Features of the Foveal Avascular Zone in Children and Young Adults with Sickle Cell Disease
DOI:
https://doi.org/10.48560/rspo.28267Keywords:
Anemia, Sickle Cell, Fovea Centralis, Fluorescein Angiography, Retinal Diseases, Tomography, Optical CoherenceAbstract
Introduction: Sickle cell disease is a pathology characterized by the occurrence of microvascular oclusions in the retina, which can lead to macular ischemia that can result in changes in the characteristics of the foveal avascular zone (FAZ). The aim of this study is to evaluate the diameter and area of the FAZ in children and young adults with sickle cell disease using optical coherence tomography angiography (OCTA).Material and Methods: The study included a review of the clinical files and OCT-A of children and young adults with sickle cell disease aged < 25 years. The control group included healthy children and young adults aged < 25 years. OCT-A scans of the sample and control group (Spectralis OCTA® Heidelberg Engineering) were obtained. The diameter of the FAZ was obtained through manual measurement and the area through a computational algorithm developed for its automatic determination. The normal distribution of the data was analyzed and statistical tests were applied to evaluate if the diameter and area of the FAZ differed in a statistically significant way in the two groups.
Results: Our study included a total of 77 eyes from 43 patients: 47 from 26 patients with sickle cell disease 18 male and 8 female, with a mean age of 16.1 ± 5.2 years (6 - 24 years of age). The control group included 30 eyes of 17 subjects, 7 males and 10 females, with a mean age of 14.0 ± 5.5 years (5 - 23 years of age). The mean diameter of the FAZ in the sickle cell group of patients was 745.5 ± 118,3 μm, while in the control group it was 648.5 ± 82.8 μm (p=0.00019). The FAZ area of the sickle-cell group was 0.4 ± 0.1 μm2, while in the control group it was 0.3 ± 0.1 μm2 (p=0.01).
Conclusion: both the diameter and the area of the FAZ in the group of children and young adults with sickle cell disease are statistically significantly higher than in the control group. These changes often precede fundoscopic findings and therefore may be considered early biomarkers of the disease.
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