II. Definitions
- Vascular Ring
- Developmental defect of the aortic arch system
- Trachea and Esophagus are completely encircled by connected segments of the aortic arch and its branches
III. Epidemiology
- Symptoms develop in first few months of life
IV. Pathophysiology
V. Causes
- Double aortic arch
- Aberrant innominate artery
- Right aortic arch
- Pulmonary Sling
VI. 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
VII. Radiology
-
Chest XRay
- Note aortic arch location (right or left)
- Note tracheal caliber
- Barium swallow
- Note Esophageal indentation
VIII. Diagnostics
- Bronchoscopy
- Angiography
IX. Course
- Respiratory symptoms worsen with increased age, size
X. Management
- Requires surgery
XI. Complications
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Related Studies
| Concepts | Congenital Abnormality (T019) |
| SnomedCT | 204426005, 253659000, 110409004, 66403007 |
| English | Vascular rings, vascular ring, vascular rings, vascular sling, Vascular ring, Vascular rings (disorder), Vascular ring (morphologic abnormality), ring; vascular, vascular; ring, Vascular ring - disorder |
| Spanish | anillos vasculares (trastorno), anillos vasculares, anillo vascular (anomalía morfológica), anillo vascular |
| Dutch | ring; vasculair, vasculair; ring |
Ontology: Origin of left pulmonary artery from right pulmonary artery (C0345041)
| Concepts | Congenital Abnormality (T019) |
| SnomedCT | 253638007 |
| English | Origin LPA from RPA, pulmonary sling, Origin of left pulmonary artery from right pulmonary artery, Pulmonary sling, Origin of left pulmonary artery from right pulmonary artery (disorder) |
| Spanish | origen en la arteria pulmonar derecha de la arteria pulmonar izquierda (trastorno), origen en la arteria pulmonar derecha de la arteria pulmonar izquierda |
