Abstract
Ischemic microangiopathy was clearly identified in sickle cell disease (SCD) using fluorescein angiography. A prospective observational clinical study was conducted to assess the foveal avascular zone (FAZ) area and explore perifoveal microvasculature changes in the superficial (SCP) and deep (DCP) capillary plexus using optical coherence tomography angiography (OCTA) and compare two genotypes—HbS/HbS (HbSS) and HbS/HbC (HbSC)-to control. All consecutive patients with electrophoretic confirmation of SCD were included. Swept-source OCTA scans (Triton Plus, Topcon, Tokyo, Japan) with a 3 × 3-mm scanning area and ultra-wide field (UWF) retinography (California, Optos, Fife, Scotland) were recorded for all patients. For OCTA analysis, preset parameters were used to segment the SCP and DCP. The FAZ area was manually assessed. The number of vascular branching points was automatically assessed based on the vascular skeletonization using ImageJ software. Eyes were staged based on Goldberg’s classification of SCD retinopathy (SCDR) using UWF imaging. Forty-six eyes of 24 patients were included in the HbSS (n = 27) and HbSC (n = 19) groups and 16 eyes of 8 unaffected patients in a control group. In the DCP, the FAZ was significantly larger in the HbSC (p = 0.0001) and HbSS (p = 0.0004) groups compared to controls. The FAZ area in the SCP, CRT and number of superficial vascular branching points did not significantly differ between both genotypes. There were less branching points in the HbSC (p = 0.034) and HbSS (p = 0.0014) groups than in controls. The Goldberg stage was significantly higher in the HbSC group than in the HbSS group (2.21 vs. 1.22, p = 0.0062). OCTA provides useful information on macular microvasculature and structural alterations associated with SCDR. Ischemic abnormalities are more predominant in the DCP in case of SCDR and no difference was found between genotypes of patients visually asymptomatic.
Highlights
Ischemic microangiopathy was clearly identified in sickle cell disease (SCD) using fluorescein angiography
In order to assess the reproducibility of the foveal avascular zone (FAZ) measurement, we evaluated the intra-class correlation coefficient (ICC) over two measures of each image
We report foveal microvasculature changes observed in SCD using swept-source optical coherence tomography angiography (OCTA)
Summary
Ischemic microangiopathy was clearly identified in sickle cell disease (SCD) using fluorescein angiography. Macular vessel occlusion due to SCD has been documented using fluorescein angiography (FA), and histopathological e xamination[4,5,6] These changes include an enlargement of the foveal avascular zone (FAZ), perifoveal capillary non-perfusion, nerve fiber layer infarcts, microaneurysm-like dot and hairpin-shaped venular loops at the posterior p ole[4,5,6,7]. These microvascular macular changes have been recently reported with optical coherence tomography angiography (OCTA), Scientific Reports | (2020) 10:11795. The distribution of macular lesions between both genotypes has not been assessed yet
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