Biometry Characteristics in Adults and Children With Marfan Syndrome: From the Marfan Eye Consortium of Chicago. Am J Ophthalmol 2017 May;177:144-149
Date
03/05/2017Pubmed ID
28257833Pubmed Central ID
PMC5648325DOI
10.1016/j.ajo.2017.02.022Scopus ID
2-s2.0-85015385280 (requires institutional sign-in at Scopus site) 37 CitationsAbstract
PURPOSE: 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).
DESIGN: 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 (Kmed of 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.
Author List
Kinori M, Wehrli S, Kassem IS, Azar NF, Maumenee IH, Mets MBMESH terms used to index this publication - Major topics in bold
AdolescentAxial Length, Eye
Biometry
Chicago
Child
Child, Preschool
Congresses as Topic
Cornea
Corneal Diseases
Corneal Topography
Cross-Sectional Studies
Female
Humans
Incidence
Male
Marfan Syndrome
Refraction, Ocular
Visual Acuity