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Updated: Jun 2, 2026
An Implantable System For Chronic In Vivo Electromyography
Published on: April 21, 2020
Facial, lingual, and pharyngeal electromyography in infants with Pierre Robin sequence
Francis Renault1, Jean-Jacques Baudon, Eva Galliani
1Clinical Neurophysiology Unit, Hôpital Armand-Trousseau, AP-HP, 28 avenue Arnold-Netter, 75571 Paris 12, France. francis.renault@trs.aphp.fr
Insights
Electromyography (EMG) effectively assessed orofacial neurological dysfunction in infants with Pierre Robin sequence (PRS). EMG findings indicated cranial nerve involvement, suggesting associated PRS forms.
Area of Science:
- Neurology
- Pediatrics
- Medical Technology
Background:
- Pierre Robin sequence (PRS) presents challenges in infant feeding and development.
- Orofacial neurological dysfunction is a key concern in PRS infants.
- Electromyography (EMG) offers a potential tool for assessing this dysfunction.
Purpose of the Study:
- To evaluate the utility of electromyography (EMG) in diagnosing orofacial neurological dysfunction in infants with PRS.
- To determine if EMG findings correlate with PRS subtypes (isolated vs. associated).
- To assess the relationship between EMG feeding parameters and clinical outcomes.
Main Methods:
- Needle EMG of facial, tongue, and soft palate muscles, along with blink responses.
- Two-channel EMG to record sucking and swallowing during bottle feeding.
- Analysis of EMG data in relation to PRS type and surgical history.
Main Results:
- Neurogenic EMG signs were significantly more prevalent in associated PRS (17/24) than isolated PRS (1/57).
- Soft palate muscle EMG showed abnormalities in 41.4% of infants requiring two-step cleft palate repair.
- Abnormal oral/pharyngeal coordination during feeding correlated with prolonged enteral feeding needs.
Conclusions:
- Combined EMG methods are valuable for managing infants with PRS.
- EMG detection of cranial nerve involvement is a strong indicator of associated PRS.
- EMG assessment aids in understanding feeding difficulties and guiding treatment in PRS infants.
Introduction:
We evaluated the role of electromyography (EMG) in assessing orofacial neurological dysfunction in 81 infants with Pierre Robin sequence (PRS).
Methods:
Needle EMG of muscles of the face, tongue, and soft palate, and blink responses were recorded. A two-channel EMG recorded sucking and swallowing during bottle feeding.
Results:
Neurogenic EMG signs were detected in facial or oral muscles in 17 of 24 associated PRS and 1 of 57 isolated PRS cases (P < 0.0001). Soft palate muscles showed low-amplitude traces in 41.4% of patients who required two surgical steps for cleft palate repair and 18.5% of those who required only one step. Regarding EMG study during bottle feeding, patients with moderate or severe abnormalities of oral/pharyngeal coordination required more prolonged enteral feeding than patients with mild abnormalities or normal coordination (P = 0.002).
Conclusion:
Combined EMG methods were useful in the treatment of infants with PRS. EMG detection of cranial nerve involvement strongly suggests an associated form of PRS.
