Sickle Cell Maculopathy: microstructural analysis using OCTA
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To identify genetic, systemic, and biological factors associated with the occurrence of sickle cell maculopathy a study was conducted. To evaluate microvascular macular alterations using optical coherence tomography angiography in sickle cell disease.

One hundred fifty-one eyes of 78 adult sickle cell disease patients (43 HbSS, 30 HbSC, 4 S/? + and 1 HbSLepore) and 40 eyes of 20 healthy controls underwent spectral-domain optical coherence tomography and optical coherence tomography angiography using Spectralis. Researchers analyzed the occurrence of sickle cell maculopathy, the foveal avascular zone area, and the severity of macular ischemia and studied their relationships with genetic, systemic, and biological parameters using multivariate logistic regression analysis.

Maculopathy occurred in 66 eyes (44%), and more frequently in HbSS patients. Multivariate analysis identified HbSS genotype and lower prothrombin ratio (PR) as independently associated with sickle cell maculopathy. Proliferative sickle cell retinopathy was also associated with sickle cell maculopathy. foveal avascular zone enlargement was associated with higher lactate dehydrogenase levels. Macular ischemia was more severe in patients with lower hemoglobin levels and lower. No flow areas were identified with optical coherence tomography angiography even in eyes with no macular thinning (36 eyes, 42%) and appeared more frequently in the temporal superior subfield (36%).

HbSS genotype, abnormal coagulation, and hemolysis increase the risk of sickle cell maculopathy. Optical coherence tomography angiography provides valuable criteria to identify potential risk factors of sickle cell maculopathy. Optical coherence tomography angiography also improves the detection of early microvascular changes before the onset of macular thinning.

Source:https://www.ajo.com/article/S0002-9394(20)30653-X/fulltext?rss=yes
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