Diagnosis and management of complete tracheal rings with concurrent tracheoesophageal fistula

Nikolaus E Wolter; Aimee A Kennedy; Michael J Rutter; Clyde Matava; Osami Honjo; Priscila L Chiu; Evan J Propst

doi:10.1016/j.ijporl.2020.109971

Diagnosis and management of complete tracheal rings with concurrent tracheoesophageal fistula

Int J Pediatr Otorhinolaryngol. 2020 Jun:133:109971. doi: 10.1016/j.ijporl.2020.109971. Epub 2020 Feb 27.

Authors

Nikolaus E Wolter¹, Aimee A Kennedy², Michael J Rutter², Clyde Matava³, Osami Honjo⁴, Priscila L Chiu⁵, Evan J Propst⁶

Affiliations

¹ Department of Otolaryngology - Head and Neck Surgery, The Hospital for Sick Children, University of Toronto, Toronto, Canada. Electronic address: nikolaus.wolter@sickkids.ca.
² Division of Pediatric Otolaryngology - Head and Neck Surgery, Cincinnati Children's Hospital Medical Centre, Department of Otolaryngology - Head and Neck Surgery, University of Cincinnati College of Medicine, Cincinnati, USA.
³ Department of Anesthesia, Perioperative Medicine, and Pain Management, Hospital for Sick Children, University of Toronto, Toronto, Canada.
⁴ Division of Cardiovascular Surgery, Department of Surgery, The Hospital for Sick Children, University of Toronto, Toronto, Canada.
⁵ Division of General and Thoracic Surgery, Department of Surgery, The Hospital for Sick Children, Department of Surgery, University of Toronto, Toronto, Canada.
⁶ Department of Otolaryngology - Head and Neck Surgery, The Hospital for Sick Children, University of Toronto, Toronto, Canada.

PMID: 32179205
DOI: 10.1016/j.ijporl.2020.109971

Abstract

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.

Keywords: Complete tracheal ring; Esophageal atresia; Tracheoesophageal fistula.

Publication types

Systematic Review

MeSH terms

Abnormalities, Multiple / diagnosis*
Abnormalities, Multiple / therapy*
Adolescent
Bronchoscopy
Child
Child, Preschool
Combined Modality Therapy
Conservative Treatment / methods
Humans
Infant
Infant, Newborn
Intubation, Intratracheal
Perioperative Care / methods
Plastic Surgery Procedures / methods
Tracheal Stenosis / congenital
Tracheal Stenosis / diagnosis*
Tracheal Stenosis / therapy*
Tracheoesophageal Fistula / congenital
Tracheoesophageal Fistula / diagnosis*
Tracheoesophageal Fistula / therapy*
Tracheostomy
Tracheotomy
Treatment Outcome