Secondary Logo

Journal Logo

Institutional members access full text with Ovid®

The Management of Obstructive Sleep Apnea in Syndromic Craniosynostosis

Nash, Robert MRCS*; Possamai, Victoria FRCS (ORL-HNS); Manjaly, Joe MRCS*; Wyatt, Michelle FRCS (ORL-HNS)*

doi: 10.1097/SCS.0000000000002097
Original Articles

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.

Supplemental Digital Content is available in the text

*Department of Paediatric Otolaryngology, Great Ormond Street Hospital

Department of Paediatric Otolaryngology, Evelina Children's Hospital, St. Thomas's Hospital, London, UK.

Address correspondence and reprint requests to Robert Nash, MRCS, Great Ormond Street Hospital For Children NHS Trust, London, UK; E-mail:

Received 2 February, 2015

Accepted 9 July, 2015

The authors report no conflicts of interest.

Supplemental digital content is available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal's Website (

© 2015 by Mutaz B. Habal, MD.