Terzis Julia K, Noah Ernst Magnus
International Institute of Reconstructive Microsurgery, Microsurgical Research Center, Department of Surgery, Division of Plastic and Reconstructive Surgery, Eastern Virginia Medical School, Norfolk 23507, USA.
Plast Reconstr Surg. 2003 Jan;111(1):40-55. doi: 10.1097/01.PRS.0000037878.89189.DB.
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.
莫比乌斯综合征的典型特征是双侧面神经和展神经麻痹并伴有肢体缺陷。在过去的110年里，医生根据症状的异质性诊断儿童患有该综合征，并将“莫比乌斯综合征”或“类莫比乌斯综合征”这一术语用于患有多组颅神经受累的患者。该综合征的病因及确切发病机制仍不清楚。基因检查、影像学检查结果以及尸检数据在莫比乌斯综合征基本病因的研究方法和结果方面存在差异。在国际文献中，在过去的一个世纪里大约散见301例病例报告。面部畸形的外观很容易识别，因为莫比乌斯综合征患者的非语言交流能力受损。尽管存在眼科问题，但正是对微笑的追求促使这些患者前来寻求整形医生的帮助。在过去的100年里，外科手术试图通过静态和动态手术，通常是局部肌肉移位，来改善面具样外观。如今，显微外科手术和美学技术相结合，通过神经和肌肉移植，试图让这些患者毫无表情的面部恢复一些活动能力。本文讨论了莫比乌斯综合征的异质性，提倡一种新的分类系统，介绍了42例会诊患者的临床检查结果，并讨论了20例接受动态修复显微手术患者的外科治疗情况。报告了说明术前检查方案及可能结果的典型病例。
