Mobius Syndrome Treatment & Management

  • Author: Cheryl Ann Palmer, MD; Chief Editor: Amy Kao, MD   more...
 
Updated: Feb 23, 2012
 

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.[34]

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.

Next

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.[35] 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.[35]

Previous
Next

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.[36] Facial mobility may increase with age, and the condition may become less embarrassing than before, easing social interactions for the patient. Family or individual counseling may prove helpful in these situations.

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

Previous
Next

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
Previous
Proceed to Medication
 
 
Contributor Information and Disclosures
Author

Cheryl Ann Palmer, MD  Professor of Pathology and Neurology, Director, Neuropathology Fellowship Program, University of Alabama at Birmingham School of Medicine; Consulting Staff, Departments of Pathology and Neurology, University of Alabama at Birmingham Hospital; Consulting Staff, Departments of Pathology and Neurology, Veteran Affairs Medical Center; Consulting Staff, Department of Pathology, Children's Hospital of Alabama

Cheryl Ann Palmer, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuropathologists, Medical Association of the State of Alabama, Society for Neuro-Oncology, and Southern Medical Association

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 Academy of Pediatrics, American Epilepsy Society, and Child Neurology Society

Disclosure: Nothing to disclose.

Additional Contributors

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

References

  1. Von Graefe A, Saemisch T. Handbuch der gesammten Augenheilkunde. Leipzig, W Engelman. 1880;6:60.

  2. Mobius PJ. Uber angeboren doppelseitige abducens-facialis-lahmung. Munchen Medizinische Wochenschrift. 1888;35:91-4.

  3. Mobius PJ. Uber infantilen kernschwund. Munchen Medizinische Wochenschrift. 1892;39:17-21, 41-3, 55-8.

  4. Henderson JL. The congenital facial diplegia syndrome: clinical features, pathology and etiology. Brain. 1939;62:381-403.

  5. Sugarman GI, Stark HH. Mobius syndrome with Poland's anomaly. J Med Genet. 1973;10(2):192-6. [Medline].

  6. Towfighi J, Marks K, Palmer E, Vannucci R. Möbius syndrome. Neuropathologic observations. Acta Neuropathol (Berl). Oct 1979;48(1):11-7. [Medline].

  7. Verzijl HT, van der Zwaag B, Cruysberg JR, Padberg GW. Möbius syndrome redefined: a syndrome of rhombencephalic maldevelopment. Neurology. Aug 12 2003;61(3):327-33. [Medline].

  8. May M, Schaitkin BM. Facial Nerve Disorders in Newborns and Children. In: The Facial Nerve. 2nd ed. New York, NY: Thieme New York; 2000:339-65.

  9. Online Mendelian Inheritance in Man. %157900 - MOEBIUS SYNDROME; MBS. OMIM - Online Mendelian Inheritance in Man. Available at http://www.ncbi.nlm.nih.gov/omim/157900.

  10. Uzumcu A, Karaman B, Toksoy G, Uyguner ZO, Candan S, Eris H. Molecular genetic screening of MBS1 locus on chromosome 13 for microdeletions and exclusion of FGF9, GSH1 and CDX2 as causative genes in patients with Moebius syndrome. Eur J Med Genet. Sep-Oct 2009;52(5):315-20. [Medline].

  11. Ziter FA, Wiser WC, Robinson A. Three-generation pedigree of a Möbius syndrome variant with chromosome translocation. Arch Neurol. Jul 1977;34(7):437-42. [Medline].

  12. Slee JJ, Smart RD, Viljoen DL. Deletion of chromosome 13 in Möbius syndrome. J Med Genet. Jun 1991;28(6):413-4. [Medline].

  13. Kremer H, Kuyt LP, van den Helm B, van Reen M, Leunissen JA, Hamel BC, et al. Localization of a gene for Möbius syndrome to chromosome 3q by linkage analysis in a Dutch family. Hum Mol Genet. Sep 1996;5(9):1367-71. [Medline].

  14. Nishikawa M, Ichiyama T, Hayashi T, Furukawa S. Mobius-like syndrome associated with a 1;2 chromosome translocation. Clin Genet. 1997;51(2):122-3. [Medline].

  15. Baraitser M. Genetics of Möbius syndrome. J Med Genet. Dec 1977;14(6):415-7. [Medline].

  16. Pastuszak AL, Schüler L, Speck-Martins CE, Coelho KE, Cordello SM, Vargas F, et al. Use of misoprostol during pregnancy and Möbius' syndrome in infants. N Engl J Med. Jun 25 1998;338(26):1881-5. [Medline].

