title: Will the right Robin patient rise, please? Definitions and criteria during management of Robin sequence patients in the Netherlands and Belgium.
abstract: BACKGROUND
  Robin Sequence (RS) is characterized by micrognathia and upper airway obstruction (UAO), with or without cleft palate, causing respiratory and feeding problems. Management options are: positioning; nasopharyngeal airway (NPA); tongue-lip adhesion (TLA); mandibular distraction (MDO); and tracheostomy. Controversy exists in literature regarding RS definition and management. Here we describe definitions, management strategies and criteria in opting for management strategies, used by Dutch and Belgian cleft teams.
  
  
  METHODS
  A specifically designed questionnaire was sent to members of all 16 Dutch and Belgian cleft teams.
  
  
  RESULTS
  14 cleft teams returned 35 questionnaires. All used micrognathia as definition criterion, 93.4% cleft palate, 51.5%glossoptosis and 45.7% UAO. Six different RS definitions were used; even within a single team >1 definition was used. All teams used different management strategies: all used positioning, 10 NPA, 6 TLA, 7 MDO, 8 tracheostomy, 5 refer patients with invasive treatment indication. Criteria in opting management modalities were: O2-saturation (89.3%), clinical presentation (86.2%), growth and feeding problems (69.0%), polysomnography (62.1%), and differed within teams.
  
  
  CONCLUSIONS
  The Dutch and Belgian cleft teams use variable RS definitions, different management modalities and criteria in choosing management strategies. A single, strict definition and evidence-based management guidelines should be formulated for optimal patient care.
authors.affiliation: Department of Plastic, Reconstructive and Hand Surgery, Academic Medical Centre, Amsterdam, The Netherlands. Electronic address: h.basart@amc.nl.
authors.forename: Hanneke
authors.initials: H
authors.lastname: Basart
authors.affiliation: Department of Pediatrics, Emma Children's Hospital, Amsterdam, The Netherlands.
authors.forename: Frea H
authors.initials: FH
authors.lastname: Kruisinga
authors.affiliation: Department of Plastic, Reconstructive and Hand Surgery, Utrecht Medical Centre/Wilhelmina Children's Hospital, Utrecht, The Netherlands.
authors.forename: Corstiaan C
authors.initials: CC
authors.lastname: Breugem
authors.affiliation: Department of Plastic, Reconstructive and Hand Surgery, VU Medical Centre, Amsterdam, The Netherlands.
authors.forename: J Peter W
authors.initials: JP
authors.lastname: Don Griot
authors.affiliation: Department of Pediatrics, Emma Children's Hospital, Amsterdam, The Netherlands.
authors.forename: Raoul C
authors.initials: RC
authors.lastname: Hennekam
authors.affiliation: Department of Plastic, Reconstructive and Hand Surgery, Academic Medical Centre, Amsterdam, The Netherlands.
authors.forename: Chantal M A M
authors.initials: CM
authors.lastname: Van der Horst
mesh_terms: D000402:Airway Obstruction / Q000175:diagnosis; D001530:Belgium; D002972:Cleft Palate / Q000175:diagnosis; D004435:Eating / Q000502:physiology; D065710:Glossoptosis / Q000175:diagnosis; D006130:Growth Disorders / Q000517:prevention & control; D006801:Humans; D007442:Intubation, Intratracheal; D008046:Lip / Q000601:surgery; D008334:Mandible / Q000601:surgery; D008844:Micrognathism / Q000175:diagnosis; D009426:Netherlands; D019857:Osteogenesis, Distraction / Q000706:statistics & numerical data; D010100:Oxygen / Q000097:blood; D010348:Patient Care Team; D056888:Patient Positioning; D010855:Pierre Robin Syndrome / Q000175:diagnosis* / Q000628:therapy; D017286:Polysomnography / Q000706:statistics & numerical data; D014059:Tongue / Q000601:surgery; D014139:Tracheostomy / Q000706:statistics & numerical data
chemical_list: D010100:Oxygen
keywords: Cleft palate; Definition; Diagnostic criteria; Micrognathia; Robin sequence; Treatment