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Motor dysfunction of the upper digestive tract in Pierre Robin sequence as assessed by sucking-swallowing

Jean-Jacques Baudon1, Francis Renault, Jean-Michel Goutet

  • 1Service de Néonatologie, Unité de Neurophysiologie Clinique, Hôpital d'Enfants Armand-Trousseau, Paris, France.

The Journal of Pediatrics
|June 20, 2002
PubMed

Insights

Infants with Pierre Robin sequence (PRS) often have motor dysfunction affecting sucking and swallowing. Diagnostic tools like electromyography and manometry reveal significant issues in the oral, pharyngeal, and esophageal phases.

Area of Science:

  • Pediatric Gastroenterology
  • Neonatology
  • Developmental Pediatrics

Background:

  • Pierre Robin sequence (PRS) is a congenital condition characterized by micrognathia, glossoptosis, and airway obstruction.
  • Infants with PRS frequently experience feeding difficulties, including congenital dysphagia, impacting nutritional intake and growth.
  • Motor dysfunction in PRS can contribute to upper airway obstruction and challenges in coordinating sucking and swallowing.

Purpose of the Study:

  • To investigate and characterize the nature of motor dysfunction in infants diagnosed with Pierre Robin sequence (PRS).
  • To evaluate the specific motor deficits in the oral and pharyngeal phases of swallowing using electromyography.
  • To assess esophageal motility and sphincter function in infants with PRS via esophageal manometry.

Main Methods:

  • Sucking-swallowing electromyography was performed on 28 term infants with nonsyndromic PRS (15-45 days old) to assess oral phase coordination.
  • Esophageal manometry was employed to evaluate lower and upper esophageal sphincter function and esophageal body motility.
  • Manometry findings in PRS infants were compared to a control group of 16 infants with gastroesophageal reflux disease (GERD).

Main Results:

  • Electromyography revealed sucking-swallowing incoordination in 24 out of 28 (85.7%) infants with PRS, ranging from mild to severe.
  • Manometry demonstrated widespread disturbances, including abnormal lower and upper esophageal sphincter relaxation and abnormal esophageal body wave patterns in all PRS patients.
  • Infants with PRS exhibited significantly higher frequencies of motor disturbances and mean resting pressures in both sphincters compared to infants with GERD.

Conclusions:

  • Sucking-swallowing electromyography and esophageal manometry are valuable tools for identifying motor dysfunction in infants with Pierre Robin sequence.
  • The study confirms significant motor organization deficits in the tongue, pharynx, and esophagus in infants with PRS.
  • These findings highlight the complex oropharyngeal and esophageal motility issues associated with PRS, contributing to feeding and airway problems.
Abstract

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