Abstract
Early detection is an essential step for effective intervention of sickle cell retinopathy (SCR). Emerging optical coherence tomography angiography (OCTA) provides excellent three-dimensional (3D) resolution to enable label-free, noninvasive visualization of retinal vascular structures, promising improved sensitivity in detecting SCR. However, quantitative analysis of SCR characteristics in OCTA images is yet to be established. In this study, we conducted comprehensive analysis of six OCTA parameters, including blood vessel tortuosity, vessel diameter, vessel perimeter index (VPI), area of foveal avascular zone (FAZ), contour irregularity of FAZ and parafoveal avascular density. Compared to traditional retinal thickness analysis, five of these six OCTA parameters show improved sensitivity for SCR detection than retinal thickness. It is observed that the most sensitive parameters were the contour irregularity of FAZ in the superficial layer and avascular density in temporal regions, while the area of FAZ, tortuosity and mean diameter of the vessel were moderately sensitive.
Highlights
Sickle Cell Disease (SCD) is an inherited red blood cell disorder that affects 90,000 – 100,000 Americans [1], making it one of the most prevalent genetic disorders in the United States [2]
A tortuosity increase of 16.56% was observed in SCD patients compared to control subjects
The analysis revealed that most of optical coherence tomography angiography (OCTA) parameters are not significantly correlated with the retinal thickness except for the area of foveal avascular zone (FAZ) in Circle 1 (1 mm diameter or 0.5 mm radius from the center) of the superficial layer
Summary
Sickle Cell Disease (SCD) is an inherited red blood cell disorder that affects 90,000 – 100,000 Americans [1], making it one of the most prevalent genetic disorders in the United States [2]. Red blood cells with normal hemoglobin have a disc shape and this round shape allows the cells to be flexible so that they can move through large blood vessels and small capillaries to deliver oxygen. In SCD, red blood cells deform into a C-shaped farm tool called a “sickle”. When sickle cells travel through small blood vessels, they can get stuck and occlude blood flow and prevent oxygen from reaching vital organs. Sickle cell retinopathy (SCR) is one of the major ocular manifestations of SCD. The underlying systemic and ocular manifestations of SCD are a result of vasoocclusive ischemia due to the blocking of blood vessels by sickle-shaped erythrocytes
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