Sleep-related disordered breathing in children with syndromic craniosynostosis

Al-Saleh, Suhail; Riekstins, Andrea; Forrest, Christopher R.; Philips, John H.; Gibbons, Jeremy; Narang, Indra

doi:10.1016/j.jcms.2010.04.011

« Previous Next »Journal of Cranio-Maxillo-Facial Surgery
Volume 39, Issue 3 , Pages 153-157, April 2011

Sleep-related disordered breathing in children with syndromic craniosynostosis

Suhail Al-Saleh
- Division of Respiratory Medicine, Hospital for Sick Children, Toronto, Ontario, Canada
- University of Toronto, Toronto, Ontario, Canada
Andrea Riekstins
- Division of Respiratory Medicine, Hospital for Sick Children, Toronto, Ontario, Canada
- University of Toronto, Toronto, Ontario, Canada
Christopher R. Forrest
- Division of Plastic Surgery, Center for Craniofacial Care or Research, Hospital for Sick Children, Toronto, Ontario, Canada
- University of Toronto, Toronto, Ontario, Canada
John H. Philips
- Division of Plastic Surgery, Center for Craniofacial Care or Research, Hospital for Sick Children, Toronto, Ontario, Canada
- University of Toronto, Toronto, Ontario, Canada
Jeremy Gibbons
- Division of Respiratory Medicine, Hospital for Sick Children, Toronto, Ontario, Canada
Indra Narang
- Division of Respiratory Medicine, Hospital for Sick Children, Toronto, Ontario, Canada
- University of Toronto, Toronto, Ontario, Canada
- Corresponding author. Division of Respiratory Medicine, The Hospital for Sick Children, 555 University Ave, Toronto, Ontario M5G 1X8, Canada. Tel.: +1 416 813 6346; fax: +1 416 813 6246.

Received 18 December 2009; accepted 23 April 2010. published online 03 June 2010.

Abstract

Background

Syndromic craniosynostosis patients are at risk for sleep-related disordered breathing (SRDB) but the role of polysomnography (PSG) in assessing these patients has not been fully explored. Our aim was to evaluate the prevalence or severity of SRDB in children with syndromic craniosynostosis or the impact of treatments on their SRDB.

Methods

We conducted a retrospective review of all patients with syndromic craniosynostosis referred between 1996 or 2008 for an initial PSG to rule out SRDB. For those with SRDB, we reviewed the interventions post PSG.

Results

35 patients (18 females) were included. Specific diagnoses were Crouzon’s (n=18), Apert’s (n=14), Pfeiffer (n=2) or Saethre-Chotzen (n=1) syndromes. Their mean age was 4.5 years or their mean body mass index (BMI) was 16.9kg/m². Of these patients, 26/35 (74%) had evidence of SRDB. The median obstructive apnoea index was 6.6/h (range 0.5–36.4/h) or median central apnoea index was 1.0/h (range 0.0–66.4/h). A total of 16 children had interventions to treat SRDB, of which 14/16 had a follow up PSG or only 10/14 (x%) had a significant improvement of their SRDB.

Conclusion

This review confirms a high prevalence SRDB in this referred population. Despite various interventions, complete resolution of SRDB could not be achieved.

Keywords: syndromic craniosynostosis, sleep-related disordered breathing, central sleep apnoea, obstructive sleep apnoea, paediatrics or polysomnography