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Mobius Syndrome Treatment & Management

  • Author: Cheryl Ann Palmer, MD; Chief Editor: Amy Kao, MD  more...
 
Updated: Jun 08, 2016
 

Approach Considerations

Möbius syndrome is congenital and nonprogressive. No definitive treatment is available, with medical care being supportive and symptomatic.

Treatment for corneal ulcerations or abrasions may be required. These occur secondary to keratitis and conjunctivitis, which result from incomplete eyelid closure.

Congenital limb amputations or other musculoskeletal abnormalities may significantly compromise patients with Möbius syndrome. Splints, prostheses, or even prophylaxis for deep venous thrombosis may be indicated.

For a number of reasons, patients may be predisposed to infections. Brainstem abnormalities can predispose patients to aspiration pneumonia, which may be complicated by pulmonary infections. Vigilance and a low threshold for treatment are required. If the patient has structural anomalies of the ear, otitis media may complicate the patient's clinical course and require intervention.

Surgical therapy

Surgical care is symptomatic or cosmetic, but it is not curative of the underlying syndrome. Clubfoot, frequently bilateral, occurs in almost one third of patients. In most cases, the deformity can be corrected partially or entirely by means of orthopedic procedures.

Airway functions commonly are compromised. Tracheotomy may be required to support the airway and permit tracheobronchial clearing.

With the diffuse incidence of feeding problems in children with Möbius syndrome, supplemental energy intake via a feeding gastrostomy tube may be required. Nissen fundoplication procedures have not been helpful in treating neurogenic dysphagia and associated aspiration.

Most ophthalmologists recommend delaying surgery for strabismus because the condition frequently improves with age. Ocular surgical procedures have been successful in some patients with Möbius syndrome. In older patients, insertion of a gold weight into the eyelid may allow lid closure to protect the cornea.[35]

Consultations

Associated deficiencies in Möbius syndrome and its variants require a multidisciplinary approach by skilled specialists, such as the following[25] :

  • Pediatricians
  • General surgeons
  • Pediatric dentists
  • Orthopedic surgeons
  • Ophthalmologists
  • Pediatric otolaryngologists
  • Psychologists
  • Physiatrists
  • Occupational and physical therapists
  • Audiologists
  • Speech therapists

Diet

If the brainstem is affected severely (enough to cause dysphagia), a dietician may assist with aspiration prevention.

Activity

Activity is not specifically limited in patients with Möbius syndrome, but it may be limited by the patient's physical abilities.

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Grafting and Reinnervation Procedures

Recognition of the fact that affected regions have no functional neuromuscular system explains why nerve grafting, cross-face grafting, and cranial nerve (CN) substitution techniques have had poor success in animating the faces of patients with Möbius syndrome.[8]

However, reanimation procedures to counteract facial nerve paralysis that involve bringing in a new neuromuscular system may be successful, although the choice of donor nerves used to reinnervate the face may be limited by the extent of CN involvement in the patient's condition.[36] Restoration of function may be more successful if surgery is performed before age 7 years.

Otolaryngologists and plastic surgeons have used the unaffected accessory nerve or the mesenteric branch of CN V as donors for reinnervation procedures.

Other surgeons have designed living devices, composed of muscle and tendon transplants combined with reinnervation procedures, that have restored at least partial control of the facial musculature. Gracilis muscle transfer with masseteric nerve innervation is currently considered one of the best surgical options for the treatment of bilateral facial palsy.[36]

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Psychological Considerations

Research into the psychological family dynamics of Möbius syndrome patients has drawn attention to the problems of parental bonding and possible disturbances of family dynamics. One contributing factor may be lack of feedback to the mother from the child, who may not be able to signal to her in the usual fashion by smiling or visual following.

When affected children reach an age of self-awareness, they realize that others notice their facial immobility. The incidence of behavioral problems is higher in patients with Möbius syndrome than in age-matched controls, and levels of caregiver stress and strain are also elevated.[37] Facial mobility may increase with age, and the condition may become less embarrassing than before, easing social interactions for the patient. A study providing data on quality of life did not show behavioral problems or reduced quality of life in children with Möbius syndrome, but results did indicate that children's emotional and social development should be closely monitored.{ref Family or individual counseling may prove helpful in these situations.[38]

Neuropsychological and intelligence testing are helpful in predicting and assisting possible learning deficiencies, autism, or various visual apraxias.

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Follow-up Care

Follow-up care with the following therapies may be beneficial in patients with Möbius syndrome:

  • Physical therapy - May be useful for managing congenital orthopedic problems or for postoperative care if orthopedic intervention is required
  • Occupational therapy - May help patients, especially those without hands or digits, to accomplish activities of daily living
  • Speech therapy - May be started if the deficits in the lower CNs are severe; severe facial nerve paralysis often mechanically affects speech
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Contributor Information and Disclosures
Author

Cheryl Ann Palmer, MD Professor of Pathology, Director of Pathology Residency Program, Director of Neuropathology, ARUP Laboratories, University of Utah School of Medicine

Cheryl Ann Palmer, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuropathologists, Society for Neuro-Oncology, International Society of Neuropathology

Disclosure: Nothing to disclose.

Chief Editor

Amy Kao, MD Attending Neurologist, Children's National Medical Center

Amy Kao, MD is a member of the following medical societies: American Academy of Neurology, American Epilepsy Society, Child Neurology Society

Disclosure: Have stock from Cellectar Biosciences; have stock from Varian medical systems; have stock from Express Scripts.

Acknowledgements

Robert J Baumann, MD Professor of Neurology and Pediatrics, Department of Neurology, University of Kentucky College of Medicine

Robert J Baumann, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, and Child Neurology Society

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

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Autopsy photograph of a 3-month-old child with Möbius syndrome who died unexpectedly demonstrates congenital amputation of the left hand at the wrist.
Low-power photomicrograph of a brainstem specimen in an infant with Möbius syndrome who died at age 3 months. Image shows bilateral lesions in the pons of the abducens nuclei (hematoxylin and eosin stain).
Medium-power photomicrograph from the abducens nucleus in an infant with Möbius syndrome who died demonstrates diffuse necrosis and neuronal loss (hematoxylin and eosin stain).
High-power photomicrograph shows a lesion of an abducens nerve nucleus in an infant with Möbius syndrome who died at age 3 months. Image shows neuronal loss, necrosis, myxoid change, and a circumferential rim of thickened glial fibrils (hematoxylin and eosin stain).
 
 
 
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