Abstract
Purpose :
Using the dynamic retinal vessel analyzer (DVA, IMEDOS Systems) retinal vessels are recorded non-invasively in real time. The measured data can be further evaluated mathematically with regard to the dynamic time- and location-dependent vessel behavior. Fabry disease is characterized by specific systemic and ocular manifestations and is accompanied by altered vascular endothelial function. In this pilot study, the principle of multimodal analysis of pathologic dynamic retinal vessel behavior was applied to reveal specific structural and functional retinal microvascular changes in Fabry disease.
Methods :
The retinal vascular response was examined with DVA in 10 Fabry patients [42.5 (34.3 – 57.3) years old (median (1st quartile – 3rd quartile)), 4 women, 6 men] and in 10 age- and sex-matched healthy subjects. The vessel width of an arterial and a venous retinal vessel segments of ~1 mm in length was assessed time- and spatial- dependent. Using mathematical signal analysis, the retinal vascular response to the flicker light (530–600 nm, 12.5 Hz, 20 s) was analyzed together with the longitudinal microstructure of retinal vessels and unstimulated vessel wall oscillations.
Results :
The entire patient group showed no difference in response to the flicker light compared to the control group. In the meantime men with Fabry disease showed pronounced arterial dilation of 6.3 (5.6 – 7.9)% vs. 3.3 (2.5 – 4.1 )%, while females with Fabry showed a reduced arterial response of 3.4 (3.1 – 3.7)% vs. 5.4 (4.0 – 6.7)% (p<0.05). Fabry patients showed higher interindividual variation in periods of myogenic vascular regulation in arteries (frequency range: 0.06 – 0.15 Hz) and longer periods in veins. Female patients had a higher and less scattering periodicity of myogenic vascular regulation both in arteries and veins. The longitudinal microstructure of retinal arteries and veins in Fabry patients was structurally and functionally altered. This was particularly visible in veins at all stages of the vascular reaction by more pronounced waves with periods of 60 – 120 µm.
Conclusions :
Multimodal dynamic retinal vessel analysis includes information beyond conventional DVA evaluation of temporal vessel response to flicker. It represents a practical possibility to investigate the microvascular status of Fabry disease in detail. This allows for individual characterization of vascular damage and further understanding of disease pathology.
This abstract was presented at the 2023 ARVO Annual Meeting, held in New Orleans, LA, April 23-27, 2023.