Airway obstruction in severe syndromic craniosynostosis.

Airway obstruction is frequent in patients with severe syndromic craniosynostosis, and must be treated for successful, complete care. The purpose of this study was to evaluate the incidence and the management of airway obstruction in patients with severe craniosynostosis. All patients with complex syndromic craniosynostosis at Chang Gung Craniofacial Center were evaluated retrospectively. Criteria for inclusion of patients were the presence of adequate follow-up and documentation. A total of 40 patients were included, of whom 13 had Apert's syndrome and 27 had Crouzon's disease. Clinical symptoms and signs related to airway obstruction and its treatment were evaluated carefully, and were verified further by telephone inquiry. The status of the airway was categorized into one of three groups: no obstruction; mild obstruction, for which positioning and medical treatment were needed; and severe obstruction, for which surgical intervention was needed. The results showed that 24 patients (60%) did not have airway obstruction, 11 patients (27.5%) had mild obstruction, and 5 patients (12.5%) had severe obstruction. There was no significant difference in the distribution of airway status between patients with Apert's syndrome and Crouzon's disease. Causes for the 5 patients with severe obstruction were midface hypoplasia, lower airway obstruction, tonsillar and adenoid hypertrophy, and choanal atresia. Tracheostomy was performed to control airway in 3 patients, and 1 patient died. Midface anterior distraction helped to decannulate 1 patient. In conclusion, airway obstruction was observed in 40% of patients with severe craniosynostotic syndromes. Most of them could be managed successfully with conservative or medical treatment, and surgical intervention should be considered on the basis of each patient's individual condition.