Comparative data on SD-OCT for the retinal nerve fiber layer and retinal macular thickness in a large cohort with Marfan syndrome

Purpose: To report the distribution of macular and optic nerve topography in the eyes of individuals with Marfan syndrome aged 8-56 years using spectral domain optical coherence tomography (SD-OCT). Methods: Thirty-three patients with Marfan syndrome underwent a full eye examination including slitlamp biomicroscopy, indirect ophthalmoscopy, and axial length measurement; and SD-OCT measurements of the retinal nerve fiber layer (RNFL) and macular thickness. Results: For patients between the ages of 8 and 12 years, the average RNFL thickness is 98 +/- 9 mu m, the vertical cup to disc (C: D) ratio is 0.50 +/- 0.10, the central subfield thickness (CST) is 274 +/- 38 mu m, and the macular volume is 10.3 +/- 0.6 mm(3). For patients between the ages of 13 and 17 years, the average RNFL is 86 +/- 16 mu m, the vertical C: D ratio is 0.35 +/- 0.20, the CST is 259 +/- 15 mu m, and the macular volume is 10.1 +/- 0.5 mm(3). For patients 18 years or older, the average RNFL is 89 +/- 12 mu m, the vertical C: D ratio is 0.46 +/- 0.18, the CST is 262 +/- 20 mu m, and the macular volume is 10.2 +/- 0.4 mm(3). When the average RNFL data are compared to a normative, age-adjusted database, 6 of 33 (18%) were thinner than the 5% limit. Conclusion: This study reports the distribution of SD-OCT data for patients with Marfan syndrome. Compared to a normative database, 18% of eyes with Marfan syndrome had RNFL thickness < 5% of the population.