莫比乌斯综合征的面部恢复-治疗双侧面瘫25年的经验

IF 2 3区 医学 Q2 SURGERY
Paul Panzenbeck , Cédric Zubler , Edel de Buitleir , Adriaan Ockert Grobbelaar , Jonathan Ian Leckenby
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引用次数: 0

摘要

莫比乌斯综合征是一种罕见的先天性神经系统疾病,以双侧面部(VII)和外展神经(VI)麻痹为特征,导致面瘫和眼外展受损。额外的脑神经受累和异常,如肢体畸形和神经发育异常,使其表现复杂化。使用自由功能肌肉移植的面部复活是恢复运动和改善社会心理结果的黄金标准。本研究介绍了超过25年的面部恢复手术治疗莫比乌斯综合征的经验。患者与方法回顾性分析1998年至2023年间接受双侧面部复生术的莫比乌斯综合征患者。结果是通过外科医生的评分、海氏量表的独立小组评分和自发微笑的评估来评估的。我们描述了我们的手术技术,术后治疗的关键方面和潜在的陷阱。结果56例患者(女性30例,男性26例)接受了面部修复术。其中49例为完全莫比乌斯综合征。93%的病例使用部分背阔肌皮瓣,91%的病例使用咬肌神经作为神经输入。96%的患者面部对称性和功能被评为优秀(n=43)或良好(n=11)。自发性微笑在59%的病例中得以实现,其中咬肌神经支配的背阔肌移植效果最好。结论双侧游离功能肌转移是治疗莫比乌斯综合征面部恢复的金标准,可显著改善儿童面部功能和美观。我们推荐双侧面部再生作为单阶段手术,因为它对这类患者的生理和心理都有好处。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Facial reanimation in Moebius syndrome – 25-Year experience in treating bilateral facial paralysis

Introduction

Moebius syndrome is a rare congenital neurological disorder characterised by bilateral facial (VII) and abducens (VI) nerve palsies, resulting in facial paralysis and impaired ocular abduction. Additional cranial nerve involvement and anomalies, such as limb deformities and neurodevelopmental abnormalities, complicate its presentation. Facial reanimation using a free functional muscle transfer is the gold standard for restoring movement and improving psychosocial outcomes. This study presents over 25 years of surgical experience in facial reanimation for Moebius syndrome management.

Patients and methods

A retrospective analysis of patients with Moebius syndrome who underwent bilateral facial reanimation between 1998 and 2023 was conducted. Outcomes were assessed using a surgeon’s score, independent panel scoring with the Hay’s rating scale and evaluation of spontaneous smile. We describe our surgical technique, key aspects of post-operative therapy and potential pitfalls.

Results

Overall, 56 patients (30 women and 26 men) underwent facial reanimation. Among them, 49 had complete Moebius syndrome. Partial latissimus dorsi muscle flaps were used in 93% of the cases, with the masseteric nerve as the neural input (91%). Facial symmetry and function were rated as excellent (n=43) or good (n=11) in 96% of the patients. A spontaneous smile was achieved in 59% of the cases, with the best outcomes in masseteric-innervated latissimus dorsi transfers.

Conclusion

Bilateral free functional muscle transfers are the gold standard for facial reanimation in Moebius syndrome management, yielding significant functional and aesthetic improvements, especially in children. We recommend performing bilateral facial reanimation as a single-stage procedure due to its physical and psychological benefits for this patient population.
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来源期刊
CiteScore
3.10
自引率
11.10%
发文量
578
审稿时长
3.5 months
期刊介绍: JPRAS An International Journal of Surgical Reconstruction is one of the world''s leading international journals, covering all the reconstructive and aesthetic aspects of plastic surgery. The journal presents the latest surgical procedures with audit and outcome studies of new and established techniques in plastic surgery including: cleft lip and palate and other heads and neck surgery, hand surgery, lower limb trauma, burns, skin cancer, breast surgery and aesthetic surgery.
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