International Journal of Oral and Maxillofacial Surgery
Clinical PaperCraniofacial SurgeryThe prevalence of obstructive sleep apnea in symptomatic patients with syndromic craniosynostosis☆
Section snippets
Materials and methods
This was a retrospective review of patients with SCS presenting to a paediatric teaching hospital between 2000 and 2014. Institutional review board approval was obtained for this study. Following approval, the charts of all patients with a diagnosis of FGFR-related SCS (Apert, Crouzon, Crouzon with acanthosis nigricans, or Pfeiffer syndromes) were identified and reviewed. Patients were excluded from the study if they had been followed for less than 1 year or if they had other causes of airway
Results
Sixty-two of 110 patients (56.4%) with FGFR-related SCS were symptomatic and underwent polysomnography for the evaluation of OSA. Of the 62 symptomatic patients, 24 were female and 38 were male; mean age at evaluation was 7.6 ± 6.5 years. The phenotypic diagnoses included Apert (n = 31), Crouzon (n = 17), Crouzon with acanthosis nigricans (n = 3), and Pfeiffer (n = 11) syndromes (Table 1).
The prevalence of OSA in symptomatic patients was 74.2%. Patients with Apert syndrome had the highest prevalence
Discussion
The purpose of this study was to determine the prevalence of OSA in patients with FGFR-related SCS using polysomnography, and to establish whether there are differences in the prevalence and severity of OSA in patients with different phenotypic diagnoses. Only patients who had clinical symptoms of OSA and underwent polysomnography evaluation were included in the analysis. Among symptomatic patients in the study cohort, the prevalence of OSA was 74.2%; OSA was most prevalent in patients with
Funding
Nothing to declare.
Competing interests
Nothing to declare.
Ethical approval
This study was approved by the Institutional Review Board of the Committee on Clinical Investigation at Boston Children's Hospital, Boston, MA, USA (protocol number 86 IRB-P00006918).
Patient consent
Not required.
References (19)
Upper airway obstruction in the syndromal craniosynostoses
Br J Plast Surg
(1993)- et al.
Sleep-related disordered breathing in children with syndromic craniosynostosis
J Craniomaxillofac Surg
(2011) - et al.
Long-term functional outcome in 167 patients with syndromic craniosynostosis; defining a syndrome-specific risk profile
J Plast Reconstr Aesthet Surg
(2010) - et al.
Airway obstruction in severe syndromic craniosynostosis
Ann Plast Surg
(1999) - et al.
Obstructive sleep apnea in Apert's and Pfeiffer's syndromes: more than a craniofacial abnormality
Plast Reconstr Surg
(1990) - et al.
Airway management in children with craniofacial anomalies
Cleft Palate Craniofac J
(1997) - et al.
Sleep-disordered breathing in children with craniosynostosis
Sleep Breath
(2013) - et al.
How does obstructive sleep apnoea evolve in syndromic craniosynostosis? A prospective cohort study
Arch Dis Child
(2013) - et al.
The American Academy of Sleep Medicine (AASM) manual for the scoring of sleep and associated events: rules, terminology and technical specifications
(2007)
Cited by (0)
- ☆
This article was presented as an abstract at the American Academy of Craniomaxillofacial Surgeons Meeting, May 2014, Portland, Oregon, USA.