Vascular ring

Background

  • Congenital anatomic abnormalities of the aortic arch
    • Compression of the trachealbronchial tree and esophagus
    • Leads to respiratory and GI symptoms.
  • Accounts for 1-3 of congenital heart disease
  • Male:Female 2:1

Clinical Features

Differential Diagnosis

Pediatric stridor

  • A minimal amount of edema or inflammation in the pediatric airway can result in significant obstruction
    • Can lead to rapid decompensation

<6mo

  • Laryngotracheomalacia
    • Accounts for 60%
    • Usually exacerbated by viral URI
    • Dx w/ flexible fiberoptic laryngoscopy
  • Vocal cord paralysis
    • Stridor associated w/ feeding problems, hoarse voice, weak and/or changing cry
    • May have cyanosis or apnea if bilateral (less common)
  • Subglottic stenosis
    • Congenital vs 2/2 prolonged intubation in premies
  • Airway hemangioma
    • Usually regresses by age 5
    • Associated w/ skin hemangiomas in beard distribution
  • Vascular ring/sling

>6mo

  • 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
    • Sx 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

  • Unlikely to be definitively diagnosed in ED
  • CXR
    • Not sensitive, but good place to start
  • Barium esophagography
  • Echocardiography
  • Bronchoscopy, cardiac catheterization

Management

  • Surgical management for all symptomatic patients

Disposition

  • Admit if symptomatic or new diagnosis

See Also

References