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Semin Plast Surg 2004; 18(1): 39-45
DOI: 10.1055/s-2004-823122
Copyright © 2004 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001 USA.

Möbius Syndrome

Janakie Singham1 , Ralph Manktelow2 , Ronald M. Zuker3
  • 1Queens University Faculty of Medicine, Kingston, ON, Canada
  • 2University of Toronto Hand Program, University of Toronto, Toronto, ON, Canada
  • 3Division of Plastic Surgery, Hospital for Sick Children, University of Toronto, Toronto, ON, Canada
Further Information

Publication History

Publication Date:
20 April 2004 (online)

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Möbius syndrome is classically defined as combined congenital bilateral facial and abducens nerve palsies, although it may also be associated with a myriad of other craniofacial, musculoskeletal, cardiothoracic, endocrinologic, and developmental disorders. The problem that most patients complain about, however, is the inability to smile and close their lips while eating. Although the etiology of this syndrome is still unknown, scientific support has been growing for the hypothesis that it is due to an embryological disruption of subclavian artery development. The treatment of choice for facial reanimation in these patients is a neurovascular free muscle transfer, ideally using the gracilis muscle with direct repair of the gracilis muscle's motor nerve to the masseteric branch of the trigeminal nerve. If the masseteric nerve is unavailable, a partial hypoglossal or accessory nerve may be used. These operations, enhanced by the effects of cerebral plasticity, may allow Möbius patients to reach their goals of satisfactory spontaneous smiles.

KEYWORDS

Möbius syndrome - facial paralysis - facial diplegia - facial reanimation - gracilis flap transfer

