Quantitative analysis of choriocapillaris flow deficits and choroidal thickness in children with Marfan syndrome

Purpose: To evaluate the characteristics and associated factors of choroidal thickness (ChT) and choriocapillaris flow deficit percentage (CC FD%) in children with Marfan syndrome (MFS).

Methods: This is a cross-sectional study. The ChT of the subfoveal area and other areas 0.5, 1.5, and 2.5 mm away from the fovea were assessed. The CC FD% of quadrant and circular regions with diameters of 0.5, 1.5 and 2.5 mm were assessed with 3×3 mm macular choriocapillaris images. Best-corrected visual acuity (BCVA) and cardiac function factor such as Z score were measured to analyse the associations with ChT and CC FD%.

Results: 51 MFS children and 50 healthy controls were enrolled in this study. Compared with the healthy controls, the ChT in the MFS group was thinner in the subfoveal area, temporal 0.5, 1.5 and 2.5 mm (all p<0.001). The CC FD% was higher in circle 0.5, 1.5 and 2.5 mm (all p<0.001). Multivariate regression analysis showed that CC FD% in the circle 2.5 mm when the Z score ≥2 was associated with BCVA (β=9.08 (95% CI 3.96 to 14.20); p=0.005) and Z score (β=4.19 (95% CI 1.28 to 12.00); p=0.012).

Conclusions: Thinner ChT and a higher CC FD% were observed in children with MFS, and an increased CC FD% in circle 2.5 mm was significantly associated with worse BCVA and cardiac function. These findings may help identify future visual impairment and early cardiac events in MFS children.