Biometry Characteristics in Adults and Children With Marfan Syndrome

From the Marfan Eye Consortium of Chicago

Michael Kinori, Sarah Wehrli, Iris S. Kassem, Nathalie F. Azar, Irene H. Maumenee, Marilyn B. Mets

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish (US)
Pages (from-to)144-149
Number of pages6
JournalAmerican Journal of Ophthalmology
Volume177
DOIs
StatePublished - May 1 2017

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Biometry
Marfan Syndrome
Ectopia Lentis
Cornea
Astigmatism
Lenses
Refractive Errors
Anterior Chamber
Cross-Sectional Studies

ASJC Scopus subject areas

  • Ophthalmology

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Biometry Characteristics in Adults and Children With Marfan Syndrome : From the Marfan Eye Consortium of Chicago. / Kinori, Michael; Wehrli, Sarah; Kassem, Iris S.; Azar, Nathalie F.; Maumenee, Irene H.; Mets, Marilyn B.

In: American Journal of Ophthalmology, Vol. 177, 01.05.2017, p. 144-149.

Research output: Contribution to journalArticle

Kinori, Michael ; Wehrli, Sarah ; Kassem, Iris S. ; Azar, Nathalie F. ; Maumenee, Irene H. ; Mets, Marilyn B. / Biometry Characteristics in Adults and Children With Marfan Syndrome : From the Marfan Eye Consortium of Chicago. In: American Journal of Ophthalmology. 2017 ; Vol. 177. pp. 144-149.
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abstract = "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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