Congenital laryngo-tracheo-esophageal clefts: updates from a quaternary care pediatric airway unit

Eur Arch Otorhinolaryngol. 2024 Jan;281(1):283-294. doi: 10.1007/s00405-023-08263-8. Epub 2023 Oct 11.

Abstract

Purpose: To review the operative techniques, outcomes, and complications following surgery in pediatric patients with laryngo-tracheo-esophageal clefts (LTEC). We describe a new combined approach to treat long LTECs.

Methods: Twenty-five patients underwent surgical repair for LTEC from March 2012 to July 2022 at our hospital. Every patient underwent a diagnostic endoscopy under general anesthesia and spontaneous ventilation to assess the LTEC and synchronous aero-digestive comorbidities/malformations. All patients underwent at least one surveillance endoscopy after the repair at our institution.

Results: The patients had multiple other malformations, specifically gastro-intestinal, synchronous airway, and cardiac. The cleft distribution according to the modified Benjamin and Inglis classification was type I (n = 5, 20%), type II (n = 6, 24%), type IIIa (n = 8, 32%), type IIIb (n = 4, 16%), and type IVa (n = 2, 8%). The median follow-up was 44.6 months. Five patients (20%) had undergone previous cleft corrective surgery(s). Seven patients (28%) had partial to complete breakdown of the repair, needing additional intervention(s), and two required a combined-open plus endoscopic repair. Preoperatively, most patients (n = 18, 72%) needed a feeding assistance. At latest follow-up, feeding assistance was weaned off in 13 out of 18 patients, which was a 72% improvement. Ten patients (40%) needed ventilation assistance before the surgery. Post-operatively, ventilatory assistance was weaned off in 6 patients, meaning a 60% improvement.

Conclusion: LTEC are rare malformations, and their management needs precise diagnosis, appropriate surgical planning, and execution, and dedicated post-operative care. Primary and revision repair of long clefts with tracheal extension may require a combined approach.

Keywords: Endoscopic surgery; LTEC; Laryngo-tracheo-esophageal cleft; Open surgery.

MeSH terms

  • Abnormalities, Multiple* / diagnosis
  • Child
  • Esophagus / surgery
  • Humans
  • Larynx* / surgery
  • Respiratory System Abnormalities*
  • Retrospective Studies
  • Trachea / abnormalities
  • Trachea / surgery