General Anesthesia Management for Adult Mucopolysaccharidosis Patients Undergoing Major Spine Surgery
General Anesthesia Management for Adult Mucopolysaccharidosis Patients Undergoing Major Spine Surgery
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DOI：
10.1159/000503051
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发表时间：
2019-11-01
影响因子：
3.2
通讯作者：
Nishikawa, Kiyonobu
中科院分区：
文献类型：
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作者：
Tsuchiya, Masahiko;Terai, Hidetomi;Nishikawa, Kiyonobu
目的：粘多糖病（Mucopolysaccharidosis， MPS）是一种罕见的全身性溶酶体积存病，伴发严重气道阻塞和心脏疾病，给麻醉治疗带来困难。当代治疗延长了受影响个体的寿命，增加了成年期大手术的需求。临床表现与干预：我们对6例接受脊柱手术的MPS成人患者进行全身麻醉。所有气道被评估为困难，2例接受清醒光纤插管，1例成功接受视频喉镜检查，而3例视频喉镜检查失败并需要转换为光纤插管。1例患者发生心室颤动。结论：成人MPS患者存在较大的麻醉风险。
Objective: Mucopolysaccharidosis (MPS) are a group of rare systemic lysosomal storage diseases associated with severe airway obstruction and cardiac disease, making anesthesia management difficult. Contemporary treatment extends the lifespan of affected individuals, increasing the need for major surgery in adulthood. Clinical Presentation and Intervention: We provided general anesthesia for 6 adult MPS patients undergoing spine surgery. The airway was assessed as difficult in all, with 2 receiving awake fiberoptic intubation and 1 successfully undergoing video-laryngoscopy, while 3 video-laryngoscopy procedures failed and required conversion to fiberoptic intubation. One patient developed ventricular fibrillation. Conclusion: Adult MPS patients have substantial anesthesia risk.