Dataset Information

Rates of decline in regions of the visual field defined by frequency-domain optical coherence tomography in patients with RPGR-mediated X-linked retinitis pigmentosa.

ABSTRACT:

Purpose

To determine whether annual decline in visual field sensitivity is greater in the transition zone at the edge of the frequency-domain optical coherence tomography (fdOCT) inner segment ellipsoid zone (EZ) than at other locations in the visual field.

Design

Prospective, longitudinal, observational study.

Participants

Forty-four patients with X-linked retinitis pigmentosa (XLRP) resulting from a mutation in the RPGR gene.

Methods

Static perimetric fields (Humphrey 30-2; Carl Zeiss Meditec, Dublin, CA) were obtained annually for 4 years. Beginning with year 2, fdOCT scans were obtained annually with a Heidelberg Spectralis HRA + OCT (Heidelberg Engineering, Heidelberg, Germany).

Main outcome measures

The rate of visual field decline at locations near the edge of the EZ compared with the rates for the macula and in the mid periphery.

Results

Sensitivity just inside and outside the edge of the EZ declined at rates of 0.84 and 0.92 dB/year, respectively. By comparison, average sensitivity in the macula and mid periphery declined by 0.38 and 0.61 dB/year, respectively.

Conclusions

The edge of the EZ in each patient with XLRP indicates a transition zone between relatively healthy and relatively degenerate retina. The annual loss of sensitivity in the transition zone is more rapid than it is elsewhere in the retina.

SUBMITTER: Birch DG

PROVIDER: S-EPMC4372456 | biostudies-literature | 2015 Apr

REPOSITORIES: biostudies-literature

ACCESS DATA

Publications

Rates of decline in regions of the visual field defined by frequency-domain optical coherence tomography in patients with RPGR-mediated X-linked retinitis pigmentosa.

Birch David G DG Locke Kirsten G KG Felius Joost J Klein Martin M Wheaton Dianna K H DK Hoffman Dennis R DR Hood Donald C DC

Ophthalmology 20141231 4

<h4>Purpose</h4>To determine whether annual decline in visual field sensitivity is greater in the transition zone at the edge of the frequency-domain optical coherence tomography (fdOCT) inner segment ellipsoid zone (EZ) than at other locations in the visual field.<h4>Design</h4>Prospective, longitudinal, observational study.<h4>Participants</h4>Forty-four patients with X-linked retinitis pigmentosa (XLRP) resulting from a mutation in the RPGR gene.<h4>Methods</h4>Static perimetric fields (Humph ...[more]

PMID: 25556114

Similar Datasets

Project description:BackgroundTo evaluate choroidal vascular changes, including choriocapillaris (CC) and middle/large choroidal vessels, in retinitis pigmentosa (RP) patients using wide-angle optical coherence tomography angiography (OCTA) and to determine whether changes in the choroidal vascularity have a relationship with visual function and retinal structural changes.Methods34 patients with a confirmed diagnosis of RP and 48 controls were recruited. All patients underwent detailed ophthalmologic and imaging examinations, including two types of OCTA (Optovue, 3 × 3 mm, 6 × 6 mm; VG-200, 12 × 12 mm). CC defects were defined according to the choroidal vascular structure in five degrees. To evaluate middle and large choroidal vascular changes, the choroidal vascularity index (CVI), which was the luminance volume to the total choroidal volume, was used.ResultsDefects of choroidal vascularity of RP eyes were detected in comparison to control eyes. The defects were observed in the CC layer with a concentric or lobular pattern at different degrees. CVI, which was used to reflect middle/large choroidal vascularity, decreased in the perifoveal, pararetinal and periretinal regions in the RP eyes. CC defects degree were correlated with the BCVA (p = 0.001, r = - 0.556), the Humphrey indexes (mean deviation, MD, p < 0.001, r = - 0.673; PVF, p = 0.003, r = - 0.639; 10° mean sensitivity, 10° MS, p = 0.002, r = - 0.651) and microperimetry index (mean sensitivity, MS, p < 0.001, r = - 0.807). The preserved CC area (mean value: 28.65 ± 12.50 mm2) was negatively correlated with MS measured by microperimetry (p = 0.005, r = - 0.449). Ordinary regression analysis revealed that the CC defect degree was associated with the CVI of perifovea (p = 0.002, 95% CI: - 102.14 to - 24.01), the EZ length (p = 0.006, 95% CI: - 0.006 to - 0.001) and the VAD (vascular area density) of the DCP (deep capillary plexus) in the fovea (p = 0.022, 95% CI: 0.038-0.478). No correlations were detected between BCVA and CVI in any retinal regions. No correlations were found between the CVI and the VAD in any retinal regions.ConclusionThe choroidal vascularity was widely defected in RP. Choriocapillaris and middle/large choroidal vascularity defects were correlated with each other. Visual function and retinal structural changes were found to be associated with choriocapillaris defects but not with middle/large choroidal vascular defects.

Project description:PurposeTranscorneal electrical stimulation (TES) is a novel treatment approach for patients with retinitis pigmentosa (RP). The aim of our study was to observe changes in optical coherence tomography angiography (OCTA) that would be attributed to TES treatment.MethodsA total of 73 eyes were included: 43 eyes of 22 subjects (11 ♀, 11 ♂) suffering from RP were examined at baseline (BL), after first stimulation (TS), 1 week (1W), and 6 months (6M) after treatment initiation and were compared with 30 control eyes of 15 subjects (8 ♀, 7 ♂). TES was performed simultaneously on both eyes for 30 min weekly. OCTA scans of 9 × 15 mm were recorded with a PLEX Elite 9000 swept-source OCTA device (Carl Zeiss Meditec AG, Jena). Vascular density metrics such as perfusion density (PD) and vessel density (VD) were calculated automatically for the macular area by using standardised extended early treatment diabetic retinopathy study (ETDRS) grids centred around the fovea. In addition, the capillary perfusion density (CPD) and the capillary flux index (CFI) of the peripapillary nerve fibre layer microvasculature in all four quadrants of an annulus centred at the optic disc were measured. All parameters were determined over all retinal layers and separately for the superficial (SCP) and deep capillary plexus (DCP). ANOVA-based linear mixed-effects models were calculated with SPSS®.ResultsThroughout the course of TES treatment, the macular VD and PD of all retinal layers in all subsections showed a slight decrement without reaching statistical significance, also when analysed separately in the SCP and DCP (p > 0.08). In analogy, the average CPD and CFI also presented with a slight decrement (p > 0.20). However, when compared with controls, most OCTA parameters showed a significant decrement (p < 0.05). When analysed systematically in all subsections of the extended ETDRS grid, the temporal macular subsections within the outer ring (radius 1.5-3 mm) and also of the peripheral C1, C2, and C3 rings (radius 3-7.5 mm) showed lower VD and PD values when compared with the other subsections (p < 0.05).ConclusionVascular density metrics in the macular region and the peripapillary microvasculature appear to remain unaffected by continuous TES treatment within a period of 6 months.

Dataset Information

Rates of decline in regions of the visual field defined by frequency-domain optical coherence tomography in patients with RPGR-mediated X-linked retinitis pigmentosa.

Purpose

Design

Participants

Methods

Main outcome measures

Results

Conclusions

Publications

Rates of decline in regions of the visual field defined by frequency-domain optical coherence tomography in patients with RPGR-mediated X-linked retinitis pigmentosa.

Similar Datasets

OmicsDI is part of the ELIXIR infrastructure

Tweets