Department of Anesthesiology, Osaka City General Hospital and Children's Hospital, Osaka, Japan.
J Anesth. 2012 Jun;26(3):445-8. doi: 10.1007/s00540-012-1342-5. Epub 2012 Feb 15.
Pena-Shokeir syndrome is a rare, early lethal disease. It is characterized by fetal growth restriction; craniofacial deformities, for example micrognathia and microcephaly; multiple ankyloses; and pulmonary hypoplasia. For patients with this syndrome, maintenance of airway and control of perioperative respiratory complications are important for anesthetic management. We report 3 pediatric cases of Pena-Shokeir syndrome undergoing tracheostomy and arthrolysis under general anesthesia using sevoflurane, nitrous oxide, fentanyl, and vecuronium bromide. Anesthetic procedures including mask ventilation, tracheal intubation, and extubation were successfully performed without complications during and after surgery. In patients with Pena-Shokeir syndrome, inhalational anesthetics can be safely used for induction and maintenance of anesthesia, although it is important to assume that difficult airway management might be encountered.
佩纳-肖克综合征是一种罕见的、早期致命性疾病。其特征为胎儿生长受限；颅面畸形，例如小颌畸形和小头畸形；多发性关节强直；以及肺发育不良。对于患有这种综合征的患者，维持气道通畅和控制围手术期呼吸并发症对于麻醉管理非常重要。我们报告了 3 例接受七氟醚、氧化亚氮、芬太尼和维库溴铵全身麻醉下行气管切开术和关节松解术的佩纳-肖克综合征儿科患者。麻醉过程包括面罩通气、气管插管和拔管，手术期间和手术后均未发生并发症。对于患有佩纳-肖克综合征的患者，可以安全地使用吸入麻醉剂进行麻醉诱导和维持，尽管必须假设可能会遇到困难气道管理。
