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
- Stridor
- Respiratory infections
- Respiratory distress
- Wheezing
- Cough
- Less common
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
- H flu type B
- 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
- 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