1. Definition & Embryology

  • Definition: A congenital malformation of the bronchial tree representing a foregut duplication cyst.
  • Etiology: Results from abnormal budding of the ventral foregut (tracheobronchial tree) between the 4th and 6th week of gestation.
  • Pathology:
    • Lined by ciliated pseudostratified columnar (respiratory) epithelium.
    • Wall contains bronchial elements: Cartilage, smooth muscle, and mucous glands.
    • Filled with mucoid or proteinaceous fluid.

2. Location & Classification

They are usually solitary and do not communicate with the tracheobronchial tree (unless infected/ruptured).

  1. Mediastinal (65-75%): Most common.
    • Typical site: Middle Mediastinum, near the Carina or paratracheal area.
  2. Intraparenchymal (15-25%): Within the lung tissue (usually lower lobes).
  3. Ectopic (Rare): Neck, pericardium, or sub-diaphragmatic.

3. Clinical Features

Presentation varies by age and size.

  • Asymptomatic: Often incidental findings on CXR/CT in older children/adults.
  • Symptomatic (Compression): Due to critical location near airways (carina).
    • Stridor, wheezing, dyspnea.
    • Dysphagia (esophageal compression).
    • Air trapping/hyperinflation of the affected lung.
  • Symptomatic (Infection): Fever, cough, purulent sputum (if ruptured into bronchus).

4. Diagnosis

  • Chest X-ray:
    • Smooth, round, well-defined opacity.
    • Usually in the mediastinum/hilar region.
    • Air-fluid level seen only if communication with airway exists (infection).
  • CT Thorax (Gold Standard):
    • Homogeneous, non-enhancing mass.
    • Fluid density (0–20 HU) usually, but high protein/calcium content can mimic soft tissue density.
    • Defines anatomical relationship to trachea/esophagus.
  • MRI: Useful if CT is indeterminate; cyst fluid is very bright on T2-weighted images (“Light bulb” sign).

5. Complications & Management

Complications:

  • Recurrent superinfection/abscess formation.
  • Massive hemoptysis (rare).
  • Malignant Transformation: Rare risk of developing carcinoma or sarcoma within the cyst wall.

Treatment:

  • Surgical Excision: The standard of care for all cysts (including asymptomatic ones).
    • Rationale: Prevention of infection, compression, and malignancy.
  • Approach:
    • Video-Assisted Thoracoscopic Surgery (VATS) is preferred.
    • Thoracotomy for complex/large adherent cysts.
    • Note: Incomplete excision can lead to recurrence.