Outer retinal layers as predictors of visual acuity in retinitis pigmentosa: a cross-sectional study

Abstract

Purpose To evaluate the integrity of the outer retinal layers-outer nuclear layer (ONL), external limiting membrane (ELM), ellipsoid (EZ), and interdigitation band (IZ)-using spectral-domain optical coherence tomography and estimate their effect on visual acuity in retinitis pigmentosa (RP). Methods A cross-sectional study was performed in the Ophthalmology Department of Hospital de Braga, Portugal. Patients with RP followed in the Hospital de Braga during January to August 2017 were included. Exclusion criteria were lack of data, macular edema due to RP, and concomitant retinal, optic nerve, or corneal disease that could interfere with visual acuity. Age, sex, time from diagnosis, phakic status, ONL thickness, and presence or absence of foveal ELM, EZ, and IZ were correlated to the bestcorrected visual acuity (BCVA). Results Forty-eight eyes were analyzed. There was a strong and positive correlation in BCVA between both eyes (p < .001*). ONL thickness was decreased in 95.8%. The EZ was the most absent layer (79.2%), followed by IZ (70.8%) and ELM (45.8%). A positive family history (p = .04*) and increased time from diagnosis (p = .037*) correlated with worse BCVA. A thicker ONL (p = .001*) and the presence of subfoveal ELM (p < .001*), EZ (p < .001*), and IZ (p = .02*) are correlated with better BCVA. There was a strong and positive correlation between the number of layers affected and a lower BCVA (p < .001). The presence of EZ was a significant predictor of BCVA (p = .02*). Conclusions The status of the outer retinal layers seems to influence BCVA. The status of the EZ was the most important predictor of BCVA but the ONL, ELM, and IZ may have a cumulative effect in the progression of visual loss.

Macular Ganglion Cell-Inner Plexiform Layer, Ganglion Cell Complex, and Outer Retinal Layer Thicknesses in a Large Cohort of Chinese Children

“…[11][12][13][14] Besides, measurement of outer retinal layer (ORL), which contain the inner nuclear layer, outer plexiform layer, outer nuclear layer, ellipsoid zone, and RPE, is a potential marker for retinal diseases such as retinitis pigmentosa, Stargardt disease, and retinopathy of prematurity. 15,16 In recent years, advances in optical coherence tomography (OCT) have enabled the accurate measurement of macular GCIPL, GCC, and ORL thicknesses with high-resolution images and minimal patient collaboration time. These measurements would constitute a significant supplementary test for pediatric disorders affecting the inner and outer retina, optic nerve, and cerebral visual pathway.…”

“…These measurements would constitute a significant supplementary test for pediatric disorders affecting the inner and outer retina, optic nerve, and cerebral visual pathway. [9][10][11][12][13][15][16][17][18][19][20][21] Despite the importance of the measurements of macular layers, the normative macular layer thicknesses in children remain unavailable. Most reported studies concerning normative macular layer thicknesses and topographic distributions have been performed in adult populations, 22,23 limiting the application of the database in pediatric patients.…”

Automated Quantification of Photoreceptor alteration in macular disease using Optical Coherence Tomography and Deep Learning

“…Regardless of how these layers are called, there is a clear consensus that the integrity of the photoreceptor bands has a substantial impact on vision. However, it is unclear to which extent photoreceptors can be restored after morphological disruption, and which degree of disruption or thickness variability is functionally relevant and leads to permanent visual compromise [25][26][27][28] . Particularly in DME, the condition of the outer retinal layers, containing the photoreceptors, has been shown to better correlate with vision than central retinal thickness 29 .…”

“…We have also presented a strategy for automatically computing en-face thickness maps of the photoreceptors. This approach is also applicable to study the effect of pathological changes in photoreceptor thickness due to other diseases such as glaucoma 33 or genetic dystrophies such as retinitis pigmentosa 25,34 , or for estimating other morphological properties such as the overall length of photoreceptors associated with several diseases, including DME 29 and RVO 28 or even early age-related macular degeneration. Our automated photoreceptor segmentation could be prospectively applied in clinical studies and allows for the first time to segment the photoreceptors on each individual voxel of the raster scan instead of using random point thickness measurement obtained manually.…”

Monitoring progression of retinitis pigmentosa: current recommendations and recent advances

“…More recently, detailed structural information, such as the integrity and the extent of the ELM and EZ as well as ONL thickness, have been shown to correlate well with function, and are thus used to monitor disease progression and serve as outcome measures in therapeutic trials. [32,36,[57][58][59][60][61][62] Figure 3A shows a progressive decrease in the lateral extend of the ELM and EZ in a left eye of a 28 year-old male patient with autosomal recessive RP during a follow up period of six years. Figure 3B is an example of a 26 year-old female patient with CRB1 associated RP that showed clinically meaningful improvement of her right eye cystoid macular oedema upon systemic acetazolamide therapy.…”