1. VELOPHARYNGEAL ELECTROMYOGRAPHY IN INFANTS WITH PIERRE ROBIN SEQUENCE
Introduction / Objectives
Pierre Robin Sequence (PRS) - retrognathia, glossoptosis, airway obstruction, and cleft palate - has more severe clinical course and worse speech outcome than isolated cleft palate.
Subjects / Methods
We designed a retrospective study of 92 children with isolated (72) or syndromic (20) PRS.
Clinical grading systems were used to classify respiratory and feeding disorders.
Outcome measures included:
the need for respiratory support
the duration of enteral feeding
phonological evaluations, using the Borel-Maisonny classification of velopharyngeal insufficiency, carried out after cleft palate repair before starting speech therapy.
Results
Neurogenic EMG pattern were detected exclusively in patients with syndromic PRS.
Low-amplitude pattern that indicate muscle hypoplasia was detected in half of the patients.
Conclusion
Neurogenic EMG signs in soft palate muscles were a marker of syndromic Pierre Robin Sequence.
Low-amplitude EMG patterns that indicate soft palate muscles hypoplasia, and neurogenic EMG signs as well, were not predictors of neither respiratory and feeding disorders, nor speech outcome.
Soft palate muscles needle EMG
VELOPHARYNGEAL ELECTROMYOGRAPHY IN INFANTS WITH PIERRE ROBIN SEQUENCE
F. Renault, MD1, Anne Morice, MD2, C. Gitiaux, MD1, R. Flores-Guevara, MD, PhD1, Jean-Jacques Baudon, MD1
(1) Pediatric Neurophysiology Unit & (2) Department of maxillo-facial and plastic surgery
Necker-Enfants Malades University Hospital, Assistance Publique –Hôpitaux de Paris, France
Contact:
Phonological outcomes: Borel-Maisonny classification according to clinical features and EMG of soft palate muscles
MAFACE
Cleft palate
EMG pattern
Isolated
PRS
Syndromic
Total
Normal 36 5 41
Low-Amplitude 8 44
Neurogenic 7
We aimed to search for possible associations of velopharyngeal EMG changes with clinical status and speech outcomes.
EMG recorded the levator veli palatini muscle bilaterally, using concentric needle electrode (25 mm x 30 G), after swabbing the area with 2% lidocaïne jelly. EMG was performed at mean age 4 ± 3.2 weeks.
Mean age at phonological evaluation: 3.4 ± 1.1 y.
Competent
Borderline-
competent
incompetent
&
Incompetent
Highly- p
Isolated/Syndromic PRS
20/6
29/5
5/3
18/6 NS
Respiratory and feeding disorders:
Mild/Moderate/severe
14/8/5
12/18/2
4/5/0
10/13/1
Clinically apparent soft palate muscles atrophy 8 3 10
Abnormal needle EMG patterns in levator veli palatini muscles 15 18
Proposal for translating Borel-Maisonny classification into Pittsburgh Weighted Speech Scale (PWSS) and Cleft Audit Protocol for Speech-Augmented (CAPS)::
Competent = PWSS 0 = CAPS dark green
Borderline-competent
= PWSS 1-2 = CAPS light green
Borderline-incompetent & Incompetent
= PWSS 3-6 = CAPS yellow-orange
Highly incompetent
= PWSS ≥ 7 = CAPS red
PRS status - isolated vs syndromic -, the frequency of respiratory complications, the duration of enteral feeding, and the grade of velopharyngeal insufficiency were not statistically associated with EMG changes.