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  • Pediatric Ophth/Strabismus, Retina/Vitreous

    This prospective, observational case series evaluated the severity of macular vascular changes in children with sickle cell disease.

    Study design

    Researchers used OCT and OCT angiography to assess retinal thinning and retinal vessel density in 17 patients with a HbS variant, 17 with HbSS and 14 age- and race-matched controls.


    Although there was no difference in visual acuity and low prevalence of proliferative retinopathy, a higher percentage of the HbSS arm showed pathologic areas of retinal thinning associated with superficial and deep capillary plexus flow loss compared with the HbS arm (55% vs. 26%; P=0.03). Patients with HbSS had decreased vessel density in the deep capillary plexus compared with controls (48%; P=0.008).


    There were only a few patients with each of the HbS variants (11 HbSC, 4 HbS alpha thalassemia and 2 HbS beta plus thalassemia). Since this cohort showed a higher incidence of retinal thinning and involvement of both the superficial and deep capillary plexus, future studies should include more patients with each of these diagnoses.

    Clinical significance

    Diagnosis of sickle cell retinopathy has previously been reliant on clinical retinal examinations and fluorescein angiography, both of which can be difficult in the pediatric population. OCT angiography is a noninvasive imaging modality that is useful for screening for vascular retinopathy in susceptible pediatric patients.