The skeletal muscle channelopathies are a group of rare diseases and include non-dystrophic myotonia and periodic paralysis. Given their rarity, little has been published on the management of anaesthesia and pregnancy in this cohort despite being important aspects of care. We have conducted a large study of over 70 patients who underwent anaesthesia and 87 pregnancies to investigate the problems encountered following anaesthesia or during pregnancy. This was performed via patient surveys sent out to genetically confirmed channelopathy patients seen at the National Hospital for Neurology and Neurosurgery. Most significantly in our cohort, patients frequently experienced a worsening or precipitation of symptoms during pregnancy (75%) or following anaesthetic (31%). None of our patients developed malignant hyperthermia, although there are confirmed reports of this in patients with periodic paralysis and mutations in RYR1. There was a significantly higher number of miscarriages compared to the normal population. There was no significant difference in antenatal or delivery complications compared to the general population. However, three neonates did have complications, all of whom were found to carry mutations in SCN4A. This study highlights the importance of counselling patients and clinicians for the possibility of worsening symptoms during pregnancy or anaesthesia and the careful management of neonates following delivery.

中文翻译：

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管理骨骼肌通道病患者的妊娠和麻醉剂

骨骼肌通道病是一组罕见的疾病，包括非营养不良性肌强直和周期性麻痹。鉴于它们的稀有性，尽管麻醉和妊娠是护理的重要方面，但在该队列中几乎没有发表关于麻醉和妊娠管理的文章。我们对 70 多名接受麻醉的患者和 87 次怀孕进行了一项大型研究，以调查麻醉后或怀孕期间遇到的问题。这是通过向在国立神经病学和神经外科医院就诊的经基因证实的通道病患者发送的患者调查进行的。在我们的队列中最重要的是，患者在怀孕期间 (75%) 或麻醉后 (31%) 经常出现症状恶化或沉淀。我们的患者均未出现恶性高热，尽管有关于周期性麻痹和 RYR1 突变的患者的确认报告。与正常人群相比，流产次数明显增多。与一般人群相比，产前或分娩并发症没有显着差异。然而，三名新生儿确实出现了并发症，他们都被发现携带 SCN4A 突变。本研究强调了就妊娠或麻醉期间症状恶化的可能性以及分娩后新生儿的谨慎管理向患者和临床医生提供咨询的重要性。与一般人群相比，产前或分娩并发症没有显着差异。然而，三名新生儿确实出现了并发症，他们都被发现携带 SCN4A 突变。本研究强调了就妊娠或麻醉期间症状恶化的可能性以及分娩后新生儿的谨慎管理向患者和临床医生提供咨询的重要性。与一般人群相比，产前或分娩并发症没有显着差异。然而，三名新生儿确实出现了并发症，他们都被发现携带 SCN4A 突变。本研究强调了就妊娠或麻醉期间症状恶化的可能性以及分娩后新生儿的谨慎管理向患者和临床医生提供咨询的重要性。