Anesthetic management of a pediatric patient with Dravet syndrome: A case report.
Anesthetic management of a pediatric patient with Dravet syndrome: A case report.
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Dravet综合征的儿科患者的麻醉管理：病例报告。
DOI：
10.1097/md.0000000000032709
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发表时间：
2023-01-27
期刊：
影响因子：
1.6
通讯作者：
中科院分区：
文献类型：
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作者：
德拉韦综合征(DS)是一种罕见而严重的肌阵挛癫痫，通常在婴儿期发病。癫痫发作是由各种原因引发的，包括发烧、洗澡和光刺激。DS对药物治疗难治性。此外，癫痫持续状态(SE)可导致严重的脑病和癫痫相关死亡。DS患者全身麻醉的报道很少。在这里，我们报告了我们的经验与麻醉管理的儿童DS患者。1例5岁男童，身高112 cm，体重19 kg，经SCN1a基因检测确诊为DS，发现一新的错义突变。他的病史包括抗药性癫痫、发育迟缓和低眼压。他的癫痫发作倾向于每天由体温上升(BT)、洗澡和光刺激触发。由于DS，他无法接受适当的牙科治疗，尽管他之前曾在我们医院的儿科牙科接受过牙科治疗。由于不合作和DS相关的限制，患者计划在全身麻醉下进行强化牙科治疗。考虑到BT升高带来的风险，避免了致痫药物，并按计划顺利完成了全身麻醉。术中BT虽有波动，但在麻醉结束时基本控制在与麻醉诱导时相同的水平。但术后第1天出现少量惊厥和单次全身惊厥伴发热。由于术前与主治儿科医生密切合作，患者在术后第3天出院时没有大问题。对于DS患者，在麻醉过程中要注意BT的管理，避免使用导致癫痫发作的药物。在癫痫发作发生的情况下，根据对患者的评估和与主治儿科医生合作的术后快速反应，谨慎的术前麻醉计划是必要的。
Dravet syndrome (DS) is a rare and severe myoclonic epilepsy, with onset commonly occurring in infancy. Seizures are triggered by various causes, including fever, bathing, and light stimulus. DS is refractory to drug treatment. Moreover, status epilepticus (SE) can cause serious encephalopathy and epilepsy-related deaths. There are very few reports of general anesthesia in DS patients. Herein, we report our experience with the anesthetic management of a pediatric patient with DS. A 5-year-old boy (height, 112 cm; weight, 19 kg) was diagnosed with DS through SCN1A genetic testing, which revealed a de novo novel missense mutation. His medical history included drug-resistant epilepsy, developmental delay, and hypotonia. His seizures tended to be triggered daily by a rise in body temperature (BT), bathing, and light stimulus. He could not receive adequate dental treatment due to DS, although he had previously undergone dental treatment under restraint at the pediatric dentistry department of our hospital. The patient was scheduled for intensive dental treatment under general anesthesia due to noncooperation, and DS-related limitations. By considering the risk posed by elevated BT, seizure-inducing drugs were avoided, and general anesthesia was completed as planned, uneventfully. Although fluctuation of BT occurred during the procedure, it was finally controlled at the end of anesthesia at about the same level as at anesthesia induction. However, small seizures and a single generalized convulsion were observed accompanied by fever on postoperative day 1. The patient was discharged from the hospital without major problems on postoperative day 3, because of detailed planning and close preoperative cooperation with the attending pediatrician. It is essential to pay attention to managing BT and to avoid drugs that induce seizures during anesthesia for patients with DS. Cautious preoperative planning for anesthesia based on evaluation of the patient and rapid postoperative response in collaboration with the attending pediatrician is necessary in case an epileptic seizure occurs.