ASSESSMENT OF MACULAR VASCULATURE OF CHILDREN WITH SICKLE CELL DISEASE COMPARED TO THAT OF HEALTHY CONTROLS USING OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY

Abstract

Purpose: 

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.

Methods: 

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.

Results: 

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.

Conclusion: 

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.

In Brief

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.