This study aims at describing the macular vascular features of the eyes of pediatric patients with the homozygous form of sickle cell disease (SCD) using optical coherence tomography angiography.
This is a retrospective case series of eyes of pediatric patients with SCD, race- and age-matched to those of healthy individuals. Using optical coherence tomography angiography images of superficial capillary plexus and deep capillary plexus, the nonflow area, vascular density, and foveal avascular zone were measured. The remodeled microvascular foveal architectures obtained on optical coherence tomography angiography and fluorescein angiography were compared.
A total of 19 eyes of patients with SCD and 18 eyes of race-matched healthy controls were examined. The best-corrected visual acuity ranged from 75 to 85 letters in all cases. In the SCD series, 8 eyes displayed signs of retinopathy on funduscopy. The foveal avascular zone was significantly enlarged, the foveal vascular density was 8% lower, and there were more perifoveal vascular alterations in the eyes of patients with SCD compared to those of controls.
The foveal vascular density was significantly reduced and area of the foveal avascular zone was significantly increased in the eyes of patients with SCD. Consistent architectural changes in the perifoveal capillaries were also observed in the pediatric patients with SCD.
This study describes sickle cell maculopathy in children using optical coherence tomography angiography. The foveal vascular density was reduced and the area of the foveal avascular zone increased in pediatric patients with sickle cell disease. Changes in the perifoveal capillaries were better elucidated by optical coherence tomography angiography than by fluorescence angiography.
Department of Ophthalmology, University of Lausanne, Jules Gonin Eye Hospital, Fondation Asile des Aveugles, Lausanne, Switzerland.
Reprint requests: Aude Ambresin, MD, Jules Gonin Eye Hospital, Avenue de France 15, Case postale 133, CH-1000, Lausanne 7, Switzerland; e-mail: firstname.lastname@example.org
Presented at the results of this study have been presented in part at the Euretina Congress, Copenhagen, September 11, 2016.
None of the authors has any financial/conflicting interests to disclose.