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Biometric and structural ocular manifestations of Marfan syndrome
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...
Autores principales: | , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Public Library of Science
2017
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5607136/ https://www.ncbi.nlm.nih.gov/pubmed/28931008 http://dx.doi.org/10.1371/journal.pone.0183370 |
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author | Gehle, Petra Goergen, Barbara Pilger, Daniel Ruokonen, Peter Robinson, Peter N. Salchow, Daniel J. |
author_facet | Gehle, Petra Goergen, Barbara Pilger, Daniel Ruokonen, Peter Robinson, Peter N. Salchow, Daniel J. |
author_sort | Gehle, Petra |
collection | PubMed |
description | 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. |
format | Online Article Text |
id | pubmed-5607136 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2017 |
publisher | Public Library of Science |
record_format | MEDLINE/PubMed |
spelling | pubmed-56071362017-10-09 Biometric and structural ocular manifestations of Marfan syndrome Gehle, Petra Goergen, Barbara Pilger, Daniel Ruokonen, Peter Robinson, Peter N. Salchow, Daniel J. PLoS One Research Article 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. Public Library of Science 2017-09-20 /pmc/articles/PMC5607136/ /pubmed/28931008 http://dx.doi.org/10.1371/journal.pone.0183370 Text en © 2017 Gehle et al http://creativecommons.org/licenses/by/4.0/ This is an open access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/) , which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. |
spellingShingle | Research Article Gehle, Petra Goergen, Barbara Pilger, Daniel Ruokonen, Peter Robinson, Peter N. Salchow, Daniel J. Biometric and structural ocular manifestations of Marfan syndrome |
title | 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_short | Biometric and structural ocular manifestations of Marfan syndrome |
title_sort | biometric and structural ocular manifestations of marfan syndrome |
topic | Research Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5607136/ https://www.ncbi.nlm.nih.gov/pubmed/28931008 http://dx.doi.org/10.1371/journal.pone.0183370 |
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