Maxillofacial Surgery, Head and Neck Department, University of Parma, 14-43100 Parma, Italy.
J Pediatr Surg. 2009 Nov;44(11):2236-42. doi: 10.1016/j.jpedsurg.2009.07.038.
Moebius syndrome, a rare congenital disorder of varying severity, involves multiple cranial nerves and is characterized predominantly by bilateral or unilateral paralysis of the facial and abducens nerves. The paralysis of the VI and VII cranial nerves leads to a lack of function in the muscles they supply. Facial paralysis often causes bilabial incompetence with speech difficulties, oral incompetence, problems with eating and drinking, including pocketing of food in the cheek and dribbling, as well as severe drooling.
In this study, we report on pediatric patients with Moebius and Moebius-like syndromes seen and treated surgically from 2003 to September 2007 at the Operative Unit of Maxillofacial Surgery, Head and Neck Department, University of Parma, Italy.
Twelve patients underwent microsurgical reconstruction for restoration of facial movement. The contralateral facial nerve was used as a motor donor nerve in 4 procedures, the motor nerve to the masseter muscle was used in 8 patients, and the gracilis muscle was used in all operations, with a total of 17 free-muscle transplantations. All free-muscle transplantations survived transfer, and no flap was lost. We observed a significant improvement in drooling, drinking, speech, and facial animation with a high degree of patient satisfaction.
The gracilis muscle free transfer is a surgical procedure well tolerated by the young patients and well accepted by their families. We consider it a safe and reliable technique for facial reanimation with good aesthetical and functional results in children with Moebius and Moebius-like syndromes.
Moebius 综合征是一种罕见的先天性疾病，严重程度不同，涉及多条颅神经，主要表现为双侧或单侧面神经和展神经麻痹。VI 及 VII 对颅神经的麻痹导致其所支配的肌肉功能丧失。面瘫常导致双唇闭合不全，出现言语困难、口腔闭合不全、吞咽困难，包括食物在颊部堆积和流涎，以及严重的流涎。
本研究报告了 2003 年至 2007 年 9 月在意大利帕尔马大学颌面外科、头颈部手术操作单位就诊和接受手术治疗的 Moebius 和 Moebius 样综合征的儿科患者。
12 例患者接受了显微外科重建以恢复面部运动。4 例手术中使用对侧面神经作为运动供体神经，8 例患者使用咬肌运动神经，所有手术均使用阔筋膜张肌，共进行了 17 次游离肌肉移植。所有游离肌肉移植均存活并转移，无皮瓣丢失。我们观察到流涎、饮水、言语和面部表情均有显著改善，患者满意度高。
阔筋膜张肌游离移植是一种年轻患者能够很好耐受且其家庭能够很好接受的手术。我们认为，对于 Moebius 和 Moebius 样综合征的儿童，该技术是一种安全可靠的面部再神经化技术，具有良好的美学和功能效果。
