title: Treatment of infants with Syndromic Robin sequence with modified palatal plates: a minimally invasive treatment option.
abstract: BACKGROUND
  Infants with Robin sequence (RS) suffer from upper airway obstruction (UAO) and feeding problems. We developed an oral appliance with a velar extension in combination with functional treatment and appropriate feeding techniques, which was proven effective in isolated RS. As the above problems are particularly challenging in syndromic RS, we set out to evaluate our treatment concept also in these patients.
  
  
  METHODS
  We searched our electronic departmental database to identify all children admitted to our department between 01/01/2003 and 31/12/2009 because of syndromic RS. UAO was quantified by cardiorespiratory sleep studies performed before and during treatment with a modified palatal plate. This appliance consists of a palatal part, covering the hard palate as well as the alveolar ridges and the potential cleft, and a velar extension shifting the tongue in a more anterior position, thereby opening the pharyngeal airway. It is adjusted by fiberoptic nasopharyngoscopy and controlled by cardiorespiratory sleep studies. Obstructive sleep apnea was defined as a mixed obstructive sleep apnea index (MOAI) >3/h. Feeding modalities before and after treatment and weight gain, determined as standard deviation score, were also evaluated.
  
  
  RESULTS
  Of 68 children meeting inclusion criteria, 56 completed treatment (46 of these being infants). Underlying diagnoses included craniofacial dysostosis (N = 13) and synostosis syndromes (N = 5), unspecified dysmorphic syndromes (N = 23) and miscellaneous rare conditions (N = 27). Median MOAI decreased from 8.5 (range 0.3-76.0) at admission to 1.1 (0.0-5.2) at discharge (p < 0.001). 51 children received only a TPP and 5 additionally continuous positive airway pressure (CPAP) or high-flow nasal cannula during sleep for mild residual OSA. Three children ultimately required tracheostomy. The number of exclusively gavage fed infants was reduced from 23 to 7. Conversely, the number of children fed exclusively by mouth increased from 18 to 30. Median SDS for weight decreased from -1.6 (-3.5-1.7) to -1.3 (-4.1-2.5). Twelve children had their treatment prematurely discontinued, e.g. due to laryngeal collapse/laryngomalacia. No patient died during treatment.
  
  
  CONCLUSIONS
  Treatment of UAO and feeding problems in these children with syndromic RS by a modified palatal plate with a velar extension was shown to be effective and safe. If confirmed in prospective studies, it may help to avoid more invasive interventions.
authors.affiliation: Interdisciplinary Centre for Craniofacial Malformations, Tuebingen University Hospital, Calwerstrasse 7, 72076, Tuebingen, Germany.|Department of Neonatology, Tuebingen University Hospital, Calwerstrasse 7, 72076, Tuebingen, Germany.|Department of Orthodontics, Tuebingen University Hospital, Osianderstrasse 2-8, 72076, Tuebingen, Germany.
authors.forename: Silvia
authors.initials: S
authors.lastname: Müller-Hagedorn
authors.affiliation: Interdisciplinary Centre for Craniofacial Malformations, Tuebingen University Hospital, Calwerstrasse 7, 72076, Tuebingen, Germany.|Department of Neonatology, Tuebingen University Hospital, Calwerstrasse 7, 72076, Tuebingen, Germany.
authors.forename: Wolfgang
authors.initials: W
authors.lastname: Buchenau
authors.affiliation: Department of Neonatology, Tuebingen University Hospital, Calwerstrasse 7, 72076, Tuebingen, Germany.
authors.forename: Jörg
authors.initials: J
authors.lastname: Arand
authors.affiliation: BIP - Orthodontic Practice, Schweickhardtstrasse 11, 72072, Tübingen, Germany.
authors.forename: Margit
authors.initials: M
authors.lastname: Bacher
authors.affiliation: Interdisciplinary Centre for Craniofacial Malformations, Tuebingen University Hospital, Calwerstrasse 7, 72076, Tuebingen, Germany. christian-f.poets@med.uni-tuebingen.de.|Department of Neonatology, Tuebingen University Hospital, Calwerstrasse 7, 72076, Tuebingen, Germany. christian-f.poets@med.uni-tuebingen.de.
authors.forename: Christian F
authors.initials: CF
authors.lastname: Poets
mesh_terms: D002648:Child; D002657:Child Development / Q000502:physiology; D002675:Child, Preschool; D016208:Databases, Factual; D005260:Female; D005500:Follow-Up Studies; D005858:Germany; D006801:Humans; D007223:Infant; D007902:Length of Stay; D008297:Male; D009967:Orthodontic Appliances / Q000706:statistics & numerical data*; D009971:Orthodontics, Corrective / Q000379:methods*; D021362:Palate, Hard / Q000002:abnormalities; D010855:Pierre Robin Syndrome / Q000150:complications / Q000000981:diagnostic imaging / Q000628:therapy*; D017286:Polysomnography / Q000379:methods; D011175:Positive-Pressure Respiration / Q000379:methods; D011474:Prosthesis Design; D012189:Retrospective Studies; D018570:Risk Assessment; D012720:Severity of Illness Index; D012891:Sleep Apnea Syndromes / Q000175:diagnosis / Q000209:etiology / Q000628:therapy*; D016896:Treatment Outcome
keywords: Orthodontic treatment; Palatal plate; Syndromic Robin sequence; Upper airway obstruction