Surgical management of tracheal agenesis

Tracheal agenesis is a rare congenital malformation that usually is fatal. This report describes our experience in two such cases. In both cases, the diagnosis was suspected at birth, because the patients had respiratory distress without an audible cry and were difficult to intubate. A gastrostomy and banding of the abdominal esophagus provided effective initial stabilization, in conjugation with respiratory management. The first patient also had complex cardiac malformations, and the infant died of cardiac failure 1 week after birth. The second infant, who had tracheal agenesis with a proximal tracheoesophageal fistula and a bronchoesophageal fistula, was managed successfully. At 9 months of age, a tracheotomy was performed, a long T tube was inserted to maintain the airway patency beyond the proximal tracheoesophageal fistula, and the patient was discharged. At 3 years of age, esophageal reconstruction was performed with a colonic interposition graft. The patient is thriving and developing normally at 4 years of age. Diagnosis at birth and maintenance of airway patency are essential for successful management of tracheal agenesis. Initial surgical interventions are palliative but lifesaving. Subsequent management focuses on improving the quality of life and allowing swallowing and speech.