Bronchopulmonary sequestration

Bronchopulmonary Sequestration

Bronchopulmonary sequestration (BPS) is a rare congenital malformation of the lower respiratory tract. It is characterized by a mass of non-functioning lung tissue that lacks normal connection with the bronchial tree and receives its blood supply abnormally from the systemic circulation, typically the aorta.

Classification

Bronchopulmonary sequestration is classified into two main types:

Intralobar Sequestration (ILS)

Intralobar sequestration is the more common type, accounting for approximately 75% of cases. In ILS, the sequestered lung tissue is located within the normal lobe and shares the visceral pleura with the surrounding lung tissue. It is usually found in the lower lobes, with a slight predilection for the left side.

Extralobar Sequestration (ELS)

Extralobar sequestration is less common and is characterized by sequestered lung tissue that is completely separate from the normal lung and has its own pleural covering. ELS can be located anywhere in the thorax or abdomen and is often associated with other congenital anomalies.

Pathophysiology

The pathogenesis of bronchopulmonary sequestration is not completely understood, but it is believed to result from an abnormal budding of the primitive foregut during embryonic development. This leads to the formation of a mass of lung tissue that does not communicate with the tracheobronchial tree and receives its blood supply from systemic arteries.

Clinical Presentation

The clinical presentation of bronchopulmonary sequestration varies depending on the type and size of the sequestration. Many cases are asymptomatic and are discovered incidentally during imaging for other reasons. Symptomatic cases may present with recurrent pulmonary infections, cough, hemoptysis, or respiratory distress.

Diagnosis

Diagnosis of bronchopulmonary sequestration is typically made through imaging studies.

Imaging

• Chest X-ray: May show an abnormal mass or opacity in the lung.
• CT Scan: Provides detailed images of the lung and can identify the systemic arterial supply to the sequestered tissue.
• MRI: Useful in delineating the anatomy and vascular supply.
• Angiography: Can be used to confirm the diagnosis by visualizing the aberrant blood supply.

Treatment

The treatment of choice for symptomatic bronchopulmonary sequestration is surgical resection. This involves removing the sequestered lung tissue and ligating the aberrant blood supply. Asymptomatic cases may be managed conservatively with regular follow-up.

Prognosis

The prognosis for patients with bronchopulmonary sequestration is generally good following surgical resection. Complications are rare but can include infection, bleeding, or recurrence if the sequestration is not completely removed.

Also see

• Congenital Pulmonary Airway Malformation
• Pulmonary Arteriovenous Malformation
• Congenital Diaphragmatic Hernia