ABSTRACT
PURPOSE: To investigate the anterior scleral thickness (AST) in patients with Marfan syndrome (MFS). METHODS: A prospective, cross-sectional study was conducted at the Department of Ophthalmology, Ghent University Hospital, Ghent, including patients with a genetically confirmed clinical diagnosis of MFS and age-, gender- and axial length-matched controls. Subjects with known corneal, conjunctival or scleral pathology and a history of ocular surgery, including pars plana vitrectomy, recent contact lens use or high-grade astigmatism were excluded. Subjects underwent non-cycloplegic autorefraction, Scheimpflug-based corneal tomography, axial length measurement and spectral-domain optical coherence tomography (OCT). AST was manually measured at 1 mm (AST1), 2 mm (AST2) and 3 mm (AST3) from the scleral spur, temporally and nasally. RESULTS: A total of 56 subjects (28 subjects in the MFS group and 28 matched subjects in the control group) were included in this study. In patients with MFS, AST was significantly reduced compared to matched controls, both overall and at every analysed measuring point in the nasal and temporal areas (p < 0.001). Central corneal thickness (CCT) and mean keratometry (Kmean) values were significantly lower in patients with MFS (p < 0.05). A positive correlation was found between nasal AST and CCT in patients with MFS. No correlation was found between AST and Kmean or between AST and axial length. In patients with MFS with ectopia lentis, compared to those without, temporal AST3 was significantly lower (p < 0.05). AST was significantly lower in patients with MFS harbouring a variant predicted to cause haploinsufficiency compared to those with a variant expected to lead to a dominant negative effect for both nasal and temporal measurements. CONCLUSION: Based on anterior segment OCT measurements, AST of patients with MFS is significantly lower compared to matched controls.
ABSTRACT
PURPOSE: To assess the correlation of the scleral shape and corneal tomographic parameters in keratoconus. METHODS: Twenty eyes of 15 keratoconus patients with no previous specialty lens wear or ocular surgery were included in this study. Corneal imaging was obtained with the Pentacam HR and three-dimensional (3D) corneoscleral maps were acquired using the Eye Surface Profiler, ESP. Sagittal height was calculated at the central corneal level (annulus of 0-4 mm radius), peripheral cornea (annulus 4-6 mm radius) and sclera (annulus 6-8 mm radius) using ESP maps and Pentacam HR (exclusively for the central cornea). The flattest and steepest regions of each annulus and the circumferential scleral asymmetry were calculated based on custom-made software. The Pearson correlation coefficient (r) was used to evaluate the correlation between corneal parameters as measured by Pentacam HR and scleral asymmetry. RESULTS: Anterior corneal parameters, such as flattest and steepest keratometry, were found to be correlated with scleral asymmetry in keratoconus (all r>0.5, p < 0.05). In contrast, anterior astigmatism showed poor correlation with the level of scleral irregularity (r=-0.11; p = 0.32). Other disease-specific parameters pertaining to the posterior corneal curvature and corneal thickness were not correlated with scleral asymmetry. The steepest regions of the central cornea, peripheral cornea, and sclera tended to share a common angle (r = 0.92; p < 0.001 for central cornea compared to sclera). CONCLUSION: Anterior corneal parameters measured by corneal imaging are associated with the level of scleral asymmetry and the orientation of the steepest area of the sclera in eyes with keratoconus.
