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

Objectives: To evaluate motor dysfunction in infants with Pierre Robin sequence (PRS) who manifest upper airway obstruction and congenital dysphagia. Study design: Term infants (n = 28) with nonsyndromic PRS were studied between days 15 and 45. Sucking-swallowing electromyography was used to evaluat...

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Published in:The Journal of pediatrics 2002-06, Vol.140 (6), p.719-723
Main Authors: Baudon, Jean-Jacques, Renault, Francis, Goutet, Jean-Michel, Flores-Guevara, Roberto, Soupre, Véronique, Gold, Francis, Vazquez, Marie-Paule
Format: Article
Language:English
Subjects:
Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy
Biological and medical sciences
Electromyography
Emergency and intensive care: neonates and children. Prematurity. Sudden death
Esophagus - physiopathology
Gastroesophageal Reflux - physiopathology
Humans
Infant
Intensive care medicine
Manometry
Medical sciences
Pharynx - physiopathology
Pierre Robin Syndrome - physiopathology
Pressure
Tongue - physiopathology
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Summary:Objectives: To evaluate motor dysfunction in infants with Pierre Robin sequence (PRS) who manifest upper airway obstruction and congenital dysphagia. Study design: 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. Conclusion: In Pierre Robin sequence, sucking-swallowing electromyography and esophageal manometry reveal dysfunction in the motor organization of the tongue, the pharynx, and the esophagus. (J Pediatr 2002;140:719-23)
ISSN:0022-3476
1097-6833
DOI:10.1067/mpd.2002.124313