Division of Neonatology, Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey.
Departments of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey.
Turk J Pediatr. 2022;64(4):775-780. doi: 10.24953/turkjped.2018.1637.
Tracheal agenesis (TA) is a rare congenital defect that consists of a complete or partial absence of the trachea below the larynx, with or without tracheoesophageal fistula (TEF). It is a severe congenital defect with a very high mortality rate. The recommended surgical approach is esophageal ligation and gastrostomy. Despite the progress in reconstructive surgical techniques, the outcome of the anomaly is still very poor. We described a case of TA with a TEF in a female newborn with a hemivertebra, single ventricle, single atrioventricular valve, single atrium, and cardiac left isomerization.
The patient, who was born at 37 weeks of age, was diagnosed with imaging methods, as the cyanosis did not improve despite being intubated many times in the delivery room; the cyanosis improved after esophageal intubation. Despite all life support treatment, the patient died on the fourth day of life. At autopsy, tracheal agenesis was diagnosed.
In newborns who cannot be intubated in the delivery room or whose lungs cannot be ventilated despite being intubated and whose cyanosis cannot be corrected, tracheal agenesis should be considered and ventilation with esophageal intubation should also be tried.
气管发育不全(TA)是一种罕见的先天性缺陷,表现为喉部以下的气管完全或部分缺失,伴有或不伴有气管食管瘘(TEF)。这是一种严重的先天性缺陷,死亡率非常高。推荐的手术方法是食管结扎和胃造口术。尽管重建外科技术取得了进展,但该异常的结果仍然非常差。我们描述了一例女性新生儿 TA 合并 TEF 的病例,该新生儿存在半椎体、单心室、单房室瓣、单心房和心脏左位异构。
该患者于 37 周龄时出生,通过影像学方法诊断,尽管在产房多次插管,但紫绀仍未改善;食管插管后紫绀改善。尽管进行了所有生命支持治疗,患者仍在出生后第四天死亡。尸检诊断为气管发育不全。
对于在产房无法插管或插管后肺部无法通气且紫绀无法纠正的新生儿,应考虑气管发育不全,并尝试食管插管通气。