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Orofacial motor dysfunction in Moebius syndrome.

Francis Renault1, Roberto Flores-Guevara2, Jean-Jacques Baudon3

  • 1Centre de Référence des Fentes et Malformations Faciales 'MAFACE', Hôpital Necker-Enfants-Malades, Paris, France.

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Summary
This summary is machine-generated.

Moebius syndrome (MBS) causes orofacial disabilities, impacting feeding and growth in children. Early interventions are crucial, as many achieve normal oral intake and growth by age five.

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Area of Science:

  • Pediatric Neurology
  • Craniofacial Disorders
  • Developmental Pediatrics

Background:

  • Moebius syndrome (MBS) is a rare congenital disorder characterized by non-progressive facial nerve (CN VII) and abducens nerve (CN VI) palsies.
  • Orofacial disabilities in MBS can lead to significant challenges in feeding, swallowing, and overall growth during infancy and early childhood.

Purpose of the Study:

  • To review the orofacial disabilities associated with Moebius syndrome in children.
  • To analyze the consequences of these disabilities on feeding, growth, and development.
  • To identify predictors of severe feeding disorders in infants with Moebius syndrome.

Main Methods:

  • Retrospective analysis of medical records from 32 pediatric patients diagnosed with Moebius syndrome.
  • Patients were examined before six months of age, with data on facial muscle involvement, cranial nerve palsies, feeding, and growth parameters collected.
  • Statistical analysis was performed to identify associations between specific impairments and feeding disorders.

Main Results:

  • Severe facial muscle involvement was noted in 17 patients; 15 had partial lower face movement. 24 patients could not smile.
  • Commonly observed issues included congenital trismus (n=20), drooling (n=18), impaired sucking (n=30) and swallowing (n=25). Palsies of cranial nerves IX, X, and XII were frequent.
  • Feeding disorders were severe/moderate in 25 infants. Severe feeding issues correlated with congenital trismus (p=0.01) and cranial nerve IX/X palsy (p=0.01). Despite initial growth failure, 25/32 patients achieved normal oral diet and 28/29 showed normal growth by 2-5 years.

Conclusions:

  • Moebius syndrome frequently leads to reduced oral intake and early failure to thrive, necessitating tailored therapeutic strategies.
  • Congenital trismus, cranial nerve IX and X palsy, and laryngeal-tracheal dysfunction are significant predictors of severe feeding disorders.
  • While early feeding and growth can be challenging, most children with Moebius syndrome achieve normal oral diet and growth parameters by 2 to 5 years of age.