Paterson R A, Tousignant M, Skene D S
Can Anaesth Soc J. 1985 Jul;32(4):418-21. doi: 10.1007/BF03011354.
The management of a patient with myotonic dystrophy undergoing Caesarean section for delivery of twins in breech position is reported. Anaesthetic management must reflect the multi-system nature of the disease in addition to the implications of pregnancy. Known triggers of myotonic crisis (succinylcholine, shivering) must be avoided. Attention to respiratory reserve is necessary in view of the restrictive defect this disease can impose. Cardiomyopathy and conduction system disturbances may be features. Upper gastrointestinal incoordination can superimpose the added risk of aspiration on the pregnant myotonic. Ineffective uterine contraction can cause vigorous post partum haemorrhage. Endocrine disturbances, especially diabetes mellitis, may be present. There may be increased sensitivity to sedative and narcotic agents. The newborn myotonic will need the expertise of neonatal care.
本文报告了一名患有强直性肌营养不良症的患者,因臀位双胞胎剖宫产分娩的管理情况。麻醉管理除了要考虑妊娠的影响外,还必须反映该疾病的多系统性质。必须避免已知的强直性肌阵挛危机触发因素(琥珀酰胆碱、寒战)。鉴于这种疾病可能导致的限制性缺陷,必须关注呼吸储备。心肌病和传导系统紊乱可能是其特征。上消化道不协调会增加妊娠强直性肌营养不良患者误吸的额外风险。子宫收缩乏力可导致产后大出血。可能存在内分泌紊乱,尤其是糖尿病。对镇静剂和麻醉剂的敏感性可能会增加。新生儿强直性肌营养不良患者需要新生儿护理方面的专业知识。