What is the most appropriate additional management to do with tracheostomy for a 2-year-old boy with severe tracheomalacia and 90% airway collapse during expiration?

Management of Severe Tracheomalacia with Tracheostomy in a 2-Year-Old

For a 2-year-old with severe tracheomalacia (90% airway collapse) and tracheostomy, non-invasive ventilation via CPAP through the tracheostomy is the most appropriate additional management.

Rationale for CPAP/Positive Pressure Support

Continuous positive airway pressure (CPAP) is the definitive non-invasive intervention for severe tracheomalacia as it immediately decreases respiratory distress and improves airway patency by preventing dynamic airway collapse during expiration. 1

Mechanism of Action

• CPAP prevents tracheal collapse by maintaining positive distending pressure throughout the respiratory cycle, which is critical when 90% collapse occurs during expiration 2
• The positive pressure acts as a pneumatic stent, keeping the malacic airway segment open during the vulnerable expiratory phase 1, 2
• CPAP increases functional residual capacity (FRC), which secondarily improves forced expiratory flows in infants with tracheomalacia 2

Clinical Evidence Supporting CPAP

• CPAP immediately reduces respiratory distress and improves airway patency in tracheomalacia patients 1
• Long-term CPAP via tracheostomy has been successfully used in children with severe tracheobronchomalacia, including infants as young as 3 months old weighing 4.9 kg 3
• A 20-year experience with 15 children using sleep apnea CPAP machines via tracheostomy showed favorable outcomes with no CPAP-related complications 3

Why Other Options Are Inappropriate

High-Flow Nasal Cannula (Option c)

• High-flow nasal cannula delivers oxygen to the face and tracheostomy in emergency situations but does not provide the positive pressure needed to stent open a severely collapsing airway 4
• This modality is appropriate for oxygen delivery and humidification but lacks the distending pressure required for 90% tracheal collapse 4
• In a patient with tracheostomy, high-flow oxygen would bypass the upper airway entirely, making nasal cannula delivery ineffective 4

Negative Pressure Ventilation (Option a)

• Negative pressure ventilation would worsen tracheomalacia by creating more negative intrathoracic pressure during inspiration, potentially increasing the dynamic collapse during expiration
• This modality is contraindicated as it would exacerbate the expiratory collapse that defines severe tracheomalacia 1

Implementation Considerations

Technical Setup

• Sleep apnea CPAP machines can be adapted for use via tracheostomy in resource-limited settings 3
• Adequate humidification must be provided to prevent airway drying and mucus plugging 5
• Initial CPAP levels typically range from 4-8 cm H₂O, titrated based on clinical response 2

Monitoring Requirements

• Continuous pulse oximetry to detect desaturation or mucus plugging 5
• Assessment for signs of respiratory distress including accessory muscle use, tracheal tug, and intercostal retractions 4
• Waveform capnography should be immediately available for monitoring ventilation adequacy 4

Caregiver Training

• Non-medical caregivers (family members) can be successfully trained to manage CPAP via tracheostomy at home 3
• Training should include emergency management protocols for tracheostomy complications 4

Long-Term Prognosis and Weaning

• Clinically significant tracheomalacia naturally decreases with age and growth, with most cases resolving within the first few years of life 1, 6
• Once stable on CPAP and the tracheomalacia improves, the patient may be a candidate for tracheostomy downsizing and eventual decannulation 5
• Surgical options (aortopexy, tracheobronchoplasty) may be considered if conservative management with CPAP fails, though these carry approximately 10% complication rates 1, 6

Critical Pitfalls to Avoid

• Do not use bronchodilators or beta-agonists as they may worsen dynamic airway collapse by relaxing central airway smooth muscle 1
• Ensure two oxygen sources are available at bedside (wall oxygen and portable supply) for emergency management 4
• Keep emergency tracheostomy equipment immediately available, including tubes one size smaller than current tube 4