Tracheal Agenesis

Abstract

Tracheal agenesis is a catastrophic congenital anomaly that invariably results in death. Forty-seven cases have been previously reported in the literature. We add five additional cases, including two type 1 cases, two type 2 cases, and one type 3 case, based on Floyd's classification scheme. We describe the features of this unusual anomaly at the time of diagnosis. We discuss a rational approach to the management of this difficult problem on an emergent basis that allows for the maintenance of the infant's life until all of the implications of this fatal condition can be assessed. While we do not advocate reconstructive surgery for this anomaly, which has been universally fatal, we discuss the potential rearrangement of the anatomy, which may offer some hope in future cases. The concomitant congenital anomalies associated with these cases are reviewed, and autopsy specimens are presented for their anatomic interest.