Yokoyama T, Tomoda M, Nishiyama T, Matsuda T, Fujimoto S, Hanaoka K, Manabe M
Department of Anesthesiology and Resuscitology, Kochi Medical School, Nankoku.
Masui. 2000 Jul;49(7):785-7.
Cornelia de Lange syndrome involves anomalies in cardio-vascular and musculo-skeletal systems, and mental retardation. In addition, a patient with this syndrome shows a peculiar look. A 22-year-old woman with Cornelia de Lange syndrome underwent general anesthesia twice. She has a small mouth, thin lips, a short neck, short limbs, and stiffness of the neck and some joints associated with slight mental retardation. She was scheduled for arthroscopy and then for rotational acetabular osteotomy and valgus osteotomy. The maximum distance between the upper and lower incisors was 34 mm, when she opened her mouth. Anesthesia was induced with sevoflurane increased slowly to 7.0% in oxygen 6 l.min-1 in both procedures. After the administration of 4% lidocaine 3.5 ml into the pharynx, orotracheal intubation was attempted, but was not successful. Then blind naso-tracheal intubation was performed successfully under spontaneous respiration under sevoflurane-oxygen inhalation. The induction of anesthesia with sevoflurane under spontaneous respiration was useful for blind naso-tracheal intubation in a case with difficult intubation such as in Cornelia de Lange syndrome.
科妮莉亚·德朗热综合征涉及心血管和肌肉骨骼系统异常以及智力发育迟缓。此外，该综合征患者还具有独特的面容。一名患有科妮莉亚·德朗热综合征的22岁女性接受了两次全身麻醉。她嘴巴小、嘴唇薄、脖子短、四肢短小，颈部和一些关节僵硬，伴有轻度智力发育迟缓。她计划先进行关节镜检查，然后进行髋臼旋转截骨术和外翻截骨术。她张嘴时上下门齿间的最大距离为34毫米。在这两次手术中，均使用七氟醚诱导麻醉，氧气流量为6升/分钟，七氟醚浓度缓慢增至7.0%。在咽部注入4%利多卡因3.5毫升后，尝试进行经口气管插管，但未成功。随后在七氟醚-氧气吸入下自主呼吸状态下成功进行了盲探鼻气管插管。对于像科妮莉亚·德朗热综合征这种插管困难的病例，在自主呼吸状态下用七氟醚诱导麻醉对盲探鼻气管插管是有用的。
