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, Vol. 256, No. 6, 06.2018, p. 1159-1163.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
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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 -