An ultrastructure study of posterior polymorphous dystrophy of the cornea. 1977

C Hanna, and F T Fraunfelder, and J R McNair

The cornea from an early developing and uncomplicated case of posterior polymorphous dystrophy in a 15-year-old boy shows a thin abnormal Descemet's membrane and reduced number of endothelial cells as the primary findings. Ultrastructurally, the membrane was composed of a very thin, three-dimensional lattice pattern layer covered by layers of fibrous material. This lattice pattern is normally formed between late fetal and early adolescent life. The 3 previously reported histologic cases of this entity were from cases with long-standing corneal opacities in the late third to fifth decade of life with additional corneal changes. However, in each case the lattice pattern layer of the membrane was abnormally thin. This report suggests that a progressive form of posterior polymorphous dystrophy of the cornea is due to cells forming abnormal Descemet's membrane beginning in late fetal life to soon after birth.

UI MeSH Term Description Entries
D008297 Male Males
D003315 Cornea The transparent anterior portion of the fibrous coat of the eye consisting of five layers: stratified squamous CORNEAL EPITHELIUM; BOWMAN MEMBRANE; CORNEAL STROMA; DESCEMET MEMBRANE; and mesenchymal CORNEAL ENDOTHELIUM. It serves as the first refracting medium of the eye. It is structurally continuous with the SCLERA, avascular, receiving its nourishment by permeation through spaces between the lamellae, and is innervated by the ophthalmic division of the TRIGEMINAL NERVE via the ciliary nerves and those of the surrounding conjunctiva which together form plexuses. (Cline et al., Dictionary of Visual Science, 4th ed) Corneas
D003317 Corneal Dystrophies, Hereditary Bilateral hereditary disorders of the cornea, usually autosomal dominant, which may be present at birth but more frequently develop during adolescence and progress slowly throughout life. Central macular dystrophy is transmitted as an autosomal recessive defect. Corneal Dystrophies,Granular Dystrophy, Corneal,Groenouw's Dystrophies,Macular Dystrophy, Corneal,Stromal Dystrophies, Corneal,Corneal Dystrophy,Corneal Dystrophy, Hereditary,Corneal Granular Dystrophies,Corneal Granular Dystrophy,Corneal Macular Dystrophies,Corneal Macular Dystrophy,Corneal Stromal Dystrophies,Corneal Stromal Dystrophy,Dystrophy, Corneal,Dystrophy, Corneal Granular,Dystrophy, Corneal Macular,Dystrophy, Corneal Stromal,Dystrophy, Hereditary Corneal,Groenouw Dystrophies,Groenouws Dystrophies,Hereditary Corneal Dystrophies,Hereditary Corneal Dystrophy,Stromal Dystrophy, Corneal
D003886 Descemet Membrane A layer of the cornea. It is the basal lamina of the CORNEAL ENDOTHELIUM (from which it is secreted) separating it from the CORNEAL STROMA. It is a homogeneous structure composed of fine collagenous filaments, and slowly increases in thickness with age. Lamina Elastica Posterior,Descemet's Membrane,Descemet Membranes,Descemets Membrane,Elastica Posterior, Lamina,Elastica Posteriors, Lamina,Lamina Elastica Posteriors,Membrane, Descemet,Membrane, Descemet's,Membranes, Descemet,Posterior, Lamina Elastica,Posteriors, Lamina Elastica
D004727 Endothelium A layer of epithelium that lines the heart, blood vessels (ENDOTHELIUM, VASCULAR), lymph vessels (ENDOTHELIUM, LYMPHATIC), and the serous cavities of the body. Endotheliums
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293 Adolescent A person 13 to 18 years of age. Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults

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