Optic disk evaluation in children with sickle cell disease

Abstract

Introduction: Sickle cell disease (SCD) is the most prevalent hemoglobinopathy in the world. SCD is associated with multi-systemic vascular occlusive events that may affect retinal microvasculature.

            The aim of this study was to make a structural and vascular analyses of the optic disk of children with SCD and try to correlate the findings with clinical data.

Methods: Prospective study including 18 eyes of 10 children with average age of 11 ±3,3 years. Optical Coherence Tomography Angiography (OCT-A) - Zeiss Cirrus HD-OCT 5000 Model with Angioplex – were performed centered in optic disk. Morphologic values and vascular density were analyzed.

Results: The best corrected visual acuity was 0,9 bilaterally. The mean cycloplegic spherical equivalent was + 0,5 in the right eye and + 0,70 in the left eye. Six eyes presented sclerotic jaundice (33%).

            Eight eyes presented with non-proliferative retinopathy (44%), none with proliferative retinopathy.

            The mean cup-to-disk ratiohigher in eyes with retinopathy than in eyes with no retinopathy (0,56 ± 0,16 vs0,41 ± 0,11; p=0,003).

            The mean central superficial and deep vascular density was inferior in eyes with retinopathy than in eyes without – 44 ± 1,08% vs50,64 ± 1,29 %; p=0,001 and 35,33 ± 1,62% vs 41,67 ± 1,02%; p=0,001, respectively.

Conclusion: This is the first study reporting data about optic disk evaluation by OCT-A in children with sickle cell disease in Portugal.

            The study concludes that the cup-to-disk ratio was higher in eyes with retinopathy than in eyes without. The vascular density was inferior in eyes with retinopathy in both superficial and deep plexus.

            More studies are needed to validate the real diagnostic and prognostic role of these exams in sickle cell disease.