title: The successful use of the nasopharyngeal airway in Pierre Robin sequence: an 11-year experience.
abstract: BACKGROUND
  Pierre Robin sequence (PRS) is a congenital anomaly presenting with micrognathia, glossoptosis and a cleft palate. This study describes a decade's experience of the management of upper airway obstruction (UAO) in PRS patients with a nasopharyngeal airway (NPA).
  
  
  METHODS
  This study was conducted by paediatric respiratory and otolaryngology departments. Children with PRS referred with UAO were evaluated according to a standard protocol. Data collected included the degree of airway obstruction, method of airway management, polysomnography data before and after intervention, and longer term follow-up.
  
  
  RESULTS
  Data were collected on 104 PRS patients referred to us for airway assessment in 2000-2010. 64/104 were aged <4 weeks at referral. Airway symptoms were managed conservatively in 27 patients (25.9%), with an NPA in 63 (60.6%) and a tracheostomy in 14 (13.4%). The average duration of NPA use was 8 months (3 weeks to 27 months). Polysomnography results improved in all 63 patients with an NPA. Fourteen severely obstructed patients underwent a tracheostomy. 86.5% (90/104) of PRS patients were managed conservatively or with the help of an NPA. There were no NPA related complications.
  
  
  CONCLUSIONS
  There is a spectrum of UAO in PRS. This study reports on long-term outcomes in 104 children with PRS and airway obstruction. In most children (86.5%), airway obstruction was managed by conservative measures or with an NPA for a few months. The natural history shows that with normal growth, airway compromise resolves without immediate surgical intervention as advocated by some practitioners. Few PRS children require a tracheostomy.
authors.affiliation: Department of Paediatric Otolaryngology, Great Ormond Street Hospital, 13 Abbotsford Gardens, Woodford Green, Essex, UK.
authors.forename: Francois
authors.initials: F
authors.lastname: Abel
authors.forename: Yogesh
authors.initials: Y
authors.lastname: Bajaj
authors.forename: Michelle
authors.initials: M
authors.lastname: Wyatt
authors.forename: Colin
authors.initials: C
authors.lastname: Wallis
mesh_terms: D058109:Airway Management / Q000295:instrumentation / Q000379:methods*; D000402:Airway Obstruction / Q000628:therapy*; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007440:Intubation / Q000295:instrumentation / Q000379:methods; D008297:Male; D009305:Nasopharynx; D010855:Pierre Robin Syndrome / Q000628:therapy*; D012189:Retrospective Studies; D014139:Tracheostomy; D016896:Treatment Outcome