Abstract
(1) Background: This paper investigates the incidence and risk factors of retinal manifestations in patients with Marfan syndrome (MFS) in a Chinese cohort. (2) Methods: This is a population-based cross-sectional study. In total, 344 eyes (172 MFS participants) were enrolled, each of whom underwent a detailed ocular examination. B-scan ultrasonography, ultra-wide-angle fundus images and optical coherence tomography images were conducted to assess posterior staphyloma, types of retinal damages and maculopathy. (3) Results: MFS patients have a high proportion (32.5%) of maculopathy, among which atrophy is the most common type (27.6%). Compared with participants without maculopathy, participants with maculopathy had a longer axial length (AL), higher incidence of posterior staphyloma, macular split and retinal detachment (RD) (p < 0.001, p < 0.001, p < 0.001 and p = 0.001). Moreover, the stage of RD has a significant correlation with longer AL and shallower anterior chamber depth (ACD) (p = 0.001 and p = 0.034, respectively). (4) Conclusions: A higher incidence and earlier onset of fundus lesions were found in MFS patients. Yearly systematic examination is recommended for MFS children with fundus manifestation until the cardiovascular and skeletal development is complete.