WOFAPS 2025 8th World Congress of Pediatric Surgery

Introduction

Airway and upper digestive tract malformations are rare, with an incidence of 1:10,000- 1:20,000 live births. The coexistence of multiple malformations is even less common. Retrospective studies report that among patients with tracheoesophageal fistula (TEF), 1.4% present with laryngomalacia, 6.7% with vocal cord paralysis, 8.3% with laryngeal cleft, 4.2% with subglottic stenosis, and 12.1% with tracheomalacia. We present the case of a patient with three simultaneous congenital aerodigestive malformations in the absence of a genetic syndrome.

Case Presentation

We report a 2-month-old female with diagnosis followed a hypoxic-ischemic event at birth and multiple failed extubation attempts requiring tracheostomy. The infant presented with 99% congenital and acquired subglottic stenosis, congenital tracheoesophageal fistula in the posterior wall of the first tracheal ring, and grade II laryngotracheal cleft. The patient underwent extended laryngotracheal reconstruction, LT-mold placement, and TEF (tracheoesophageal fistula) closure in a single surgical procedure. Multidisciplinary management determined optimal surgical timing, antibiotic therapy, intraoperative strategy, and rehabilitation. The patient recovered well, achieving fistula closure, and improving airway function. Decannulation is pending until full airway healing.

Conclusion

The rarity of these malformations complicates management. Previous studies have described isolated cases of multiple anomalies, but none identical to this case. One series found that 62% of 139 tracheoesophageal fistula patients had upper airway abnormalities, highlighting the need for multidisciplinary care. Triple endoscopy optimizes diagnosis, while single-stage extended cricotracheal resection appears to be a viable approach prioritizing aspiration prevention, regardless of patient age. A multidisciplinary approach is essential for optimal preoperative and postoperative care.