Marfan Syndrome: Biomechanics of the cornea as new noninvasive method for assessment of disease progression

Abstract

Purpose : To evaluate corneal air-puff deformation responses as predictor of disease status and progression in Marfan Syndrome (MFS).

Methods : This prospective observational clinical study monitored right eyes of 44 individuals (19 female, 25 male; mean age 42±12) diagnosed with MFS. The revised Ghent criteria, with particular attention being paid to the degree of aortic root dilatation (Z-Score) as correlates of disease status, were correlated with ocular biometry, corneal topography, tomography (Pentacam [Oculus]; Galilei G6 [Ziemer]) and dynamic Scheimpflug-based biomechanical analyses (CorvisST [CST; Oculus]). These findings were compared to 30 control subjects. A t-test or Wilcoxon matched-pairs test were used to test for statistically significant differences between these groups. Our study was approved by the local Ethics Committee Hamburg.

Results : Comparing control- and MFS-eyes, the corneal biomechanical parameters A1 length, A2 length, radius of the inward-bended cornea, and deflection length at the highest concavity revealed statistically significant differences (P<0.05). In addition, the comparison between low- and high-risk patients demonstrated consistently increasing differences with increasing statistical significance between mild and those with advance MFS stages.

Conclusions : In vivo noninvasive biomechanical analyses (CST) offer a new reliable method to describe and distinct
features of corneal deformation responses between low- and high-risk patients with MFS. In the future corneal air-puff deformation responses might therefore be used in early identification of critically ill patients at high risk of disease progression as an adjunct to existing diagnostic tests.

This is an abstract that was submitted for the 2017 ARVO Annual Meeting, held in Baltimore, MD, May 7-11, 2017.