NF-1脊柱侧凸伴肋骨头脱位：肋骨头切除还是保留？

Type 1 neurofibromatosis scoliosis associated with ribs head dislocation: resected or reserved?

中文关键词:  先天性多发性关节挛缩症  脊柱侧凸  困难气道  呼吸管理

英文关键词:Arthrogryposis multiplex congenital  Scoliosis  Difficult airway  Management of respiratory function

中文摘要:

  【摘要】 目的：探讨先天性多发性关节挛缩症（arthrogryposis multiplex congenital，AMC）伴脊柱侧凸患者围手术期的呼吸管理特点。方法：回顾性分析2006年10月～2015年10月我院行脊柱矫形手术的22例AMC伴脊柱侧凸患者的病例资料。男12例，女10例，平均年龄13.7±4.6岁（5～26岁）。记录患者的一般情况、气道评估（Mallampati评分、颈椎后仰受限、张口受限）结果、术前肺功能、麻醉诱导时的插管情况、手术时间及拔管时间等呼吸管理的相关临床资料，总结此类患者围手术期呼吸管理的特点。结果：22例患者中，Mallampati评分Ⅰ级4例（18%），Ⅱ级7例（32%），Ⅲ级8例（36%），Ⅳ级3例（14%），颈椎后仰受限6例（27%），张口受限4例（18%）。所有患者术前均存在不同程度的肺通气功能减退，其中轻度6例（27%），中度9例（41%），重度7例（32%）。22例患者均成功进行气管内插管，其中采用普通喉镜插管成功4例（18%）、普通喉镜导芯辅助3例（14%），视频喉镜5例（23%），光棒6例（27%），纤维支气管镜引导插管4例（18%）。3例术前气道评估预计严重困难气道患者表面麻醉下纤维支气管镜引导插管，8例患者麻醉诱导保留自主呼吸进行了气管插管。术后最短拔管时间为10min，最长达245min。术前重度肺功能减退患者较术前轻、中度肺功能减退者拔管时间明显延长（P＜0.05）。结论：AMC伴脊柱侧凸患者困难气道多见，常合并肺功能障碍，手术及麻醉风险高，在麻醉前需充分进行气道评估，优化麻醉呼吸管理策略，减少围手术期呼吸系统并发症。

英文摘要:

  【Abstract】 Objectives: To investigate the characteristics of management of respiratory function of scoliosis associated with arthrogryposis multiplex congenital(AMC). Methods: The medical records of 22 AMC patients underwent spinal corrective surgery were reviewed, 12 males and 10 females were included with the age ranging from 5 to 28 years(average, 13.7±4.6 years). The data on the management of respiratory function were recorded: demographic data, airway assessment(Mallampati score, reduced neck extension, small month opening), pulmonary function test, intubation information, operation time and extubation time, the characteristics of perioperative management of respiratory function of such patients were summarized. Results: The case of Mallampati Ⅰ, Ⅱ, Ⅲ and Ⅳ grade was 4(18%), 7(32%), 8(36%) and 3(14%), respectively. There were 6 cases of reduced neck extension and 4 cases of small month opening. All patients suffered from pulmonary dysfunction. The case of mild, moderate and severe preoperative pulmonary dysfunction was 6(27%), 9(41%) and 7(32%) respectively. All patients were successfully intubated. A variety of difficult airway tools was used in intubation macintosh laryngoscope: 4 cases(18%); macintosh laryngoscope with stylet: 3 cases(14%); visualization laryngoscope: 5 cases(23%); light wand, 6 cases(27%); and fiber bronchoscope, 4 cases(18%). 3 patients with anticipated difficult airway were successfully awake intubated with fiber bronchoscope. The time from the end of surgery to trachead extubation varied greatly, ranging from 10min to 245min. Extubation time was significantly longer in patients with severe pulmonary dysfunction than those with mild or moderate pulmonary dysfunction(P＜0.05) and longer respiratory support was needed. Conclusions: Scoliosis patients associated with AMC have high incidence of pulmonary dysfunction and difficult airway. In consideration of high risk of surgery and anesthesia, adequation and optimization of perioperative management are needed to ensure the patient′s safety.