Abstract
Optical coherence tomography angiography (OCT-A) represents the most recent tool in ophthalmic imaging. It allows for a non-invasive, depth-selective and quantitative visualization of blood flow in central retinal vessels and it has an enormous diagnostic potential not only in ophthalmology but also with regards to neurologic and systemic diseases. Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a hereditary vascular small-vessel disease caused by Notch3 mutations and represents the most common form of hereditary stroke disorder. In this study, CADASIL patients prospectively underwent OCT-A imaging to evaluate retinal and choriocapillaris blood flow as well as blood flow at the optic nerve head. The vessel density of the macular region and the size of the foveal avascular zone in the superficial and deep retinal plexus were determined as well as the vessel density at the optic nerve head and in the choriocapillaris. Additionally, cerebral magnetic resonance images were evaluated. The main finding was that vessel density of the deep retinal plexus was significantly decreased in CADASIL patients compared to healthy controls which may reflect pericyte dysfunction in retinal capillaries.
Highlights
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a hereditary vascular small-vessel disease caused by Notch[3] mutations[1]
OCT-Angiography (OCT-A) represents a novel non-invasive, depth-selective modality that allows for visualization of retinal blood flow without dye injection[8]
The device used in this study provides a scheme that defines en-face slabs according to a set of reference planes automatically segmented by the integrated software including the internal limiting membrane (ILM), the inner plexiform layer (IPL) and the retinal pigment epithlium (RPE) ref.[10]
Summary
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a hereditary vascular small-vessel disease caused by Notch[3] mutations[1]. It is distinguished from other vascular disorders by the characteristic accumulation of granular osmiophilic material in brain vasculature[2]. A study by Fang and co-workers confirmed these results in a chinese CADASIL population They found a correlation between retinal vessel changes and findings in brain magnetic resonance imaging (MRI)[7]. The standard retinal imaging techniques for visualizing retinal and choroidal perfusion are fluorescein angiography (FA) and indocyanine green angiography (ICGA) Both are invasive, two-dimensional en-face modalities that cannot show retinal blood flow depth-selectively. This study aims to evaluate macular retinal and choriocapillaris (CC) blood flow as well as blood flow at the ONH in CADASIL patients using OCT-A
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