Cornea and Uveitis Division, Jules Stein Eye Institute, University of California, Los Angeles, CA 90095, USA.
Cornea. 2012 Apr;31(4):437-41. doi: 10.1097/ICO.0b013e318239ad37.
To describe the in vivo confocal microscopic and clinicopathologic correlations in Lisch corneal dystrophy.
This is a retrospective case series of 2 patients with Lisch corneal dystrophy. The diagnosis was made based on clinical findings in both cases and was confirmed histopathologically following epithelial debridement in case 1. In vivo laser scanning confocal microscopy using the Heidelberg Retina Tomograph III with the Rostock Cornea Module was carried out in both cases.
Clinical examination of the corneas revealed areas of epithelial opacification that were sharply demarcated in juxtaposition with normal corneal epithelium. The gray feathery appearance of the epithelial lesions in both cases was characteristic of Lisch corneal dystrophy. The central visual axis was involved in case 1, and corneal topography showed irregular astigmatism. Histological analysis of the epithelial cells in this case showed intracytoplasmic vacuoles, confirming the diagnosis of Lisch corneal dystrophy. In vivo confocal microscopy in both cases demonstrated highly hyperreflective epithelial cytoplasm with hypo-reflective nuclei. There was involvement of all epithelial layers extending to the limbus and findings on imaging were confined to the clinically observed areas of corneal opacity. The lesion in case 1 recurred after epitheliectomy of the central cornea without removal of affected limbal cells.
The unique features on in vivo confocal microscopy correlated with the clinical and histopathologic features of Lisch corneal dystrophy may be used to distinguish this disorder from other corneal epithelial conditions. The affected epithelial cells appear to originate from abnormal limbal stem cells.
描述 Lisch 角膜营养不良的体内共焦显微镜和临床病理相关性。
这是一项回顾性的 2 例 Lisch 角膜营养不良患者的病例系列研究。在这两种情况下，基于临床发现诊断出该疾病，并在第 1 例中通过上皮清创术确认了组织病理学诊断。对这两种情况均使用 Heidelberg Retina Tomograph III 与 Rostock Cornea Module 进行了体内激光扫描共焦显微镜检查。
对角膜的临床检查显示，上皮混浊区域与正常角膜上皮紧密相邻，界限分明。在这两种情况下，上皮病变的灰色羽毛状外观是 Lisch 角膜营养不良的特征。在第 1 例中，中央视觉轴受到影响，角膜地形图显示不规则散光。对该病例的上皮细胞进行组织学分析显示，细胞质内有空泡，证实了 Lisch 角膜营养不良的诊断。在这两种情况下的体内共焦显微镜检查均显示出高度高反射上皮细胞质和低反射核。所有上皮层均受累，延伸至角膜缘，并且影像学发现仅限于临床上观察到的角膜混浊区域。在没有去除受影响的角膜缘细胞的情况下，对中央角膜进行上皮切除术，导致第 1 例中的病变复发。
体内共焦显微镜的独特特征与 Lisch 角膜营养不良的临床和组织病理学特征相关，可用于将这种疾病与其他角膜上皮疾病区分开来。受影响的上皮细胞似乎来源于异常的角膜缘干细胞。
