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Orofacial motor dysfunction in Moebius syndrome.

Authors :
Renault, Francis
Flores‐Guevara, Roberto
Baudon, Jean‐Jacques
Sergent, Bernard
Charpillet, Valérie
Denoyelle, Françoise
Thierry, Briac
Amiel, Jeanne
Gitiaux, Cyril
Vazquez, Marie‐Paule
Flores-Guevara, Roberto
Baudon, Jean-Jacques
Vazquez, Marie-Paule
Source :
Developmental Medicine & Child Neurology. Apr2020, Vol. 62 Issue 4, p521-527. 7p.
Publication Year :
2020

Abstract

<bold>Aim: </bold>To review orofacial disabilities and their consequences in children with Moebius syndrome (MBS).<bold>Method: </bold>We retrospectively analysed the records of 32 patients (21 males, 11 females) with non-progressive bilateral facial and abducens palsies who had been examined before 6 months of age.<bold>Results: </bold>All facial muscles were severely involved in 17 patients; in the 15 others, partial movements were found in the lower face. Most patients (n=24) were unable to smile. Patients frequently presented with congenital trismus (n=20) and drooling (n=18). Additional palsies involved cranial nerves IX and X (n=18) and XII (n=25). Sucking was absent or weak in 30 patients; swallowing was impaired in 25. During the first month of life, feeding disorders were graded as severe/moderate in 25. Respiratory complications occurred in 17. Severe feeding disorders were associated with congenital trismus (p=0.01) and with cranial nerve IX and X palsy (p=0.01). Growth failure between 1 and 6 months of age, followed by catch-up growth between 6 and 12 months, was observed in 20 patients. Between 2 and 5 years of age, 25 out of 32 patients attained normal oral diet and 28 out of 29 showed normal growth.<bold>Interpretation: </bold>Children with MBS frequently require adjusted therapeutic options to prevent failure to thrive. Congenital trismus, cranial nerve IX and X palsy, and laryngeal-tracheal dysfunctions are predictors of severe feeding disorders.<bold>What This Paper Adds: </bold>Moebius syndrome frequently induces reduced oral intake and early failure to thrive. Normal oral diet and growth parameters are attained at 2 to 5 years of age. Congenital trismus, pharyngeal palsy, and laryngeal disorders predict dysphagia. [ABSTRACT FROM AUTHOR]

Details

Language :
English
ISSN :
00121622
Volume :
62
Issue :
4
Database :
Academic Search Index
Journal :
Developmental Medicine & Child Neurology
Publication Type :
Academic Journal
Accession number :
142039627
Full Text :
https://doi.org/10.1111/dmcn.14379