Abstract
Purpose :
To date, there is limited literature on detection of microvascular alterations in children with sickle cell disease (SCD) using OCTA. This retrospective, consecutive series compares macular thickness and microvascular changes between SCD children with and without peripheral retinopathy (SCR) as well as between different sickle genotypes.
Methods :
A total of 606 eyes of 303 children with SCD aged 18 years old or less (53.1% males and 46.9% females) who had an ophthalmic exam at Boston Children’s Hospital between January 1998 and August 2022 were included. Primary outcomes included total retinal thickness measured on macular optical coherence tomography (OCT), as well as superficial capillary plexus (SCP) and deep capillary plexus (DCP) vessel density (VD), and foveal avascular zone (FAZ) area measured on 6 x 6 mm OCT angiography (OCTA) scans.
Results :
Two hundred eighty (46%) HbSS, 106 (18%) HbSC, 50 (8%) HbS thalassemia, 154 (25%) sickle trait, and 16 (3%) eyes with unknown genotype were included. OCT and OCTA images were acquired on 104 (17.2%) and 60 (9.9%) eyes at presentation and on 159 (26.2%) and 100 (16.5%) eyes at final visit. Overall, temporal retinal thinning was noted qualitatively in 35.6% and 60.6% of patients with SCD at presentation and final visit, respectively. Of those, 5.4% and 9.5% did not have peripheral SCR at presentation and final visit, respectively. Macular thinning was more common in HbSS eyes compared with HbSC eyes at initial (56.8% vs. 29.7%, P < 0.001) and final (63.5% vs. 30.2%, P < 0.001) visits. On quantitative OCT analysis, HbSS eyes had a lower retinal thickness in most of the quadrants compared with HbSC and HbS thalassemia, P ≤ 0.01. Eyes with peripheral SCR had a larger FAZ at presentation (P = 0.003) and lower DCP VD at final visit in the superior nasal (P = 0.012) and inferior temporal (P = 0.017) macular quadrants compared to eyes without peripheral SCR. SCP and DCP VD and FAZ size did not differ between sickle genotypes.
Conclusions :
This is the largest study of pediatric sickle cell patients imaged with OCTA. Temporal retinal thinning in the macula was observed in eyes without peripheral SCR, suggesting the importance of adding OCT macular thickness as a diagnostic criterion for SCR. Eyes with peripheral SCR had lower macular DCP VD and larger FAZ compared to eyes without peripheral SCR, highlighting that OCTA may help in early identification of SCR.
This abstract was presented at the 2023 ARVO Annual Meeting, held in New Orleans, LA, April 23-27, 2023.