Scheimpflug Tomographic Indices for Classifying Normal, Down Syndrome and Clinical Keratoconus in Pediatric Patients

TL;DR

This study finds that specific corneal measurements can help diagnose keratoconus in children with Down Syndrome, but different cutoff values are needed for accurate diagnosis.

Contribution

The study identifies optimal Pentacam indices and cutoff values for diagnosing keratoconus in pediatric Down Syndrome patients.

Findings

Indices like average pachymetric progression and anterior elevation showed high accuracy (AUC > 0.94) for keratoconus in Down Syndrome patients.

Thresholds for these indices differ significantly from non-Down Syndrome patients.

No indices accurately distinguished forme fruste keratoconus in Down Syndrome patients.

Abstract

The study aimed to evaluate the precision of different Pentacam indices in diagnosing keratoconus (KC) in pediatric patients with and without Down Syndrome (DS) and determine suitable cutoff values. This prospective multicenter cross-sectional study evaluated 216 eyes of 131 patients aged 6–18 years (mean age 12.5 ± 3.2 years) using Pentacam. Patients were categorized into four groups: KC, forme fruste keratoconus (FK), DS, and control, excluding DS patients with topographic KC. Receiver operating characteristic curves were generated to determine the optimal cutoff points and compare the accuracy in identifying KC and FK in patients with and without DS. In DS patients, corneal morphology resembled KC features. The most effective indices for distinguishing KC in DS patients were the average pachymetric progression index (AUC = 0.961), higher-order aberration of the anterior cornea (AUC = 0.953), anterior elevation (AUC = 0.946), posterior elevation (AUC = 0.947), index of vertical asymmetry (AUC = 0.943), and Belin/Ambrosio enhanced ectasia total derivation value (AUC = 0.941). None of the indices showed good accuracy for distinguishing FK in DS patients. The thresholds of these indices differed significantly from non-DS patients. The results highlighted the need for DS-specific cutoff values to avoid false-positive or false-negative diagnoses in this population.

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