Dynamic restoration in Möbius and Möbius-like patients.
Möbius 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 "Möbius syndrome" or "Möbius-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 Möbius 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 Möbius 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 and 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 Möbius 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.
Terzis
Julia K
JK
International Institute of Reconstructive Microsurgery, Microsurgical Research Center, Department of Surgery, Division of Plastic and Reconstructive Surgery, Eastern Virginia Medical School, Norfolk 23507, USA. mrc1@erols.com
Noah
Ernst Magnus
EM
D000328:Adult; D002648:Child; D002675:Child, Preschool; D003391:Cranial Nerves / Q000503:physiopathology; D004576:Electromyography; D005149:Facial Expression; D005152:Facial Muscles / Q000294:innervation*; D005154:Facial Nerve / Q000503:physiopathology / Q000601:surgery*; D005158:Facial Paralysis / Q000150:complications / Q000503:physiopathology / Q000601:surgery*; D005260:Female; D006085:Graft Survival; D006801:Humans; D008297:Male; D008866:Microsurgery; D020331:Mobius Syndrome / Q000150:complications / Q000503:physiopathology / Q000601:surgery*; D018482:Muscle, Skeletal / Q000637:transplantation*; D016067:Nerve Transfer; D011183:Postoperative Complications; D012189:Retrospective Studies