REFERENCES

  • 1 Von Graefe A. In: Von Graefe A, Saemisch T Handbuch der Gesamten Augenheilkunde. (Handbook of Ophthalmology). Vol 6. Leipzig; W Engelmann 1880: 60-67
    Google Scholar
  • 2 Möbius P J. Über angeborene doppelseitige Abducens-Facialis lähmung.  Muench Med Wochenschr. 1888;  35 91-108
    PubMedGoogle Scholar
  • 3 Möbius P J. Über infantilen Kernschwund.  Muench Med Wochenschr. 1892;  39 17-58
    PubMedGoogle Scholar
  • 4 Carr M M, Ross D A, Zuker R M. Cranial nerve defects in congenital facial palsy.  J Otolaryngol. 1997;  26 80-87
    PubMedGoogle Scholar
  • 5 Henderson J L. The congenital facial diplegia syndrome: clinical features, pathology and aetiology. A review of 61 cases.  Brain. 1939;  62 381-403
    PubMedGoogle Scholar
  • 6 Kumar D. Moebius syndrome.  J Med Genet. 1990;  27 122-126
    PubMedGoogle Scholar
  • 7 Reed H, Grant W. Möbius's syndrome.  Br J Ophthalmol. 1957;  41 731-740
    PubMedGoogle Scholar
  • 8 Van Allen M W, Blodi F C. Neurologic aspects of the Möbius syndrome. A case study with electromyography of the extraocular and facial muscles.  Neurology. 1960;  10 249-259
    PubMedGoogle Scholar
  • 9 Bedrossian E H, Lachman B E. Congenital paralysis of sixth and seventh nerve.  Am J Ophthalmol. 1956;  41 304-307
    PubMedGoogle Scholar
  • 10 Rubin L R. The Moebius syndrome: bilateral facial diplegia.  Clin Plast Surg. 1976;  3 625-636
    PubMedGoogle Scholar
  • 11 Cronemberger M F, de Castro Moreira J B, Brunoni D et al.. Ocular and clinical manifestations of Möbius' syndrome.  J Pediatr Ophthalmol Strabismus. 2001;  38 156-162
    PubMedGoogle Scholar
  • 12 Miller M T, Stromland K. The Möbius sequence: a relook.  J AAPOS. 1999;  3 (4) 199-207
    PubMedGoogle Scholar
  • 13 Evans P R. Nuclear agenesis: Möbius syndrome: the congenital facial diplegia syndrome.  Arch Dis Child. 1955;  30 237-242
    PubMedGoogle Scholar
  • 14 Bosch-Banyeras J M, Zuasnabar A, Puig A, Catala M, Cuatrecasas J M. Poland-Möbius syndrome associated with dextrocardia.  J Med Genet. 1984;  21 70-71
    PubMedGoogle Scholar
  • 15 Gadoth N, Biedner B, Torok G. Möbius syndrome and Poland anomaly: case report and review of literature.  J Pediatr Ophthalmol Strabismus. 1979;  16 374-376
    PubMedGoogle Scholar
  • 16 Hanissian A S, Fuste F, Hayes W T, Duncan J M. Möbius syndrome in twins.  Am J Dis Child. 1970;  120 472-475
    PubMedGoogle Scholar
  • 17 Kuhn M J. Radiologic findings in Möbius syndrome.  Mt Sinai J Med. 1988;  55 (2) 166-170
    PubMedGoogle Scholar
  • 18 Raroque Jr H G, Hershewe G L, Snyder R D. Möbius syndrome and transposition of the great vessels.  Neurology. 1988;  38 1894-1895
    PubMedGoogle Scholar
  • 19 Parker D L, Mitchell P R, Holmes G L. Poland-Möbius syndrome.  J Med Genet. 1981;  18 317-320
    PubMedGoogle Scholar
  • 20 Caravella L, Rogers G. Dextrocardia and ventricular septal defect in the Möbius syndrome.  Ann Ophthalmol. 1978;  10 572-575
    PubMedGoogle Scholar
  • 21 Sugarman G I, Stark H H. Möbius syndrome with Poland's anomaly.  J Med Genet. 1973;  10 192-196
    PubMedGoogle Scholar
  • 22 Saito H, Takeda T, Kishimoto S. Neonatal facial nerve defect.  Acta Otolaryngol Suppl. 1994;  510 77-81
    PubMedGoogle Scholar
  • 23 Bouwes-Bavinck J N, Weaver D D. Subclavian artery supply disruption sequence: hypothesis of a vascular etiology for Poland, Klippel-Feil, and Möbius anomalies.  Am J Med Genet. 1986;  23 903-918
    CrossrefPubMedGoogle Scholar
