A case report of type II Floyd tracheal agenesis with staged tracheal and alimentary reconstructions.

Abstract

Background: Tracheal agenesis is a rare and lethal congenital airway anomaly. In particular, very few patients with type II Floyd tracheal agenesis experience long-term survival. We present the case of a male infant who was diagnosed with type II disease, underwent staged reconstruction and was discharged home with a tolerance for oral intake.

Case presentation: The patient was delivered via emergency cesarean section at 33 week gestation due to fetal distress. Initial attempts to intubate the trachea failed, but ventilation was successfully achieved through esophageal intubation, prompting suspicion of tracheal agenesis with a tracheoesophageal fistula. The diagnosis was confirmed via CT and laryngoesophagoscopy. On the same day, abdominal esophageal banding and gastrostomy were performed. For long-term management, staged operations, including pseudotracheostomy, internal and external stenting of the trachealized esophagus, and reconstruction of the alimentary tract, were performed. Despite being discharged home with a tolerance for oral intake, the patient experienced cardiac arrest at 41 months due to tracheal tube displacement.

Conclusions: Early detection of tracheal agenesis in postnatal patients experiencing respiratory distress is crucial. Type II Floyd tracheal agenesis remains challenging because of the absence of a trachea and the narrow diameter of the tracheoesophageal fistula.