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Fleck Corneal Dystrophy

Fleck Corneal Dystrophy Abstract • Fourteen members of four families with fleck dystrophy of the corneal stroma were examined and studied. Corneal and lens changes were noted in all patients. Corneal sensation was normal. Corneal biopsies were performed on two patients and a corneal button of a patient with fleck dystrophy and keratoconus was studied. Light and electron microscopy and histochemical studies showed this dystrophy to consist of abnormal keratocytes with variable numbers of membrane-limited intracytoplasmic vacuoles containing a granular to fibrogranular material that stains positively for mucopolysaccharide. This appears to be the first reported incidence of a dominantly inherited disorder of mucopolysaccharides affecting the eyes. (Arch Ophthalmol 95:440-444, 1977) References 1. François J, Neetens A: Nouvelle dystrophie hérédo-familiale du parenchyme cornéen (hérédodystrophie Mouchetée.) Bull Soc Belge Ophthalmol 114:641-646, 1956. 2. Aracena T: Hereditary fleck dystrophy of the cornea: Report of a family . J Ped Ophthalmol 12:223-227, 1975. 3. Streeten BW, Falls HF: Hereditary fleck dystrophy of the cornea . Am J Ophthalmol 51:275-278, 1961. 4. Birndorf LA, Ginsberg SP: Hereditary fleck dystrophy associated with decreased corneal sensitivity . Am J Ophthalmol 73:670-672, 1972. 5. Stankovic I, Stojanovic D: L'Héredo-Dystrophie Mouchetée du parenchyme Cornéen Ann d' Oculist 197:52-57, 1964. 6. Collier M: Dystrophie Mouchetée parenchyme cornéen avec dystrophie nuageuse centrale . Bull Soc Ophtalmol Fr 64:608-611, 1964. 7. Goldberg M, (ed): Genetic and Metabolic Eye Diseases . Boston, Little Brown & Co, 1974. 8. Kenyon KR, Sensenbrenner JA: Mucolipidosis II (I-cell disease): Ultrastructural observations of conjunctiva and skin . Invest Ophthalmol 10:555-567, 1971. 9. Patten JT, Hyndiuk RA, Donaldson DD, et al: Fleck (Mouchetée) dystrophy of the cornea . Ann Ophthalmol 8:25-32, 1976. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Ophthalmology American Medical Association

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Publisher
American Medical Association
Copyright
Copyright © 1977 American Medical Association. All Rights Reserved.
ISSN
0003-9950
eISSN
1538-3687
DOI
10.1001/archopht.1977.04450030082009
Publisher site
See Article on Publisher Site

Abstract

Abstract • Fourteen members of four families with fleck dystrophy of the corneal stroma were examined and studied. Corneal and lens changes were noted in all patients. Corneal sensation was normal. Corneal biopsies were performed on two patients and a corneal button of a patient with fleck dystrophy and keratoconus was studied. Light and electron microscopy and histochemical studies showed this dystrophy to consist of abnormal keratocytes with variable numbers of membrane-limited intracytoplasmic vacuoles containing a granular to fibrogranular material that stains positively for mucopolysaccharide. This appears to be the first reported incidence of a dominantly inherited disorder of mucopolysaccharides affecting the eyes. (Arch Ophthalmol 95:440-444, 1977) References 1. François J, Neetens A: Nouvelle dystrophie hérédo-familiale du parenchyme cornéen (hérédodystrophie Mouchetée.) Bull Soc Belge Ophthalmol 114:641-646, 1956. 2. Aracena T: Hereditary fleck dystrophy of the cornea: Report of a family . J Ped Ophthalmol 12:223-227, 1975. 3. Streeten BW, Falls HF: Hereditary fleck dystrophy of the cornea . Am J Ophthalmol 51:275-278, 1961. 4. Birndorf LA, Ginsberg SP: Hereditary fleck dystrophy associated with decreased corneal sensitivity . Am J Ophthalmol 73:670-672, 1972. 5. Stankovic I, Stojanovic D: L'Héredo-Dystrophie Mouchetée du parenchyme Cornéen Ann d' Oculist 197:52-57, 1964. 6. Collier M: Dystrophie Mouchetée parenchyme cornéen avec dystrophie nuageuse centrale . Bull Soc Ophtalmol Fr 64:608-611, 1964. 7. Goldberg M, (ed): Genetic and Metabolic Eye Diseases . Boston, Little Brown & Co, 1974. 8. Kenyon KR, Sensenbrenner JA: Mucolipidosis II (I-cell disease): Ultrastructural observations of conjunctiva and skin . Invest Ophthalmol 10:555-567, 1971. 9. Patten JT, Hyndiuk RA, Donaldson DD, et al: Fleck (Mouchetée) dystrophy of the cornea . Ann Ophthalmol 8:25-32, 1976.

Journal

Archives of OphthalmologyAmerican Medical Association

Published: Mar 1, 1977

References