Multimodal Imaging of Central Retinal Disease Progression in a 2-Year Mean Follow-up of Retinitis Pigmentosa

Abstract

Purpose To determine the rate of progression and optimal follow up time in patients with advanced stage retinitis pigmentosa (RP) comparing the use of fundus autofluorescence imaging and spectral domain optical coherence tomography. Design Retrospective analysis of progression rate. Methods Longitudinal imaging follow up in 71 patients with retinitis pigmentosa was studied using the main outcome measurements of hyperautofluoresent ring horizontal diameter and vertical diameter along with ellipsoid zone line width from spectral domain optical coherence tomography. Test-retest reliability and the rate of progression were calculated. The interaction between the progression rates was tested for sex, age, mode of inheritance, and baseline measurement size. Symmetry of left and right eye progression rate was also tested. Results Significant progression was observed in >75% of patients during the 2 year mean follow up. The mean annual progression rates of ellipsoid zone line, and hyperautofluorescent ring horizontal diameter and vertical diameter were 0.45° (4.9%), 0.51° (4.1%), and 0.42° (4.0%), respectively. The e llipsoid zone line width, and hyperautofluorescent ring horizontal diameter and vertical diameter had low test-retest variabilities of 8.9%, 9.5% and 9.6%, respectively. This study is the first to demonstrate asymmetrical structural progression rate between right and left eye, which was found in 19% of patients. The rate of progression was significantly slower as the disease approached the fovea, supporting the theory that RP progresses in an exponential fashion. No significant interaction between progression rate and patient age, sex, or mode of inheritance was observed. Conclusions Fundus autofluorescence and optical coherence tomography detect progression in patients with RP reliably and with strong correlation. These parameters may be useful alongside functional assessments as the outcome measurements for future therapeutic trials. Follow-up at 1 year intervals should be adequate to efficiently detect progression.

Comparison of 5-year progression of retinitis pigmentosa involving the posterior pole among siblings by means of SD-OCT: a retrospective study

“…In general, the mean annual rate of progression is our dataset is comparable with previous studies: the EZ line width reduction was 4.16% per year in accordance with 4.9–10.9% [34, 36, 38] reported in published literature. Similarly to published data, we observed that the progression rate of the EZ band atrophy decreases when the margins of the atrophic retina approach the foveal region [34, 36].…”

“…Similarly to published data, we observed that the progression rate of the EZ band atrophy decreases when the margins of the atrophic retina approach the foveal region [34, 36]. …”

Quantification of Ellipsoid Zone Changes in Retinitis Pigmentosa Using en Face Spectral Domain–Optical Coherence Tomography

“…Sujirakul et al 15 observed a significantly lower mean rate of structural progression in patients with RP who had a smaller preserved EZ band, which suggests that the progression rate decreases as the disease approaches the fovea. We observed a tendency toward higher variability in grading of the EZ area in eyes with a smaller preserved EZ area in our study.…”

Quantifying Fundus Autofluorescence in Patients With Retinitis Pigmentosa

“…1–4 With time the diameter of the ring progressively constricts. 5 Outside the SW-AF ring, photoreceptor cell degeneration is evidenced by loss of the ellipsoid zone (EZ) and external limiting membrane (ELM) and thinning or absence of the reflectivity layer corresponding to outer nuclear layer (ONL) in SD-OCT scans. 6,7 This thinning of photoreceptor-attributable reflectivity layers in SD-OCT scans also corresponds to a reduction in multi-focal ERG amplitudes.…”