  • 24 Preis S, Majewski F, Hantschmann R, Schumacher H, Lenard H G. Goldenhar, Möbius and hypoglossia-hypodactyly anomalies in a patient: syndrome or association?.  Eur J Pediatr. 1996;  155 385-389
    CrossrefPubMedGoogle Scholar
  • 25 Schimke R N, Collins D L, Hiebert J M. Congenital nonprogressive myopathy with Möbius and Robin sequence-the Carey-Fineman-Ziter syndrome: a confirmatory report.  Am J Med Genet. 1993;  46 721-723
    CrossrefPubMedGoogle Scholar
  • 26 Kawai M, Momoi T, Nakano S, Itagaki Y, Mikawa H. The syndrome of Möbius sequence, peripheral neuropathy, and hypogonadotropic hypogonadism.  Am J Med Genet. 1990;  37 578-582
    CrossrefPubMedGoogle Scholar
  • 27 Rubinstein A E, Lovelace R E, Behrens M M, Weisberg L A. Moebius syndrome in association with peripheral neuropathy and Kallmann syndrome.  Arch Neurol. 1975;  32 480-482
    PubMedGoogle Scholar
  • 28 Olson W H, Bardin C W, Walsh G O, Engel W K. Moebius syndrome. Lower motor neuron involvement and hypogonadotrophic hypogonadism.  Neurology. 1970;  20 1002-1008
    PubMedGoogle Scholar
  • 29 Ichiyama T, Handa S, Hayashi T, Furukawa S. Premature thelarche in Möbius syndrome.  Clin Genet. 1995;  47 108-109
    PubMedGoogle Scholar
  • 30 De Serpa Pinto M V, De Magalhaes M H, Nunes F D. Moebius syndrome with oral involvement.  Int J Paediatr Dent. 2002;  12 446-449
    CrossrefPubMedGoogle Scholar
  • 31 Sjogreen L, Andersson-Norinder J, Jacobsson C. Development of speech, feeding, eating, and facial expression in Möbius sequence.  Int J Pediatr Otorhinolaryngol. 2001;  60 197-204
    CrossrefPubMedGoogle Scholar
  • 32 Rizos M, Negron R J, Serman N. Möbius syndrome with dental involvement: a case report and literature review.  Cleft Palate Craniofac J. 1998;  35 (3) 262-268
    PubMedGoogle Scholar
  • 33 Gusek-Shneider G, Langenbucher A, Seits B. Association of keratoconus and Möbius syndrome.  J Pediatr Ophthalmol Strabismus. 2001;  38 47-48
    PubMedGoogle Scholar
  • 34 Fujita I, Koyanagi T, Yamashita H et al.. Moebius syndrome with central hypoventilation and brainstem calcification: a case report.  Eur J Pediatr. 1991;  150 582-583
    PubMedGoogle Scholar
  • 35 Gilmore R L, Falace P, Kanga J, Baumann R. Sleep disordered breathing in Möbius syndrome.  J Child Neurol. 1991;  6 73-77
    PubMedGoogle Scholar
  • 36 Konkol R J, D'Cruz O, Splainguard M. Central respiratory dysfunction in Möbius syndrome.  J Child Neurol. 1991;  6 278-279
    PubMedGoogle Scholar
  • 37 Gorlin R J, Chen M M, Levin L S. Syndromes of the Head and Neck. 3rd ed. New York; Oxford University Press 1990
    Google Scholar
  • 38 Szajnberg N M. Möbius syndrome: alternatives in affective communication.  Dev Med Child Neurol. 1994;  36 459-462
    PubMedGoogle Scholar
  • 39 Giannini A J, Tamulonis D, Giannini M C, Loiselle R H, Spirtos G. Defective response to social cues in Möbius syndrome.  J Nerv Ment Dis. 1984;  172 174-175
    PubMedGoogle Scholar
  • 40 Kahane J C. Pathophysiological effects of Möbius syndrome on speech and hearing.  Arch Otolaryngol. 1979;  105 29-34
    PubMedGoogle Scholar
  • 41 Saito S, Kishimoto S, Furuta M. Temporal bone findings in a patient with Möbius syndrome.  Ann Otol Rhinol Laryngol. 1981;  90 80-84
    PubMedGoogle Scholar
  • 42 MacDermot K D, Winter R M, Baraitser M. Oculofacialbulbar palsy in mother and son: review of 26 reports of familial transmission within the ‘Möbius spectrum of defects’.  J Med Genet. 1991;  28 18-26
