Airway obstruction in severe syndromic craniosynostosis

Airway obstruction is frequent in patients with severe syndromic craniosyno stosis, and must be treated for successful, complete care. The purpose of t his study was to evaluate the incidence and the management of airway obstru ction in patients with severe craniosynostosis. All patients with complex s yndromic craniosynostosis at Chang Gung Craniofacial Center were evaluated retrospectively. Criteria for inclusion of patients were the presence of ad equate follow-up and documentation. A total of 40 patients were included, o f whom 13 had Apert's syndrome and 27 had Crouzon's disease. Clinical sympt oms and signs related to airway obstruction and its treatment were evaluate d carefully, and were verified further by telephone inquiry. The status of the airway was categorized into one of three groups: no obstruction; mild o bstruction, for which positioning and medical treatment were needed; and se vere obstruction, for which surgical intervention was needed. The results s howed 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 b etween patients with Apert's syndrome and Crouzon's disease. Causes for the 5 patients with severe obstruction were midface hypoplasia, lower airway o bstruction, tonsillar and adenoid hypertrophy, and choanal atresia. Tracheo stomy was performed to control airway in 3 patients, and 1 patient died. Mi dface anterior distraction helped to decannulate 1 patient. In conclusion, airway obstruction was observed in 40% of patients with severe craniosynost otic syndromes. Most of them could be managed successfully with conservativ e or medical treatment, and surgical intervention should be considered on t he basis of each patient's individual condition.