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

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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® [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.

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Author notes

  1. Dido Scheibenberger and Andreas Frings contributed equally to this work

Authors and Affiliations

  1. Department of Ophthalmology, University Hospital Hamburg-Eppendorf, Hamburg, Germany

    Dido Scheibenberger, Johannes Steinberg, Vasyl Druchkiv, Toam Katz & Stephan Linke

  2. University of Hamburg, Medical School, University Hospital Hamburg-Eppendorf, Hamburg, Germany

    Dido Scheibenberger

  3. Department of Ophthalmology, Heinrich-Heine-University Düsseldorf, Moorenstraße 5, 40225, Düsseldorf, Germany

    Andreas Frings

  4. zentrumsehstärke, Martinistraße 64, 20251, Hamburg, Germany

    Johannes Steinberg & Stephan Linke

  5. Clínica Baviera, Valencia, Spain

    Johannes Steinberg, Vasyl Druchkiv, Toam Katz & Stephan Linke

  6. German Aortic Centre Hamburg at the University Heart Centre, University Hospital Hamburg-Eppendorf, Hamburg, Germany

    Helke Schüler & Yskert von Kodolitsch

Authors
  1. Dido Scheibenberger
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  2. Andreas Frings
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  3. Johannes Steinberg
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  4. Helke Schüler
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  5. Vasyl Druchkiv
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  6. Toam Katz
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  7. Yskert von Kodolitsch
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  8. Stephan Linke
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Corresponding author

Correspondence to Andreas Frings.

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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.

Additional information

The present work was performed in fulfillment of the requirements for obtaining the degree “Dr. Med.” (Dido Scheibenberger)

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Scheibenberger, D., Frings, A., Steinberg, J. et al. Ocular manifestation in Marfan syndrome: corneal biomechanical properties relate to increased systemic score points. Graefes Arch Clin Exp Ophthalmol 256, 1159–1163 (2018). https://doi.org/10.1007/s00417-018-3946-4

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  • DOI: https://doi.org/10.1007/s00417-018-3946-4

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