scholarly journals Optical Coherence Tomography Angiography Findings in Fabry Disease

2019 ◽  
Vol 8 (4) ◽  
pp. 528 ◽  
Author(s):  
Gilda Cennamo ◽  
Laura Giovanna Di Maio ◽  
Daniela Montorio ◽  
Fausto Tranfa ◽  
Camilla Russo ◽  
...  

Background: Fabry disease (FD) is a X-linked recessive lysosomal storage disorder characterized by altered biodegradation of glycosphingolipids. It is a multisystem pathology, also involving ophthalmological systems that show modifications of the vessel wall due to glycosphingolipid deposits. Optical coherence tomography angiography (OCT-A) allows for an objective analysis of retinal microvasculature alterations, evaluating retinal vessel density in macular region. Methods: A total of 54 FD patients (34 females, 20 males, mean age 44.1 ± 15.6 years) and 70 controls (36 females, 34 males, mean age 42.3 ± 15.6 years) were included in this study. We evaluated vessel density in different macular areas (whole image, fovea, and parafovea) of both the superficial capillary plexus (SCP) and of the deep capillary plexus (DCP). Results: In the SCP there was a significantly lower vascular density in patients compared with controls in whole image (49.95 ± 5.17% vs. 51.99 ± 2.52%; p < 0.001), parafovea (52.01 ± 6.69% vs. 54.30 ± 2.61%; p = 0.002), and fovea (22.38 ± 9.01% vs. 29.31 ± 5.84%; p < 0.0001). In the DCP the vessel density was statistically increased in each macular area in patients compared with controls (54.82 ± 8.07% vs. 50.93 ± 5.46%; p = 0.005, 57.76 ± 7.26% vs. 53.59 ± 5.46%; p = 0.0001, and 39.75 ± 8.59% vs. 34.43 ± 8.68%; p < 0.0001 for whole image, parafovea, and fovea, respectively). Conclusion: OCT-A analysis showed that the macular vessel density was significantly reduced in the SCP and increased in the DCP in FD patients compared with controls. These findings, which might be a consequence of the alteration of vascular wall occurring in FD, support the hypothesis that the evaluation of early retinal microvascular network changes could be a useful tool in the clinical evaluation of the disease.

2020 ◽  
Vol 9 (12) ◽  
pp. 4087
Author(s):  
Gilda Cennamo ◽  
Daniela Montorio ◽  
Ciro Santoro ◽  
Sirio Cocozza ◽  
Letizia Spinelli ◽  
...  

In this study, we evaluated the possible relationship between the changes in retinal vessel density (VD) by optical coherence tomography angiography (OCTA) and the vascular alterations involving renal, cardiovascular and central nervous systems in patients affected by Fabry disease (FD). In 50 FD patients, the retinal superficial capillary plexus (SCP) and deep capillary plexus (DCP) in macular region were evaluated by OCTA examination. The patients also underwent a brain magnetic resonance imaging scan, renal and echocardiographic examinations with quantification of systolic pulmonary arterial pressure (PAPs) and left atrial volume index (LAVi). The VD of SCP and DCP was inversely related with E/e’ ratio, LAVi, interventricular septal thickness, global longitudinal strain (GLS) and PAPs (p < 0.05). No relationship was found, with a multivariate analysis, between OCTA parameters and kidney function and neuroradiological signs of central nervous system involvement. OCTA could be a new vascular biomarker in FD, revealing a strong correlation between retinal capillary damage and myocardial impairment, possibly preceding both renal dysfunction and cerebrovascular involvement.


2019 ◽  
Vol 2019 ◽  
pp. 1-7 ◽  
Author(s):  
Jingyuan Yang ◽  
Mingzhen Yuan ◽  
Erqian Wang ◽  
Youxin Chen

The aim of this study was to compare the repeatability of optical coherence tomography angiography (OCT-A) measurements of macular vessel density using four OCT-A systems, including Heidelberg Spectralis HRA, Optovue RTVue XR, Zeiss Cirrus HD-OCT 5000, and Topcon DRI OCT Triton. A cross-sectional design was used for this study. The vascular density and vascular length density of the superficial and deep retinal capillary plexuses were imaged with OCT-A using 3 mm and 6 mm scan patterns and were calculated using ImageJ. Comparisons of intraclass correlation coefficients (ICC) were conducted. We found that the OCT-A systems had various levels of repeatability. Zeiss had better repeatability for vessel density than the other systems (overall ICC = 0.936). Optovue had better repeatability for vessel length density when the 6 mm scan pattern was used (ICC = 0.680 and 0.700 for retinal superficial and deep capillary plexus, respectively). We concluded that repeatability varied when different scan patterns of various OCT-A systems were used for imaging the superficial retinal and deep capillary plexuses. Results should be seen as valid only for a given method. The repeatability of various OCT-A systems should be considered in clinical practice and in clinical trials that use OCT-A metrics as outcome measures.


