Diagnosis and management of complete tracheal rings with concurrent tracheoesophageal fistula

Objective: Characterize patients with complete tracheal rings and tracheoesophageal fistula (TEF) and summarize management options. Methods: A systematic review of patients under 18 years of age with complete tracheal rings and TEF was conducted. Authors were contacted for additional patient information and new cases were added. Patients with iatrogenic TEF and tracheal stenosis due to other causes were excluded. Results: Sixteen patients with a median (IQR) follow-up of 10 months (3-12 months) were identified. All had a distal TEF with complete tracheal rings distal to the TEF. There were 10 (63%) type C esophageal atresia + TEF (EA/TEF), and 1 (6%) type D (5 missing data). Median (IQR) airway diameter was 2 mm (1.5-2.2 mm). Complete tracheal rings were diagnosed prior to TEF repair in 5 (31.3%) patients, after >= 1 failed extubation in 3 (12.5%) patients, and intra-operatively during respiratory distress in 1 patient. Ten patients (62.5%) were intubated with an endotracheal tube and one with a 6 Fr flexible aortic canula (5 missing data). Four patients with an endotracheal tube for TEF repair developed ventilatory problems. Complete tracheal rings were repaired in 9 (56%) patients (8 slide tracheoplasty, 1 pericardial patch) and followed conservatively in 3 (19%). One patient required tracheotomy. Four patients died. Conclusions: Complete tracheal rings with concurrent TEF is a rare entity that pose challenges for ventilatory management during operative repair. Bronchoscopy prior to TEF repair is critical to allow for proper preoperative planning.