Early ligation of the gastroesophageal junction to manage respiratory instability in tracheal agenesis: A case report

Introduction: Tracheal agenesis (TA) is an exceedingly rare life-threatening congenital malformation. It can present in isolation, as part of congenital syndromes, or in the setting of chromosomal abnormalities and is associated with recognized risk factors such as maternal diabetes. An associated tracheoesophageal fistula (TEF) can allow for ventilation at birth via the esophagus. However, mortality among patients with TA remains high, even with prompt surgical intervention. Case Report: We present the case of a male infant who had delayed diagnosis of TA. The patient underwent emergent exploratory laparotomy and ligation of the gastroesophageal junction to temporize his respiratory instability but ultimately was transitioned to comfort focused care following multidisciplinary discussion with his family. Conclusion: This case highlights the importance of early recognition of TA. Temporizing measures, including surgical ligation of the gastroesophageal junction, can allow time for more definitive treatments.