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

ABSTRACT

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 slit-lamp 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 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 mm³. 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 mm³. 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 mm³. 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.

KEYWORDS:

• Connective tissue disease
• Marfan syndrome
• OCT

Acknowledgments

Marfan Eye Exam Consortium: Janice Lasky Zeid, MD; Bahram Rahmani, MD, MPH; Hawke Yoon, MD; Mahsa Sohrab, MD; Sarah Wehrli, MD; Elizabeth Chiang, MD, PhD; Dina Johnson, Heath Barto, CO; Aaliyah Hamidullah, MS; Nathalie Azar, MD; Iris Kassem, MD; PhD, Alice Lyon; MD; Joshua Fernandes, MD; Safa Rahmani, MD; Evica Simjanoski, CRA; Jessica Dougall; Ernie Zuno and Hantamalala Ralay Ranaivo, PhD. Statistical help was provided by Karen Rychlik.

Declaration of interest

Mary K. Durbin is an employee of Carl Zeiss Meditec, Inc. The remaining authors report no conflicts of interest. The authors alone are responsible for the content and writing of this article.