Cogbill T H, Moore F A, Accurso F J, Lilly J R
Ann Thorac Surg. 1983 May;35(5):538-41. doi: 10.1016/s0003-4975(10)60430-4.
Tracheomalacia is a rare congenital malformation of the tracheobronchial cartilages in which the supporting cartilaginous rings permit expiratory collapse of the airway. The condition is usually mild and self-limited. There is a severe variant, however, that is life-threatening and warrants separate categorization. Four children with severe primary tracheomalacia were treated recently. The clinical symptoms, diagnostic findings, and eventual treatment of these patients were highly distinctive and almost identical in all 4, permitting us to make the following observations: (1) primary severe tracheomalacia must be suspected in infants with unexplained respiratory distress manifested by stridor and cyanosis; (2) symptoms are not present at birth but appear insidiously after the first weeks of life, are markedly aggravated by respiratory tract infections, and are made worse by agitation; (3) bronchoscopy is essential for definitive diagnosis and should be employed early in the diagnostic process; (4) tracheostomy is probably essential in most instances; and (5) resolution, although spontaneous, does not occur until after 2 years of age.
气管软化是一种罕见的气管支气管软骨先天性畸形，其中支撑性软骨环可导致气道在呼气时塌陷。这种情况通常较轻且具有自限性。然而，有一种严重的变体，它会危及生命，需要单独分类。最近对4例严重原发性气管软化患儿进行了治疗。这4例患者的临床症状、诊断结果及最终治疗情况高度独特且几乎相同，使我们能够得出以下观察结果：（1）对于出现喘鸣和发绀等不明原因呼吸窘迫的婴儿，必须怀疑原发性严重气管软化；（2）症状并非出生时就存在，而是在出生后几周内隐匿出现，呼吸道感染会使其明显加重，烦躁也会使其恶化；（3）支气管镜检查对于明确诊断至关重要，应在诊断过程早期进行；（4）在大多数情况下，气管切开术可能是必要的；（5）尽管可自发缓解，但直到2岁以后才会出现。
