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Tailored Management of Airway Abnormalities in Children with Congenital Single Functional Lung

Madhavan Ramaswamy1, Pilar Anton-Martin2, Laura Garcia Martinez1

  • 1Department of Cardiothoracic Surgery, Great Ormond Street Hospital for Children, London, UK.

Journal of Pediatric Surgery
|December 12, 2018
PubMed

Insights

Managing airway abnormalities in pediatric patients with a single functional lung is achievable. Surgical interventions, including staged repairs and mediastinal repositioning, lead to acceptable outcomes and improved airway stability post-discharge.

Area of Science:

  • Pediatric Surgery
  • Thoracic Surgery
  • Congenital Abnormalities

Background:

  • Pediatric patients with a single functional lung often present with complex airway abnormalities.
  • Management of these conditions is technically and ethically challenging.

Purpose of the Study:

  • To report the surgical experience and outcomes of managing symptomatic airway abnormalities in pediatric patients with congenital single functional lung.

Main Methods:

  • Retrospective cohort study of 16 pediatric patients (0-18 years) with congenital single functional lung and airway abnormalities (tracheal stenosis, tracheomalacia) requiring surgery.
  • Surgical interventions included slide tracheoplasty, aortopexy, rudimentary lung excision, and mediastinal repositioning.
  • Analysis of one-stage versus staged repair strategies.

Main Results:

  • 16 patients (8 with agenesis, 8 with hypoplasia) were included, with right-sided lung abnormalities in 62.5%.
  • Common airway issues were long segment congenital tracheal stenosis (12 patients) and tracheomalacia (4 patients).
  • Overall survival to discharge was 87.5%, with 64.2% of survivors having stable airways without home respiratory support.

Conclusions:

  • Surgical management of airway abnormalities in pediatric patients with congenital single functional lung is feasible.
  • Staged repairs and mediastinal repositioning are crucial for patients with comorbidities.
  • Treatment should not be withheld if no contraindications exist.
Abstract

Keywords:
Congenital tracheal stenosisECMOMechanical ventilationOutcomesSingle lungTracheomalacia

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