Division of Dento-Oral Anesthesiology, Tohoku University Graduate School of Dentistry, Seiryomachi 4-1, Aoba, Sendai, Miyagi, 980-8575, Japan.
Department of Anesthesiology 38 Morohongo, Saitama Medical University Hospital, Moroyama, Iruma, Saitama, 350-0495, Japan.
J Anesth. 2020 Oct;34(5):773-776. doi: 10.1007/s00540-020-02808-5. Epub 2020 Jun 5.
Monoamine oxidase (MAO) deficiency is an X-linked hereditary disease characterized by spontaneous deletion of MAO-A and/or MAO-B on the X chromosome. Here, we describe the first reported case of a patient with MAO-A and MAO-B deficiency managed under general anesthesia in dental treatment. The patient was aged 11 years old when he was scheduled for dental treatment. He was diagnosed with MAO-A and MAO-B deficiency on genetic testing at 2 years of age. He was not given premedication, and standard monitoring with noninvasive blood pressure monitoring, pulse oximetry, and ECG was instituted. We also preemptively prepared a cardioverter-defibrillator. General anesthesia was induced with propofol 46 mg (2 mg/kg), then rocuronium 10 mg (0.4 mg/kg) and remifentanil 0.30 μg/kg/min were administered via separate infusion pumps. Orotracheal intubation was performed without complications. Anesthesia was maintained uneventfully with a continuous infusion of remifentanil 0.15-0.2 μg/kg/min and propofol 5.0-7.0 mg/kg. Fresh gas flow included oxygen and air. End-tidal CO concentration was maintained at around 35 mmHg throughout the procedure. We administered sugammadex 92 mg (4 mg/kg) for reversal of neuromuscular blockade and the patient was extubated. We achieved successful anesthetic management without any appreciable clinical signs of fatal arrhythmias in this patient with MAO-A and MAO-B deficiency.
单胺氧化酶（MAO）缺乏症是一种 X 连锁遗传性疾病，其特征是 X 染色体上的 MAO-A 和/或 MAO-B 自发性缺失。在这里，我们描述了首例 MAO-A 和 MAO-B 缺乏症患者在牙科治疗中接受全身麻醉管理的病例。该患者在 11 岁时接受了牙科治疗。他在 2 岁时通过基因检测被诊断为 MAO-A 和 MAO-B 缺乏症。他没有接受术前用药，采用标准监测，包括非侵入性血压监测、脉搏血氧饱和度和心电图。我们还预先准备了除颤器。全身麻醉诱导用丙泊酚 46mg（2mg/kg），然后通过单独的输注泵给予罗库溴铵 10mg（0.4mg/kg）和瑞芬太尼 0.30μg/kg/min。气管插管无并发症。通过持续输注瑞芬太尼 0.15-0.2μg/kg/min 和丙泊酚 5.0-7.0mg/kg 平稳维持麻醉。新鲜气体包括氧气和空气。呼气末二氧化碳浓度在整个手术过程中维持在 35mmHg 左右。我们给予舒更葡糖钠 92mg（4mg/kg）逆转神经肌肉阻滞，患者拔管。我们成功地对这名 MAO-A 和 MAO-B 缺乏症患者进行了麻醉管理，没有出现明显的致命心律失常的临床迹象。
