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. 2017 May:177:144-149.
doi: 10.1016/j.ajo.2017.02.022. Epub 2017 Feb 28.

Biometry Characteristics in Adults and Children With Marfan Syndrome: From the Marfan Eye Consortium of Chicago

Michael Kinori  1 , Sarah Wehrli  1 , Iris S Kassem  2 , Nathalie F Azar  3 , Irene H Maumenee  3 , Marilyn B Mets  4
Affiliations

Biometry Characteristics in Adults and Children With Marfan Syndrome: From the Marfan Eye Consortium of Chicago

Michael Kinori et al. Am J Ophthalmol. 2017 May.

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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Figures

FIGURE 1
FIGURE 1
Categorical analyses of corneal parameters between adults and children with Marfan syndrome. Percentage of patients with flat corneas (Kmed, top) central corneal thickness (CCT, middle) and corneal astigmatism (Cast, bottom) was not statistically significant.
FIGURE 2
FIGURE 2
A negative correlation between the axial length and the corneal curvature (Kmed) in patients with Marfan syndrome (r = −0.33, P < .001).
See this image and copyright information in PMC

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