SURGICAL-MANAGEMENT OF TRACHEAL AGENESIS

Tracheal agenesis is a rare congenital malformation that usually is fa tal. This report describes our experience in two such cases. In both c ases, the diagnosis was suspected at birth, because the patients had r espiratory distress without an audible cry and were difficult to intub ate. A gastrostomy and banding of the abdominal esophagus provided eff ective initial stabilization, in conjugation with respiratory manageme nt. 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 an d a bronchoesophageal fistula, was managed successfully. At 9 months o f age, a tracheotomy was performed, a long T tube was inserted to main tain the airway patency beyond the proximal tracheoesophageal fistula, and the patient was discharged. At 3 years of age, esophageal reconst ruction was performed with a colonic interposition graft. The patient is thriving and developing normally at 4 years of age. Diagnosis at bi rth and maintenance of airway patency are essential for successful man agement of tracheal agenesis. Initial surgical interventions are palli ative but lifesaving. Subsequent management focuses on improving the q uality of life and allowing swallowing and speech.