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In Vivo Confocal Microscopic Findings in Lisch Corneal Dystrophy

Kurbanyan, Kristina MD; Sejpal, Kunjal D. MD; Aldave, Anthony J. MD; Deng, Sophie X. MD, PhD

doi: 10.1097/ICO.0b013e318239ad37
Case Report

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.

Cornea and Uveitis Division, Jules Stein Eye Institute, University of California, Los Angeles

Reprints: Sophie X. Deng, MD, PhD, Jules Stein Eye Institute, 100 Stein Plaza, Los Angeles, CA 90095 (email:

Financial disclosures/conflicts of interest: None reported.

Received December 31, 2010

Accepted June 2, 2011

Copyright © 2012 Wolters Kluwer Health, Inc. All rights reserved.