Fagerholm, Per
目的.研究进行性角膜缘干细胞缺乏的沃格特角膜缘栅栏组织的形态学改变。对20例（40眼）先天性无虹膜的挪威受试者和9例（18眼）无虹膜的健康家庭成员进行了检查。通过裂隙灯生物显微镜评估无虹膜相关性角膜病变（方舟）的临床分级，并额外测量泪液产生和质量、角膜厚度和敏感性。用激光扫描共聚焦显微镜（IVCM）观察沃格特角膜缘上级和下栅栏层及角膜中央的形态。无虹膜0级方舟患者角膜透明，角膜缘栅栏结构正常，1级方舟患者12只眼中有5只眼栅栏结构退化。在剩余的1级眼和所有20只患有2、3和4期方舟的眼中，IVCM显示栅栏结构缺失。方舟损伤分级的增加与视力和角膜敏感性降低、角膜厚度增加、上级和下级栅栏结构退化程度、周围神经减少、炎性细胞浸润增加、基底上皮细胞和中央基底下神经密度降低显著相关。此外，与健康角膜相比，无虹膜患者的角膜缘基底上皮细胞密度和中央角膜基底下神经密度均显著降低（分别为P = 0.002和0.003）。无虹膜患者角膜缘干细胞缺乏的进展与栅栏结构的降解、上皮表型的逐渐转化、炎症的发生和角膜神经缺损相关。IVCM可用于监测干细胞缺陷患者的早期至晚期退行性变化。
PURPOSE. To investigate morphologic alterations in the limbal palisades of Vogt in a progressive form of limbal stem cell deficiency.METHODS. 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).RESULTS. 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).CONCLUSIONS. 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.