Abstract

BackgroundTo 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).MethodsIn 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.ResultsAnalyses 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).ConclusionsIn 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.

Highlights

  • 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)

  • This difference, in addition to age and genetic predisposition, can be due to diagnostic criteria. Given these uncertainties in the diagnosis, the term KC-compatible has been used to describe findings, and it has been shown that 71.3% of DS patients may have findings suggestive of KC [11]

  • 250 DS patients were recruited through special needs schools, the National Down Syndrome Society, and relevant non-profit organizations, and 225 of them were enrolled into Down syndrome keratoconus (DS-KC), DSKCS, and DS comparison (DS-C) groups

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Summary

Introduction

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). The reported prevalence of KC in DS ranges from 0% [9] to 21.1% [10] This difference, in addition to age and genetic predisposition, can be due to diagnostic criteria. Given these uncertainties in the diagnosis, the term KC-compatible has been used to describe findings, and it has been shown that 71.3% of DS patients may have findings suggestive of KC [11]. Given the lack of literature on this topic, this study was designed in an attempt to define diagnostic criteria for differentiating KC and KCS from non-ectatic corneas in DS patients

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