Levy, Simon Gabriel; (1996) The iridocorneal-endothelial syndrome: A study of cell and basement membrane pathology. Doctoral thesis (M.D), UCL (University College London).

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Abstract

This thesis is a detailed investigation of the pathology of the iridocorneal-endothelial syndrome. The clinical features of this disease are a "hammered-silver" appearance of the corneal endothelium, corneal failure, glaucoma, multiple iris nodules and progressive iris destruction. Specular photomicroscopy of the corneal endothelium has shown a unique population of abnormal cells, called "ICE-cells", which are believed to be of central importance in this disorder. Ultrastructural examination of keratoplasty and trabecular meshwork specimens from patients with the iridocorneal-endothelial syndrome demonstrated cells with epithelial features which were named "epithelial-like cells" and postulated to be the histological equivalent of the ICE-cell. The origin of epithelial-like cells was investigated by using light and electron microscopic immunocytochemistry to identify the differentiation markers they express. Comparison with normal corneal tissues showed that epithelial-like cells express the same differentiation markers as normal limbal epithelial cells. These findings suggest that the epithelial-like or ICE-cells arise from an embryological ectopia of ocular surface epithelium. Alternatively, they may represent a metaplastic response of corneal endothelial cells to an unknown stimulus. The morphology and composition of Descemet's membrane in the iridocorneal-endothelial syndrome were investigated. In the majority of specimens ultrastructural examination demonstrated a posterior collagenous layer, a region of abnormal basement membrane containing lattice-material (wide-spaced collagen) and microfibrils, located between the endothelial cells and Descemet's membrane proper. Electron microscopic immunocytochemistry and histochemistry showed that the abnormal lattice-material contains collagen Type VIII whilst collagen Types I, III, IV, V and VI, fibronectin, tenascin and oxytalan are components of microfibrils.

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