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Neurophysiological brainstem investigations in isolated Pierre Robin sequence.
F Renault1, R Flores-Guevara, V Soupre
1Unité de neurophysiologie clinique, Hôpital d'enfants Armand-Trousseau, Paris, France. neuro.physiol@trs.ap-hop-paris.fr
Early Human Development
|June 16, 2000
Summary
Newborns with Pierre Robin sequence (PRS) often experience obstructive sleep apnea and feeding difficulties. Neurophysiological tests reveal motor organization dysfunction in the tongue and pharynx, but not the brainstem.
Area of Science:
- Neuroscience
- Pediatrics
- Sleep Medicine
Background:
- Pierre Robin sequence (PRS) is a congenital condition characterized by micrognathia, glossoptosis, and cleft palate.
- Infants with PRS are at risk for airway obstruction and feeding difficulties due to craniofacial abnormalities.
Purpose of the Study:
- To evaluate neurophysiological function in newborns with isolated PRS.
- To identify potential underlying mechanisms for respiratory and feeding issues in PRS.
Main Methods:
- Polysomnography, electromyography (EMG) of facial, tongue, and soft palate muscles, blink reflexes (BRs), EMG during bottle-feeding, and brainstem auditory evoked responses (BAERs) were performed.
- 25 newborn infants with isolated PRS were included in the study.
Main Results:
- Obstructive apneas were present in 23/24 infants.
- Sucking-swallowing disorders were observed in 20/25 infants during bottle-feeding.
- EMG showed dysfunction in soft palate muscles in 11/25 infants and denervation in 1/25. BAERs indicated bilateral conductive hearing impairment in 5/19 infants.
Conclusions:
- Infants with isolated PRS exhibit neurophysiological evidence of lingual and pharyngeal motor organization dysfunction.
- These findings suggest that PRS involves functional, rather than structural, impairments in the brainstem pathways controlling swallowing and breathing.