Manschot H J, van den Anker J N, Tibboel D
Department of Anaesthesia, Sophia Children's Hospital, Rotterdam, The Netherlands.
Anaesthesia. 1994 Sep;49(9):788-90. doi: 10.1111/j.1365-2044.1994.tb04453.x.
Agenesis of the trachea is a rare anomaly. The main signs are respiratory distress and cyanosis, inability to vocalize and impossible tracheal intubation. In most cases concomitant congenital anomalies of the heart, digestive tract or genitourinary tract are present. Endoscopy and X ray studies will confirm the diagnosis. There is no long-term surgical solution because no suitable material for a tracheal prosthesis is available at present, therefore the condition is ultimately fatal. We report a case of tracheal agenesis. After the diagnosis was established the baby's lungs were ventilated for several hours via an oesophageal tube and two broncho-oesophageal fistulae, but she finally died from untreatable respiratory acidosis. Autopsy revealed a Floyd's type III tracheal agenesis and a laryngeal cleft.
气管缺如是一种罕见的异常情况。主要体征为呼吸窘迫和发绀、无法发声以及无法进行气管插管。大多数情况下，还伴有心脏、消化道或泌尿生殖道的先天性异常。内镜检查和X线研究将确诊。由于目前没有合适的气管假体材料，因此不存在长期的手术解决方案，所以这种情况最终是致命的。我们报告一例气管缺如病例。确诊后，通过食管管和两个支气管食管瘘为婴儿的肺部通气数小时，但她最终死于无法治疗的呼吸性酸中毒。尸检显示为弗洛伊德III型气管缺如和喉裂。
