The Management of Obstructive Sleep Apnea in Syndromic Craniosynostosis

Abstract

Syndromic craniosynostosis comprises a group of rare conditions often associated with fibroblast growth factor receptor gene mutations. Premature fusion of cranial sutures leads to facial and cranial dysmorphism, which is associated with upper airway compromise and a high incidence of obstructive sleep apnea. The authors performed a literature search to determine the evidence base for interventions used to treat obstructive sleep apnea in this patient group.A search strategy identified 503 papers of which 23 were included. There was evidence for craniofacial surgery, adenotonsillectomy and palatal surgery, and the use of continuous positive airway pressure and nasopharyngeal airways. The level of evidence was low in all studies, but this is likely to be a manifestation of a rare, heterogenous disease in a pediatric population. The largest volume of evidence supports craniofacial surgery; however, patients undergoing this surgery are commonly older, and there is evidence for alternative treatment strategies in younger patients.