• 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[1]
      • Characterized by cyanosis, apnea, bradycardia, and hypotonia that require resuscitation[2]
  • Severe
    • Stridor at rest
    • Biphasic stridor
    • Dyspnea with feeding
    • Expiratory wheezing with respiratory infections

Differential Diagnosis



Infectious Disorders


Neoplastic Disorders

  • Neoplasms/tumors

Allergic and Auto-Immune Disorders

  • Spasmodic/tracheobronchitis
  • Angioedema/Angioneurotic edema

Metabolic, Storage Disorders

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

Biochemical Disorders

Congenital, Developmental Disorders

Psychiatric Disorders

  • Somatization disorder

Anatomical or Mechanical

  • Foreign body aspiration
  • Acute gastric acid/aspiration syndrome
  • Airway obstruction
  • Neck compartment hemorrhage/hematoma
  • Vocal Cord Dysfunction [3]

Vegetative, Autonomic, Endocrine Disorders


  • Smoke inhalation
  • Chemical burn/esophagus

Chronic Pediatric Conditions


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


  • CXR


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


  • Admit


    • Kovesi T, Rubin S. Long-Term Complications of Congenital Esophageal Astresia and/or Tracheoesophageal Fistula. Chest. 2004;915-925.
    • Boudewyns A, Claes J, Van de Heyning P. Clinical Practic: An approach to stridor in infants and children. Eur J Pediatr. 2010;169:135-141
  1. Vocal Cord Dysfunction on Internet Book of Critical Care
  2. Ernst A, Feller-Kopman D, Becker HD, Mehta AC. Central airway obstruction. Am J Respir Crit Care Med 2004
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