scholarly journals Retinal vessel tortuosity as a prognostic marker for disease severity in Fabry disease

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Yevgeniya Atiskova ◽  
Jan Wildner ◽  
Martin Stephan Spitzer ◽  
Charlotte Aries ◽  
Nicole Muschol ◽  
...  

Abstract Purpose The aim of this case control study was to evaluate the prognostic value of automatically quantified retinal vessel tortuosity from fundus images and vessel density from OCT-A in Fabry disease and to evaluate the correlation of these with systemic disease parameters. Methods Automatically quantified perimacular retinal vessel tortuosity (MONA REVA software), acquired by fundus imaging, and perifoveal retinal vessel density, acquired by optic coherence tomography angiography (OCT-A) were compared between 26 FD patients and 26 controls. Gender and FD phenotype were analyzed to the obtained retinovascular data and correlated to the Mainz severity score index (MSSI) and plasma lyso-Gb3. Results Automatically quantified retinal vessel tortuosity indices of FD patients were significantly lower, reflecting an increased vessel tortuosity, compared to controls (p = 0.008). Males with a classical phenotype showed significantly lower retinal vessel tortuosity indices compared to males with an oligosymptomatic phenotype and females with a classical or oligosymptomatic phenotype (p < 0.001). The retinal vessel tortuosity index correlated significantly with systemic disease severity parameters [global MSSI (r = − 0.5; p < 0.01), cardiovascular MSSI (r = − 0.5; p < 0.01), lyso-Gb3 (r = − 0.6; p < 0.01)]. Conclusion We advocate fundus imaging based automatically quantified retinal vessel tortuosity index over OCT-A imaging as it is a quick, non-invasive, easily assessable, objective and reproducible marker.

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.


Author(s):  
Noemi Guemes-Villahoz ◽  
Barbara Burgos-Blasco ◽  
Beatriz Vidal-Villegas ◽  
Juan Donate-López ◽  
Francisco Javier Martín-Sánchez ◽  
...  

2020 ◽  
Author(s):  
Qian Wang ◽  
Lili Liu ◽  
Jost B. Jonas ◽  
Bixia Gao ◽  
Shou Ling Wu ◽  
...  

2021 ◽  
Vol 156 (11) ◽  
pp. 541-546
Author(s):  
Noemi Guemes-Villahoz ◽  
Barbara Burgos-Blasco ◽  
Beatriz Vidal-Villegas ◽  
Juan Donate-López ◽  
Francisco Javier Martín-Sánchez ◽  
...  

2021 ◽  
Vol 10 (24) ◽  
pp. 5756
Author(s):  
Daniela Montorio ◽  
Roberta Lanzillo ◽  
Antonio Carotenuto ◽  
Maria Petracca ◽  
Marcello Moccia ◽  
...  

Optical Coherence Tomography Angiography (OCTA) abnormalities occur in multiple sclerosis (MS) over the course of the disease. OCTA investigations at early MS stages are lacking. We aimed to investigate vessel density in macular and papillary regions over two years after an initial demyelinating event (IDE). Vessel density was analyzed in superficial, deep, choriocapillaris and radial peripapillary plexus at baseline, and after one and two years. We also evaluated structural OCT parameter changes of the ganglion cell complex (GCC) and retinal nerve fiber layer (RNFL). We evaluated 30 eyes from 15 IDE patients (7 females, 8 males, mean age 28.4 ± 9.6 years) and 30 eyes from 15 healthy controls. After 2 years, we reported in the IDE group a reduced vessel density in the superficial capillary plexus, deep capillary plexus and radial peripapillary capillary plexus with respect to the baseline (coeff. β = −2.779, p = 0.013; coeff. β = −4.055, p = 0.018 and coeff. β = −2.687, p ≤ 0.001; respectively), while GCC and RNFL thicknesses did not change. Vessel density reduction was not associated with an expanded disability status scale (EDSS) change, relapse occurrence or magnetic resonance imaging activity. The analysis of healthy controls did not reveal any impairment in OCT and OCTA parameters over 2 years of follow-up. Retinal vascular loss occurs in patients with an IDE independently from clinical and radiological disease activity. Retinal vessel density could represent a novel early biomarker to monitor the MS pathological burden.


2020 ◽  
Vol 70 (3) ◽  
pp. 79-85
Author(s):  
Robert C. Gunzenhauser ◽  
Irena Tsui ◽  
Federico G. Velez ◽  
Simon SM Fung ◽  
Joseph L. Demer ◽  
...  

2020 ◽  
Vol 2020 ◽  
pp. 1-9
Author(s):  
Hyung Bin Lim ◽  
Tae Seen Kang ◽  
Yeo Kyoung Won ◽  
Jung Yeul Kim

Purpose. To evaluate the difference in the repeatability of automated superficial retinal vessel density and foveal avascular zone (FAZ) metrics according to the measurement area of optical coherence tomography angiography (OCTA). Methods. A total of 127 normal eyes from 127 healthy subjects were included. Macular angiography images were acquired from all subjects using the Zeiss Cirrus 5000 with AngioPlex™ OCTA software. Scans of 3 × 3 mm and 6 × 6 mm were each performed twice in a randomly arranged sequence. Vessel density (VD), perfusion density (PD), and FAZ metrics of the superficial capillary plexus were calculated automatically for all scans, and the repeatabilities for both scan patterns were assessed based on intraclass correlation (ICC), coefficient of variation (CV), and coefficient of repeatability (CR) parameters. The average measured values in the two scan patterns were also compared. Results. VD was significantly greater in the 3 × 3 mm scan than in the 6 × 6 mm scan according to all parameters, whereas PD was significantly less in the 3 × 3 mm scan than in the 6 × 6 mm scan. The ICCs for VDs in the central fovea were 0.826 and 0.741 for the 3 × 3 and 6 × 6 mm scans, respectively, and the CVs were 8.00% and 12.75%. For PDs, the ICCs were 0.839 and 0.762 and the CVs were 9.32% and 14.90%. The FAZ metrics in the 3 × 3 mm scan showed good repeatability with an ICC >0.75 and a CV <10.0%. However, all ICCs for the 6 × 6 mm scans were <0.75, and the CVs were all >10%. Conclusions. The 6 × 6 mm macular angiography scans resulted in lower repeatabilities than the 3 × 3 mm scans according to all OCTA parameters, particularly in the central fovea and FAZ metrics. The 3 × 3 mm scan was more suitable than the 6 × 6 mm scan for analyzing macular microvascular density and FAZ metrics.


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