Faculty Collaboration Database

Quantitative Analysis of Retinal Structure Using Spectral-Domain Optical Coherence Tomography in RPGR-Associated Retinopathy. Am J Ophthalmol 2017 Jun;178:18-26

Date

03/23/2017

Pubmed ID

28322733

Pubmed Central ID

PMC5451208

DOI

10.1016/j.ajo.2017.03.012

Scopus ID

2-s2.0-85016633761 (requires institutional sign-in at Scopus site)   29 Citations

Abstract

PURPOSE: To quantify retinal structure and progression using spectral-domain optical coherence tomography (SDOCT) in patients with retinitis pigmentosa (RP) associated with retinitis pigmentosa GTPase regulator gene (RPGR) mutations.

DESIGN: Retrospective observational case series.

METHODS: Setting: Moorfields Eye Hospital, London, United Kingdom.

SUBJECTS: Both eyes of 32 patients. SDOCT follow-up period of >1 year (3.1 ± 1.4 years).

MAIN OUTCOME MEASURES: Ellipsoid zone (EZ) width (EZW) and outer nuclear layer (ONL) and inner retinal layer (IRL) thickness measurements. Progression rates, interocular symmetry, and association with age and genotype were investigated.

RESULTS: Significant differences were observed between baseline and final measurements of EZW and ONL thickness, but not for IRL thickness. Baseline and final EZWs were 2438 ± 1646 μm and 1901 ± 1423 μm for right eyes (P < .0001); 2420 ± 1758 μm and 1922 ± 1482 μm for left eyes (P < .0001). EZW constriction rates were 176.6 ± 130.1 μm/year and 173.1 ± 146.8 μm/year for right and left eyes. ONL thinning rates were 2.58 ± 2.85 μm/year and 2.52 ± 3.54 μm/year for right and left eyes. Interocular differences in EZW and ONL progression were not significant (P = .8609 and P = .6735, respectively). Strong correlations were found between EZW constriction rates of right and left eyes (r_s = 0.627, P = .0002) and between EZW constriction and baseline EZW (r_s = 0.714, P < .0001). There was moderate negative correlation between EZW constriction and age (r_s = -0.532, P < .0001). Correlation between ONL thinning and age was not significant, as were differences between EZW and ONL progression rates with respect to genotype.

CONCLUSIONS: This study provides SDOCT progression rates for RPGR-associated RP. There is overall interocular symmetry with implications for future treatment trials where 1 eye could serve as a control.

Author List

Tee JJL, Carroll J, Webster AR, Michaelides M

Author

Joseph J. Carroll PhD Director, Professor in the Ophthalmology and Visual Sciences department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Adult
Disease Progression
Eye Proteins
Female
Genotype
Humans
Male
Mutation
Retina
Retinitis Pigmentosa
Retrospective Studies
Tomography, Optical Coherence
Visual Acuity