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PLoS One. 2017 Sep 20;12(9):e0183370. doi: 10.1371/journal.pone.0183370. eCollection 2017.

Biometric and structural ocular manifestations of Marfan syndrome.

Author information

1
Department of Cardiology, Charité -University Medicine Berlin, Augustenburger Platz 1, Berlin, Germany.
2
Department of Ophthalmology, Charité -University Medicine Berlin, Augustenburger Platz 1, Berlin, Germany.
3
Private Practice Zehlendorf, Berlin, Germany.
4
The Jackson Laboratory for Genomic Medicine, Farmington, United States of America.

Abstract

BACKGROUND:

To study biometric and structural ocular manifestations of Marfan syndrome (MFS).

METHODS:

Observational, retrospective, comparative cohort study in a tertiary referral center on 285 MFS patients and 267 controls. Structural and biometric ocular characteristic were compared.

RESULTS:

MFS eyes were longer (axial length 24.25 ± 1.74 mm versus 23.89 ± 1.31 mm, p < 0.001) and had a flatter cornea than control eyes (mean keratometry 41.78 ± 1.80 diopters (D) versus 43.05 ± 1.51 D, p < 0.001). Corneal astigmatism was greater and the central cornea was thinner in MFS eyes (530.14 ± 41.31 μm versus 547.02 ± 39.18 μm, p < 0.001). MFS eyes were more myopic than control eyes (spherical equivalent -2.16 ± 3.75 D versus -1.17 ± 2.58 D, p < 0.001). Visual acuity was reduced (0.13 ± 0.25 logMAR versus 0.05 ± 0.18 logMAR, p < 0.001) and intraocular pressure was lower in MFS eyes (14.6 ± 3.4 mmHg versus 15.1 ± 3.2 mmHg, p = 0.01). Iris transillumination defects (ITD) were significantly more common in MFS eyes (odds ratio for MFS in the presence of ITD, 3.7). Ectopia lentis (EL) was only present in MFS eyes (33.4%). History of retinal detachment was significantly more common in MFS eyes. Glaucoma was equally common in both groups.

CONCLUSIONS:

ITD and EL are most characteristic findings in MFS. ITD and corneal curvature should be studied as diagnostic criteria for MFS. Visual acuity is reduced in MFS. MFS patients need regular eye exams to identify serious ocular complications.

PMID:
28931008
PMCID:
PMC5607136
DOI:
10.1371/journal.pone.0183370
[Indexed for MEDLINE]
Free PMC Article

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