Ocular manifestation in Marfan syndrome

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Ocular manifestation in Marfan syndrome : corneal biomechanical properties relate to increased systemic score points. / Scheibenberger, Dido; Frings, Andreas; Steinberg, Johannes; Schüler, Helke; Druchkiv, Vasyl; Katz, Toam; von Kodolitsch, Yskert; Linke, Stephan.

in: GRAEF ARCH CLIN EXP, Jahrgang 256, Nr. 6, 06.2018, S. 1159-1163.

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@article{04f5d3dffb8a4e5c82a036efcc302c89,
title = "Ocular manifestation in Marfan syndrome: corneal biomechanical properties relate to increased systemic score points",
abstract = "PURPOSE: To evaluate corneal deformation to an air puff as a new noninvasive tool to document disease status in Marfan syndrome (MFS) METHODS: Prospective observational cohort study. We included patients diagnosed with MFS who had their routine cardiovascular follow-up and applied the revised Ghent nosology to define two subgroups according to a high (≥ 7) and a low (< 7 points) systemic score. Dynamic Scheimpflug-based biomechanical analyses (CorvisST{\textregistered} [CST; Oculus GmbH]) were performed. The main outcome measure was the displacement of the corneal apex as given by the parameters highest concavity (HC; in ms), peak distance (PD; in mm), and highest concavity deformation amplitude (DA; mm).RESULTS: Forty-three eyes of 43 individuals (19 female, 24 male; mean age 42.0 ± 12.0 years, range 18-67 years) diagnosed with MFS were included. Applying the Ghent criteria, 21 patients had an advanced systemic score of ≥ 7, and 22 had score points < 7. There were no differences in age or sex between both groups. In contrast, HC was faster (P = 0.004), and PD (P < 0.001) was longer in those individuals with systemic score ≥ 7; maximum DA did not result in a statistically significant difference between the groups (P = 0.250).CONCLUSIONS: In vivo noninvasive biomechanical analyses with CST offer a new, non-invasive method to identify pathologic corneal deformation responses in adults with MFS. In the future, corneal deformation to an air puff could thus assist early identification of patients with high Ghent score as an adjunct to existing diagnostic tests.",
keywords = "Adolescent, Adult, Aged, Cornea, Corneal Diseases, Corneal Topography, Elasticity, Female, Follow-Up Studies, Humans, Intraocular Pressure, Male, Marfan Syndrome, Middle Aged, Prospective Studies, Severity of Illness Index, Young Adult, Journal Article, Observational Study",
author = "Dido Scheibenberger and Andreas Frings and Johannes Steinberg and Helke Sch{\"u}ler and Vasyl Druchkiv and Toam Katz and {von Kodolitsch}, Yskert and Stephan Linke",
year = "2018",
month = jun,
doi = "10.1007/s00417-018-3946-4",
language = "English",
volume = "256",
pages = "1159--1163",
journal = "GRAEF ARCH CLIN EXP",
issn = "0721-832X",
publisher = "Springer",
number = "6",

}

RIS

TY - JOUR

T1 - Ocular manifestation in Marfan syndrome

T2 - corneal biomechanical properties relate to increased systemic score points

AU - Scheibenberger, Dido

AU - Frings, Andreas

AU - Steinberg, Johannes

AU - Schüler, Helke

AU - Druchkiv, Vasyl

AU - Katz, Toam

AU - von Kodolitsch, Yskert

AU - Linke, Stephan

PY - 2018/6

Y1 - 2018/6

N2 - PURPOSE: To evaluate corneal deformation to an air puff as a new noninvasive tool to document disease status in Marfan syndrome (MFS) METHODS: Prospective observational cohort study. We included patients diagnosed with MFS who had their routine cardiovascular follow-up and applied the revised Ghent nosology to define two subgroups according to a high (≥ 7) and a low (< 7 points) systemic score. Dynamic Scheimpflug-based biomechanical analyses (CorvisST® [CST; Oculus GmbH]) were performed. The main outcome measure was the displacement of the corneal apex as given by the parameters highest concavity (HC; in ms), peak distance (PD; in mm), and highest concavity deformation amplitude (DA; mm).RESULTS: Forty-three eyes of 43 individuals (19 female, 24 male; mean age 42.0 ± 12.0 years, range 18-67 years) diagnosed with MFS were included. Applying the Ghent criteria, 21 patients had an advanced systemic score of ≥ 7, and 22 had score points < 7. There were no differences in age or sex between both groups. In contrast, HC was faster (P = 0.004), and PD (P < 0.001) was longer in those individuals with systemic score ≥ 7; maximum DA did not result in a statistically significant difference between the groups (P = 0.250).CONCLUSIONS: In vivo noninvasive biomechanical analyses with CST offer a new, non-invasive method to identify pathologic corneal deformation responses in adults with MFS. In the future, corneal deformation to an air puff could thus assist early identification of patients with high Ghent score as an adjunct to existing diagnostic tests.

AB - PURPOSE: To evaluate corneal deformation to an air puff as a new noninvasive tool to document disease status in Marfan syndrome (MFS) METHODS: Prospective observational cohort study. We included patients diagnosed with MFS who had their routine cardiovascular follow-up and applied the revised Ghent nosology to define two subgroups according to a high (≥ 7) and a low (< 7 points) systemic score. Dynamic Scheimpflug-based biomechanical analyses (CorvisST® [CST; Oculus GmbH]) were performed. The main outcome measure was the displacement of the corneal apex as given by the parameters highest concavity (HC; in ms), peak distance (PD; in mm), and highest concavity deformation amplitude (DA; mm).RESULTS: Forty-three eyes of 43 individuals (19 female, 24 male; mean age 42.0 ± 12.0 years, range 18-67 years) diagnosed with MFS were included. Applying the Ghent criteria, 21 patients had an advanced systemic score of ≥ 7, and 22 had score points < 7. There were no differences in age or sex between both groups. In contrast, HC was faster (P = 0.004), and PD (P < 0.001) was longer in those individuals with systemic score ≥ 7; maximum DA did not result in a statistically significant difference between the groups (P = 0.250).CONCLUSIONS: In vivo noninvasive biomechanical analyses with CST offer a new, non-invasive method to identify pathologic corneal deformation responses in adults with MFS. In the future, corneal deformation to an air puff could thus assist early identification of patients with high Ghent score as an adjunct to existing diagnostic tests.

KW - Adolescent

KW - Adult

KW - Aged

KW - Cornea

KW - Corneal Diseases

KW - Corneal Topography

KW - Elasticity

KW - Female

KW - Follow-Up Studies

KW - Humans

KW - Intraocular Pressure

KW - Male

KW - Marfan Syndrome

KW - Middle Aged

KW - Prospective Studies

KW - Severity of Illness Index

KW - Young Adult

KW - Journal Article

KW - Observational Study

U2 - 10.1007/s00417-018-3946-4

DO - 10.1007/s00417-018-3946-4

M3 - SCORING: Journal article

C2 - 29525839

VL - 256

SP - 1159

EP - 1163

JO - GRAEF ARCH CLIN EXP

JF - GRAEF ARCH CLIN EXP

SN - 0721-832X

IS - 6

ER -