Nasal Airway Measurements Predict Early Nasal Intervention in Fibroblast Growth Factor Receptor-Related Craniosynostosis

INTRODUCTION: Fibroblast growth factor receptor (FGFR)-related craniosynostosis syndromes are frequently associated with midfacial hypoplasia and multilevel nasal airway stenosis, often resulting in early respiratory compromise. While CT-based nasal measurements are well established for isolated congenital nasal pyriform aperture stenosis, their role in predicting airway intervention in FGFR-related syndromes remains poorly defined. This study aimed to evaluate whether CT-derived nasal airway widths can predict the need for early nasal intervention and prolonged nasopharyngeal airway (NPA) support in this population. METHODS: A retrospective observational study was conducted at a tertiary pediatric craniofacial center. Children with genetically confirmed FGFR-related craniosynostosis who had diagnostic craniofacial CT imaging and documented airway outcomes were included. Linear nasal airway widths were measured at the pyriform aperture (PA), mid-nasal 50%, and mid-nasal 75% levels. Associations with nasal dilatation, NPA insertion, and duration of NPA support were analyzed using nonparametric testing, correlation analysis, and receiver operating characteristic (ROC) curves. Inter- and intrarater reliability were assessed using intraclass correlation coefficients. RESULTS: Eighteen patients were included, of whom 11 underwent CT imaging before 2 months of age. Narrower nasal airway measurements at all levels were significantly associated with nasal dilatation and NPA insertion. Pyriform aperture width correlated strongly with duration of NPA dependence in early-imaged infants (ρ=-0.80, P=0.002). The ROC analysis demonstrated good discriminatory performance for predicting early nasal intervention, with mid-nasal 75% measurements providing the strongest overall prediction. Measurement reliability was high, with intraclass correlation coefficients >0.94. CONCLUSION: Computed tomography (CT)-based nasal airway measurements, particularly at the pyriform aperture and mid-nasal levels, are reliable predictors of early nasal intervention and prolonged airway support in FGFR-related craniosynostosis. These findings support the use of objective imaging thresholds to inform early airway management and family counseling.