  17. Smets K, Zecic A, Willems J. Ergotamine as a possible cause of Möbius sequence: additional clinical observation. J Child Neurol. May 2004;19(5):398. [Medline].

  18. Puvabanditsin S, Garrow E, Augustin G, Titapiwatanakul R, Kuniyoshi KM. Poland-Möbius syndrome and cocaine abuse: a relook at vascular etiology. Pediatr Neurol. Apr 2005;32(4):285-7. [Medline].

  19. Kanemoto N, Kanemoto K, Kamoda T, Hasegawa M, Arinami T. A case of Moebius syndrome presenting with congenital bilateral vocal cord paralysis. Eur J Pediatr. Aug 2007;166(8):831-3. [Medline].

  20. Broussard AB, Borazjani JG. The faces of Moebius syndrome. Am J Matern Child Nurs. Sept/Oct, 2008;33(5):272-278.

  21. Moebius Syndrome Foundation. Available at http://www.moebiussyndrome.com/. Accessed February 21, 2012.

  22. Strömland K, Sjögreen L, Miller M, Gillberg C, Wentz E, Johansson M, et al. Mobius sequence--a Swedish multidiscipline study. Eur J Paediatr Neurol. 2002;6(1):35-45. [Medline].

  23. Kumar D. Moebius syndrome. J Med Genet. Feb 1990;27(2):122-6. [Medline].

  24. Briegel W. Neuropsychiatric findings of Mobius sequence -- a review. Clin Genet. Aug 2006;70(2):91-7. [Medline].

  25. Cohen SR, Thompson JW. Variants of Mobius' syndrome and central neurologic impairment. Lindeman procedure in children. Ann Otol Rhinol Laryngol. 1987;96(1 Pt 1):93-100. [Medline].

  26. Gillberg C, Steffenburg S. Autistic behaviour in Moebius syndrome. Acta Paediatr Scand. Mar 1989;78(2):314-6. [Medline].

  27. Johansson M, Wentz E, Fernell E, Strömland K, Miller MT, Gillberg C. Autistic spectrum disorders in Möbius sequence: a comprehensive study of 25 individuals. Dev Med Child Neurol. May 2001;43(5):338-45. [Medline].

  28. Verzijl HT, van Es N, Berger HJ, Padberg GW, van Spaendonck KP. Cognitive evaluation in adult patients with Möbius syndrome. J Neurol. Feb 2005;252(2):202-7. [Medline].

  29. Griz S, Cabral M, Azevedo G, Ventura L. Audiologic results in patients with Moebiüs sequence. Int J Pediatr Otorhinolaryngol. Sep 2007;71(9):1457-63. [Medline].

  30. Dooley JM, Stewart WA, Hayden JD, Therrien A. Brainstem calcification in Möbius syndrome. Pediatr Neurol. Jan 2004;30(1):39-41. [Medline].

  31. Singh B, Shahwan SA, Singh P, al-Deeb SM, Sharif H. Mobius syndrome with basal ganglia calcification. Acta Neurol Scand. Jun 1992;85(6):436-8. [Medline].

  32. Cattaneo L, Chierici E, Bianchi B, Sesenna E, Pavesi G. The localization of facial motor impairment in sporadic Möbius syndrome. Neurology. Jun 27 2006;66(12):1907-12. [Medline].

  33. Jaradeh S, D'Cruz O, Howard JF Jr, Haberkamp TJ, Konkol RJ. Mobius syndrome: electrophysiologic studies in seven cases. Muscle Nerve. Sep 1996;19(9):1148-53. [Medline].

  34. Magli A, Bonavolontà P, Forte R, Vassallo P. Lower eyelid surgery for lagophthalmos in Möbius and Poland-Möbius syndromes. J Craniofac Surg. Nov 2011;22(6):e53-4. [Medline].

  35. Marre D, Hontanilla B. Brain plasticity in Möbius syndrome after unilateral muscle transfer. Ann Plast Surg. 2011;Publish Ahead of Print.

  36. Briegel W, Hofmann C, Schwab KO. Behaviour problems of patients with Moebius sequence and parental stress. J Paediatr Child Health. Apr 2010;46(4):144-8. [Medline].

 
Previous
Next
 
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).
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.