Dynamic restoration in Mobius and Mobius-Like patients

被引:67
作者
Terzis, JK
Noah, EM
机构
[1] Eastern Virginia Med Sch, Div Plast & Reconstruct Surg, Microsurg Res Ctr, Int Inst Reconstruct Microsurg,Dept Surg, Norfolk, VA 23507 USA
[2] Univ Aachen, Dept Plast Hand & Reconstruct Surg, D-5100 Aachen, Germany
关键词
D O I
10.1097/01.PRS.0000037878.89189.DB
中图分类号
R61 [外科手术学];
学科分类号
摘要
Mobius syndrome is classically characterized by bilateral facial nerve and abducens nerve paralysis in combination with limb defects. In the past 110 years, physicians diagnosed children as having the syndrome on the basis of heterogeneity of symptoms and used the term "Mobius syndrome" or "Mobius-like syndrome" for patients with multiple cranial nerve involvement. The cause and the exact pathogenesis of the syndrome still elude understanding. Genetic work-ups, radiological findings, and data from autopsies differ in their approaches and their findings of the basic causes of Mobius syndrome. In the international literature, about 301 case reports are found scattered through the past century. The appearance of the facial deformity is easy to recognize, because the Mobius patient is impaired in his or her ability to communicate nonverbally. Despite ophthalmologic problems, it is the search for a smile that brings these patients to the reconstructive surgeon. Over the past 100 years, surgical efforts attempted to improve the mask-like appearance by static and dynamic procedures, usually local muscle transpositions. Today, combinations of microsurgical procedures mid aesthetic techniques are being used to restore some movement to the expressionless face of these patients by nerve and muscle transplantation. This article discusses the heterogeneity of Mobius syndrome, advocates a new classification system, presents the clinical findings of 42 patients who were seen and examined in consultation, and discusses the surgical management of 20 patients who underwent dynamic restorative microsurgery. Exemplary cases illustrating the preoperative work-up regimen and possible outcomes are reported.
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页码:40 / 55
页数:16
相关论文
共 94 条
[1]   Mobius syndrome: Classification and grading system [J].
Abramson, DL ;
Cohen, MM ;
Mulliken, JB .
PLASTIC AND RECONSTRUCTIVE SURGERY, 1998, 102 (04) :961-967
[2]   GENETICS OF MOBIUS SYNDROME [J].
BARAITSER, M .
JOURNAL OF MEDICAL GENETICS, 1977, 14 (06) :415-417
[3]   SUBCLAVIAN ARTERY SUPPLY DISRUPTION SEQUENCE - HYPOTHESIS OF A VASCULAR ETIOLOGY FOR POLAND, KLIPPEL-FEIL, AND MOBIUS ANOMALIES [J].
BAVINCK, JNB ;
WEAVER, DD .
AMERICAN JOURNAL OF MEDICAL GENETICS, 1986, 23 (04) :903-918
[4]  
Blanchard K, 1998, NEW ENGL J MED, V339, P1553
[5]   Molecular cytogenetic characterisation of a complex 46,XY,t(7;8;11;13) chromosome rearrangement in a patient with Moebius syndrome [J].
Borck, G ;
Wirth, J ;
Hardt, T ;
Tönnies, H ;
Brondum-Nielsen, K ;
Bugge, M ;
Tommerup, N ;
Nothwang, HG ;
Ropers, HH ;
Haaf, T .
JOURNAL OF MEDICAL GENETICS, 2001, 38 (02) :117-120
[6]  
Bothra G C, 1972, J Assoc Physicians India, V20, P187
[7]  
CARAVELLA L, 1978, ANN OPHTHALMOL, V10, P572
[8]  
Carr MM, 1997, J OTOLARYNGOL, V26, P80
[9]  
CASTROVIEJO IP, 1968, ARCH NEUROBIOL MADR, V31, P287
[10]  
Chrisholm JJ, 1882, ARCH OPHTHALMOL-CHIC, V11, P323