In vivo confocal microscopic findings in Lisch corneal dystrophy.

PURPOSE

To describe the in vivo confocal microscopic and clinicopathologic correlations in Lisch corneal dystrophy.

METHODS

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.

RESULTS

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.

CONCLUSIONS

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.