December 1995
Volume 36, Issue 13
Free
Articles  |   December 1995
Pathology of the iridocorneal-endothelial syndrome. The ICE-cell.
Author Affiliations
  • S G Levy
    Department of Histopathology, Charing Cross and Westminster Medical School, London, United Kingdom.
  • A C McCartney
    Department of Histopathology, Charing Cross and Westminster Medical School, London, United Kingdom.
  • M H Baghai
    Department of Histopathology, Charing Cross and Westminster Medical School, London, United Kingdom.
  • M C Barrett
    Department of Histopathology, Charing Cross and Westminster Medical School, London, United Kingdom.
  • J Moss
    Department of Histopathology, Charing Cross and Westminster Medical School, London, United Kingdom.
Investigative Ophthalmology & Visual Science December 1995, Vol.36, 2592-2601. doi:
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      S G Levy, A C McCartney, M H Baghai, M C Barrett, J Moss; Pathology of the iridocorneal-endothelial syndrome. The ICE-cell.. Invest. Ophthalmol. Vis. Sci. 1995;36(13):2592-2601.

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Abstract

PURPOSE: The iridocorneal-endothelial (ICE) syndrome is characterized by glaucoma, corneal failure, and iris destruction. Specular photomicroscopy of the corneal endothelium in this disease shows a population of abnormal cells named ICE-cells. Comparison between ultrastructural examination and specular photomicroscopy demonstrates that the histologic equivalent of ICE-cells are cells with an epithelial phenotype. The authors have studied the differentiation markers expressed by ICE-cells using an ultrastructural immunocytochemical technique. METHODS: Seven keratoplasty specimens from patients with the ICE syndrome were examined by scanning and transmission electron microscopy and light and electron microscopic immunocytochemistry. Comparison was made with three normal corneas. Immunocytochemical studies were performed with monoclonal antibodies to broad-spectrum cytokeratins, cytokeratins 3, 5/8, 8/18 and 19, vimentin, and epithelial membrane antigen. RESULTS: ICE-cells were morphologically similar to epithelial cells and expressed the same profile of differentiation markers as did normal limbal epithelial cells. CONCLUSIONS: ICE-cells may arise from an embryologic ectopia of ocular surface epithelium. Alternatively, these findings are consistent with a metaplastic stimulus resulting in a profound change in the phenotype of normal corneal endothelial cells.

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