Biometric and structural ocular manifestations of Marfan syndrome.

To study biometric and structural ocular manifestations of Marfan syndrome (MFS).Observational, retrospective, comparative cohort study in a tertiary referral center on 285 MFS patients and 267 controls. Structural and biometric ocular characteristic were compared.MFS eyes were longer (axial length...

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Main Authors: Petra Gehle, Barbara Goergen, Daniel Pilger, Peter Ruokonen, Peter N Robinson, Daniel J Salchow
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2017-01-01
Series:PLoS ONE
Online Access:http://europepmc.org/articles/PMC5607136?pdf=render
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spelling doaj-8f92de66fadf43c3a10716404c7e55dc2020-11-25T02:08:04ZengPublic Library of Science (PLoS)PLoS ONE1932-62032017-01-01129e018337010.1371/journal.pone.0183370Biometric and structural ocular manifestations of Marfan syndrome.Petra GehleBarbara GoergenDaniel PilgerPeter RuokonenPeter N RobinsonDaniel J SalchowTo study biometric and structural ocular manifestations of Marfan syndrome (MFS).Observational, retrospective, comparative cohort study in a tertiary referral center on 285 MFS patients and 267 controls. Structural and biometric ocular characteristic were compared.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.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.http://europepmc.org/articles/PMC5607136?pdf=render
collection DOAJ
language English
format Article
sources DOAJ
author Petra Gehle
Barbara Goergen
Daniel Pilger
Peter Ruokonen
Peter N Robinson
Daniel J Salchow
spellingShingle Petra Gehle
Barbara Goergen
Daniel Pilger
Peter Ruokonen
Peter N Robinson
Daniel J Salchow
Biometric and structural ocular manifestations of Marfan syndrome.
PLoS ONE
author_facet Petra Gehle
Barbara Goergen
Daniel Pilger
Peter Ruokonen
Peter N Robinson
Daniel J Salchow
author_sort Petra Gehle
title Biometric and structural ocular manifestations of Marfan syndrome.
title_short Biometric and structural ocular manifestations of Marfan syndrome.
title_full Biometric and structural ocular manifestations of Marfan syndrome.
title_fullStr Biometric and structural ocular manifestations of Marfan syndrome.
title_full_unstemmed Biometric and structural ocular manifestations of Marfan syndrome.
title_sort biometric and structural ocular manifestations of marfan syndrome.
publisher Public Library of Science (PLoS)
series PLoS ONE
issn 1932-6203
publishDate 2017-01-01
description To study biometric and structural ocular manifestations of Marfan syndrome (MFS).Observational, retrospective, comparative cohort study in a tertiary referral center on 285 MFS patients and 267 controls. Structural and biometric ocular characteristic were compared.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.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.
url http://europepmc.org/articles/PMC5607136?pdf=render
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