II. Epidemiology
- Symptoms develop in first few months of life
III. Pathophysiology
- Large vessels apply local pressure
- Compress trachea and Bronchial tree
- Compress esophagus
IV. Causes
- Double aortic arch
- Aberrant innominate artery
- Right aortic arch
- Pulmonary Sling
V. Signs
- Low pitched inspiratory and expiratory gargling sounds
- Inspiratory Stridor
- Expiratory Wheezes
- Obstructive Sleep Apnea
- Feeding difficulties may occur
- Sounds present since birth
- Established over first month of life
- Parent may not be aware of sounds
- Provocative maneuvers that increase Dyspnea
- Neck flexion
- Upper Respiratory Infections
- Palliative maneuvers that decrease Dyspnea
- Neck extension
VI. Radiology
-
Chest XRay
- Note aortic arch location (right or left)
- Note tracheal caliber
- Barium swallow
- Note Esophageal indentation
VII. Diagnostics
- Bronchoscopy
- Angiography
VIII. Course
- Respiratory symptoms worsen with increased age, size
IX. Management
- Requires surgery