Ocular manifestation in Marfan syndrome: corneal biomechanical properties relate to increased systemic score points
- 41 Downloads
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.
KeywordsMarfan syndrome Corvis ST Corneal biomechanics Ghent criteria
Compliance with ethical standards
Conflict of interest
The authors declare that they have no conflict of interest.
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 was obtained from all individual participants included in the study.
- 10.Hjortdal JO (1998) On the biomechanical properties of the cornea with particular reference to refractive surgery. Acta Ophthalmol Scand Suppl 225:1–23Google Scholar
- 12.Randleman JB, Dawson DG, Grossniklaus HE, McCarey BE, Edelhauser HF (2008) Depth-dependent cohesive tensile strength in human donor corneas: implications for refractive surgery. J Refract Surg 24:85–89Google Scholar
- 13.Dawson DG, Grossniklaus HE, McCarey BE, Edelhauser HF (2008) Biomechanical and wound healing characteristics of corneas after excimer laser keratorefractive surgery: is there a difference between advanced surface ablation and sub-Bowman’s keratomileusis? J Refract Surg 24:90–96Google Scholar