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(Investigative Ophthalmology and Visual Science. 1973;12:88-97.)
© 1973 by The Association for Research in Vision and Ophthalmology, Inc.

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Original Articles: Macular Comeal Dystrophy: Ultrastructural Pathology of Corneal Endothelium and Descemet's Membrane

ROBERT C. SNIP 1, KENNETH R. KENYON 1, and W. RICHARD GREEN 1

1 Eye Pathology Laboratory of the Wilmer Institute, Johns Hopkins Hospital Baltimore, Md.

Comeal buttons from four patients with macular corneal dystrophy (MCD) were examined histochemicaUy and uhrastructuraUu with particular emphasis on the abnormalities of the endothelium and Descemet's membrane. Abnormal deposits of acid mucopolysaccharide (AMP) were histochemically demonstrable within both the endothelial cells and Descemet's membrane. By electron microscopy, these deposits corresponded to fibrillogranular material visible as membrane-bound vacuoles within the endothelial cytoplasm and as vesicular deposits throughout Descemet's membrane. The similarity of these alterations to those of the keratocytes and stroma in MCD indicates that the endothelium may be primarily involved in this disorder and may constitute the source of the extraceUular AMP in Descemet's membrane. Guttate excrescences of Descemet's membrane in MCD ultrastructurally resembled those of Fuchs’ combined dystrophy. Comparison of the corneal lesions in MCD and in systemic mucopolysaccharidoses suggests different biochemical lesions in these disease states

Key Words: macular corneal dystrophy • mucopolysaccharidosis • acid mucopolysaccharide • Descemet's membrane • corneal endothelium • corneal guttata • histochemistry • electron microscopy

Submitted on September 1, 1972
Accepted on October 17, 1972




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