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

• Published in: Ophthalmic genetics 2017-01, Vol.38 (1), p.34-38
• Main Authors: Xu, WanWan, Kurup, Sudhi P, Fawzi, Amani A, Durbin, Mary K, Maumenee, Irene H, Mets, Marilyn B
• Format: Article
• Language: English
• Subjects: Adolescent; Adult; Axial Length, Eye; Child; Female; Humans; Macula Lutea - pathology; Male; Marfan Syndrome - diagnosis; Middle Aged; Nerve Fibers - pathology; Retinal Ganglion Cells - pathology; Slit Lamp; Tomography, Optical Coherence
• ISSN: 1381-6810; 1744-5094
• DOI: 10.1080/13816810.2016.1275017

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). Thirty-three patients with Marfan syndrome underwent a full eye examination including slit-lamp biomicroscopy, indirect ophthalmoscopy, and axial length measurement; and SD-OCT measurements of the retinal nerve fiber layer (RNFL) and macular thickness. For patients between the ages of 8 and 12 years, the average RNFL thickness is 98 ± 9 μm, the vertical cup to disc (C:D) ratio is 0.50 ± 0.10, the central subfield thickness (CST) is 274 ± 38 μm, and the macular volume is 10.3 ± 0.6 mm . For patients between the ages of 13 and 17 years, the average RNFL is 86 ± 16 μm, the vertical C:D ratio is 0.35 ± 0.20, the CST is 259 ± 15 μm, and the macular volume is 10.1 ± 0.5 mm . For patients 18 years or older, the average RNFL is 89 ± 12 μm, the vertical C:D ratio is 0.46 ± 0.18, the CST is 262 ± 20 μm, and the macular volume is 10.2 ± 0.4 mm . When the average RNFL data are compared to a normative, age-adjusted database, 6 of 33 (18%) were thinner than the 5% limit. 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.