The corneoscleral shape in Marfan syndrome

Purpose: To investigate the corneoscleral shape in Marfan syndrome (MFS) patients.

Methods: Thirty eyes of 15 participants with molecularly proven MFS were included in this prospective, cross-sectional study. Optical biometry, Scheimpflug imaging, and corneoscleral topography (Eye Surface Profiler) were performed in all patients. Topographic data were compared to data from controls (25 emmetropes and 17 myopes). The raw three-dimensional anterior height data from MFS eyes and control eyes were exported for further analysis. Custom-made software was used to demarcate the limbal radius and to calculate the sagittal height in different concentric annuli centred at the corneal apex, placed in a pupil plane, for the central cornea (0-4 mm radius), peripheral cornea (4-6 mm radius) and sclera (6-8 mm radius) and the corneoscleral asymmetry.

Results: Marfan syndrome (MFS) eyes had significantly lower values of mean sagittal height compared to non-MFS eyes in all three annuli (central cornea, corneal periphery and sclera (independent t-test, p < 0.01 except for the inferior area of the scleral radius: p > 0.05). The sclera was significantly more asymmetric in MFS eyes compared to myopes (independent t-test, p < 0.01 for both eyes), but similar to emmetropes (independent t-test, p = 0.17 and p = 0.93 for right and left eyes, respectively). In MFS eyes, scleral asymmetry was not found to be correlated with axial length (Pearson correlation coefficient, r < 0.30, p > 0.05).

Conclusion: The peripheral cornea and sclera of Marfan syndrome patients have a significantly different shape compared to healthy controls.