Pizarro Christian, Davies Ryan R, Theroux Mary, Spurrier Ellen A, Averill Lauren W, Tomatsu Shunji
Nemours/Alfred I. duPont Hospital for Children, Wilmington, Delaware.
Nemours/Alfred I. duPont Hospital for Children, Wilmington, Delaware.
Ann Thorac Surg. 2016 Oct;102(4):e329-31. doi: 10.1016/j.athoracsur.2016.02.113.
Progressive tracheal obstruction is commonly seen in Morquio A syndrome and can lead to life-threatening complications. Although tracheostomy can address severe upper airway obstruction, lower airway obstruction, commonly associated with a narrow thoracic inlet and vascular compression, requires an alternative approach. We describe the case of a 16-year-old patient with Morquio A syndrome whose near-fatal tracheal obstruction was relieved by timely surgical tracheal vascular reconstruction with dramatic resolution of his respiratory symptoms.
进行性气管梗阻在莫尔基奥A综合征中很常见，可导致危及生命的并发症。虽然气管切开术可以解决严重的上气道梗阻，但与胸廓入口狭窄和血管受压相关的下气道梗阻需要另一种治疗方法。我们描述了一名16岁莫尔基奥A综合征患者的病例，其近乎致命的气管梗阻通过及时的气管血管重建手术得以缓解，呼吸症状显著改善。
