背景:Alström综合征是一种罕见的遗传性疾病,以肥胖为特征,糖尿病,心肌病,和肝功能障碍。Further,脊柱侧弯,Alström综合征的常见症状,通常需要对功能障碍进行手术干预。在进行功能性脊柱侧凸手术时,运动诱发电位(MEP)监测和其他电生理测试至关重要。然而,关于Alström综合征如何维持全身麻醉的报道很少。这里,我们描述了一名Alström综合征患者,他在全身麻醉下接受了脊柱侧凸手术,并接受了瑞咪唑安定和MEP监测.
方法:一名17岁女性(身高,140厘米,体重,64.5kg)诊断为Alström综合征,计划进行后路脊柱融合术以治疗功能性脊柱侧凸。Alström综合征的其他相关并发症是扩张型心肌病,2型糖尿病,肥胖(体重指数,32.1kg/m2),弱视(光感知),和听力障碍(每只耳朵的语音意识阈值为50dBHL)。
方法:对功能性脊柱侧凸进行后路脊柱融合术。在调查扩张型心肌病时,经胸超声心动图显示全壁运动功能减退,左心室射血分数为45%。左心室扩张,左心室舒张末期和收缩末期直径为55和42毫米,分别。这一发现以及与Alström综合征相关的高甘油三酯血症使我们得出结论,应避免使用异丙酚。因此,我们用瑞马唑仑诱导全身麻醉。进行了MEP监测,患者在手术过程中没有运动障碍。
结论:心肌和肝功能障碍决定了Alström综合征患者的预后。因此,在这种情况下,应选择保留肝功能的麻醉。在高甘油三酯血症患者中,应该避免使用异丙酚,使用雷米唑仑,一种超短作用的苯并二氮卓类,可能是合适的。在这种情况下,使用SedLine检查患者状态指数使我们能够顺利地进行MEP监测,后路脊柱融合术完成,无任何运动障碍。
BACKGROUND: Alström syndrome is a rare genetic disorder characterized by obesity, diabetes mellitus, cardiomyopathy, and liver dysfunction. Further, scoliosis, a common symptom of Alström syndrome, often requires surgical intervention for functional impairments. Motor evoked potential (MEP) monitoring and other electrophysiological tests are essential when performing surgery for functional scoliosis. However, there are few
reports on how to maintain general anesthesia in Alström syndrome. Here, we describe a patient with Alström syndrome who underwent surgery for scoliosis under general anesthesia with remimazolam and MEP monitoring.
METHODS: A 17-year-old woman (height, 140 cm, weight, 64.5 kg) diagnosed with Alström syndrome was scheduled for a posterior spinal fusion for functional scoliosis. Other associated comorbidities of Alström syndrome present were dilated cardiomyopathy, type 2 diabetes mellitus, obesity (body mass index, 32.1 kg/m2), amblyopia (light perception), and hearing impairment (speech awareness threshold 50 dBHL in each ear).
METHODS: Posterior spinal fusion was planned for functional scoliosis. While investigating the dilated cardiomyopathy, transthoracic echocardiography showed global wall hypokinesis, with 45% left ventricular ejection fraction. The left ventricle was dilated, with left ventricular end-diastolic and end-systolic diameters of 55 and 42 mm, respectively. This finding along with the hypertriglyceridemia associated with Alström syndrome led us to conclude that propofol should be avoided. Thus, we induced general anesthesia using remimazolam. MEP monitoring was performed, and the patient experienced no motor impairments during the surgery.
CONCLUSIONS: Myocardial and hepatic dysfunction determine the prognosis of patients with Alström syndrome. Thus, anesthesia that preserves liver function should be selected in such cases. In patients with hypertriglyceridemia, propofol should be avoided, and using remimazolam, an ultrashort-acting benzodiazepine, may be appropriate. In this
case, reviewing the Patient State Index with SedLine allowed us to perform MEP monitoring uneventfully, and the posterior spinal fusion was completed without any motor impairment.