Mobius syndrome; congenital facial diplegia; oromandibular-limb hypogenesis

Etiology

• birth defect caused by the absence or underdevelopment of cranial nerve 6 & cranial nerve 7

Epidemiology

rare

Pathology

• small or absent brain stem nuclei
• decreased numbers of muscle fibers
• deformities of the tongue, jaw, & limb

Clinical manifestations

• suckling defect 1st symptom present at birth
• feeding, swallowing, & choking
• excessive drooling
• crossed eyes
• lack of facial expression
• inability to smile
• eye sensitivity
• motor delays
• high or cleft palate
• hearing impairment
• clubfoot
• missing or webbed fingers
• speech impairment
• as children get older, lack of facial expression & inability to smile become the dominant visible symptoms

Management

• no specific treatment
• Infants may require feeding tubes or special bottles to maintain sufficient nutrition
• physical therapy & speech therapy improves coordination & ability to eat & speak
• surgery may correct crossed eyes & improve limb & jaw deformities
• plastic reconstructive surgery may be beneficial
  • nerve & muscle transfers to the corners of the mouth may provide limited ability to smile
• prognosis is generally good; life expectancy is normal

More general terms

• facial disorder/injury/anomaly
• developmental disorder

Patient information

Mobius syndrome patient information