2019 ◽  
Author(s):  
Ehsan Vaghefi ◽  
Sophie Hill ◽  
Hannah M Kersten ◽  
David Squirrell

AbstractPurposeTo determine whether vessel density (VD) as measured by optical coherence tomography angiography (OCT-A) provide insights into retinal and choriocapillaris vascular changes with ageing and intermediate dry age related macular degeneration (AMD).MethodsSeventy-five participants were recruited into three cohorts; young healthy (YH) group, old healthy (OH) and those at high-risk for exudative AMD. Raw OCT and OCT-A data from TOPCON DRI OCT Triton were exported using Topcon IMAGENET 6.0 software, and 3D datasets were analysed to determine retinal thickness and vessel density.ResultsCentral macular thickness measurements revealed a trend of overall retinal thinning with increasing age. VD through the full thickness of the retina was highest in ETDRS sector 4 (the inferior macula) in all the cohorts. Mean VD was significantly higher in the deep capillary plexus than the superficial capillary plexus in all ETDRS sectors in all cohorts but there was no significant difference noted between groups. Choriocapillaris VD was significantly lower in all ETDRS sectors in the in the AMD group compared with the YH and the OH groups.ConclusionsRetinal vessel density maps, derived from the retinal plexi are not reliable biomarkers for assessing the ageing macular. Our non-proprietary analysis of the vascular density of the choriocapillaris revealed a significant drop off of VD with age and disease but further work is required to corroborate this finding. If repeatable, choriocapillaris VD may provide a non-invasive biomarker of healthy ageing and disease.Brief SummaryIn this manuscript, we have studied the potential of retinal vessel density as measured by optical coherence tomography angiography (OCT-A), as a biomarker for detection of high-risk of developing exudative age-related macular degeneration (AMD).


2021 ◽  
Author(s):  
Junyan Xiao ◽  
Yi Qu ◽  
Chan Zhao ◽  
Hang Song ◽  
Anyi Liang ◽  
...  

Abstract Purpose: Using spectral domain optical coherence tomography angiography (SD-OCTA) to evaluate tomographic and microvascular parameters in the macula in quiescent unilateral anterior pediatric uveitis (APU) patients.Methods: Forty-two eyes of 21 patients diagnosed with unilateral APU and 21 eyes of 21 normal controls (NC) were included in this study. 6*6 mm macular scanning mode of SD-OCTA was used for all subjects. The central macular thickness (CMT), subfoveal choroidal thickness (SFCT), vascular density (VD) of superficial capillary plexus (SCP) and the deep capillary plexus (DCP), the foveal avascular zone (FAZ) area, and choriocapillary flow density (CFD) were analyzed and compared among affected, fellow, and NC eyes. Correlation analysis were used to evaluate the potential correlating factors with CFD.Results: DCP VD and CFD were significantly lower in quiescent affected eyes as compared to fellow and NCs eyes (DCP VD both p<0.001; CDF1.0: p=0.012 and p=0.003; CDF1.5: p=0.015 and p=0.006; CDF3.0: p=0.036 and p=0.010, respectively). SCP VD, DCP VD and CMT were significantly lower in the fellow eyes as compared to NC eyes (p=0.021; p<0.001; p=0.037, respectively); CFD was negatively correlated with FAZ and CMT in affected eyes and fellow eyes. No significant differences were detected in FAZ among the 3 groups (p > .05).Conclusions: As compared to NC eyes, both retinal and choroidal microvasculature were impaired in eyes with quiescent APU; retinal microvasculature in unaffected fellow eyes of unilateral APU was also impaired. OCTA is a useful technology for detection of subclinical microvascular changes in APU and may be useful as an additional prognostic tool.


2018 ◽  
Vol 159 (8) ◽  
pp. 320-326
Author(s):  
Cecília Czakó ◽  
Gábor László Sándor ◽  
Mónika Ecsedy ◽  
Zsuzsanna Szepessy ◽  
Ágnes Borbándy ◽  
...  

