临床儿科杂志 ›› 2022, Vol. 40 ›› Issue (3): 161-164.doi: 10.12372/jcp.2022.21e0061

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脊髓性肌萎缩症合并脊柱侧凸的临床诊治

杨军林, 隋文渊, 张天元   

  1. 上海交通大学医学院附属新华医院脊柱中心(上海 200092)
  • 收稿日期:2022-01-10 出版日期:2022-03-15 发布日期:2022-03-09
  • 基金资助:
    国家自然科学基金面上项目(82072519);上海申康医院发展中心临床科技创新项目(SHDC22020206)

Clinical diagnosis and treatment of spinal muscular atrophy with scoliosis

YANG Junlin, SUI Wenyuan, ZHANG Tianyuan   

  1. Spine Center, Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai 200092, China
  • Received:2022-01-10 Online:2022-03-15 Published:2022-03-09

摘要:

脊髓性肌萎缩症(SMA)是一种以脊髓前角α运动神经元退行性病变、进行性近端肌无力为特征的严重神经肌肉疾病,随着疾病的进展,还会引发包括骨骼系统、呼吸系统、消化系统等其他多系统疾病,其中脊柱侧凸是最常见的骨骼系统并发症。文章介绍SMA合并脊柱侧凸的自然史,以及不同程度脊柱侧凸的临床保守治疗和手术治疗方法,重点阐述了多学科团队术前评估在SMA治疗中的重要意义和严重脊柱侧凸的外科手术策略,总结和介绍SMA合并脊柱侧凸的临床诊治经验,为精准外科治疗提供可参考的思路。

关键词: 脊髓性肌萎缩症, 脊柱侧凸, 保守治疗, 手术治疗

Abstract:

Spinal muscular atrophy (SMA) is a severe neuromuscular disease characterized by the degeneration of α motor neuron in the spinal cord and progressive proximal muscle weakness. It will also cause other multisystem diseases such as skeletal system, respiratory system and digestive system with the development of the disease process. Among them, scoliosis is the commonest orthopaedic complication. This article introduces the natural history of SMA combined with scoliosis, as well as the conservative treatment and surgical treatment methods for different degrees of scoliosis, and focuses on the significance of preoperative evaluation of multidisciplinary team and the surgical strategies of severe scoliosis. The purpose is to provide a clinical algorithm for reference about the precise surgical treatment of SMA combined with scoliosis.

Key words: spinal muscular atrophy, scoliosis, conservative management, surgery