Ohshita Naohiro, Tomiyama Yoshinobu, Iseki Akio, Kawano Hiroaki, Kakuta Nami, Tsutsumi Yasuo M, Oshita Shuzo P
Department of Anesthesiology, Tokushima University Hospital, Tokushima 770-8503.
Masui. 2010 Apr;59(4):484-6.
Angelman syndrome is a hereditary disease described by Angelman. The clinical features of Angelman syndrome are characterized by mental retardation, puppet-like ataxia, easily excitable personality, seizures, paroxysmal laughter, strabismus and macroglossia. A 4-year-old girl with Angelman syndrome underwent strabismus repair under general anesthesia. Anesthesia was slowly induced with sevoflurane in oxygen and maintained with air, oxygen, propofol and remifentanil. Tracheal intubation was performed after administration of rocuronium. During and after anesthesia, no adverse events regarding circulatory and respiratory systems occurred. However, this case demonstrates that it is necessary to pay attention to airway troubles including the difficulty of tracheal intubation, management of body temperature and chronotropic action or respiratory depression by anesthetic agents.
安吉尔曼综合征是一种由安吉尔曼描述的遗传性疾病。安吉尔曼综合征的临床特征表现为智力发育迟缓、木偶样共济失调、易激惹的性格、癫痫发作、阵发性大笑、斜视和巨舌症。一名患有安吉尔曼综合征的4岁女孩在全身麻醉下接受了斜视修复手术。用七氟醚在氧气中缓慢诱导麻醉，并用空气、氧气、丙泊酚和瑞芬太尼维持麻醉。给予罗库溴铵后进行气管插管。麻醉期间及麻醉后，未发生循环和呼吸系统的不良事件。然而，该病例表明，有必要注意气道问题，包括气管插管困难、体温管理以及麻醉药物引起的变时作用或呼吸抑制。
