Department of Anesthesia and Pain Management, Toronto Western Hospital, University Health Network, 399 Bathurst St., Toronto, ON M5T 2S8, Canada.
Can J Anaesth. 2011 Nov;58(11):1034-8. doi: 10.1007/s12630-011-9580-9. Epub 2011 Aug 25.
Central alveolar hypoventilation syndrome (CAHS) is a rare disease characterized by the loss of autonomic control of breathing. This condition causes hypoventilation and obstruction during sleep. Throughout their lives, these patients require ventilatory assistance by means of positive pressure ventilation to their lungs via mask, tracheotomy, or other means, such as phrenic nerve pacers. The diaphragm pacing stimulation system (DPSS) is a new treatment where electrodes are implanted into the diaphragm and cause contraction on stimulation. The DPSS has been used successfully in tetraplegic patients and patients suffering from amyotrophic lateral sclerosis (ALS). It has been shown to improve quality of life and to extend survival in patients with advanced respiratory muscle weakness. In our case, we describe the perioperative management of an adult patient with acquired CAHS who presented for laparoscopic DPSS insertion.
Our patient was a 50-yr-old female who developed CAHS at age thirteen after contracting encephalitis. Since the onset of her disease, she had been managed with positive pressure ventilation to her lungs via mask. Due to her longstanding disease, she presented with pulmonary hypertension and cor pulmonale and was scheduled for laparoscopic DPSS implantation. Our anesthetic technique included a total intravenous technique with remifentanil and propofol, and her trachea was intubated without the use of muscle relaxants. The pacemakers were switched on when the patient emerged from anesthesia, which provided her with ventilatory support and allowed us to extubate her trachea.
We present the successful anesthetic management of an adult patient with CAHS undergoing laparoscopic DPSS insertion.
中枢性肺泡通气不足综合征（CAHS）是一种罕见的疾病，其特征是呼吸的自主控制丧失。这种情况会导致睡眠期间通气不足和阻塞。这些患者一生都需要通过面罩、气管切开术或其他方式（如膈神经起搏器）对肺部进行正压通气来提供通气辅助。膈肌起搏刺激系统（DPSS）是一种新的治疗方法，其中将电极植入膈肌，通过刺激引起收缩。DPSS 已成功用于四肢瘫痪患者和肌萎缩侧索硬化症（ALS）患者。它已被证明可以改善生活质量并延长晚期呼吸肌无力患者的生存时间。在我们的病例中，我们描述了一名成年获得性 CAHS 患者接受腹腔镜 DPSS 插入术的围手术期管理。
我们的患者是一名 50 岁女性，13 岁时因脑炎后患上 CAHS。自发病以来，她一直通过面罩对肺部进行正压通气。由于她的长期疾病，她出现了肺动脉高压和肺心病，并计划进行腹腔镜 DPSS 植入。我们的麻醉技术包括使用瑞芬太尼和丙泊酚的全静脉技术，并且在没有使用肌肉松弛剂的情况下对她的气管进行插管。当患者从麻醉中苏醒时，起搏器被打开，为她提供通气支持，并允许我们拔出她的气管。
我们成功地管理了一名成年 CAHS 患者接受腹腔镜 DPSS 插入术的麻醉。
