Categorization and repair of recurrent and acquired tracheoesophageal fistulae occurring after esophageal atresia repair

Purpose Recurrent trachea-esophageal fistula (recTEF) is a frequent (5%–10%) complication of congenital TEF (conTEF) and esophageal atresia (EA) repair. In addition, postoperative acquired TEF (acqTEF) can occur in addition to or even in the absence of prior conTEF in the setting of esophageal anastomotic complications. Reliable repair often proves difficult by endoluminal or standard surgical techniques. We present the results of an approach that reliably identifies the TEF and facilitates airway closure as well as repair of associated tracheal and esophageal problems. Methods Retrospective review of 66 consecutive patients 2009–2016 (55 referrals and 11 local) who underwent repair via reoperative thoracotomy or cervicotomy for recTEF and acqTEF (IRB P00004344). Our surgical approach used complete separation of the airway and esophagus, which reliably revealed the TEF (without need for cannulation) and freed the tissues for primary closure of the trachea and frequently resection of the tracheal diverticulum. For associated esophageal strictures, stricturoplasty or resection was performed. Separation of the suture lines by rotational pexy of the both esophagus and the trachea, and/or tissue interposition were used to further inhibit re-recurrence. For associated severe tracheomalacia, posterior tracheopexy to the anterior spinal ligament was utilized. Results The TEFs were recurrent (77%), acquired from esophageal leaks (26%), in addition to persistent or missed H-type (6%). Seven patients in this series had multiple TEFs of more than one category. Of the acqTEF cases, 6 were esophagobronchial, 10 esophagopulmonic, 2 esophagotracheal (initial pure EA cases), and 2 from a gastric conduit to the trachea. Upon referral, 18 patients had failed endoluminal treatments; and open operations for recTEF had failed in 18 patients. Significant pulmonary symptoms were present in all. During repairs, 58% were found to have a large tracheal diverticulum, and 51% had posterior tracheopexy for significant tracheomalacia. For larger esophageal defects, 32% were treated by stricturoplasty and 37% by segmental resection. Rotational pexy of the trachea and/or esophagus was utilized in 62% of cases to achieve optimal suture line separation. Review with a mean follow-up of 35 months identified no recurrences, and resolution of pulmonary symptoms in all. Stricture treatment required postoperative dilations in 30, and esophageal replacement in 6 for long strictures. There was one death. Conclusion This retrospective review of 66 patients with postoperative recurrent and acquired TEF following esophageal atresia repair is the largest such series to date and provides a new categorization for postoperative TEF that helps clarify the diagnostic and therapeutic challenges for management.