TY - JOUR
T1 - Biometry Characteristics in Adults and Children With Marfan Syndrome
T2 - From the Marfan Eye Consortium of Chicago
AU - Kinori, Michael
AU - Wehrli, Sarah
AU - Kassem, Iris S.
AU - Azar, Nathalie F.
AU - Maumenee, Irene H.
AU - Mets, Marilyn B.
N1 - Funding Information:
Funding/Support: Publication of this article was supported by a grant from Research to Prevent Blindness, Inc, New York, New York (to the University of Illinois at Chicago); a grant from the Children's Surgical Foundation, Chicago, Illinois (to Ann & Robert H. Lurie Children's Hospital of Chicago); and National Eye Institute grants K12 EY021475 and K08EY024645 (to I.S.K.). Financial Disclosures: The following authors have no financial disclosures: Michael Kinori, Sarah Wehrli, Iris S. Kassem, Nathalie F. Azar, Irene H. Maumenee, and Marilyn B. Mets. All authors attest that they meet the current ICMJE criteria for authorship.
Publisher Copyright:
© 2017 Elsevier Inc.
PY - 2017/5/1
Y1 - 2017/5/1
N2 - 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.
AB - 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.
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U2 - 10.1016/j.ajo.2017.02.022
DO - 10.1016/j.ajo.2017.02.022
M3 - Article
C2 - 28257833
AN - SCOPUS:85015385280
SN - 0002-9394
VL - 177
SP - 144
EP - 149
JO - American journal of ophthalmology
JF - American journal of ophthalmology
ER -