June 2013
Volume 54, Issue 15
Free
ARVO Annual Meeting Abstract  |   June 2013
Macular and Optic Nerve Optical Coherence Tomography in Marfan Patients: from the Marfan Eye Consortium of Chicago
Author Affiliations & Notes
  • WanWan Xu
    Ophthalmology, Northwestern University, Chicago, IL
  • Sudhi Kurup
    Ophthalmology, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL
  • Amani Fawzi
    Ophthalmology, Northwestern University, Chicago, IL
  • Mary Durbin
    Carl Zeiss Meditec, Inc, Dublin, CA
  • Irene Maumenee
    Ophthalmology and Visual Sciences, University of Illinois at Chicago, Chicago, IL
  • Marilyn Mets
    Ophthalmology, Northwestern University, Chicago, IL
    Ophthalmology, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL
  • Footnotes
    Commercial Relationships WanWan Xu, None; Sudhi Kurup, None; Amani Fawzi, None; Mary Durbin, Carl Zeiss Meditec, Inc. (E); Irene Maumenee, None; Marilyn Mets, None
  • Footnotes
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Investigative Ophthalmology & Visual Science June 2013, Vol.54, 5851. doi:https://doi.org/
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      WanWan Xu, Sudhi Kurup, Amani Fawzi, Mary Durbin, Irene Maumenee, Marilyn Mets; Macular and Optic Nerve Optical Coherence Tomography in Marfan Patients: from the Marfan Eye Consortium of Chicago. Invest. Ophthalmol. Vis. Sci. 2013;54(15):5851. doi: https://doi.org/.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract

Purpose: To report the distribution of macular and optic nerve topography in the eyes of individuals with Marfan syndrome aged 2 to 56 years using optical coherence tomography (OCT) measurements.

Methods: 72 patients were seen on 8/2/2012 at the Ann & Robert H. Lurie Children's Hospital Ophthalmology Clinic as part of the Annual National Marfan Foundation Conference. 60 patients consented to the study and underwent a full eye examination including evaluation with slit-lamp, indirect ophthalmoscopy, axial length measurement using the IOL Master (Carl Zeiss Meditec, Dublin, CA), and OCT measurements using the Macular Cube 512x128 and Optic Disc Cube 200x200 of the Cirrus HD-OCT (Carl Zeiss Meditec, Dublin, CA). OCT data included right eyes that had signal strength ≥ 6 out of 10 without artifacts. If the right eye was excluded, the left eye was used.

Results: 17/60 patients were excluded for having scans of insufficient signal strength. For patients between the ages of 7 to 12, the average retinal nerve fiber layer (RNFL) thickness is 90 ± 15 um, vertical cup to disc (C:D) ratio is 0.46 ± 0.17, central subfield thickness (CST) is 269 ± 43 um, and macular volume is 10.3 ± 0.55 mm3. For patients between the ages of 13 to 17, average RNFL is 83 ± 16 um, vertical C:D ratio is 0.34 ± 0.18, CST is 241 ± 63 um, and macular volume is 9.7 ± 1.6 mm3. For patients 18 years or older, average RNFL is 87.5 ± 13 um, vertical C:D ratio is 0.47 ± 0.18, CST is 358 ± 23 um, and macular volume is 10.1 ± 0.5 mm3. When the average RNFL data is compared to a normative database adjusted for age, overall, 28 of 43 eyes (65%) were greater than the 5% limit; 5 of 43 (12%) were between the 1% and 5% limits; and 10 out of 43 eyes (23%) were thinner than the 1% limit. For vertical C:D ratio, 36 of 43 eyes (83%) were > 5% limit; 4 of 43 eyes (9%) were between 1-5%; and 3 of 43 eyes (7%) were <1%. For CST, 39 of 43 eyes (90%) were >5% limit; 2 of 43 eyes (5%) were between 1-5%; and 2 of 43 eyes (5%) were <1%. For macular volume, 39 out of 43 (91%) were >5% limit, while 4 out of 43 eyes (9%) were <1%.

Conclusions: This study reports distribution of OCT data for patients with Marfan syndrome. Compared to normative database, 23% of eyes with Marfan syndrome had RNFL <1 % of population, suggestive of RNFL loss, with overall less significant macular changes. We are currently comparing this data to clinical exam findings.

Keywords: 550 imaging/image analysis: clinical  
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