Pulmonary Sequestration
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Pulmonary Sequestration

Cardio-thoracic SurgeryPaediatricsPaediatric Surgery

Pulmonary sequestration is a rare congenital lung anomaly, characterized by a portion of lung tissue that does not connect to the normal bronchial tree and receives its blood supply from the systemic circulation (e.g., aorta) rather than the pulmonary arteries. This sequestered lung tissue is non-functioning and prone to infection or abscess formation.

Types of Pulmonary Sequestration

Pulmonary sequestration is a congenital lung anomaly characterized by a portion of lung tissue that does not participate in normal respiration and receives its blood supply from systemic arteries (e.g., the aorta) rather than the pulmonary artery.

Normal Anatomy
 

  • Trachea: Delivers air from the throat to the lungs.
  • Lungs: Conduct gas exchange, inhaling oxygen and exhaling carbon dioxide.
  • Heart: Pumps blood, including to and from the lungs.
  • Diaphragm: Moves up and down during breathing to help the lungs expand and contract.

Intralobar Sequestration

The sequestered tissue lies within a normal lobe, without a separate pleural covering (more common).

  • Location: Within a normal lung lobe, shares the pleura with surrounding normal lung tissue.
  • Blood Supply: From systemic arteries (e.g., branches of the aorta).
  • Venous Drainage: Drains into pulmonary veins.
  • Typical Age of Discovery: Often found in adolescents or adults.
  • Clinical Presentation: Recurrent lung infections, cough, sputum production, sometimes chest pain.
  • Imaging: Cystic or solid lung mass with systemic arterial supply.

Extralobar Sequestration

The sequestered tissue is completely separate from normal lung and has its own pleura.

  • Location: Outside the normal lung lobes, with its own separate pleural covering.
  • Blood Supply: Also from systemic arteries.
  • Venous Drainage: Into systemic veins (e.g., azygos vein, inferior vena cava).
  • Typical Age of Discovery: Usually diagnosed during newborn or infancy, often asymptomatic.
  • Appearance: Appears as a separate lung-like mass, disconnected from the normal lung.

Common Symptoms

Symptoms are often related to recurrent lung infections, though some cases may be asymptomatic and discovered incidentally:

  • Persistent or recurrent coughing
  • Chronic productive cough, sometimes with blood
  • Shortness of breath or wheezing
  • Fever
  • Chest pain (occasional)
  • In infants: respiratory distress or poor growth

Untreated, the abnormal tissue may lead to lung abscesses or bleeding.

Causes & Risk Factors

Pulmonary sequestration is a congenital malformation, present from fetal development. Though the exact cause is unclear, it is believed to be due to abnormal budding of the lung tissue during embryogenesis.

Risk factors include:

  • Abnormal thoracic development in the fetus
  • May occur with other congenital anomalies (e.g., diaphragmatic hernia)

Diagnosis

Accurate diagnosis requires imaging studies to assess anatomy and blood supply:

  1. Chest X-ray: May show abnormal lung shadows, but cannot identify the vascular supply.
  2. CT Thorax with Contrast / CT Angiography: Commonly used; clearly shows the abnormal tissue and feeding artery.
  3. Magnetic Resonance Angiography (MRA): Radiation-free, suitable for children or specific patient groups.
  4. Prenatal Ultrasound: May be detected during fetal scans.
  5. Conventional Angiography: Used when planning interventional treatment.

Treatments

Surgery

  • Intralobar sequestration: Surgery is recommended to remove the abnormal segment and prevent infection.
     
  • Extralobar sequestration: May be monitored if asymptomatic, but resection is often preferred to avoid future complications.
     
  • Video-Assisted Thoracoscopic Surgery (VATS) is the minimally-invasive approach of first choice. Its advantages include: Small incisions, shorter recovery time, less postoperative pain

Interventional Embolization

For patients who are unfit for surgery, embolization of the feeding artery can cause the abnormal tissue to shrink.

FAQ

No. It is a non-cancerous congenital anomaly. Malignant transformation only in very rare circumstances.
While some cases are asymptomatic, most doctors recommend preventive surgery due to infection or bleeding risks.
Recovery is typically excellent after resection, especially with minimally invasive techniques. Lung function impairment is mild.
There is no evidence suggesting a hereditary pattern.
No. If no other anomalies are present, the fetus can be monitored until birth. Termination is usually unnecessary.

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