Kato Shinnya, Mizuno Ju, Ino Kentaro, Kuroki Kanako, Morita Shigeho
Department of Anesthesiology, Teikyo University School of Medicine, Tokyo 173-8606.
Masui. 2010 May;59(5):632-4.
Williams syndrome is caused by the deletion of genetic material from chromosome 7, including the elastin gene. Affected individuals frequently have connective tissue disorders and skeletal hypoplasia, resulting in micrognathia and mandibular retrusion. These conditions hinder adequate visualization of the larynx and render intubation difficult. We used different methods of tracheal intubation for two patients with Williams syndrome. The first case was a 35-year-old woman with micrognathia, mandibular retrusion, and a Mallampati class III airway, scheduled for mitral valve replacement. She had a three fingerbreadth oral aperture, 3.0 cm mandibulohyoid distance, and 30 degree neck flexion. Since she had previously been successfully intubated at age 18 for patch enlargement of the aorta and extended aortoplasty, we elected to use intravenous anesthetic agents for this intubation. After administration of diazepam and fentanyl, mask ventilation was difficult and the direct laryngoscopic view was Cormack grade IV. Fortunately, blind orotracheal intubation using a Mallinckrodt tracheal tube with direct laryngoscopy was accomplished on the first attempt. The second case was a 71-year-old woman with micrognathia, slight mandibular retrusion, macroglossia, and a Mallampati class IV airway, scheduled for sigmoidectomy and insertion of a ureteral stent. She had a 2.5 fingerbreadth oral aperture, 4.0 cm mandibulohyoid distance, and 30 degree neck flexion. From our experience with the first patient, we expected difficult ventilation and intubation. After 8% lidocaine spray to the larynx, and then 4% lidocaine spray to the vocal cords and trachea using a bronchoscope, we accomplished awake, fibreoptic-guided orotracheal intubation easily and quickly on the first attempt using a Parker Flex-Tip tube. Difficult tracheal intubation should be anticipated in Williams syndrome patients. Awake, fibreoptic-guided technique is easier and safer than direct laryngoscopy for intubating such patients.
威廉姆斯综合征是由7号染色体上的遗传物质缺失引起的，其中包括弹性蛋白基因。受影响的个体经常患有结缔组织疾病和骨骼发育不全，导致小颌畸形和下颌后缩。这些情况妨碍了对喉部的充分观察，并使插管变得困难。我们对两名威廉姆斯综合征患者采用了不同的气管插管方法。第一例是一名35岁女性，患有小颌畸形、下颌后缩和马兰帕蒂III级气道，计划进行二尖瓣置换术。她的口腔开口为三指宽，下颌舌骨距离为3.0厘米，颈部屈曲30度。由于她曾在18岁时成功接受过主动脉补片扩大和主动脉成形术插管，我们选择在此次插管时使用静脉麻醉剂。给予地西泮和芬太尼后，面罩通气困难，直接喉镜视野为科马克IV级。幸运的是，在直接喉镜引导下使用马林克罗特气管导管进行盲探经口气管插管首次尝试就成功了。第二例是一名71岁女性，患有小颌畸形、轻度下颌后缩、巨舌症和马兰帕蒂IV级气道，计划进行乙状结肠切除术和输尿管支架置入术。她的口腔开口为2.5指宽，下颌舌骨距离为4.0厘米，颈部屈曲30度。根据我们对第一名患者的经验，我们预计通气和插管会很困难。在对喉部喷洒8%利多卡因，然后使用支气管镜对声带和气管喷洒4%利多卡因后，我们首次尝试就使用帕克Flex-Tip导管轻松快速地完成了清醒纤维支气管镜引导下经口气管插管。威廉姆斯综合征患者应预料到气管插管困难。对于此类患者，清醒纤维支气管镜引导技术比直接喉镜插管更容易、更安全。
