Changi General Hospital, Singhealth. 2 Simei Street 3, 529889, Singapore, Singapore.
National Healthcare Group (NHG) Anaesthesiology Residency, 11 Jalan Tan Tock Seng, 308433, Singapore, Singapore.
BMC Anesthesiol. 2021 Feb 18;21(1):57. doi: 10.1186/s12871-021-01262-4.
Paramyotonia congenita is a rare autosomal dominant myopathy which presents with periodic weakness due to cold and exercise. It is caused by mutations of the SCN4 gene which encodes the sodium channel in skeletal muscles.
We report a full term obstetric patient with both paramyotonia congenita and terminal filum lipoma who presents for induction of labour followed by an emergency caesarean section performed under epidural anesthesia. Her recovery is subsequently complicated by a 3-day history of postpartum paraparesis attributed to hypokalemic periodic paralysis.
We describe the perioperative anesthesia considerations and challenges in this case with a review of the current literature. This case report highlights the importance of early proactive and collaborative multidisciplinary approach, maintaining normal temperature and electrolytes with a heightened vigilance for muscle-related perioperative complications.
先天性肌强直是一种罕见的常染色体显性遗传性肌病,表现为寒冷和运动引起的周期性无力。它是由编码骨骼肌钠离子通道的 SCN4 基因突变引起的。
我们报告了一例足月产科患者,患有先天性肌强直和终丝脂肪瘤,因产程发动而行引产,继而紧急行硬膜外麻醉剖宫产术。她随后出现了 3 天的产后截瘫,归因于低钾性周期性瘫痪。
我们描述了该病例的围手术期麻醉注意事项和挑战,并回顾了当前文献。该病例报告强调了早期积极的多学科协作方法的重要性,保持正常体温和电解质,对与肌肉相关的围手术期并发症保持高度警惕。