The systemic mucopolysaccharidoses. Ultrastructural and histochemical studies of conjunctiva and skin.
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Biomedical subjects
Publications and source records attributed to K R Kenyon.
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The clinicopathologic features of Chandler's syndrome are elucidated based on a study of nine patients. Keratoplasty, trabeculectomy and/or iris specimens were studied by light, electron and, in six cases, specular microscopy. The prominent pathologic features were abnormalities of the endothelially-derived cells lining the posterior corneal surface, the inner surface of the trabecular meshwork, and the anterior iris surface. In all four corneal buttons, the endothelium was diffusely attenuated and focally absent, and posterior collagen layers were present. Extension of the endothelial cell layer and Descemet's membrane deposition over the trabecular meshwork were observed in two trabeculectomy specimens, and similar proliferation onto the anterior surface of the iris was evident in four iris specimens. Other abnormalities of the endothelial cells included increased intracytoplasmic filaments. These pathologic alterations are consistent with the concept of Chandler's syndrome as representing one variant of the iridocorneal endothelial (ICE) syndrome. Interestingly, although both endothelial proliferative and degenerative responses can be observed in Chandler's syndrome, their occurrence within the same eye is apparently not simultaneous.
Based on their own study of nine corneas with clinically documented posterior polymorphous dystrophy and a review of the literature, the authors describe the morphologic features of this entity. Study by phase contrast light microscopy and transmission and scanning electron microscopy found that changes were primarily in the endothelium and consisted of endothelial cell degeneration and loss with focal fibroblastic and epithelial-like cell transformation. Secondary alterations of Descemet's membrane were seen; they consisted of abnormal lamination with deposition of abnormal collagen material, particularly in the posterior collagen layer, and formation of guttate excrescences and pits.
The clinical and pathologic findings of a case of congenital bilateral, peripheral corneal leukoma associated with iris coloboma and anterior subluxation of the lens are reported. The changes in the region of keratolenticular apposition include partial absence of Bowman's membrane, stromal vascularization and thinning with large-diameter collagen fibrils, and absence of Descemet's membrane and endothelium. The authors postulate that dysembryogenesis of the tertiary vitreous, with iris coloboma, resulted in apposition of the lens to cornea during the second and third month of gestation and that this apposition resulted in the observed peripheral corneal abnormalities.
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To secure a penetrating keratoplasty donor button, a continuous suture is both less time consuming to place and easier to remove. Infrequently, however, the continuous suture may loosen or break prematurely. For this purpose we propose the anchor suture as a simple technique to restore a tight, continuous suture and a secure wound.
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The fellow normal-appearing eyes of 23 patients with unilateral aphakic bullous keratopathy were examined by wide-field specular microscopy and pachymetry. All patients had: pseudophakic or aphakic bullous keratopathy in one eye; unoperated fellow eye on no medication; no other ocular disease; and no endothelial guttata seen by slitlamp biomicroscopy or specular microscopy. Three of 23 patients had endothelial cell counts less than 1,000 cells/mm2. There was no correlation between cell density and corneal thickness, but a negative correlation existed between age and endothelial cell density. Preoperative specular microscopy is indicated in patients who have developed bullous keratopathy in the fellow eye after cataract surgery.