Tomography-based definition of keratoconus for Down syndrome patients

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RESEARCH

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Tomography-based definition of keratoconus for Down syndrome patients Soheila Asgari1, Shiva Mehravaran2, Mohammadreza Aghamirsalim3 and Hassan Hashemi1*

Abstract Background: To assess the diagnostic ability of Pentacam HR (Oculus Optikgeräte, GmbH, Wetzlar, Germany) tomographic indices in discriminating keratoconus (KC) and KC suspect (KCS) in 10- to 30-year-old patients with Down syndrome (DS). Methods: In this study, DS patients were enrolled through special needs schools, the National Down Syndrome Society, and relevant non-profit organizations. Diagnoses were made independently by two experienced specialists. Forty Pentacam indices related to corneal thickness, volume, density, keratometry, power, shape, aberration, and elevation were extracted. For each index, the accuracy for KC and KCS diagnosis was evaluated using discriminant analysis and the area under receiver operating characteristic curve (AUROC). From each enrolled case, data from only one eye was entered in the analyses. Results: Analyses were performed on data from 25 KC, 46 KCS, and 154 non-ectatic DS eyes. The best discriminants for KC were anterior higher order aberrations (HOA) (cutoff > 0.643, AUROC = 0.879), posterior vertical coma (cutoff > 0.0702 μm, AUROC = 0.875), anterior vertical coma (cutoff > 0.4124 μm, AUROC = 0.868), and total HOA (cutoff > 0.608, AUROC = 0.867). The difference between AUROCs were not statistically significant (all P > 0.05). For KCS, the best discriminants were minimum corneal thickness (cutoff ≤ 480.0 μm, AUROC = 0.775), corneal volume (cutoff ≤ 55.3 μm, AUROC = 0.727) and Belin Ambrosio display-total deviation (BAD-D) (cutoff > 2.23, AUROC = 0.718) with no significant difference between AUROCs (all P > 0.05). Conclusions: In this sample of DS patients, best KC discriminators were HOA and coma which showed good diagnostic ability. For KCS, best predictors were minimum corneal thickness, corneal volume, and BAD-D with relatively good diagnostic ability. Defining a new set of KC diagnostic criteria for DS patients is suggested. Keywords: Down syndrome, Keratoconus, Diagnostic criteria, Tomography, Discriminant analysis

Background Keratoconus (KC) is a degenerative inflammatory corneal pathology [1] that is usually associated with progressive thinning and steepening of the cornea [2]. Advanced techniques and 3-D assessment of the cornea have significantly enhanced our ability to detect KC and even KC suspects (KCS). Corneal topography is currently an important diagnostic tool, and its combination with pachymetry and * Correspondence: [email protected] 1 Noor Ophthalmology Research Center, Noor Eye Hospital, No. 96 Esfandiar Blvd., Vali’asr Ave., Tehran, Iran Full list of author information is available at the end of the article

assessment of corneal stiffness has improved diagnostic accuracy [3–5]. However, the diagnosis of KCS remains complicated and uncertain. There are several KC-related indices and classifications, especially for identifying cases in the early stages, but it can be a c