Long-Term Outcomes of Airway Management in 6 Children With Campomelic Dysplasia.

Abstract

Objective: This case series describes the outcomes of airway management, including airway reconstruction, in 6 patients with campomelic dysplasia and tracheostomy/ventilator dependence secondary to multilevel airway obstruction.

Methods: Case series and clinical guidelines are provided for the airway management of patients with campomelic dysplasia.

Results: Average age of individuals is 19.4 years. Mean follow-up was 12.2 years. Four individuals underwent open airway reconstruction and achieved decannulation. One patient underwent airway reconstruction with improvement of a complete subglottic stenosis but remains ventilator dependent due to severe scoliosis. The remaining 2 patients did not require additional airway reconstruction, have been liberated from ventilator support, and are under evaluation for tracheostomy tube decannulation.

Conclusion: Although campomelic dysplasia was historically considered a lethal form of congenital skeletal dysplasia, with many patients succumbing to respiratory failure due to tracheobronchomalacia in the neonatal period, airway reconstruction and long-term survivorship is feasible in children with campomelic dysplasia and significant airway disease.