title: Surgical versus nonsurgical interventions to relieve upper airway obstruction in children with Pierre Robin sequence.
abstract: BACKGROUND
  Newborns with Pierre Robin sequence (PRS) often experience chronic intermittent hypoxemia/hypoventilation associated with airway obstruction. The heterogeneity of the severity of upper airway obstruction makes management a challenge; the optimal intervention in individual cases is not clear.
  
  
  OBJECTIVE
  To investigate the prevalence of surgical/nonsurgical interventions for PRS at two children's hospitals. Patient characteristics and outcomes were examined.
  
  
  METHODS
  The present retrospective chart review identified 139 patients with PRS born between 2000 and 2010. Demographic information, mode of airway management, associated anomalies and syndromes, polysomnography results, length of intensive care unit and hospital stay, complications and deaths were extracted.
  
  
  RESULTS
  Interventions included prone positioning (alone [61%]), tongue-lip adhesion (45%), nasopharyngeal intubation (28%), continuous positive airway pressure (20%), tracheostomy (19%) and mandibular distraction osteogenesis (5%). Tracheostomies were more prevalent in syndromic patients (P=0.03). Patients who underwent tracheostomy had a lower birth weight (P=0.03) compared with newborns with other interventions. Patients who underwent surgical interventions had longer intensive care unit stays (P<0.001). No intervention was associated with a statistically significant likelihood of requiring a subsequent intervention. Thirty percent of patients underwent polysomnography, with a higher proportion of these using continuous positive airway pressure (n=15) (P<0.01).
  
  
  CONCLUSIONS
  In the present descriptive study, patients with syndromic PRS or low birth weight underwent early intervention, which included a tracheostomy. Objective measures of airway obstruction were underutilized. Decision making regarding evaluation and management of upper airway obstruction in this population remains clinician and resource dependent. Reporting data obtained from a large cohort of PRS patients is important to compare experiences and motivate future studies investigating this complex condition.
authors.forename: Karen
authors.initials: K
authors.lastname: Kam
authors.forename: Meghan
authors.initials: M
authors.lastname: McKay
authors.forename: Joanna
authors.initials: J
authors.lastname: MacLean
authors.forename: Manisha
authors.initials: M
authors.lastname: Witmans
authors.forename: Sheldon
authors.initials: S
authors.lastname: Spier
authors.forename: Ian
authors.initials: I
authors.lastname: Mitchell
mesh_terms: D058109:Airway Management; D000402:Airway Obstruction / Q000175:diagnosis / Q000209:etiology / Q000628:therapy*; D003422:Critical Care; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D007234:Infant, Premature; D007235:Infant, Premature, Diseases / Q000175:diagnosis / Q000628:therapy*; D007902:Length of Stay; D008297:Male; D010855:Pierre Robin Syndrome / Q000175:diagnosis / Q000628:therapy*; D017286:Polysomnography; D012189:Retrospective Studies
references.citation: Otolaryngol Head Neck Surg. 2004 Dec;131(6):810-3
references.pmid: 15577773
references.citation: Int J Pediatr Otorhinolaryngol. 2006 Feb;70(2):319-24
references.pmid: 16112206
references.citation: Int J Pediatr Otorhinolaryngol. 2006 Jun;70(6):973-80
references.pmid: 16443284
references.citation: J Craniofac Surg. 2009 Mar;20(2):315-20
references.pmid: 19276814
references.citation: J Craniofac Surg. 2009 Sep;20 Suppl 2:1817-21
references.pmid: 19816357
references.citation: J Pediatr Surg. 2009 Nov;44(11):2112-8
references.pmid: 19944218
references.citation: Pediatr Pulmonol. 2010 May;45(5):487-93
references.pmid: 20425857
references.citation: Cleft Palate Craniofac J. 2010 Jul;47(4):378-81
references.pmid: 20590459
references.citation: Otolaryngol Head Neck Surg. 2010 Jul;143(1):90-6
references.pmid: 20620625
references.citation: Clin Pediatr (Phila). 2010 Dec;49(12):1117-22
references.pmid: 20837617
references.citation: Arch Facial Plast Surg. 2011 May-Jun;13(3):173-9
references.pmid: 21242420
references.citation: Int J Pediatr Otorhinolaryngol. 2011 Sep;75(9):1152-5
references.pmid: 21764465
references.citation: Cleft Palate Craniofac J. 2011 Sep;48(5):614-8
references.pmid: 20815709
references.citation: J Pediatr. 2012 Apr;160(4):645-650.e2
references.pmid: 22048048
references.citation: Arch Dis Child. 2012 Apr;97(4):331-4
references.pmid: 22331679
references.citation: Otolaryngol Clin North Am. 2012 Jun;45(3):695-710, ix
references.pmid: 22588044
references.citation: Int J Pediatr Otorhinolaryngol. 2013 Apr;77(4):499-503
references.pmid: 23313433
references.citation: Otolaryngol Head Neck Surg. 2013 May;148(5):847-51
references.pmid: 23426711
references.citation: Cleft Palate Craniofac J. 2003 Mar;40(2):180-5
references.pmid: 12605525
references.citation: Plast Reconstr Surg. 2004 Apr 1;113(4):1113-25
references.pmid: 15083010
references.citation: Br J Plast Surg. 1983 Oct;36(4):434-7
references.pmid: 6626822
references.citation: Int J Pediatr Otorhinolaryngol. 1986 Apr;11(2):135-46
references.pmid: 3744695
references.citation: Pediatrics. 1990 Aug;86(2):294-301
references.pmid: 2371106
references.citation: Int J Pediatr Otorhinolaryngol. 1991 Jul;22(1):29-37
references.pmid: 1917336
references.citation: Cleft Palate Craniofac J. 1992 May;29(3):220-3
references.pmid: 1591254
references.citation: Cleft Palate Craniofac J. 2003 Jan;40(1):13-8
references.pmid: 12498601
references.citation: Otolaryngol Head Neck Surg. 1998 May;118(5):630-5
references.pmid: 9591861