Tracheomalacia

Background

• Congenital narrowing of the larygeal cartilage that causes upper airway obstruction and problems with swallowing.
  • A frequent complication of surgical repair of esophageal atresia (EA) and TEF
• Most common cause of stridor in infancy
  • Typically infant to <2 years
• Affects the epiglottis and/or arytenoid cartilages
• When the epiglottis is usually folds in on itself because it is abnormally elongated, and the arytenoid cartilages are abnormally large. Because the misshapen epiglottis does not function properly, it can prolapse during inspiration, causing a high pitched "stridor".

Clinical Features

• Infants
  • Brassy, barking cough
  • Can also develop “dying/death spells” that begin after 2–3 months of age
    • Characterized by cyanosis, apnea, bradycardia, and hypotonia that require resuscitation
• Severe
  • Stridor at rest
  • Biphasic stridor
  • Dyspnea with feeding
  • Expiratory wheezing with respiratory infections

Differential Diagnosis

Stridor

Trauma

• Larynx fracture
• Tracheobronchial tear/injury
• Thyroid gland injury/trauma
• Trachea injury
• Electromagnetic or radiation exposure
• Burns, inhalation

Infectious Disorders

• Bacterial tracheitis
• Diphtheria
• Tetanus
• Tracheobronchial tuberculosis
• Poliomyelitis, paralytic, bulbar
• Poliomyelitis, acute
• Fungal laryngitis

Abscesses

• Retropharyngeal abscess
• Epiglottitis, acute
• Peritonsillar abscess
• Laryngotracheobronchitis, acute
• Retropharyngeal abscess

Neoplastic Disorders

• Neoplasms/tumors

Allergic and Auto-Immune Disorders

• Croup, spasmodic/tracheobronchitis
• Angioedema/Angioneurotic edema

Metabolic, Storage Disorders

• Cerebral Gaucher's of infants (acute)
• Tracheobronchial amyloidosis

Biochemical Disorders

• Tetany

Congenital, Developmental Disorders

• Angioedema/Angioneurotic edema, hereditary

Psychiatric Disorders

• Somatization disorder

Anatomical or Mecanical

• Foreign Body Aspiration
• Acute gastric acid/aspiration syndrome
  • Airway obstruction
  • Neck compartment hemorrhage/hematoma

Vegetative, Autonomic, Endocrine Disorders

• Esophageal free reflux/GERD syndrome
• Laryngospasm, acute
  • Bilateral vocal cord paralysis
• Hypoparathyroidism

Poisoning

• Smoke inhalation
• Chemical burn/esophagus

Chronic Pediatric Conditions

• Laryngomalacia
• Tracheomalacia
• Subglottic stenosis or prior intubation
• Vascular ring (double aortic arch)
• Vocal cord dysfunction/paroxysmal vocal fold movement

Evaluation

• High clinical suspicion
• ENT consult for nasopharyngoscopy vs. bronchoscopy

Workup

• CXR

Management

• Close observation
• Recurrent “death spells”
  • May require more invasive intervention such as nasal CPAP temporarily
• Aortopexy or tracheostomy for long-term relief

Disposition

• Admit

References

Authors

Ross Donaldson, Anthony Lucero, Michael Holtz