    PubMedGoogle Scholar
  • 43 Gillberg C, Winnergard I. Childhood psychosis in a case of Möbius syndrome.  Neuropediatrics. 1984;  15 147-149
    PubMedGoogle Scholar
  • 44 Lipson A H, Gillerot Y, Tannenberg A E, Giurgea S. Two cases of maternal antenatal splenic rupture and hypotension associated with Moebius syndrome and cerebral palsy in offspring. Further evidence for a utero placental vascular aetiology for the Moebius syndrome and some cases of cerebral palsy.  Eur J Pediatr. 1996;  155 800-804
    CrossrefPubMedGoogle Scholar
  • 45 Johansson M, Wentz E, Fernell E, Stromland K, Miller M T, Gillberg C. Autistic spectrum disorders in Möbius sequence: a comprehensive study of 25 individuals.  Dev Med Child Neurol. 2001;  43 338-345
    PubMedGoogle Scholar
  • 46 Keane J R. Bilateral seventh nerve palsy: analysis of 43 cases and review of the literature.  Neurology. 1994;  44 1198-1202
    PubMedGoogle Scholar
  • 47 Yoon K H, Yoo S J, Suh D C, Kim K S, Choe G. Möbius syndrome with brainstem calcification: prenatal and neonatal sonographic findings.  Pediatr Radiol. 1997;  27 150-152
    CrossrefPubMedGoogle Scholar
  • 48 Sherer D M, Spafford P. Prenatal sonographic evidence supporting an in utero developmental etiology of Möbius sequence.  Am J Perinatol. 1994;  11 (2) 157-159
    PubMedGoogle Scholar
  • 49 Miller M T, Ray V, Owens P, Chen F. Möbius and Möbius-like syndromes (TTV-OFM, OMLH).  J Pediatr Ophthalmol Strabismus. 1989;  26 176-188
    PubMedGoogle Scholar
  • 50 Hanson P A, Rowland L P. Möbius syndrome and fascioscapulohumeral muscular dystrophy.  Arch Neurol. 1971;  24 31-39
    PubMedGoogle Scholar
  • 51 Hall B D. Aglossia-adactylia.  Birth Defects Orig Artic Ser. 1971;  7 233-236
    PubMedGoogle Scholar
  • 52 Wallis P G. Creatinuria in Möbius syndrome.  Arch Dis Child. 1960;  35 393-395
    PubMedGoogle Scholar
  • 53 Hopper K D, Haas D K, Rice M M, Freeley D A, Taubner R W, Ghaed N. Poland-Möbius syndrome: evaluation by computerized tomography.  South Med J. 1985;  78 523-527
    PubMedGoogle Scholar
  • 54 Merz M, Wojtowicz S. The Möbius syndrome: report of electromyographic examinations in two cases.  Am J Ophthalmol. 1967;  63 837-840
    PubMedGoogle Scholar
  • 55 D'Çruz O F, Swisher C N, Jaradeh S, Tang T, Konkol R J. Möbius syndrome: evidence for a vascular etiology.  J Child Neurol. 1993;  8 260-265
    PubMedGoogle Scholar
  • 56 Lammens M, Moerman P, Fryns J P et al.. Neuropathological findings in Moebius syndrome.  Clin Genet. 1998;  54 136-141
    PubMedGoogle Scholar
  • 57 Singh B, Shawan S A, Singh P, Al-Deep S M, Sharif H. Möbius syndrome with basal ganglia calcifications.  Acta Neurol Scand. 1992;  85 436-438
    PubMedGoogle Scholar
  • 58 Harbord M G, Finn J P, Hall-Craggs M A, Baraitser M. Moebius syndrome with unilateral cerebellar hypoplasia.  J Med Genet. 1989;  26 579-582
    PubMedGoogle Scholar
  • 59 Pitner S E, Edwards J F, McCormick W F. Observations on the pathology of the Moebius syndrome.  J Neurol Neurosurg Psychiatry. 1965;  28 362-374
    PubMedGoogle Scholar
  • 60 Kuhn M J, Clark B, Morales A, Shekar P C. Group III Möbius syndrome: CT and MR findings.  AJNR. 1990;  11 903-904
    PubMedGoogle Scholar
  • 61 Govaert P, Vanhaesebrouck P, De Praeter C, Frankel U, Leroy J. Moebius sequence and prenatal brain ischemia.  Pediatrics. 1989;  84 570-573
    PubMedGoogle Scholar
