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HEREDITARY POLYMORPHOUS DEEP DEGENERATION OF THE CORNEA

HEREDITARY POLYMORPHOUS DEEP DEGENERATION OF THE CORNEA Abstract IT HAS been our privilege to study two families certain members of which exhibited a rarely observed form of endothelial dystrophy of the cornea. A careful survey of the families revealed that the aberration is hereditary and most likely congenital. It is to be differentiated from the usually described endothelial dystrophy, or corneal guttata. REVIEW OF LITERATURE Polymorphous degeneration of the deep layers of the cornea is characterized by the presence of polymorphic opacities, vesicles, and concavities of the endothelium, and possibly Descemet's membrane.Koeppe1 (1916) was probably the first to describe this form of endothelial dystrophy. He presented six cases of his own in which keratitis bullosa interna was exhibited. This author postulated that the concavities were congenital pits on the inner corneal surface. In 1920 Koeppe reported 18 cases in which similar lesions were present. Since the eyes exhibited no other pathologic condition, the author concluded that References 1. Koeppe, L.: Klinische Beobachtungen mit der Nernst Spaltlampe und dem Hornhautmikroskop , Arch. opht. 91:375, 1916. 2. Triebenstein, O.: Veränderung an der Hornhauthinterfläche , Klin. Monatsbl. Augenh. 74:777, 1925. 3. Freudenthal, E.: Über zwei Fälle von familiärer Endotheldystrophie der Hornhaut bei Vorhandensein allgemein degenerativer Veränderungen , Ztschr. Augenh. 78:224, 1932. 4. Theodore, F. H.: Congenital Type of Endothelial Dystrophy , Arch. Ophth. 21:626, 1939.Crossref 5. Berliner, M. L.: Biomicroscopy of the Eye: Slit Lamp Microscopy of the Living Eye , New York, Paul B. Hoeber, Inc., 1949. 6. Schlichting, H.: Blasen und dellenförmige Endotheldystrophie der Hornhaut , Klin. Monatsbl. Augenh. 107:425, 1941. 7. Cuntz-Schüssler, E.: Über zwei Sippen mit eigenartiger erblicher Hornhautdystrophie , Klin. Monatsbl. Augenh. 112:70, 1947. 8. Forni, S.: Dégénérescence polymorphe familiale de la membrane limitante posterieure de la cornée , Arch. opht. 11:162, 1951. 9. Franceschetti, A.; Klein, D., and Babel, J.: Clinical and Social Aspects of Heredity in Ophthalmology , reprinted from Acta XVI Concilium Ophthalmologicum Britannia, 1950 http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png A.M.A. Archives of Ophthalmology American Medical Association

HEREDITARY POLYMORPHOUS DEEP DEGENERATION OF THE CORNEA

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Publisher
American Medical Association
Copyright
Copyright © 1953 American Medical Association. All Rights Reserved.
ISSN
0096-6339
DOI
10.1001/archopht.1953.00920030470006
Publisher site
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Abstract

Abstract IT HAS been our privilege to study two families certain members of which exhibited a rarely observed form of endothelial dystrophy of the cornea. A careful survey of the families revealed that the aberration is hereditary and most likely congenital. It is to be differentiated from the usually described endothelial dystrophy, or corneal guttata. REVIEW OF LITERATURE Polymorphous degeneration of the deep layers of the cornea is characterized by the presence of polymorphic opacities, vesicles, and concavities of the endothelium, and possibly Descemet's membrane.Koeppe1 (1916) was probably the first to describe this form of endothelial dystrophy. He presented six cases of his own in which keratitis bullosa interna was exhibited. This author postulated that the concavities were congenital pits on the inner corneal surface. In 1920 Koeppe reported 18 cases in which similar lesions were present. Since the eyes exhibited no other pathologic condition, the author concluded that References 1. Koeppe, L.: Klinische Beobachtungen mit der Nernst Spaltlampe und dem Hornhautmikroskop , Arch. opht. 91:375, 1916. 2. Triebenstein, O.: Veränderung an der Hornhauthinterfläche , Klin. Monatsbl. Augenh. 74:777, 1925. 3. Freudenthal, E.: Über zwei Fälle von familiärer Endotheldystrophie der Hornhaut bei Vorhandensein allgemein degenerativer Veränderungen , Ztschr. Augenh. 78:224, 1932. 4. Theodore, F. H.: Congenital Type of Endothelial Dystrophy , Arch. Ophth. 21:626, 1939.Crossref 5. Berliner, M. L.: Biomicroscopy of the Eye: Slit Lamp Microscopy of the Living Eye , New York, Paul B. Hoeber, Inc., 1949. 6. Schlichting, H.: Blasen und dellenförmige Endotheldystrophie der Hornhaut , Klin. Monatsbl. Augenh. 107:425, 1941. 7. Cuntz-Schüssler, E.: Über zwei Sippen mit eigenartiger erblicher Hornhautdystrophie , Klin. Monatsbl. Augenh. 112:70, 1947. 8. Forni, S.: Dégénérescence polymorphe familiale de la membrane limitante posterieure de la cornée , Arch. opht. 11:162, 1951. 9. Franceschetti, A.; Klein, D., and Babel, J.: Clinical and Social Aspects of Heredity in Ophthalmology , reprinted from Acta XVI Concilium Ophthalmologicum Britannia, 1950

Journal

A.M.A. Archives of OphthalmologyAmerican Medical Association

Published: Oct 1, 1953

References