Ocular manifestation in Marfan syndrome: corneal biomechanical properties relate to increased systemic score points

  • Dido Scheibenberger
  • Andreas Frings
  • Johannes Steinberg
  • Helke Schüler
  • Vasyl Druchkiv
  • Toam Katz
  • Yskert von Kodolitsch
  • Stephan Linke



To evaluate corneal deformation to an air puff as a new noninvasive tool to document disease status in Marfan syndrome (MFS)


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).


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).


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.


Marfan syndrome Corvis ST Corneal biomechanics Ghent criteria 


Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed consent

Informed consent was obtained from all individual participants included in the study.


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  • Dido Scheibenberger
    • 1
    • 2
  • Andreas Frings
    • 3
  • Johannes Steinberg
    • 1
    • 4
    • 5
  • Helke Schüler
    • 6
  • Vasyl Druchkiv
    • 1
    • 5
  • Toam Katz
    • 1
    • 5
  • Yskert von Kodolitsch
    • 6
  • Stephan Linke
    • 1
    • 4
    • 5
  1. 1.Department of OphthalmologyUniversity Hospital Hamburg-EppendorfHamburgGermany
  2. 2.University of Hamburg, Medical School, University Hospital Hamburg-EppendorfHamburgGermany
  3. 3.Department of OphthalmologyHeinrich-Heine-University DüsseldorfDüsseldorfGermany
  4. 4.zentrumsehstärkeHamburgGermany
  5. 5.Clínica BavieraValenciaSpain
  6. 6.German Aortic Centre Hamburg at the University Heart CentreUniversity Hospital Hamburg-EppendorfHamburgGermany

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