  • 62 St. Charles S, Di Mario Jr F J, Grunnet M L. Möbius sequence: further in vivo support for subclavian artery supply disruption sequence.  Am J Med Genet. 1993;  47 289-293
    CrossrefPubMedGoogle Scholar
  • 63 Pedraza S, Gamez J, Zamora A, Grive E, Raguer N, Ruscalleda J. MRI findings in Möbius syndrome: correlation with clinical features.  Neurology. 2000;  55 1058-1060
    PubMedGoogle Scholar
  • 64 Mitter N S, Chudley A E. Facial weakness and oligosyndactyly:? independent variable features of familial type of the Möbius syndrome.  Clin Genet. 1983;  24 350-354
    PubMedGoogle Scholar
  • 65 Shepard T H. Möbius syndrome after misoprostol: a possible teratogenic mechanism.  Lancet. 1995;  346 780
    PubMedGoogle Scholar
  • 66 Gonzalez C H, Vargas F R, Perez A B et al.. Limb deficiency with or without Möbius sequence in seven Brazilian children associated with misoprostol use in the first trimester of pregnancy.  Am J Med Genet. 1993;  47 59-64
    CrossrefPubMedGoogle Scholar
  • 67 Lipson A H, Webster W S, Brown-Woodman P D et al.. Möbius syndrome: animal model-human correlations and evidence for a brainstem vascular etiology.  Teratology. 1989;  40 339-350
    CrossrefPubMedGoogle Scholar
  • 68 Graham J M, Edwards M J, Lipson A H, Webster W S. Gestational hyperthermia as a cause for Moebius syndrome.  Teratol. 1988;  37 461-462
    PubMedGoogle Scholar
  • 69 Graf W D, Shepard T H. Uterine contraction in the development of Möbius syndrome.  J Child Neurol. 1997;  12 225-227
    PubMedGoogle Scholar
  • 70 Courtens W, Vamos E, Hainaut M, Vergauwen P. Moebius syndrome in an infant exposed to in utero benzodiazepines.  J Pediatr. 1992;  121 833-834
    PubMedGoogle Scholar
  • 71 Elsahy N I. Moebius syndrome associated with the mother taking thalidomide during gestation.  Plast Reconstr Surg. 1973;  51 93-95
    PubMedGoogle Scholar
  • 72 Vargas F R, Schuler-Faccini L, Brunoni D et al.. Prenatal exposure to misoprostol and vascular disruption defects: a case control study.  Am J Med Genet. 2000;  95 302-306
    CrossrefPubMedGoogle Scholar
  • 73 Pastuszak A L, Schuler L, Speck-Martins C E et al.. Use of misoprostol during pregnancy and Möbius syndrome in infants.  N Engl J Med. 1998;  338 1881-1885
    CrossrefPubMedGoogle Scholar
  • 74 Carlson B M. The development of facial muscles and nerves in relation to Möbius syndrome.  Otolaryngol Head Neck Surg. 1981;  89 903-906
    PubMedGoogle Scholar
  • 75 Legum C, Godel V, Nemet P. Heterogeneity and pleiotropism in the Moebius syndrome.  Clin Genet. 1981;  20 254-259
    PubMedGoogle Scholar
  • 76 Becker-Christensen F, Lund H T. A family with Möbius syndrome.  J Pediatr. 1974;  84 115-117
    PubMedGoogle Scholar
  • 77 Journel H, Roussey M, Le Marec B. MCA/MR syndrome with oligodactyly and Möbius anomaly in first cousins: new syndrome or familial facial-limb disruption sequence?.  Am J Med Genet. 1989;  34 506-510
    CrossrefPubMedGoogle Scholar
  • 78 Nishikawa M, Ichiyama T, Hayashi T, Furukawa S. Möbius syndrome associated with a I;2 chromosome translocation.  Clin Genet. 1997;  51 122-123
    PubMedGoogle Scholar
  • 79 Slee J J, Smart R D, Viljoen D L. Deletion of chromosome 13 in Moebius syndrome.  J Med Genet. 1991;  28 413-414
    PubMedGoogle Scholar
  • 80 Ziter F A, Wiser W C, Robinson A. Three-generation pedigree of a Möbius syndrome variant with chromosomal translocation.  Arch Neurol. 1977;  34 437-442
    PubMedGoogle Scholar
  • 81 Donahue S P, Wenger S L, Steele M W, Gorin M B. Broad-spectrum Möbius syndrome associated with a 1;11 chromosome translocation.  Ophthalmic Paediatr Genet. 1993;  14 17-21
