title: Dysphagia and nutrition problems in infants with apert syndrome.
abstract: OBJECTIVE
  The purpose of this study was to identify and describe the nature of dysphagia and nutrition difficulties in infants with Apert syndrome.
  
  
  METHODS
  The study comprised a review of the medical, nutrition, and feeding records of 13 consecutive infants still feeding by the bottle who had been referred to the Craniofacial Unit and analyses of swallow function from videofluoroscopic swallow investigations.
  
  
  METHODS
  Outcome measures included qualitative analyses of bottle-feeding and nutritional status and quantitative functional severity ratings of dysphagia based on videofluoroscopic swallow investigations using the O'Neil et al. (1999) Dysphagia Outcome Severity Scale.
  
  
  RESULTS
  The main qualitative descriptors of oral feeding in this cohort included uncoordinated suck-swallow-breathe patterns, inability to maintain sucking bursts, and changes in respiratory patterns as the feed progressed. Videofluoroscopic evaluations (N = 7) showed silent laryngeal penetration or aspiration in more than half of the cohort. Failure to thrive was a frequent occurrence seen in seven infants, and 9 of the 10 required dietetic intervention and enteral supplements. (Nutritional records were not located for three infants.)
  
  
  CONCLUSIONS
  In view of the small sample size and retrospective nature of the study, the results need to be interpreted with caution. However, the study adds to current limited knowledge on feeding and nutrition in Apert syndrome. Further prospective multidisciplinary and objective research is clearly warranted.
authors: North Thames Regional Cleft Service, London, UK. Pereiv@gosh.nhs.uk
authors: Valerie
authors: V
authors: Pereira
authors: Paul
authors: P
authors: Sacher
authors: Martina
authors: M
authors: Ryan
authors: Richard
authors: R
authors: Hayward
mesh_terms: D000168:Acrocephalosyndactylia / Q000150:complications*; D000402:Airway Obstruction / Q000209:etiology; D001903:Bottle Feeding; D002675:Child, Preschool; D015331:Cohort Studies; D003371:Cough / Q000209:etiology; D003679:Deglutition / Q000502:physiology; D003680:Deglutition Disorders / Q000209:etiology*; D004750:Enteral Nutrition; D005183:Failure to Thrive / Q000209:etiology / Q000628:therapy; D005260:Female; D005471:Fluoroscopy / Q000379:methods; D006801:Humans; D007223:Infant; D007830:Larynx / Q000503:physiopathology; D008297:Male; D009748:Nutrition Disorders / Q000209:etiology*; D044623:Nutrition Therapy; D009752:Nutritional Status; D010614:Pharynx / Q000503:physiopathology; D012119:Respiration; D053120:Respiratory Aspiration / Q000209:etiology; D012189:Retrospective Studies; D013391:Sucking Behavior / Q000502:physiology; D014741:Video Recording; D014939:Work of Breathing / Q000502:physiology