Adenotonsillectomy for obstructive sleep apnea in children with syndromic craniosynostosis.

BACKGROUND: The role of adenotonsillectomy in the treatment of obstructive sleep apnea in patients with craniosynostosis is not well established.

METHODS: A retrospective review was conducted of all children with syndromic craniosynostosis (Apert, Crouzon, and Pfeiffer syndromes) seen at Boston Children's Hospital from January 2001 through April 2011. The primary outcome measure was the apnea/hypopnea index before and after adenotonsillectomy.

RESULTS: There were 47 patients (66 percent Apert syndrome, 19 percent Pfeiffer syndrome, and 15 percent Crouzon syndrome) less than 21 years of age (mean, 1.2 ± 3.3 years at first visit) who were followed for a mean of 14.6 ± 8.6 years. Of children with at least one polysomnogram, 83 percent had obstructive sleep apnea (42 percent severe, 19 percent moderate, 22 percent mild). Adenotonsillectomy was performed in 62 percent of patients. Preoperative and postoperative polysomnography was performed in 45 percent of children undergoing adenotonsillectomy. Following adenotonsillectomy, the mean apnea/hypopnea index was not significantly different, and obstructive sleep apnea persisted in 11 of 13 children.

CONCLUSIONS: Obstructive sleep apnea is commonly present in children with syndromic craniosynostosis and is a complex disease with airway obstruction at several different levels. Adenotonsillectomy often does not improve the severity of obstructive sleep apnea in this population.