Tracheal Agenesis: Vertical Division of the Native Esophagus – A Novel Surgical Approach and Review of the Literature

Abstract

Introduction: Tracheal agenesis (TA) is rare and usually fatal. Few survivors with concomitant tracheoesophageal fistulae (TEF) who underwent ligation of the distal esophagus with creation of a spit-fistula and neo-trachea from the proximal esophagus exist. We report a novel surgical technique whereby the esophagus is divided longitudinally to preserve a functional alimentation tract and a parallel neo-trachea. We review the literature of reported cases, including survivors beyond 12 months. Methods: Case report and literature review. Results: A female infant with prenatal polyhydramnios was born at 35 weeks gestation with immediate respiratory distress and absent cry. Oxygenation was maintained with a laryngeal mask airway. Despite a normal appearing larynx, she could not be intubated and emergent neck exploration disclosed no cervical trachea. The patient was placed on extra corporeal membranous oxygenation (ECMO), and later diagnosed with TA, Floyd Type I. Parental desire for reconstruction but refusal of a spit-fistula necessitated a novel procedure. The esophagus was divided longitudinally via a microstapler to preserve the original alimentary tract and create a parallel neo-trachea originating from the TEF and terminating as a cervical stoma. The healing process was complicated but the baby was ultimately discharged to home where she developed normally neurologically until succumbing one night to accidental decannulation at 16 months of age. Conclusion: We describe a novel surgical approach to manage TA. This includes avoiding creation of a spit fistula and preserving the native esophagus. We then survey the literature, reporting the survivorship duration and operative management of 174 reported cases of TA.

External Esophageal Stenting Technique in Palliation for Tracheal Agenesis in a Case of Esophageal Lung: A Lesson Learned from the Experience for Tracheomalacia

“…Tracheal agenesis is a rare condition, with 118 English reports of 175 cases since its discovery in 1900 [11]. Most of the patients with this condition died within a few hours after the birth because of respiratory distress and unsuccessful resuscitation, and only ten patients have been reported as surviving more than 12 months to date [4,5,7,[11][12][13][14][15].…”

“…In type III, two mainstem bronchi arise individually from the esophagus. The relative incidence of these three types is 19%, 51%, and 30% [11]. Floyd's type III TA may be described as esophageal lung [10].…”

“…Regarding the long-term prognosis of TA, mechanical respiratory support and oxygen administration were not necessary in a few long-term survivors [15], whereas some patients died because of airway troubles such as accidental extubation [11], airway obstruction due to esophageal ulceration and granulation at the distal end of the airway tube [12], and a total collapse of the reconstructed trachea [6]. In TA patients achieving stabilization of the respiratory status, esophageal/alimentary reconstruction using a gastric tube, the jejunum, or the patient's own esophagus was performed to enable oral feeding.…”

Neonatal Airway Abnormalities

“…While several methods of reconstruction have been attempted, few have yielded promising results, rendering this condition largely fatal [ 73 ]. Creations of a neotracheal utilizing esophageal tissue in patients with existing tracheoesophageal fistula have been reported but should not be considered a standard of care [ 74 ].…”

Tracheal agenesis versus tracheal atresia: anatomical conditions, pathomechanisms and causes with a possible link to a novel MAPK11 variant in one case

“…Perinatal intervention via "extrauterine intrapartum therapy" (EXIT) allows for targeted oesophageal intubation and life-saving postnatal oxygenation [ 5 , 10 ]. Attempts at surgical neotrachealization of the oesophagus by vertical division or by internal or external stenting and oesophageal reconstruction have thus far been unable to provide long-term survival without neurological impairment [ 12 , 13 ].…”