Tracheobronchopexy to Avoid Tracheostomy in Esophageal Atresia Patients With Severe Life-Threatening Tracheobronchomalacia

Hester F Shieh; Russell W Jennings; Thomas E Hamilton; Shawn Izadi; Benjamin Zendejas; C Jason Smithers

doi:10.1016/j.jpedsurg.2024.162152

Tracheobronchopexy to Avoid Tracheostomy in Esophageal Atresia Patients With Severe Life-Threatening Tracheobronchomalacia

J Pediatr Surg. 2025 Jan 3;60(4):162152. doi: 10.1016/j.jpedsurg.2024.162152. Online ahead of print.

Authors

Hester F Shieh¹, Russell W Jennings², Thomas E Hamilton³, Shawn Izadi⁴, Benjamin Zendejas⁴, C Jason Smithers²

Affiliations

¹ Division of Specialized Thoracic Care, Department of Surgery, Johns Hopkins All Children's Hospital, 501 6th Ave S, St. Petersburg, FL 33701, United States. Electronic address: hshieh1@jhmi.edu.
² Division of Specialized Thoracic Care, Department of Surgery, Johns Hopkins All Children's Hospital, 501 6th Ave S, St. Petersburg, FL 33701, United States.
³ Department of Surgery, Children's Hospital of Philadelphia, 3401 Civic Center Blvd, Philadelphia, PA 19104, United States.
⁴ Department of Surgery, Boston Children's Hospital, 300 Longwood Ave, Boston, MA 02115, United States.

PMID: 39826297
DOI: 10.1016/j.jpedsurg.2024.162152

Abstract

Background: Esophageal atresia (EA) is associated with tracheobronchomalacia (TBM), which in its most severe form, causes blue spells, brief resolved unexplained events (BRUEs) that can require cardiopulmonary resuscitation (CPR), and positive pressure ventilation (PPV) or ventilator dependence, often requiring tracheostomy. We study the role of tracheobronchopexy, as an alternative to tracheostomy, in EA patients with severe life-threatening TBM.

Methods: We reviewed EA patients who underwent tracheobronchopexy for blue spells, BRUEs, and failure to wean PPV or extubate from February 2013 to September 2021 at two institutions. Patient characteristics, surgical techniques, and respiratory outcomes were reviewed.

Results: 80 EA patients (most Gross type C 92.5 %) underwent 91 tracheobronchopexies at median age 6 (IQR 3-14) months for blue spells/BRUEs (53 %), PPV (21 %), and ventilator dependence (26 %). On preoperative dynamic bronchoscopy, most (90 %) demonstrated complete airway collapse. Surgical approach for tracheobronchopexy was posterior (73 %), anterior (23 %), and simultaneous posterior and anterior (4 %). Tracheobronchopexy included thoracic trachea alone (58 %), trachea and bronchi (41 %), and bronchi alone (1 %). At latest follow up of median 39 (IQR 14-64) months, there were no recurrent blue spells/BRUEs (p < 0.001) and significantly reduced PPV and ventilator dependence (p < 0.001). Nearly all patients (n = 75, 94 %) avoided tracheostomy. Mortality was 5 %, one 30-day operative mortality and three long-term mortalities related to underlying comorbidities.

Conclusions: In EA patients with severe life-threatening TBM, tracheobronchopexy significantly reduces blue spells/BRUEs, PPV, and ventilator dependence, and avoids tracheostomy. This surgical strategy should be considered the treatment of choice for EA patients with severe life-threatening TBM symptoms.

Level of evidence: Level IV Treatment Study.

Keywords: Esophageal atresia; Tracheobronchomalacia; Tracheobronchopexy; Tracheostomy.