Epidural Anesthesia and Continuous Epidural Analgesia in a Pediatric Patient With Pelizaeus-Merzbacher Disease: A Case Report.
Epidural Anesthesia and Continuous Epidural Analgesia in a Pediatric Patient With Pelizaeus-Merzbacher Disease: A Case Report.
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DOI：
10.7759/cureus.29983
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发表时间：
2022-10
期刊：
影响因子：
--
通讯作者：
Tsuneyoshi I
中科院分区：
文献类型：
--
作者：
Maruta T;Watanabe Y;Nagata Y;Kashino R;Tsuneyoshi I
Pelizaeus-Merzbacher病（PMD）是一种遗传性白质营养不良，是一种由髓鞘发育异常引起的进行性和退行性中枢神经系统异常。由于PMD的发病率极低，关于其麻醉管理的病例报告很少。特别地，硬膜外麻醉仅在一例全身麻醉合并尾侧麻醉中被报道。我们采用全麻联合硬膜外麻醉对一名六岁男性双侧髋关节半脱位患者进行软组织松解手术治疗。七氟醚在氧化亚氮和氧气中诱导全身麻醉。给予罗库溴铵以促进气管插管。插管后，七氟醚在空气和氧气中维持全身麻醉。从L3/4开始放置硬膜外导管。硬膜外麻醉和镇痛，根据需要使用1%甲哌卡因，并在手术结束前1小时开始2ml /h 0.2%罗哌卡因。术中仅给予硬膜外镇痛作为术后镇痛，患者拔管后无疼痛主诉。麻醉持续3小时55分钟。没有发生明显的血流动力学或呼吸变化。术后患者持续硬膜外镇痛，定期口服对乙酰氨基酚，疼痛控制良好。术后第二天拔除硬膜外导管。术后疗程良好，患者于术后第5天转至儿科康复医院。住院期间无不良事件发生，无神经功能缺损。总之，麻醉医师应注意围手术期误吸、痉挛和癫痫发作的可能性，即使是轻度PMD。正确的术前评估、术中监测和麻醉技术将确保PMD患者的麻醉安全。尽管区域麻醉对已有神经功能缺陷的患者存在争议，但我们能够安全地对患有PMD的儿科患者进行硬膜外麻醉和术后持续硬膜外镇痛。
Pelizaeus-Merzbacher disease (PMD) is a genetic leukodystrophy, which is a progressive and degenerative central nervous system abnormality caused by dysmyelination. Because the incidence of PMD is extremely low, only a few case reports have been published regarding its anesthetic management. In particular, epidural anesthesia has only been reported in one case of general anesthesia combined with caudal anesthesia. We performed general anesthesia combined with epidural anesthesia for the soft-tissue release surgery for bilateral hip subluxation in a six-year-old male patient diagnosed with PMD. General anesthesia was induced with sevoflurane in nitrous oxide and oxygen. Rocuronium was administered to facilitate tracheal intubation. After intubation, general anesthesia was maintained with sevoflurane in the air and oxygen. An epidural catheter was placed from L3/4. For epidural anesthesia and analgesia, 1% mepivacaine was used as needed, and 2 ml/h of 0.2% ropivacaine was started one hour before the end of surgery. During surgery, only epidural analgesia was provided as postoperative analgesia, and the patient did not complain of pain after extubation. Anesthesia lasted three hours and 55 minutes. No significant hemodynamic or respiratory changes occurred. Postoperatively, the patient received continuous epidural analgesia and regular oral acetaminophen, and pain control was good. The epidural catheter was removed on the second postoperative day. The postoperative course was good, and the patient was transferred to a pediatric rehabilitation hospital on the fifth postoperative day. No adverse events occurred and no neurological deficits were observed during hospitalization. In conclusion, anesthesiologists should pay attention to the possibility of perioperative aspiration, spasticity, and seizure, even with mild PMD. Proper preoperative evaluations, intraoperative monitoring, and anesthetic techniques will ensure safe anesthesia for PMD patients. Although regional anesthesia in patients with pre-existing neurologic deficits is controversial, we were able to safely perform epidural anesthesia and postoperative continuous epidural analgesia in a pediatric patient with PMD.
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影响因子：
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作者：
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