Vascular Ring

Aka: Vascular Ring, Vascular Sling, Pulmonary Sling

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II. Definitions

  1. Vascular Ring
    1. Developmental defect of the aortic arch system
    2. Trachea and Esophagus are completely encircled by connected segments of the aortic arch and its branches

III. Epidemiology

  1. Symptoms develop in first few months of life

IV. Pathophysiology

  1. Large vessels apply local pressure compressing mediastinal structures
    1. Compress trachea and Bronchial tree
    2. Compress Esophagus

V. Causes

  1. Double aortic arch
  2. Aberrant innominate artery
  3. Right aortic arch
  4. Pulmonary Sling

VI. Signs

  1. Low pitched inspiratory and expiratory gargling sounds
  2. Inspiratory Stridor
  3. Expiratory Wheezes
  4. Obstructive Sleep Apnea
  5. Feeding difficulties may occur
  6. Sounds present since birth
    1. Established over first month of life
    2. Parent may not be aware of sounds
  7. Provocative maneuvers that increase Dyspnea
    1. Neck flexion
    2. Upper Respiratory Infections
  8. Palliative maneuvers that decrease Dyspnea
    1. Neck extension

VII. Radiology

  1. Chest XRay
    1. Note aortic arch location (right or left)
    2. Note tracheal caliber
  2. Barium swallow
    1. Note Esophageal indentation

VIII. Diagnostics

  1. Bronchoscopy
  2. Angiography

IX. Course

  1. Respiratory symptoms worsen with increased age, size

X. Management

  1. Requires surgery

XI. Complications

  1. Obstructive Sleep Apnea
  2. Tracheomalacia

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