Foveal avascular zone changes in children with sickle cell disease

Purpose To study the morphological changes in the foveal avascular zone (FAZ) in children diagnosed with sickle cell disease (SCD) via the optical coherence tomography angiography (OCTA). Patients and methods This was a prospective case-control study that was done in a tertiary hospital. A total of 15 children with SCD (confirmed with electrophoresis) and 15 matched healthy children were included. Ophthalmological assessment was done. RTVue XR Avanti was employed to obtain 6x6 macular OCTA scans. Foveal parameters including FAZ area (mm2), perimeter (mm) (PERIM), acircularity index (AI), and foveal density were analyzed. Pediatric assessment including the disease variant, sickling crisis, and current treatment was done. Results A total of 15 eyes of 15 children with SCD and 15 eyes of healthy children were included. Six eyes showed stage 1 retinopathy. Children with SCD had wider FAZ area (P=0.001) with larger PERIM (P=0.00) and higher AI (P=0.030) in comparison with the control children. No significant changes in the FAZ parameters between patients with SCD with stage 1 retinopathy and patients without retinopathy were found. Conclusion Children with SCD have a wide FAZ area with large PERIM and high AI in comparison with normal controls. OCTA macular changes might be an early predictor of sickle cell retinopathy. Further follow-up studies are recommended to understand the effect of early macular changes on the future development of retinopathy.