线粒体脑肌病,乳酸性酸中毒,卒中样发作(MELAS)综合征是一种复杂且罕见的线粒体细胞病变。MELAS患者常表现为多系统表现,使他们的麻醉管理特别具有挑战性。在这个案例报告中,我们详细描述了我们对一名19岁男性的麻醉方法,该男性已证实MELAS与m.3243A>G突变相关.该患者在中风样发作后12岁时被诊断出患有MELAS,并出现进行性脊柱畸形。他表现出70°的胸椎弯曲和80°的后凸,需要T1-L2后路脊柱融合术.手术计划包括具有体感和运动诱发电位的神经监测。在这种情况下,静脉麻醉剂如丙泊酚通常是优选的,因为与挥发性麻醉剂相比,它们对神经监测的干扰减少。预计手术持续时间为六到七个小时,然而,由于丙泊酚在MELAS患者中存在乳酸性酸中毒的潜在风险,我们犹豫是否要在这一延长的时间段内依赖丙泊酚.鉴于丙泊酚长时间输注(>48小时)或高剂量输注(≥5mg·kg-1·hour-1)已知可诱发丙泊酚相关性输注综合征,再加上我们对该患者乳酸性酸中毒风险的担忧,我们被迫设计了一种麻醉方案,在不过度使用挥发性麻醉剂的情况下完全避免使用异丙酚.这种主动的方法确保了保持一致的神经监测信号和病人的安全,尤其是考虑到他潜在的线粒体功能障碍.我们提出此病例报告的主要理由是强调MELAS在扩展手术中带来的挑战。在神经监测过程中重点考虑麻醉因素。对于通常严重依赖静脉麻醉药的长时间手术,与挥发性麻醉药相比,对神经监测的干扰较小,在MELAS环境中使用异丙酚应谨慎,因为其与乳酸性酸中毒的风险相关.据我们所知,这是第一例病例报告,描述了接受这种持续时间的MELAS手术的患者的麻醉管理,需要体感和运动诱发电位神经监测。我们相信我们的经验将为麻醉医师和面临类似挑战性临床情况的围手术期团队提供参考。
Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome is a complex and infrequently encountered mitochondrial cytopathy. Patients with MELAS often present with multi-systemic manifestations, making their anesthetic management particularly challenging. In this
case report, we describe in detail our anesthetic approach for a 19-year-old male with confirmed MELAS linked to an m.3243A>G mutation. The patient had been diagnosed with MELAS at age 12 following a stroke-like episode and presented with progressive spinal deformities. He exhibited a 70° thoracic spine curvature and an 80° kyphosis, requiring a T1-L2 posterior spinal fusion. The surgical plan included neuromonitoring with both somatosensory and motor evoked potentials. Intravenous anesthetics such as propofol are typically preferred in this context due to their reduced interference with neuromonitoring compared to volatile anesthetics. Anticipating a surgical duration of six to seven hours, however, we hesitated to rely on propofol for this extended period due to its potential risks of lactic acidosis in the context of MELAS. Given that propofol infusion for extended periods (>48 hours) or at high doses (≥5 mg·kg-1·hour-1) is known to induce propofol-related infusion syndrome, and coupled with our concerns about the risk of lactic acidosis in this patient, we were compelled to design an anesthetic plan that avoided propofol altogether without excessive use of volatile anesthetics. This proactive approach ensured the maintenance of consistent neuromonitoring signals and the patient\'s safety, especially given his underlying mitochondrial dysfunction. Our primary rationale in presenting this
case report is to highlight the challenges posed by MELAS in the setting of extended surgery, with a focus on anesthetic considerations during neuromonitoring. For prolonged surgeries that typically rely heavily on intravenous anesthetics, which interfere less with neuromonitoring than volatile anesthetics, the use of propofol should be approached with caution in MELAS contexts due to its associated risk of lactic acidosis. To our knowledge, this is the first
case report that described the anesthetic management of a patient with MELAS undergoing a procedure of such duration, requiring both somatosensory and motor evoked potential neuromonitoring. We believe our experiences will serve as a reference for anesthesiologists and perioperative teams faced with similar challenging clinical situations.