II. Epidemiology

  1. Presents in infancy

III. Pathophysiology

  1. Weakness and softness of the trachea's cartilage and walls
  2. Abnormally soft cartilage more easily collapses, narrowing the trachea, especially with expiration
  3. May result in difficult clearance of airway secretions

IV. Causes

  1. Congenital (most common)
  2. Other congenital anomalies
    1. Tracheoesophageal fistula
    2. Vascular Ring
  3. Prolonged intubation
  4. Prolonged Pediatric Gastroesophageal Reflux disease
  5. Iatrogenic injury (e.g. tracheotomy)

V. Findings

  1. Barking cough
  2. Expiratory Wheeze or Inspiratory Stridor
  3. Provocative factors (increase Wheezing)
    1. Crying
    2. Feeding
    3. Cough
    4. Nebulized beta Agonists

VII. Diagnosis

  1. Bronchoscopy (3 phase dynamic)
    1. Child breathes spontaneously
    2. Airway lumen narrows >50% with coughing or forced exhalation

VIII. Management

  1. Consult pediatric pulmonology
  2. Supportive care (most cases)
  3. Bethanechol
    1. Muscarinic Agonist increases trachealis Muscle tone, decreasing trachea compliance
  4. Severe Tracheomalacia
    1. Tracheostomy (risk of acquired tracheal stenosis)
    2. CPAP

IX. Course

  1. Self limited
  2. Improves by age 12 to 24 months

X. Complications

  1. Pneumonia (due to insufficient mucus clearance)

XI. References

  1. Magafas (2026) Crit Dec Emerg Med 40(6): 17-8
  2. Mehta and Eliason (2024) Crit Dec Emerg Med 38(6): 27-35
  3. Benjamin (1984) Ann Otol Rhinol Laryngol 93:438-42 [PubMed]

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