The long-term outcome of tracheal agenesis following reconstruction of the airway and alimentary tract

摘要

We herein report the 14-year-follow-up of a patient with tracheal agenesis (TA) after reconstruction of the airway and alimentary tract. A 2536-g female infant was diagnosed with TA of Floyd’-s type 1. Her condition was stabilized by the direct insertion of a ready-made endotracheal tube into a tracheo-esophageal fistula, after birth. At seven days old, she underwent esophageal transection, gastrostomy, and cervical esophageal division, and distal cervical esophagostomy was created as a neo-trachealized esophagus.During early infancy, a ready-made endotracheal tube had been used as an internal esophageal stent. At 7 months old, a custom-made long tracheostomy tube was substituted, which was revised according to her growth, and her respiratory condition became stable thereafter. At 7 years old, her alimentary tract was reconstructed using a gastric tube, and the post-operative course was uneventful; she eventually achieved oral intake 5 years after this surgery. She is now 14 years and 5 months old, and shows a substandard height and weight, with a slower-than-normal neurologic and physiologic development for her age. As a result, she currently attends a special-needs school. We also reviewed six other long-term survival cases to date. The present report seems to describe the longest-term outcome of a patient with TA.