Sleep-related disordered breathing in children with syndromic craniosynostosis

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 16.9 kg/m(2). 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.