title: Comparative data on SD-OCT for the retinal nerve fiber layer and retinal macular thickness in a large cohort with Marfan syndrome.
abstract: 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 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.
  
  
  
  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.
authors.affiliation: a Department of Ophthalmology , Northwestern University , Chicago , Illinois , USA.|b Division of Ophthalmology , Ann & Robert H. Lurie Children's Hospital of Chicago , Chicago , Illinois , USA.
authors.forename: WanWan
authors.initials: W
authors.lastname: Xu
authors.affiliation: a Department of Ophthalmology , Northwestern University , Chicago , Illinois , USA.|b Division of Ophthalmology , Ann & Robert H. Lurie Children's Hospital of Chicago , Chicago , Illinois , USA.
authors.forename: Sudhi P
authors.initials: SP
authors.lastname: Kurup
authors.affiliation: a Department of Ophthalmology , Northwestern University , Chicago , Illinois , USA.
authors.forename: Amani A
authors.initials: AA
authors.lastname: Fawzi
authors.affiliation: c Carl Zeiss Meditec, Inc ., Dublin , California , USA.
authors.forename: Mary K
authors.initials: MK
authors.lastname: Durbin
authors.affiliation: d Department of Ophthalmology and Visual Sciences , University of Illinois at Chicago , Chicago , Illinois , USA.
authors.forename: Irene H
authors.initials: IH
authors.lastname: Maumenee
authors.affiliation: a Department of Ophthalmology , Northwestern University , Chicago , Illinois , USA.|b Division of Ophthalmology , Ann & Robert H. Lurie Children's Hospital of Chicago , Chicago , Illinois , USA.
authors.forename: Marilyn B
authors.initials: MB
authors.lastname: Mets
mesh_terms: D000293:Adolescent; D000328:Adult; D058452:Axial Length, Eye; D002648:Child; D005260:Female; D006801:Humans; D008266:Macula Lutea / Q000473:pathology*; D008297:Male; D008382:Marfan Syndrome / Q000175:diagnosis*; D008875:Middle Aged; D009412:Nerve Fibers / Q000473:pathology*; D012165:Retinal Ganglion Cells / Q000473:pathology*; D066167:Slit Lamp; D041623:Tomography, Optical Coherence
keywords: Connective tissue disease; Marfan syndrome; OCT