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LONG-TERM FOLLOW-UP OF PATIENTS WITH CHOROIDEREMIA WITH SCLERAL PITS AND TUNNELS AS A NOVEL OBSERVATION
- Source :
- Retina (Philadelphia, Pa.), 38(9), 1713-1724. Lippincott Williams and Wilkins, Retina, 38, 1713-1724. Lippincott Williams & Wilkins, RETINA, The Journal of Retinal and Vitreous Diseases, 38(9), 1713-1724
- Publication Year :
- 2017
-
Abstract
- PURPOSE: To evaluate the long-term clinical course and visual outcome of patients with choroideremia. METHODS: Clinical examination, a social questionnaire, and medical records review of 21 patients with choroideremia from 14 families. RESULTS: The mean follow-up time was 25.2 years (SD: 13.3; range 2-57 years). The mean age at symptom onset was 15.1 years (SD: 10.1; range 5-40 years). Best-corrected visual acuity was stable until the age of 35 (P = 0.96), but declined significantly faster after the age of 35 (11%/year, P = 0.001), with a high variability between individual patients. The mean age at which patients discontinued working was 48.1 years (SD: 11.7, range 25-65 years). The reason for work discontinuation was vision related in 60% of cases. Most patients (70%) reported visual field constriction as the most debilitating symptom. The authors report scleral pits and tunnels as a novel finding visible on spectral domain optical coherence tomography and ophthalmoscopy. CONCLUSION: Choroideremia is a severely debilitating disease showing a rapid decline of visual acuity generally after the age of 35, but a more gradual decline for other abnormalities.
- Subjects :
- 0301 basic medicine
Male
social participation
Visual acuity
progression rate
Visual Acuity
Choroideremia
0302 clinical medicine
Fluorescein Angiography
Child
medicine.diagnostic_test
Cysts
Medical record
Clinical course
General Medicine
Middle Aged
Cleft Palate
Child, Preschool
Disease Progression
Female
medicine.symptom
Tomography, Optical Coherence
Adult
medicine.medical_specialty
Adolescent
Long term follow up
phenotype
Fundus Oculi
Cleft Lip
clinical course
Physical examination
Retina
Ophthalmoscopy
03 medical and health sciences
Young Adult
Ophthalmology
medicine
Journal Article
Humans
Abnormalities, Multiple
Aged
Retrospective Studies
business.industry
Choroid
medicine.disease
eye diseases
Lip
Discontinuation
030104 developmental biology
030221 ophthalmology & optometry
Visual Fields
business
Follow-Up Studies
Forecasting
Subjects
Details
- ISSN :
- 15392864 and 0275004X
- Volume :
- 38
- Issue :
- 9
- Database :
- OpenAIRE
- Journal :
- Retina (Philadelphia, Pa.)
- Accession number :
- edsair.doi.dedup.....8e474655ec4f73f007e66b8ca59d1514