Tracheomalacia

Background

Cross section of a trachea and esophagus anatomy.

Tracheal anatomy.

• 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

Pediatric stridor

<6 Months Old

• Laryngotracheomalacia
  • Accounts for 60%
  • Usually exacerbated by viral URI
  • Diagnosed with flexible fiberoptic laryngoscopy
• Vocal cord paralysis
  • Stridor associated with feeding problems, hoarse voice, weak and/or changing cry
  • May have cyanosis or apnea if bilateral (less common)
• Subglottic stenosis
  • Congenital vs secondary to prolonged intubation in premies
• Airway hemangioma
  • Usually regresses by age 5
  • Associated with skin hemangiomas in beard distribution
• Vascular ring/sling

>6 Months Old

• Croup
  • viral laryngotracheobronchitis
  • 6 mo - 3 yr, peaks at 2 yrs
  • Most severe on 3rd-4th day of illness
  • Steeple sign not reliable- diagnose clinically
• Epiglottitis
  • H flu type B
    • Have higher suspicion in unvaccinated children
  • Rapid onset sore throat, fever, drooling
  • Difficult airway- call anesthesia/ ENT early
• Bacterial tracheitis
  • Rare but causes life-threatening obstruction
  • Symptoms of croup + toxic-appearing = bacterial tracheitis
• Foreign body (sudden onset)
  • Marked variation in quality or pattern of stridor
• Retropharyngeal abscess
  • Fever, neck pain, dysphagia, muffled voice, drooling, neck stiffness/torticollis/extension

Evaluation

(A) Tracheal structure with normal C-shape rings. (B) U-shaped rings with a wider posterior membrane, demonstrating posterior intrusion. (C) Bow-shaped rings with a broad posterior membrane and severe posterior intrusion.

Bronchoscopy in a neonate showing narrowing of tracheomalacia.

Workup

• CXR

Diagnosis

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

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

See Also

• Laryngomalacia