Dido Scheibenberger1,2, Andreas Frings3, Johannes Steinberg1,4,5, Helke Schüler6, Vasyl Druchkiv1,5, Toam Katz1,5, Yskert von Kodolitsch6, Stephan Linke1,4,5. 1. Department of Ophthalmology, University Hospital Hamburg-Eppendorf, Hamburg, Germany. 2. University of Hamburg, Medical School, University Hospital Hamburg-Eppendorf, Hamburg, Germany. 3. Department of Ophthalmology, Heinrich-Heine-University Düsseldorf, Moorenstraße 5, 40225, Düsseldorf, Germany. andi.frings@gmail.com. 4. zentrumsehstärke, Martinistraße 64, 20251, Hamburg, Germany. 5. Clínica Baviera, Valencia, Spain. 6. German Aortic Centre Hamburg at the University Heart Centre, University Hospital Hamburg-Eppendorf, Hamburg, Germany.
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.
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.
Authors: Bart L Loeys; Harry C Dietz; Alan C Braverman; Bert L Callewaert; Julie De Backer; Richard B Devereux; Yvonne Hilhorst-Hofstee; Guillaume Jondeau; Laurence Faivre; Dianna M Milewicz; Reed E Pyeritz; Paul D Sponseller; Paul Wordsworth; Anne M De Paepe Journal: J Med Genet Date: 2010-07 Impact factor: 6.318
Authors: J Bradley Randleman; Daniel G Dawson; Hans E Grossniklaus; Bernard E McCarey; Henry F Edelhauser Journal: J Refract Surg Date: 2008-01 Impact factor: 3.573
Authors: Yskert von Kodolitsch; Meike Rybczynski; Marina Vogler; Thomas S Mir; Helke Schüler; Kerstin Kutsche; Georg Rosenberger; Christian Detter; Alexander M Bernhardt; Axel Larena-Avellaneda; Tilo Kölbel; E Sebastian Debus; Malte Schroeder; Stephan J Linke; Bettina Fuisting; Barbara Napp; Anna Lena Kammal; Klaus Püschel; Peter Bannas; Boris A Hoffmann; Nele Gessler; Eva Vahle-Hinz; Bärbel Kahl-Nieke; Götz Thomalla; Christina Weiler-Normann; Gunda Ohm; Stefan Neumann; Dieter Benninghoven; Stefan Blankenberg; Reed E Pyeritz Journal: J Multidiscip Healthc Date: 2016-11-03
Authors: Ruchi Shah; Cynthia Amador; Kati Tormanen; Sean Ghiam; Mehrnoosh Saghizadeh; Vaithi Arumugaswami; Ashok Kumar; Andrei A Kramerov; Alexander V Ljubimov Journal: Exp Eye Res Date: 2021-01-21 Impact factor: 3.467