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Relationship of Vessel Density to Vessel Length Density in Patients with Treated Fabry Disease.

ABSTRACT:

Background

Fabry disease (FD) is a potentially lethal lysosomal disorder with systemic vascular changes. Previous studies demonstrated retinal vascular involvement using optical coherence tomography angiography (OCTA) in affected patients; Aim: To analyze and quantify the retinal vasculature measuring vessel density (VD), vessel length density (VLD), and the ratio of VD to VLD (VD/VLD) in superficial capillary plexuses (SCP) and deep capillary plexuses (DCP) using OCTA in patients with FD and to show whether they differ from healthy controls (HC); Patients and methods: Single-center, retrospective, consecutive cohort study of patients with genetically proven FD. Patients underwent an ophthalmological examination including OCTA. VD, VLD, foveal avascular zone (FAZ), and the VD/VLD were compared to an HC group using a linear mixed model; Results: A statistically significant difference in the VLD and VD/VLD of DCP was observed between the two groups (p < 0.001). Using ROC curves with AUC and Youden's Index, a cut-off value for differentiating both groups using VD/VLD in DCP FD with high specificity and high sensitivity was established; Conclusions: FD and HC groups seem to be separable using the VD/VLD ratio in DCP. This new biomarker might differentiate changes in the retinal microvasculature that are not detectable by VD or VLD alone.

SUBMITTER: Wiest MRJ

PROVIDER: S-EPMC10093750 | biostudies-literature | 2023 Mar

REPOSITORIES: biostudies-literature

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Relationship of Vessel Density to Vessel Length Density in Patients with Treated Fabry Disease.

Wiest Maximilian Robert Justus MRJ Toro Mario Damiano MD Nowak Albina A Bajka Anahita A Fasler Katrin K Al-Sheikh Mayss M Hamann Timothy T Zweifel Sandrine Anne SA

Diagnostics (Basel, Switzerland) 20230324 7

<h4>Background</h4>Fabry disease (FD) is a potentially lethal lysosomal disorder with systemic vascular changes. Previous studies demonstrated retinal vascular involvement using optical coherence tomography angiography (OCTA) in affected patients; Aim: To analyze and quantify the retinal vasculature measuring vessel density (VD), vessel length density (VLD), and the ratio of VD to VLD (VD/VLD) in superficial capillary plexuses (SCP) and deep capillary plexuses (DCP) using OCTA in patients with F ...[more]

PMID: 37046445

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Relationship of Vessel Density to Vessel Length Density in Patients with Treated Fabry Disease.

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Relationship of Vessel Density to Vessel Length Density in Patients with Treated Fabry Disease.

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