title: Awake Flexible Fiberoptic Laryngoscopy to diagnose glossoptosis in Robin Sequence patients.
abstract: BACKGROUND
  Robin Sequence (RS) is usually defined as the combination of micrognathia, glossoptosis and upper airway obstruction. No objective criteria to diagnose RS exist. To compare management strategy results, a single RS definition using objective criteria is needed. The most frequently used primary diagnostic tool for glossoptosis is awake Flexible Fiberoptic Laryngoscopy (aFFL).
  
  
  OBJECTIVE
  To determine the reliability of the aFFL videos as an independent diagnostic tool itself, rather than on the complete evaluation of a patient.
  
  
  METHODS
  All RS individuals from an existing cohort with an available aFFL video were included retrospectively. Thirty age-matched patients without pathologic findings on aFFL were used as controls. aFFL videos were scored by six otolaryngologists as: a. Marked glossoptosis, b. Mild glossoptosis, c. Severity unknown, d. No glossoptosis, e. Insufficient video quality. Videos were anonymised and rated twice, in altered sequences, after a washout period of minimally 2 weeks.
  
  
  METHODS
  Inter-rater and intrarater agreement.
  
  
  RESULTS
  Twenty-six videos of 16 RS patients and 30 videos of controls were included. Inter-rater agreement was fair in the whole group (κ: 0.320) and RS group (κ: 0.226), and fair to moderate in determining presence of glossoptosis (total group κ: 0.430; RS κ: 0.302; controls κ: 0.212). The intrarater agreement for the presence of glossoptosis in RS was moderate (κ: 0.541).
  
  
  CONCLUSIONS
  aFFL offers fair to moderate inter-rater agreement, with moderate intrarater agreement, in evaluating glossoptosis in RS. Using aFFL as the single tool in choosing management strategies in RS seems insufficient. There is need for a more reliable, patient friendly diagnostic tool or an internationally accepted aFFL scoring system, to diagnose glossoptosis in RS.
authors.affiliation: Department of Pediatrics, Academic Medical Center/Emma Pediatric Hospital, Amsterdam, Netherlands.|Department of Plastic, Reconstructive and Hand Surgery, Academic Medical Center, University of Amsterdam, Amsterdam, Netherlands.
authors.forename: H
authors.initials: H
authors.lastname: Basart
authors.affiliation: Department of Otolaryngology, Academic Medical Center, Amsterdam, Netherlands. a.m.konig-jung@amc.uva.nl.
authors.forename: A M
authors.initials: AM
authors.lastname: König
authors.affiliation: Department of Otolaryngology, VU Medical Center, Amsterdam, Netherlands.
authors.forename: J H
authors.initials: JH
authors.lastname: Bretschneider
authors.affiliation: Department of Otolaryngology, Academic Medical Center, Amsterdam, Netherlands.
authors.forename: C E L
authors.initials: CE
authors.lastname: Hoekstra
authors.affiliation: Department of Otolaryngology, Utrecht Medical Center/Wilhelmina Children's Hospital, Utrecht, Netherlands.
authors.forename: K P Q
authors.initials: KP
authors.lastname: Oomen
authors.affiliation: Department of Otolaryngology, Erasmus Medical Center/Sophia Pediatric Hospital, Rotterdam, Netherlands.
authors.forename: B
authors.initials: B
authors.lastname: Pullens
authors.affiliation: Department of Otolaryngology, VU Medical Center, Amsterdam, Netherlands.
authors.forename: R N P M
authors.initials: RN
authors.lastname: Rinkel
authors.affiliation: Department of Otolaryngology, VU Medical Center, Amsterdam, Netherlands.
authors.forename: C D L
authors.initials: CD
authors.lastname: van Gogh
authors.affiliation: Department of Plastic, Reconstructive and Hand Surgery, Academic Medical Center, University of Amsterdam, Amsterdam, Netherlands.
authors.forename: C M A M
authors.initials: CM
authors.lastname: van der Horst
authors.affiliation: Department of Pediatrics, Academic Medical Center/Emma Pediatric Hospital, Amsterdam, Netherlands.
authors.forename: R C
authors.initials: RC
authors.lastname: Hennekam
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D005336:Fiber Optic Technology; D065710:Glossoptosis / Q000175:diagnosis* / Q000209:etiology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007828:Laryngoscopy / Q000379:methods*; D008297:Male; D010855:Pierre Robin Syndrome / Q000150:complications*; D015203:Reproducibility of Results; D012189:Retrospective Studies; D014741:Video Recording