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Choroidal Vasculature in Bietti Crystalline Dystrophy With CYP4V2 Mutations and in Retinitis Pigmentosa With EYS Mutations
Choroidal Vasculature in Bietti Crystalline Dystrophy With CYP4V2 Mutations and in Retinitis Pigmentosa With EYS Mutations
- Source :
- Investigative Opthalmology & Visual Science. 58:3871
- Publication Year :
- 2017
- Publisher :
- Association for Research in Vision and Ophthalmology (ARVO), 2017.
-
Abstract
- Purpose We compare the choroidal vascular area between Bietti crystalline dystrophy (BCD) patients with CYP4V2 mutations, retinitis pigmentosa (RP) patients with EYS mutations, and normal controls, and investigate the correlation between choroidal vascular area and associated parameters. Methods This prospective case-series study included consecutive nine eyes of nine BCD patients with CYP4V2 mutations (BCD group), 16 eyes of 16 RP patients with EYS mutations (EYS-RP group), and 16 eyes of 16 normal volunteers matched for age and axial length (control group). Using swept-source optical coherence tomography, we obtained en face images of the choroidal vasculature at the midpoint of the choriocapillaris layer-Sattler's layer (inner choroid) and Haller's layer (outer choroid). After binarization, we compared the inner and outer choroidal vascular areas among the three groups and identified associated factors. Results The outer choroidal vascular area was 43.34 ± 5.76%, 53.73 ± 4.92%, and 52.80 ± 4.10% in the BCD, EYS-RP, and control groups, respectively. This value was significantly smaller in the BCD group than in the EYS-RP and control groups (P < 0.001 in both; no significant difference between the EYS-RP and control groups). In the BCD group, the outer choroidal vascular area was correlated strongly with the subfoveal inner choroidal thickness (P = 0.001, r = 0.91, respectively). The inner choroidal vasculature could not be identified in eight of nine eyes in the BCD group. Conclusions The outer choroidal vascular narrowing might progress with the inner choroidal thinning in BCD, and the inner choroidal vasculature might be extinguished in advanced-stage BCD. Our findings may help to clarify the etiology of BCD.
- Subjects :
- Adult
Male
0301 basic medicine
medicine.medical_specialty
animal structures
03 medical and health sciences
0302 clinical medicine
BIETTI CRYSTALLINE DYSTROPHY
Retinal Diseases
Ophthalmology
Retinitis pigmentosa
Humans
Medicine
Cytochrome P450 Family 4
Prospective Studies
Eye Proteins
Prospective cohort study
Aged
Corneal Dystrophies, Hereditary
Choroid
business.industry
Significant difference
Axial length
Middle Aged
medicine.disease
eye diseases
Normal volunteers
030104 developmental biology
Vascular narrowing
medicine.anatomical_structure
Case-Control Studies
Mutation
embryonic structures
030221 ophthalmology & optometry
Blood Vessels
Female
sense organs
business
Retinitis Pigmentosa
Subjects
Details
- ISSN :
- 15525783
- Volume :
- 58
- Database :
- OpenAIRE
- Journal :
- Investigative Opthalmology & Visual Science
- Accession number :
- edsair.doi.dedup.....6ac61761d699b1b21a03f0f93f0a681d
- Full Text :
- https://doi.org/10.1167/iovs.17-21515