Current management and outcome of tracheobronchial malacia and stenosis presenting to the paediatric intensive care unit

Objective: To identify factors associated with mortality and prolonged ventilatory requirements in patients admitted to our paediatric intensive care unit (PICU) with tracheobronchial malacia and stenosis diagnosed by dynamic contrast bronchograms. Design: Retrospective review Setting: Tertiary paediatric intensive care unit. Patients: Forty-eight cases admitted to our PICU over a 5-year period in whom a diagnosis of tracheobronchial malacia or stenosis was made by dynamic contrast bronchography (1994-1999). Interventions: Conservative management, tracheostomy and longterm ventilation, surgical correction, internal or external airway stenting. Measurements and results: Recording of clinical details, length of invasive ventilation and appearance at contrast bronchography. Five groups of patients were defined: isolated primary airway pathology (n = 7), ex-premature infants (n = 11), vascular rings (n = 9), complex cardiac and/or syndromic pathology (n = 11\) and tracheooesophageal fistulae (n = 4). The overall mortality was 29%. Median length of invasive ventilation in survivors was 38 days and in patients who died 45, Mortality was highest in the patients with complex cardiac ;and/or syndromic pathology (P = 0.039 Cox regression analysis) but was not related to any other factor, Patients with stenosis required a significantly longer period of ventilatory support (median length of ventilation 59 days) than patients with malacia (39 days). Conclusions: Length of ventilation and bronchographic diagnosis did not predict survival. The only factor found to contribute significantly to mortality was the presence of complex cardiac and/or syndromic pathology However; patients with stenosis required longer ventilatory support than patients with malacia.