Airway obstruction in the Crouzon syndrome: case report and review of the literature

Abstract

Craniofacial dysostosis (Crouzon syndrome) is a syndrome characterized by premature closing of calvarial and cranial base sutures as well as those of the orbit and maxillary complex. Orofacial manifestations of this disease include maxillary hypoplasia, external nasal deformity, and prognathism. Airway distress is a well described feature of this syndrome, and both upper and lower airway obstruction may be present in the Crouzon syndrome. Obstructions in the upper airway have been reported secondary to septal deviation, midnasal and choanal abnormalities, and nasopharyngeal narrowing. This study presents a child with the Crouzon syndrome who required tracheotomy during the first 2 years of life for airway obstruction caused in the newborn period (first 6 months) by midnasal stenosis and later (6 months to 2 years) compounded by soft palate obstruction at the level of the oro/hypopharynx. A review of the English language literature revealed one similar patient presentation but no comprehehsive review of this subject. Previously reported airway anomalies are reviewed and airway management is discussed in patients with Crouzon syndrome.