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

English Keywords:Arthrogryposis multiplex congenital  Scoliosis  Difficult airway  Management of respiratory function

English Abstract:

  【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.