Abstract
We reported a case of tracheal agenesis with complicated heart anomaly and otic imperforation. A female infant weighing 1938g, was born at 31 weeks' gestation with respiratory distress. Endoscopic examination demonstrated agenesis of trachea and existence of bronchoesophageal fistula. With esophageal ventilation, abdominal esophageal banding and gastrostomy was performed. Because of aggravation of respiratory status, we performed cervical esophagostomy, but she died at 8 days of age of esophageal rupture and pneumomediastinum. We have experienced 4 cases of tracheal agenesis and one of them has been alive for 5 years and 9 months. Three dimension helical CT, performed in the living case and the recent case, showed clear anatomical structure. In the former case, tracheoesophageal fistula was large, and there was the lower part of trachea. On the other hand, in the later case, the bronchoesopahgeal fistula was very narrow, and the trachea was completely absent. It is considered that the existence of large tracheoesophageal fistula or insert of endotracheal tube into the fistula would be required for stability of the endotracheal tube. In addition, we summarized 23 domestic cases of tracheal atresia, including our 4 cases.
