Mandai H, Kinouchi K
Department of Anesthesiology, Osaka Medical Center and Research Institute for Maternal and Child Health, Izumi 594-1101.
Masui. 2001 Jul;50(7):773-5.
Aglossia-adactylia syndrome is a rare disorder characterized by aglossia and deformity of the limbs of variable degree. We managed a 2-day-old neonate with ileojejunal atresia with aglossia-adactylia syndrome. He was scheduled for the repair of the intestinal atresia, gastrostomy and palatal impression taking, under general anesthesia with sevoflurane supplemented with fentanyl. In this case we could achieve tracheal intubation by direct laryngoscopy with cricoid pressure applied, and could manage without any complications. But many of the patients with this syndrome are complicated with cleft palate and micrognathia, and we should be careful of the difficult airway at the induction of general anesthesia and aspiration during perioperative period.
无舌-无指（趾）综合征是一种罕见的疾病，其特征为无舌和不同程度的肢体畸形。我们治疗了一名患有回肠空肠闭锁合并无舌-无指（趾）综合征的2日龄新生儿。他计划在七氟醚复合芬太尼全身麻醉下进行肠闭锁修复、胃造口术和取腭印模。在这种情况下，我们通过直接喉镜检查并施加环状软骨压迫实现了气管插管，且未出现任何并发症。但许多患有该综合征的患者合并腭裂和小颌畸形，我们在全身麻醉诱导时应注意困难气道问题以及围手术期的误吸情况。