    PubMedGoogle Scholar
  • 82 Borck G, Wirth J, Hardt T et al.. Molecular cytogenetic characterization of a complex 46XY, t(7;8;11;13) chromosome rearrangement in a patient with Moebius syndrome.  J Med Genet. 2001;  38 117-120
    CrossrefPubMedGoogle Scholar
  • 83 Verzijl H TFM, van den Helm B, Hamel B CJ et al.. A second gene for autosomal dominant Möbius syndrome is localized to chromosome 10q in a Dutch family.  Am J Hum Genet. 1999;  65 752-756
    CrossrefPubMedGoogle Scholar
  • 84 Kremer H, Kuyt L P, van den Helm B et al.. Localization of a gene for Möbius syndrome to chromosome 3q by linkage analysis in a Dutch family.  Hum Mol Genet. 1996;  5 1367-1371
    CrossrefPubMedGoogle Scholar
  • 85 Wilmore H P, Smith M J, Wilcox S J, Bell K M, Sinclair A H. Sox 14 is a candidate gene for limb defects associated with BPES and Möbius syndrome.  Hum Genet. 2000;  106 269-276
    PubMedGoogle Scholar
  • 86 Temtamy S A, McKusick V A. The genetics of hand malformations.  Birth Defects Orig Artic Ser. 1978;  14 (3) i-xviii 1-619
    PubMedGoogle Scholar
  • 87 Towghfi J, Marks K, Palmer E, Vannucci R. Möbius syndrome: neuropathological observations.  Acta Neuropathol (Berl). 1979;  48 11-17
    CrossrefPubMedGoogle Scholar
  • 88 Abramson D L, Cohen Jr M M, Mulliken J B. Möbius syndrome: classification and grading system.  Plast Reconstr Surg. 1998;  102 961-967
    CrossrefPubMedGoogle Scholar
  • 89 Terzis J K, Noah E M. Möbius and Möbius-like patients: etiology, diagnosis and treatment options.  Clin Plast Surg. 2002;  29 497-514
    PubMedGoogle Scholar
  • 90 Terzis J K, Noah E M. Dynamic restoration in Möbius and Möbius-like patients.  Plast Reconstr Surg. 2003;  111 40-55
    CrossrefPubMedGoogle Scholar
  • 91 Waterhouse W J, Enzenhauer R W, Martyak A P. Successful strabismus surgery in a child with Moebius syndrome.  Ann Ophthalmol. 1993;  25 292-294
    PubMedGoogle Scholar
  • 92 Puckett C L, Beg S A. Facial reanimation in Möbius syndrome.  South Med J. 1978;  71 1498-1501
    PubMedGoogle Scholar
  • 93 Morello D C, Converse J M. Moebius syndrome. Case report was a 30 year follow-up.  Plast Reconstr Surg. 1977;  60 451-453
    PubMedGoogle Scholar
  • 94 Edgerton M T, Tuerk D B, Fisher J C. Surgical treatment of Moebius syndrome by platysma and temporalis muscle transfers.  Plast Reconstr Surg. 1975;  55 305-311
    PubMedGoogle Scholar
  • 95 Eppley B L, Zuker R M. Salvage of facial reanimation with vascularized adductor magnus flap: clinical experience and anatomical studies.  Plast Reconstr Surg. 2002;  110 (7) 1693-1696
    PubMedGoogle Scholar
  • 96 Zuker R M, Goldberg C S, Manktelow R T. Facial animation in children with Möbius syndrome after segmental gracilis muscle transplant.  Plast Reconstr Surg. 2000;  106 (1) 1-8
    CrossrefPubMedGoogle Scholar
  • 97 Harrison D H. In discussion: Zuker RM, Goldberg CS, Manktelow RT Facial animation in children with Möbius syndrome after segmental gracilis muscle transplant. Plast Reconstr Surg. 2000 106: 9
    Google Scholar
  • 98 Zuker R M, Manktelow R T. A smile for the Möbius syndrome patient.  Ann Plast Surg. 1989;  22 188-194
    PubMedGoogle Scholar
  • 99 Manktelow R T. Microvascular Reconstruction. New York; Springer-Verlag 1986
    Google Scholar

Janakie SinghamB.Sc. 

EN7-228, 200 Elizabeth Street, Toronto, ON

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