Abstract |
Between August 1, 1982, and December 31, 1988, 487 patients with clinically established or suspected diagnosis of a hereditary degenerative or dystrophic disorder of the ocular fundus or of a disorder of the optic pathways were evaluated with an extensive set of clinical and electrophysiological tests, 67% of the patients were referred from other eye departments. In order of magnitude the most frequently encountered diagnoses were: 1) 'functional' disturbances (18%), 2) disorders of the optic nerve (9%), 3) retinitis pigmentosa (8%), 4), 5) and 6) progressive cone dystrophy, disorders of the central optic pathways, and fundus flavimaculatus/Stargardt's disease (5% each), and 7) choriocapillaris atrophy (4%). Choriocapillaris atrophy affected older patients than retinitis pigmentosa (P less than 0.001). A male preponderance was observed for juvenile retinoschisis (P less than 0.005) and congenital stationary night blindness (P less than 0.05), and a female dominance in the groups of patients with 'functional' symptoms (P less than 0.01). A specific diagnosis was established in 469 patients. After exclusion of 16 patients referred because of a known hereditary disposition, 196 (43%) of the remaining 453 patients were referred under the same diagnosis as our final diagnosis, 153 (34%) under a different diagnosis and 104 (23%) without any clear diagnosis. Obviously, there is a need for regional centers specialized in these disorders.
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Authors | K O Skoog, O Textorius, S E Nilsson |
Journal | Acta ophthalmologica
(Acta Ophthalmol (Copenh))
Vol. 68
Issue 2
Pg. 131-8
(Apr 1990)
ISSN: 0001-639X [Print] Denmark |
PMID | 2356699
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
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Topics |
- Adolescent
- Adult
- Aged
- Aged, 80 and over
- Child
- Child, Preschool
- Choroid Diseases
(classification, diagnosis)
- Color Perception Tests
- Dark Adaptation
- Electroretinography
- Female
- Humans
- Infant
- Male
- Middle Aged
- Pigment Epithelium of Eye
- Retinal Degeneration
(diagnosis)
- Retinal Diseases
(classification, diagnosis)
- Sex Factors
- Visual Acuity
- Visual Fields
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