A structure-function correlation between vessel density on OCTA and macular sensitivity on microperimetry in sickle cell retinopathy in children, adolescents, and young adults

• OCTA and microperimetry can link structure-function in retinal disease. • This study is the first to use both tools in pediatric sickle cell disease (pSCD). • Vessel density on OCTA correlated with microperimetry deficits in pSCD. • OCTA and microperimetry may be sensitive, noninvasive endpoints in pSCD trials. To identify associations between microvascular changes and functional macular sensitivity in children, adolescents, and young adults with sickle cell disease (SCD). This analysis included patients (8–22 years) with SCD with optical coherence tomography-angiography (OCTA) and microperimetry testing between March 2023 and December 2024. Superficial (SCP) and deep (DCP) capillary plexi scans were divided into four quadrants: supero-temporal (ST), supero-nasal (SN), infero-temporal (IT), and infero-nasal (IN). Microperimetry examinations were analyzed using the 4–2 visual field method and a 68-point, 10-degree grid using the Macular Integrity Assessment (MAIA). Spearman correlation and mixed effects regression models assessed associations between vessel density (VD, measured in mm/mm²) and microperimetry sensitivity. Thirty-two eyes were included. Most were Black, non-Hispanic patients (71.9 %) with hemoglobin SS disease (62.5 %). The median age and best-corrected visual acuity (BCVA) at testing were 14.6 years (IQR, 12.2–17.5) and 0 logMAR (IQR, 0–0.1) (Snellen 20/20), respectively. Half of eyes (50 %) had some degree of sickle cell retinopathy. There were few cases of severe retinopathy (9.4 %). Median age was 14.54 (IQR, 9.34–13.61). Median VD in the SCP and DCP were lowest in the infero-temporal quadrant (27.64 and 23.80, respectively). After adjusting for covariates, microperimetry sensitivity remained positively associated with VD in the supero-temporal region, both for the SCP (β=0.44, p = 0.010) and the DCP (β =0.36, p = 0.04). In children, adolescents, and young adults with sickle cell disease, loss of vessel density in the supero-temporal region of the SCP and DCP was associated with decreased sensitivity on microperimetry. This may support a structure-function correlation, compelling further investigation of the impact of impaired retinal perfusion on subclinical vision loss.