Van Meter C H, Lusk R M, Muntz H, Spray T L
Children's Hospital, Washington University Medical Center, St. Louis, Missouri.
Am Surg. 1991 Mar;57(3):157-60.
Congenital long-segment intrathoracic tracheal stenosis (CTS) is a rare life-threatening obstruction in infancy and childhood. From July 1983 to March 1988 six infants aged 14 days to 14 months with CTS were identified. Symptoms ranged from recurrent stridor and wheezing to severe respiratory compromise and hypercarbia. Routine chest x-rays were not diagnostic. Definitive diagnosis was made by bronchoscopy, which showed complete tracheal rings in all patients with severely compromised tracheobronchial lumens. In three patients, pericardium was successfully used for anterior tracheoplasty with one early death due to fulminant sepsis in an infant with undiagnosed sickle cell disease. The other two died late, at 3 and 9 months from problems unrelated to the repair. In three patients a rib graft was used for repair; in one, tracheoplasty was required after earlier repair of tetralogy of Fallot. All are late survivors with no postoperative symptoms. After recognition of CTS, prompt surgery is warranted to avoid the late complications of tracheostomy for long-term ventilatory support. Rigid repair with rib cartilage is preferable to use of pericardium. Proper rib harvesting with intact perichondrium, intraoperative bronchoscopy, oxygenation by cardiopulmonary bypass, and meticulous graft alignment are necessary for successful postoperative outcome.
先天性长节段胸段气管狭窄（CTS）是婴幼儿期一种罕见的危及生命的梗阻性疾病。1983年7月至1988年3月，共确诊6例年龄在14天至14个月的CTS婴儿。症状从反复喘鸣和喘息到严重的呼吸功能不全及高碳酸血症不等。常规胸部X线检查无诊断价值。通过支气管镜检查做出明确诊断，所有气管支气管腔严重受损的患者均显示有完整的气管环。3例患者成功采用心包进行前路气管成形术，其中1例患有未诊断出的镰状细胞病的婴儿因暴发性败血症早期死亡。另外2例患者后期分别于3个月和9个月时死于与修复无关的问题。3例患者采用肋骨移植进行修复；1例患者在早期法洛四联症修复术后需要进行气管成形术。所有患者均为后期存活者，术后无任何症状。认识到CTS后，应及时进行手术，以避免为长期通气支持而行气管造口术的后期并发症。采用肋软骨进行刚性修复优于使用心包。为获得成功的术后效果，完整保留软骨膜正确取肋骨、术中支气管镜检查、体外循环氧合以及精心的移植物对齐是必要的。
