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

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 Automati...

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Autores principales:	Yevgeniya Atiskova, Jan Wildner, Martin Stephan Spitzer, Charlotte Aries, Nicole Muschol, Simon Dulz
Formato:	article
Lenguaje:	EN
Publicado:	BMC 2021
Materias:	Fabry disease Vascular tortuosity Retina OCT-A MONA REVA Medicine R
Acceso en línea:	https://doaj.org/article/e30d04021ccf4b299d8990bb1dc3f90f
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spelling	oai:doaj.org-article:e30d04021ccf4b299d8990bb1dc3f90f2021-11-21T12:26:06ZRetinal vessel tortuosity as a prognostic marker for disease severity in Fabry disease10.1186/s13023-021-02080-01750-1172https://doaj.org/article/e30d04021ccf4b299d8990bb1dc3f90f2021-11-01T00:00:00Zhttps://doi.org/10.1186/s13023-021-02080-0https://doaj.org/toc/1750-1172Abstract 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.Yevgeniya AtiskovaJan WildnerMartin Stephan SpitzerCharlotte AriesNicole MuscholSimon DulzBMCarticleFabry diseaseVascular tortuosityRetinaOCT-AMONA REVAMedicineRENOrphanet Journal of Rare Diseases, Vol 16, Iss 1, Pp 1-9 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Fabry disease Vascular tortuosity Retina OCT-A MONA REVA Medicine R
spellingShingle	Fabry disease Vascular tortuosity Retina OCT-A MONA REVA Medicine R Yevgeniya Atiskova Jan Wildner Martin Stephan Spitzer Charlotte Aries Nicole Muschol Simon Dulz Retinal vessel tortuosity as a prognostic marker for disease severity in Fabry disease
description	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.
format	article
author	Yevgeniya Atiskova Jan Wildner Martin Stephan Spitzer Charlotte Aries Nicole Muschol Simon Dulz
author_facet	Yevgeniya Atiskova Jan Wildner Martin Stephan Spitzer Charlotte Aries Nicole Muschol Simon Dulz
author_sort	Yevgeniya Atiskova
title	Retinal vessel tortuosity as a prognostic marker for disease severity in Fabry disease
title_short	Retinal vessel tortuosity as a prognostic marker for disease severity in Fabry disease
title_full	Retinal vessel tortuosity as a prognostic marker for disease severity in Fabry disease
title_fullStr	Retinal vessel tortuosity as a prognostic marker for disease severity in Fabry disease
title_full_unstemmed	Retinal vessel tortuosity as a prognostic marker for disease severity in Fabry disease
title_sort	retinal vessel tortuosity as a prognostic marker for disease severity in fabry disease
publisher	BMC
publishDate	2021
url	https://doaj.org/article/e30d04021ccf4b299d8990bb1dc3f90f
work_keys_str_mv	AT yevgeniyaatiskova retinalvesseltortuosityasaprognosticmarkerfordiseaseseverityinfabrydisease AT janwildner retinalvesseltortuosityasaprognosticmarkerfordiseaseseverityinfabrydisease AT martinstephanspitzer retinalvesseltortuosityasaprognosticmarkerfordiseaseseverityinfabrydisease AT charlottearies retinalvesseltortuosityasaprognosticmarkerfordiseaseseverityinfabrydisease AT nicolemuschol retinalvesseltortuosityasaprognosticmarkerfordiseaseseverityinfabrydisease AT simondulz retinalvesseltortuosityasaprognosticmarkerfordiseaseseverityinfabrydisease
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Retinal vessel tortuosity as a prognostic marker for disease severity in Fabry disease

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