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: docteur.frenault@wanadoo.fr 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.