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Midface Hypoplasia and Cranial Base Morphology in Syndromic Craniosynostosis: A Comparative Analysis Study Using a Predictive Regression Model
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Sleep-related disordered breathing in children with syndromic craniosynostosis

Suhail Al-Saleh1, Andrea Riekstins, Christopher R Forrest

  • 1Division of Respiratory Medicine, Hospital for Sick Children, Toronto, Ontario, Canada.

Journal of Cranio-Maxillo-Facial Surgery : Official Publication of the European Association for Cranio-Maxillo-Facial Surgery
|July 15, 2010
PubMed

Insights

Children with syndromic craniosynostosis have a high prevalence of sleep-related disordered breathing (SRDB). Treatments for SRDB in these patients showed limited success in achieving complete resolution.

Area of Science:

  • Pediatric Sleep Medicine
  • Craniofacial Surgery
  • Genetics

Background:

  • Syndromic craniosynostosis is associated with an increased risk of sleep-related disordered breathing (SRDB).
  • The diagnostic utility of polysomnography (PSG) for SRDB in this population requires further investigation.

Purpose of the Study:

  • To evaluate the prevalence and severity of SRDB in children with syndromic craniosynostosis.
  • To assess the impact of interventions on SRDB in this patient group.

Main Methods:

  • Retrospective review of patients with syndromic craniosynostosis undergoing initial polysomnography (PSG) between 1996 and 2008.
  • Analysis of interventions and follow-up PSG data for patients diagnosed with SRDB.

Main Results:

  • 74% of the 35 included patients (Crouzon's, Apert's, Pfeiffer, Saethre-Chotzen syndromes) exhibited SRDB.
  • The median obstructive apnea index was 6.6/h and the median central apnea index was 1.0/h.
  • Interventions improved SRDB in only 10 out of 14 patients, with no complete resolution reported.

Conclusions:

  • A high prevalence of SRDB is confirmed in children with syndromic craniosynostosis referred for PSG.
  • Current interventions demonstrate limited efficacy in fully resolving SRDB in this population.
Abstract

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