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Cardiology11 papers

Congenital anomaly of lung

Last edited: 4/15/2026

Overview

Congenital lobar emphysema (CLE) is a rare developmental anomaly characterized by overdistension of one or more lobes of the lung due to airway obstruction or malformation. In this case, it presented alongside a tracheal bronchus and Tetralogy of Fallot in a three-month-old infant 1.

Diagnosis

  • Clinical Presentation: Respiratory distress, heart murmur 1.
  • Imaging: Chest X-ray showing hyperaeration of a specific lung lobe 1.
  • Bronchoscopy: Essential for identifying anomalous bronchial connections 1.
  • Bronchography: Can confirm the origin and nature of the anomalous bronchus 1.

    • Management

  • Surgical Intervention: Lobectomy or segmentectomy for definitive treatment 1.
  • Supportive Care: Mechanical ventilation and respiratory support as needed 1.
  • Cardiac Management: Addressing associated congenital heart defects concurrently 1.

    • Special Populations

  • Pediatrics: Early surgical intervention is crucial in infants due to respiratory compromise 1.

    • Key Recommendations

  • Perform bronchoscopy and imaging (chest X-ray, bronchography) for definitive diagnosis of congenital lobar emphysema with tracheal bronchus 1 (Evidence: Weak).
  • Consider simultaneous management of associated congenital heart defects, such as Tetralogy of Fallot, during surgical intervention 1 (Evidence: Weak).
  • Early surgical resection (lobectomy or segmentectomy) is recommended to prevent respiratory failure in pediatric patients 1 (Evidence: Weak).

    • References

    1 Keller MS. Congenital lobar emphysema with tracheal bronchus. Journal of the Canadian Association of Radiologists 1983. link

    Original source

    1. [1]
      Congenital lobar emphysema with tracheal bronchus. — Keller MS Journal of the Canadian Association of Radiologists (1983)
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