title: Motor dysfunction of the upper digestive tract in Pierre Robin sequence as assessed by sucking-swallowing electromyography and esophageal manometry.
abstract: OBJECTIVE
  To evaluate motor dysfunction in infants with Pierre Robin sequence (PRS) who manifest upper airway obstruction and congenital dysphagia.
  
  
  METHODS
  Term infants (n = 28) with nonsyndromic PRS were studied between days 15 and 45. Sucking-swallowing electromyography was used to evaluate suction and coordination between the oral and pharyngeal phases of swallowing. Esophageal manometry was used to study the lower esophageal sphincter, esophageal body, and upper esophageal sphincter functions. Manometry results were compared with those of 16 infants with gastroesophageal reflux disease (GERD).
  
  
  RESULTS
  Electromyography showed incoordination of sucking and swallowing in 24 of 28 patients. The disorder was mild in 6, moderate in 6, and severe in 12 patients. All patients showed manometry disturbances: incomplete or asynchronous lower sphincter relaxation (15), multipeaked esophageal body waves (17), very high amplitude waves (14), and asynchronous upper sphincter relaxation (19). The frequency of disturbances and mean resting pressures of both lower and upper sphincters were significantly higher than GERD patients.
  
  
  CONCLUSIONS
  In Pierre Robin sequence, sucking-swallowing electromyography and esophageal manometry reveal dysfunction in the motor organization of the tongue, the pharynx, and the esophagus.
authors.affiliation: Service de Néonatologie, Unité de Neurophysiologie Clinique, Hôpital d'Enfants Armand-Trousseau, Paris, France.
authors.forename: Jean-Jacques
authors.initials: JJ
authors.lastname: Baudon
authors.forename: Francis
authors.initials: F
authors.lastname: Renault
authors.forename: Jean-Michel
authors.initials: JM
authors.lastname: Goutet
authors.forename: Roberto
authors.initials: R
authors.lastname: Flores-Guevara
authors.forename: Véronique
authors.initials: V
authors.lastname: Soupre
authors.forename: Francis
authors.initials: F
authors.lastname: Gold
authors.forename: Marie-Paule
authors.initials: MP
authors.lastname: Vazquez
mesh_terms: D004576:Electromyography; D004947:Esophagus / Q000503:physiopathology*; D005764:Gastroesophageal Reflux / Q000503:physiopathology; D006801:Humans; D007223:Infant; D008365:Manometry; D010614:Pharynx / Q000503:physiopathology*; D010855:Pierre Robin Syndrome / Q000503:physiopathology*; D011312:Pressure; D014059:Tongue / Q000503:physiopathology*