肌强直性营养不良患者脊柱畸形手术后的灾难性并发症

Rae Hyung Kim, Sung Bae Park
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摘要

肌强直性营养不良(MD)是一种遗传性疾病,可导致进行性肌肉无力和萎缩,通常导致脊柱畸形和生活质量下降。虽然脊柱畸形的手术矫正在MD患者中并非绝对禁忌,但全面的麻醉前评估是至关重要的,特别是考虑到心血管和呼吸健康。我们报告一位50岁女性MD患者,她接受脊柱畸形手术以矫正后凸和减轻严重的背痛。尽管手术期间没有发生重大事件和术前危险因素,但患者在重症监护室出现了术后呼吸衰竭、低血压、虚弱加重和精神恶化。患者因呼吸肌无力住院期间接受长期呼吸机支持。在多学科支持和康复治疗下,患者的力量、活动能力和呼吸功能有了显著改善,并在术后6个月内实现了功能独立。作者讨论了这些并发症可能的病因机制,并强调了MD患者术前评估和多学科护理的必要性。
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Catastrophic Complications Following Spinal Deformity Surgery in a Patient with Myotonic Dystrophy
Myotonic dystrophy (MD) is a genetic disorder that causes progressive muscle weakness and atrophy, often leading to spinal deformities and decreased quality of life. While the surgical correction of spinal deformities is not absolutely contraindicated in MD patients, a comprehensive pre-anesthetic evaluation is crucial, particularly considering cardiovascular and respiratory health. We present a case of a 50-year-old woman with MD who underwent spinal deformity surgery to correct kyphosis and alleviate severe back pain. Despite the absence of significant events during surgery and preoperative risk factors, the patient experienced postoperative respiratory failure, hypotension, aggravated weakness, and mental deterioration in the intensive care unit. The patient received long-term ventilator support during hospitalization for respiratory muscle weakness. She demonstrated significant improvements in strength, mobility, and respiratory function with multidisciplinary support and rehabilitation, achieving functional independence within 6 months postoperatively. The authors discuss the possible etiological mechanisms underlying these complications and highlight the need for preoperative evaluations and multidisciplinary care for patients with MD.
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