title: Upper airway endoscopy to optimize obstructive sleep apnea treatment in Apert and Crouzon syndromes.
abstract: BACKGROUND
  Obstructive sleep apnea (OSA) is highly prevalent in children with Apert and Crouzon syndromes. Although often related to midface hypoplasia, it is a multi-level problem for which routine midface advancement might be a suboptimal treatment choice. We therefore wished to: 1.) use upper airway endoscopy to examine the level of obstruction in children with OSA; 2.) determine the relationship between endoscopic assessment and OSA severity; and 3.) evaluate the effect of surgery on endoscopic assessment and OSA severity.
  
  
  METHODS
  Prospective observational cohort study of patients considered for midface advancement, underwent upper airway endoscopy. Endoscopy findings were scored according to the system of Bachar, based on level (nose, uvulopalatine plane, tongue base, hypopharynx and larynx); and severity (no, partial or complete obstruction). Polysomnography was used to diagnose OSA.
  
  
  RESULTS
  We included 22 children (Apert N = 10, Crouzon N = 12), 17 had OSA, 14 of whom had multilevel obstruction and 3 single-level obstruction. The endoscopy findings were correlated with OSA severity: R = 0.56, P = 0.01. Midface advancement (N = 8) reduced Bachar's severity index in 7 of 8 patients, and OSA in all patients.
  
  
  CONCLUSIONS
  OSA in children with Apert or Crouzon syndrome is often a multi-level problem. Upper airway endoscopy is essential to optimizing OSA treatment.
authors.affiliation: Erasmus MC - Sophia Children's Hospital, University Medical Center Rotterdam, Dutch Craniofacial Center, Department of Plastic and Reconstructive Surgery and Hand Surgery, The Netherlands. Electronic address: p.doerga@erasmusmc.nl.
authors.forename: Priya N
authors.initials: PN
authors.lastname: Doerga
authors.affiliation: Erasmus MC - Sophia Children's Hospital, University Medical Center Rotterdam, Dutch Craniofacial Center, Department of Plastic and Reconstructive Surgery and Hand Surgery, The Netherlands.
authors.forename: Bart
authors.initials: B
authors.lastname: Spruijt
authors.affiliation: Erasmus MC - Sophia Children's Hospital, University Medical Center Rotterdam, Dutch Craniofacial Center, Department of Plastic and Reconstructive Surgery and Hand Surgery, The Netherlands.
authors.forename: Irene M J
authors.initials: IM
authors.lastname: Mathijssen
authors.affiliation: Erasmus MC - Sophia Children's Hospital, University Medical Center Rotterdam, Department of Oral and Maxillofacial Surgery, The Netherlands.
authors.forename: Eppo B
authors.initials: EB
authors.lastname: Wolvius
authors.affiliation: Erasmus MC - Sophia Children's Hospital, University Medical Center Rotterdam, Pediatric Intensive Care Unit, The Netherlands.
authors.forename: Koen F M
authors.initials: KF
authors.lastname: Joosten
authors.affiliation: Erasmus MC - Sophia Children's Hospital, University Medical Center Rotterdam, Department of Otorhinolaryngology, The Netherlands.
authors.forename: Marc P
authors.initials: MP
authors.lastname: van der Schroeff
mesh_terms: D000168:Acrocephalosyndactylia / Q000150:complications*; D004724:Endoscopy / Q000379:methods*; D006801:Humans; D017286:Polysomnography; D011446:Prospective Studies; D020181:Sleep Apnea, Obstructive / Q000175:diagnosis / Q000209:etiology / Q000628:therapy*
keywords: Apert syndrome; Craniosynostoses; Crouzon syndrome; Monobloc; Obstructive sleep apnea; Respiratory system