Abstract
ObjectiveTo describe and quantify the structural and functional consequences of retinal vasculopathy and cerebral leukoencephalopathy (RVCL) on the neurosensory retina. DesignCross-sectional descriptive study from December 2021 to December 2022 ParticipantsRVCL patients (n=9, 18 eyes) recruited from the RVCL Research Center at Washington University in St. Louis MethodsRVCL patients underwent comprehensive ophthalmological evaluation including Optical Coherence Tomography (OCT), Optical Coherence Tomography Angiography (OCT-A), ultra-wide-field (UWF) fundus imaging, retinal autofluorescence, dark adaptation, electroretinography (ERG), Goldmann kinetic perimetry, and fluorescein angiography (FA). Main Outcomes MeasuresComprehensive characterization from various modalities including: best corrected visual acuity, central subfield thickness (μm) from OCT, foveal avascular zone (mm2) from OCT-A, dark adaptation rod intercept (sec), cone response in ERG, and presence or absence of vascular abnormalities, leakage, neovascularization, and nonperfusion on FA. ResultsA total of 18 eyes from 9 individuals were included in this study. The best-corrected visual acuity ranged from 20/15 to 20/70. The mean central subfield thickness from OCT was 275.8 μm (range 217-488 μm). The mean foveal avascular zone (FAZ) from OCT-A was 0.65 mm2 (range 0.18-1.76 mm2). On dark adaptometry, the mean time was 5.02 minutes (range 2.9 - 6.5 min) with 1 individual with impaired dark adaptation. ERG demonstrated mild cone response impairment in 4 eyes. On FA, there was evidence of macular and peripheral capillary non-perfusion in 16 of 18 eyes and notable areas of vascular leakage and retinal edema in 5 of the 18 eyes. ConclusionsThis study illustrates the phenotypic spectrum of disease and may be clinically valuable for aiding diagnosis, monitoring disease progression, and further elucidating the pathophysiology of RVCL to aid in the development of therapies.
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