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Retinal degeneration associated with ectopia lentis
- Source :
- Scopus-Elsevier
-
Abstract
- Two brothers had retinal degeneration, lens subluxation, and myopia since early life. There was no evidence of Marfan syndrome, homocystinuria, or other systemic disease. They had nystagmus, myopia, inferior dislocation of the lens, and posterior subcapsular opacities in both eyes. Fundus examination showed attenuated retinal vessels, macular atrophy with occasional pigment accumulation as clumps, and perivascular sleeves. Electroretinography revealed decreased photopic and scotopic responses. The visual fields were constricted. We believe this to be the first report of retinal degeneration with bilateral lens subluxation in a family. It appears to be inherited in an autosomal recessive fashion.
- Subjects :
- Adult
Male
Retinal degeneration
Marfan syndrome
medicine.medical_specialty
genetic structures
Fundus Oculi
Fundus (eye)
Ectopia Lentis
Ophthalmology
Pigment accumulation
medicine
Humans
Ectopia lentis
Genetics (clinical)
medicine.diagnostic_test
business.industry
Retinal Degeneration
Anatomy
Middle Aged
medicine.disease
eye diseases
Pedigree
medicine.anatomical_structure
Lens (anatomy)
Pediatrics, Perinatology and Child Health
sense organs
Visual Fields
business
Electroretinography
Photopic vision
Subjects
Details
- Database :
- OpenAIRE
- Journal :
- Scopus-Elsevier
- Accession number :
- edsair.doi.dedup.....0fdc94a01d354d1da164d8fe7a3a00a5