Abstract: Introduction: Optical coherence tomography angiography is a non-invasive imaging technique that is able to visualize the different retinal vascular layers using motion contrast to detect blood flow without intravenous dye injection. This method might help to assess microangiopathy in diabetic retinopathy during screening and follow-up. Aim: To quantify retinal microvasculature alterations in both eyes of diabetic patients in relation to systemic risk factors using optical coherence tomography angiography. Method: Both eyes of 36 diabetic patients and 45 individuals without diabetes were examined. Duration of diabetes, insulin therapy, blood pressure, HbA1c, dyslipidemia, axial length and the presence of diabetic retinopathy were recorded. Retinal vessel density was measured by optical coherence tomography angiography. The effect of risk factors on vessel density and between-eye asymmetry was assessed using multivariable regression analysis. Results: Vessel density was significantly lower and between-eye difference was significantly higher in diabetic patients compared to controls (p<0.05). Both vessel density and between-eye asymmetry significantly correlated with diabetes duration (p<0.05) after controlling for the effect of risk factors. The between-eye asymmetry in vessel density was significantly higher in patients without clinically detectable diabetic retinopathy compared to control subjects (p<0.001). Conclusions: There is a decrease in retinal vessel density and an increase in between-eye asymmetry in patients with diabetes compared to healthy subjects. By using optical coherence tomography angiography, the detection of these microvascular alterations is possible before clinically detectable diabetic retinopathy and might serve as a useful tool in both screening and timing of treatment. Orv Hetil. 2018; 159(8): 320–326.


2021 ◽  
Vol 18 (2) ◽  
pp. 147916412110044
Author(s):  
Marta Wysocka-Mincewicz ◽  
Joanna Gołębiewska ◽  
Marta Baszyńska-Wilk ◽  
Andrzej Olechowski ◽  
Aleksandra Byczyńska ◽  
...  

Background: This study aimed to assess the influence of pubertal status on the results of optical coherence tomography angiography (OCTA) in children with type 1 diabetes (T1D). Methods: We enrolled 167 consecutive children with T1D. Retinal superficial capillary plexus (SCP) and deep capillary plexus (DCP) vessel density data underwent analysis. We divided the study population into three subgroups depending on the pubertal status. Results: Analysis of the prepubertal and pubertal subgroups revealed statistically significant differences in foveal thickness (FT) (p < 0.05) and foveal SCP (p < 0.02). Analyzing subgroups of the prepubertal and postpubertal children, we observed statistically significant differences in FT (p < 0.03), whole SCP (p < 0.02), and foveal SCP (p < 0.02). Comparison of the pubertal and postpubertal subjects revealed differences in parafoveal DCP (p < 0.003). In the groups matched depending on diabetes duration, we observed differences between prepubertal, pubertal, and postpubertal children in FT, PFT, and parafoveal SCP and DCP. Conclusion: Our data suggest that in a cohort of pubertal children with a short duration of diabetes, alterations in retinal vessel density occur early and progress during puberty.


2018 ◽  
Vol 30 (1) ◽  
pp. 48-57 ◽  
Author(s):  
Jaeryung Oh ◽  
Daniel Jinhag Baik ◽  
Jaemoon Ahn

Purpose: To quantify vascular and structural macular variables in healthy eyes and to investigate correlations between these variables and age using optical coherence tomography angiography. Materials and methods: A total of 261 eyes of 261 subjects with normal fundus were included. Central macular thickness, ganglion cell layer to inner plexiform layer thickness, outer retina layer thickness, subfoveal choroidal thickness, and choroidal vascularity index were measured using optical coherence tomography. Foveal avascular zone area, vascular density, and flow void area were measured using optical coherence tomography angiography. Results: Vascular density in the superficial capillary plexus was correlated with central macular thickness, ganglion cell layer to inner plexiform layer thickness, and outer retina layer thickness ( P < 0.001, P = 0.004, and P < 0.001, respectively). Vascular density in the deep capillary plexus was correlated with central macular thickness and outer retina layer thickness ( P = 0.003 and P = 0.001, respectively). Vascular density of choriocapillaris was correlated with vascular density of superficial capillary plexus and deep capillary plexus ( P < 0.001 and P = 0.001, respectively). Conclusion: Vascular density of choriocapillaris varies with retinal vascular density rather than the structure of choroid using optical coherence tomography angiography. In contrast, retinal vascular density changes as the retinal structure. Our results provide more information about the relationship between retina and choroid.


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