title: Corneal Deformation Response and Ocular Geometry: A Noninvasive Diagnostic Strategy in Marfan Syndrome.
abstract: OBJECTIVE
  To evaluate corneal air-puff deformation responses and ocular geometry as predictors of Marfan syndrome.
  
  
  METHODS
  Prospective observational clinical study.
  
  
  METHODS
  Sixteen investigator-derived, 4 standard Ocular Response Analyzer (ORA), and geometric variables from corneal tomography and optical biometry using Oculus Pentacam and IOL Master were assessed for discriminative value in Marfan syndrome, measuring right eyes of 24 control and 13 Marfan syndrome subjects. Area under the receiver operating characteristic (AUROC) curve was assessed in univariate and multivariate analyses.
  
  
  RESULTS
  Six investigator-derived ORA variables successfully discriminated Marfan syndrome. The best lone disease predictor was Concavity Min (Marfan syndrome 47.5 ± 20, control 69 ± 14, P = .003; AUROC = 0.80). Corneal hysteresis (CH) and corneal resistance factor (CRF) were decreased (Marfan syndrome CH 9.45 ± 1.62, control CH 11.24 ± 1.21, P = .01; Marfan syndrome CRF 9.77 ± 1.65, control CRF 11.03 ± 1.72, P = .01) and corneas were flatter in Marfan syndrome (Marfan syndrome Kmean 41.25 ± 2.09 diopter, control Kmean 42.70 ± 1.81 diopter, P = .046). No significant differences were observed in central corneal thickness, axial eye length, or intraocular pressure. A multivariate regression model incorporating corneal curvature and hysteresis loop area (HLA) provided the best predictive value for Marfan syndrome (AUROC = 0.85).
  
  
  CONCLUSIONS
  This study describes novel biodynamic features of corneal deformation responses in Marfan syndrome, including increased deformation, decreased bending resistance, and decreased energy dissipation capacity. A predictive model incorporating HLA and corneal curvature shows greatest potential for noninvasive clinical diagnosis of Marfan syndrome.
authors.affiliation: Case Western Reserve University School of Medicine, Cleveland, Ohio; Center for Genetic Eye Diseases, Cole Eye Institute, Cleveland Clinic Foundation, Cleveland, Ohio.
authors.forename: Lauren C
authors.initials: LC
authors.lastname: Beene
authors.affiliation: Center for Genetic Eye Diseases, Cole Eye Institute, Cleveland Clinic Foundation, Cleveland, Ohio.
authors.forename: Elias I
authors.initials: EI
authors.lastname: Traboulsi
authors.affiliation: Ocular Biomechanics & Imaging Laboratory, Cole Eye Institute, Cleveland Clinic Foundation, Cleveland, Ohio.
authors.forename: Ibrahim
authors.initials: I
authors.lastname: Seven
authors.affiliation: Ocular Biomechanics & Imaging Laboratory, Cole Eye Institute, Cleveland Clinic Foundation, Cleveland, Ohio; Department of Biomedical Engineering, Case Western Reserve University, Cleveland, Ohio.
authors.forename: Matthew R
authors.initials: MR
authors.lastname: Ford
authors.affiliation: Ocular Biomechanics & Imaging Laboratory, Cole Eye Institute, Cleveland Clinic Foundation, Cleveland, Ohio.
authors.forename: Abhijit
authors.initials: A
authors.lastname: Sinha Roy
authors.affiliation: Quantitative Health Sciences, Cleveland Clinic Lerner Research Institute, Cleveland, Ohio.
authors.forename: Robert S
authors.initials: RS
authors.lastname: Butler
authors.affiliation: Ocular Biomechanics & Imaging Laboratory, Cole Eye Institute, Cleveland Clinic Foundation, Cleveland, Ohio; Department of Biomedical Engineering, Case Western Reserve University, Cleveland, Ohio; Department of Biomedical Engineering, Cleveland Clinic Lerner Research Institute, Cleveland, Ohio. Electronic address: bjdupps@sbcglobal.net.
authors.forename: William J
authors.initials: WJ
authors.lastname: Dupps
mesh_terms: D000328:Adult; D019540:Area Under Curve; D001696:Biomechanical Phenomena / Q000502:physiology; D001699:Biometry; D003315:Cornea / Q000503:physiopathology*; D063171:Corneal Pachymetry; D004548:Elasticity / Q000502:physiology*; D054459:Elasticity Imaging Techniques; D005260:Female; D006801:Humans; D007429:Intraocular Pressure / Q000502:physiology; D008297:Male; D008382:Marfan Syndrome / Q000175:diagnosis* / Q000503:physiopathology; D008875:Middle Aged; D011446:Prospective Studies; D012372:ROC Curve