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Quantitative progression of retinitis pigmentosa by optical coherence tomography angiography.

ABSTRACT: Optical coherence tomography angiography (OCT-A) is a non-invasive alternative to fluorescein angiography that allows for the study of the retinal and choroidal vasculatures. In this retrospective cohort study of 28 patients with retinitis pigmentosa (RP), we used OCT-A to quantify changes in perfusion density, foveal avascular zone (FAZ) area, and choriocapillaris blood flow over time and correlated these variables with ellipsoid zone (EZ) line width and best-corrected visual acuity (BCVA). Perfusion density decreased by 2.42?±?0.62% per year at the superior capillary plexus (SCP) (P?=?0.001) and 2.41?±?0.76% per year at the deep capillary plexus (DCP) (P?=?0.004). FAZ area increased by 0.078?±?0.021 mm2 per year (P?=?0.001) at the SCP and 0.152?±?0.039 mm2 per year (P?=?0.001) at the DCP. No changes were observed in the choriocapillaris blood flow. EZ line width had the strongest correlation to perfusion density at the SCP (r?=?0.660 and 0.635, first and second visit, respectively, P?=?0.001), while BCVA most strongly correlated with FAZ area at the SCP (r?=?0.679 and 0.548, P?=?0.001 and 0.003). Our results suggest that OCT-A is a useful tool for monitoring RP disease progression and may be used to measure retinal vascular parameters as outcomes in clinical trials.

SUBMITTER: Jauregui R

PROVIDER: S-EPMC6120860 | biostudies-other | 2018 Sep

REPOSITORIES: biostudies-other

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Quantitative progression of retinitis pigmentosa by optical coherence tomography angiography.

Jauregui Ruben R Park Karen Sophia KS Duong Jimmy K JK Mahajan Vinit B VB Tsang Stephen H SH

Scientific reports 20180903 1

Optical coherence tomography angiography (OCT-A) is a non-invasive alternative to fluorescein angiography that allows for the study of the retinal and choroidal vasculatures. In this retrospective cohort study of 28 patients with retinitis pigmentosa (RP), we used OCT-A to quantify changes in perfusion density, foveal avascular zone (FAZ) area, and choriocapillaris blood flow over time and correlated these variables with ellipsoid zone (EZ) line width and best-corrected visual acuity (BCVA). Per ...[more]

PMID: 30177829

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Project description:Background To 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. Methods 34 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. Results Defects 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. Conclusion The 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.

Project description:PurposeTo use projection-resolved optical coherence tomographic angiography (PR-OCTA) to characterize the microvascular changes in 3 distinct retinal plexuses in retinitis pigmentosa (RP) patients.DesignProspective cross-sectional study.MethodsA commercial 70-kHz spectral-domain optical coherence tomography (OCT) system was used to acquire 6-mm macular scans from RP patients and age-matched healthy participants at a tertiary academic center. Blood flow was detected using a commercial version of split-spectrum amplitude-decorrelation angiography (SSADA) algorithm. The PR-OCTA algorithm was used to suppress projection artifacts and resolve microvasculature in 3 plexuses around the macula. Vessel density was calculated from en face OCTA of the parafoveal and perifoveal regions in each of the 3 plexuses, as well as the all-plexus inner retinal slab. Inner and outer retinal thicknesses were measured form structural OCT scans. Generalized estimating equations and Spearman's rank correlation statistical methods were used.ResultsForty-four eyes from 26 RP patients and 34 eyes from 26 healthy subjects were included. Significant reduction in vessel density was detected in the perifovea but not the parafovea of inner retinal slab of RP patients (P = .001 and P = .56, respectively) compared to controls. We also found deeper retinal plexuses (intermediate and deep capillary plexuses, ICP and DCP) were primarily damaged by RP, compared to superficial vascular complex (SVC). Significant thickening of the inner retina and thinning of the outer retina were also observed. Strong correlation was found between the vessel density in the perifoveal ICP and DCP and outer retinal thickness in RP patients with no history of cystoid macular edema.ConclusionsPR-OCTA enables the detection of microvascular changes in the perifoveal regions of the ICP and DCP in RP, with relative sparing of the SVC. OCT and OCTA parameters might be able to provide better understanding of the pathophysiology of the disease, as well as monitoring disease progression and the response to experimental treatments.

Project description:Determining the annual rate of change in the width of the inner segment ellipsoid zone (EZ; ie, inner/outer segment border) in the context of short-term variability should allow us to better understand the value of this measure for future treatment trials in X-linked retinitis pigmentosa (XLRP).To identify the width of the central region showing an EZ and to determine the short-term repeat variability and the annual rate of change in the width of the EZ from spectral-domain optical coherence tomography (SD-OCT) measures in RP.Patients with recessive or simplex RP (age range, 8-65 years; mean age, 40.5 years) underwent scanning twice on the same day to evaluate test-retest variability. Patients with XLRP (age range, 8-27 years; mean age, 15.2 years) from a larger group participating in an ongoing double-blind treatment trial (docosahexaenoic acid vs placebo; clinicaltrials.gov NCT00100230) underwent spectral-domain optical coherence tomography line scanning across the horizontal meridian at 3 yearly intervals.Research center specializing in medical retina.Forty-eight patients with RP, including 20 with recessive or simplex RP and 28 with XLRP, and 23 healthy control subjects.Widths of the EZ calculated and compared among the 3 annual visits.Test-retest differences were normally distributed, and the magnitude of the difference was independent of mean EZ width. The mean (SD) for test-retest differences in EZ width was 0.08° (0.22°) (range, -0.30° to 0.60°). Thus, 95% of all test-retest differences fall within ± 0.43° (124 ?m). Of the 28 patients with XLRP, 27 showed a significant decrease in EZ width after 2 years. Patients with XLRP showed a mean annual decrease in EZ width of 0.86° (248 ?m, or 7%).The mean rate of decline in EZ width (7%) translates into a mean rate of change of 13% for the equivalent area of functioning retina. This rate of change is consistent with that reported for visual fields and full-field electroretinograms. Unlike visual fields and electroretinograms, however, the repeat variability is less than the annual rate of change. These results support the validity of EZ width as an outcome measure in prospective clinical trials in RP.

Dataset Information

Quantitative progression of retinitis pigmentosa by optical coherence tomography angiography.

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Quantitative progression of retinitis pigmentosa by optical coherence tomography angiography.

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