Upper Airway Obstruction Trends in Craniofacial Syndromes: A Comparative Study.

Abstract

Craniosynostosis is a congenital disorder marked by the early fusion of one or more cranial sutures, potentially leading to functional impairments, including airway obstruction. This is particularly common in syndromic craniosynostosis, such as in Apert, Crouzon, Pfeiffer, Saethre-Chotzen, and Muenke syndromes, where midface hypoplasia and retrusion are often present. Airway obstruction, including obstructive sleep apnea, affects up to 68% of individuals with syndromic craniosynostosis. Understanding the severity and variability of airway compromise across these syndromes is critical for guiding interventions, such as Le Fort advancements. This study retrospectively reviewed preoperative computed tomography scans from patients diagnosed with Apert, Crouzon, Pfeiffer, Saethre-Chotzen, Muenke, and craniofrontonasal dysplasia syndromes. Key airway metrics, including nasopharyngeal and oropharyngeal anteroposterior diameter, volume, and minimal cross-sectional area, were measured and compared across syndromes. The analysis also explored age-related changes in airway parameters to assess how obstruction evolves over time. Significant variability in airway obstruction was observed across the syndromes. Pfeiffer syndrome exhibited the most severe obstruction, with a 50% to 70% reduction in airway dimensions. Apert syndrome showed milder obstruction, with a 30% to 40% reduction in younger individuals, improving to 15% to 20% with age. Saethre-Chotzen and Muenke syndromes demonstrated the mildest obstruction, with reductions of 10% to 25%. These findings highlight the diverse severity of airway obstruction across craniosynostosis syndromes. Pfeiffer syndrome shows the most significant early childhood obstruction, while Saethre-Chotzen and Muenke syndromes experience milder airway compromise. Surgical interventions, like Le Fort advancements, are essential in managing severe cases, emphasizing the importance of individualized surgical and airway management strategies.