Motomura Yuka, Idei Masafumi, Sato Hitoshi, Goto Takahisa
Department of Anesthesiology, Yokohama City University Hospital, 236-0004 3-9 Fukuura, Kanazawa-ku, Yokohama-shi, Kanagawa-ken, Japan.
, 235-0033 602 2-2-2 Sugita, Isogo-ku, Yokohama-shi, Kanagawa-ken, Japan.
JA Clin Rep. 2018 Apr 18;4(1):32. doi: 10.1186/s40981-018-0169-y.
Rett syndrome, which is a progressive, central nervous system disease that is caused by a gene mutation, is known to present with various symptoms. This case is that of a 15-year-old girl who was diagnosed with Rett syndrome at the age of 2 years. Laryngotracheal isolation under general anesthesia was planned due to recurrent aspiration pneumonia. Since the patient's nutritional status and control of convulsions were good, this was deemed an appropriate time for the surgery. Following careful preoperative evaluation of her airway, we performed oral endotracheal intubation using a video laryngoscope after rapid induction. Since postoperative pain control was important to prevent apneic attacks and convulsions, we used a multimodal analgesic regimen including carefully titrated fentanyl, acetaminophen, nonsteroidal anti-inflammatory drug, and wound infiltration with a local anesthetic. Postoperatively, the patient returned to the intensive care unit under spontaneous ventilation and followed a good course. Patients with Rett syndrome present several symptoms. Thus, several points must be considered during the preoperative evaluation, anesthetic management, and postoperative care of these patients.
瑞特综合征是一种由基因突变引起的进行性中枢神经系统疾病，已知会出现各种症状。该病例是一名15岁女孩，她在2岁时被诊断出患有瑞特综合征。由于反复发生吸入性肺炎，计划在全身麻醉下进行喉气管隔离。由于患者的营养状况和惊厥控制良好，这被认为是进行手术的合适时机。在对她的气道进行仔细的术前评估后，我们在快速诱导后使用视频喉镜进行了口腔气管插管。由于术后疼痛控制对于预防呼吸暂停发作和惊厥很重要，我们采用了多模式镇痛方案，包括仔细滴定的芬太尼、对乙酰氨基酚、非甾体抗炎药以及用局部麻醉药进行伤口浸润。术后，患者在自主通气下返回重症监护病房，恢复过程良好。瑞特综合征患者会出现多种症状。因此，在对这些患者进行术前评估、麻醉管理和术后护理时，必须考虑几个要点。
