Department of Dental Anesthesiology, Division of Oral and Maxillofacial Surgery and Oral Medicine, Hiroshima University Hospital, Hiroshima, Japan.
J Craniofac Surg. 2023 Sep 1;34(6):e580-e582. doi: 10.1097/SCS.0000000000009407. Epub 2023 May 29.
Spinal muscular atrophy (SMA) is an autosomal recessive neuromuscular disease characterized by the degeneration of alpha motor neurons in the spinal cord, resulting in progressive proximal muscle weakness and paralysis. SMA is classified into types I-IV based on the age at symptom onset or maximum motor function achieved, and its clinical manifestations vary. SMA affects maxillofacial growth because of muscle dysfunction and results in abnormal maxillofacial morphology. In addition, definitive diagnosis is not often made because of the older onset age and symptoms are rarely severe. Therefore, the possibility of undiagnosed SMA in craniofacial surgeries must be considered. This report described a case of an SMA type III recognized after delayed recovery from the neuromuscular blockade in an orthognathic surgery under general anesthesia.
脊髓性肌萎缩症（SMA）是一种常染色体隐性神经肌肉疾病，其特征是脊髓中的α运动神经元退化，导致进行性近端肌肉无力和瘫痪。SMA 根据症状出现的年龄或最大运动功能的获得情况分为 I-IV 型，其临床表现也有所不同。SMA 由于肌肉功能障碍而影响颌面生长，导致异常的颌面形态。此外，由于发病年龄较大且症状很少严重，因此通常无法做出明确诊断。因此，在颅面外科手术中必须考虑到未确诊的 SMA 的可能性。本报告描述了一例 SMA III 型患者，该患者在全身麻醉下接受正颌手术时，神经肌肉阻滞延迟恢复后被确诊。
