Tracheal Ultrasound for Diagnosis of Tracheal Cartilaginous Sleeve in Patients with Syndromic Craniosynostosis

Objective. The aim of this study was to assess the utility of ultrasound (US) imaging for diagnosis of abnormal tracheal morphology, such as tracheal cartilaginous sleeves (TCS), in patients with syndromic craniosynostosis (SC). Study Design. Age-matched cohort study. Setting. Tertiary pediatric hospital. Methods. Two age-matched cohorts were identified: patients with SC and known TCS based upon airway endoscopy and normal controls without tracheal pathology. Enrolled patients underwent awake US of the neck which were randomized and reviewed by blinded pediatric radiologists and rated on presence or absence of normal tracheal cartilage morphology and visualization or nonvisualization of a tracheostomy tube. Fisher's exact test was used to assess pooled data. Fleiss' Kappa (kappa) was calculated to assess inter-rater reliability. Results. Ten patients were included in each cohort. Control patients were gender and age-matched to TCS patients with a mean difference of 3.7 months (+/- 3.9 months). Across all raters, cartilage type was correctly identified in 93% (95% confidence interval [CI]: 84%-98%) and tracheostomy visualization in 97% (95% CI: 89%-99%). The sensitivity and specificity for detection of abnormal cartilage pathology was 87% and 100%, respectively. Inter-rater reliability for cartilage assessment was kappa = 0.88 (95% CI: 0.67-1.00, P < .05) and 0.83 (95% CI: 0.58-1.00, P < .05) for tracheostomy presence. Conclusion. This study demonstrated that tracheal US is a feasible, accurate screening tool for TCS, and can be successfully performed non-sedated in patients up to 18 years of age, both with and without tracheostomy tubes in place.