Tracheal agenesis is a rare, congenital anomaly that may present to the anaesthesiologist in the operating room or in the delivery suite. It was first reported by Payne in 1900. Since then, more than 150 cases worldwide have been reported in literature. The incidence is 1/50,000 births, male: female ratio is 2:1. Several types have been described in literature. It usually manifests in the new born with severe respiratory distress and an absent cry. There is failure to perform endotracheal intubation. Ventilation via a bag-valve-mask and oesophageal intubation (i.e. only in the presence of a trachea-oesophageal fistula) may enable supportive ventilation until a complete evaluation is made. Further, the airway management of laryngeal agenesis and tracheal agenesis at birth, in the delivery room differs significantly.
Embryologically, during the 3rd and 4th week of gestation, the oesophagus and trachea begin their development. Ventral displacement of the trachea-oesophageal septum at this stage causes the tracheal agenesis. Various anomalies of the larynx or trachea or both may develop.