title: Biometry Characteristics in Adults and Children With Marfan Syndrome: From the Marfan Eye Consortium of Chicago.
abstract: OBJECTIVE
  To report on the biometric findings of adults and children with Marfan syndrome (MFS) recruited from 2 annual National Marfan Foundation conferences (2012 and 2015).
  
  
  METHODS
  Cross-sectional study.
  
  
  METHODS
  Subjects diagnosed with MFS by Ghent 2 nosology were included for analysis. Subjects were divided into "adults" (≥16 years of age) and "children" (5-15 years of age). Biometric data included values for refractive error, axial length (AL), corneal curvature, anterior chamber depth, lens thickness, and central corneal thickness.
  
  
  RESULTS
  Of the 117 subjects evaluated, 74 (35 adults, 32 children, and 7 children <5 years of age) had a definite diagnosis of MFS and were included in the study. The AL was longer (25.25 ± 0.32 mm vs 24.24 ± 0.33 mm, P = .03) and the lens was thicker (3.94 ± 0.09 mm vs 3.62 ± 0.10 mm, P = .03) in adults. Both groups had flat corneas (average keratometry [Kmed] of 41.59 ± 0.35 diopters [D] in adults vs 40.89 ± 0.36 D in children, P = .17). A negative correlation was found between AL and Kmed(-0.33, P < .001). The corneas of patients with MFS with ectopia lentis (EL) were significantly flatter and with higher degree of corneal astigmatism compared to patients without EL (Kmedof 40.68 ± 0.31 D vs 41.75 ± 0.28 D, P < .01 and corneal astigmatism of 1.68 ± 0.16 D vs 1.13 ± 0.14 D, P = .01).
  
  
  CONCLUSIONS
  Children with established MFS have flat corneas at least to the same degree as adults. Corneas of patients with MFS with EL are flatter and have a higher degree of corneal astigmatism. We strongly suggest that corneal parameters should be measured if MFS is suspected, especially in children that may not yet have developed EL.
authors.affiliation: Northwestern University Feinberg School of Medicine, Chicago, Illinois; Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, Illinois.
authors.forename: Michael
authors.initials: M
authors.lastname: Kinori
authors.affiliation: Northwestern University Feinberg School of Medicine, Chicago, Illinois; Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, Illinois.
authors.forename: Sarah
authors.initials: S
authors.lastname: Wehrli
authors.affiliation: University of Illinois at Chicago, Chicago, Illinois; Medical College of Wisconsin, Milwaukee, Wisconsin.
authors.forename: Iris S
authors.initials: IS
authors.lastname: Kassem
authors.affiliation: University of Illinois at Chicago, Chicago, Illinois.
authors.forename: Nathalie F
authors.initials: NF
authors.lastname: Azar
authors.affiliation: University of Illinois at Chicago, Chicago, Illinois.
authors.forename: Irene H
authors.initials: IH
authors.lastname: Maumenee
authors.affiliation: Northwestern University Feinberg School of Medicine, Chicago, Illinois; Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, Illinois. Electronic address: mmets@luriechildrens.org.
authors.forename: Marilyn B
authors.initials: MB
authors.lastname: Mets
mesh_terms: D000293:Adolescent; D058452:Axial Length, Eye / Q000473:pathology*; D001699:Biometry / Q000379:methods*; D002641:Chicago / Q000453:epidemiology; D002648:Child; D002675:Child, Preschool; D003226:Congresses as Topic; D003315:Cornea / Q000473:pathology*; D003316:Corneal Diseases / Q000175:diagnosis* / Q000453:epidemiology / Q000209:etiology; D019781:Corneal Topography / Q000379:methods*; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D015994:Incidence; D008297:Male; D008382:Marfan Syndrome / Q000150:complications*; D012029:Refraction, Ocular / Q000502:physiology*; D014792:Visual Acuity / Q000502:physiology