What causes pulmonary sequestration?

Pulmonary sequestration is a rare congenital (present from birth) malformation where non-functioning lung tissue is separated from the rest of the lung and supplied with blood from an unusual source, often an artery from systemic circulation.

What is Intralobar pulmonary sequestration?

Intralobar pulmonary sequestration is characterized by the presence of nonfunctional parenchymal lung tissue, receiving systemic arterial blood supply. It lacks normal communication with tracheobronchial tree. Failure to diagnose and treat this condition can lead to recurrent pneumonia and fatal hemoptysis.

How is pulmonary sequestration diagnosed?

Pulmonary sequestrations are diagnosed with a prenatal ultrasound showing a mass in the chest of the fetus. The mass may displace the heart from its normal position or push the diaphragm downward, but the key feature of a sequestration is the artery leading from the cystic mass directly to the aorta.

What is a pulmonary sequestration?

Bronchopulmonary sequestration, also known as BPS or pulmonary sequestration, is a rare birth defect in which an abnormal mass of nonfunctioning lung tissue forms during prenatal development. It can form outside (extralobar) or inside (intralobar) the lungs, but is not connected directly to the airways.

Is Pulmonary sequestration life threatening?

While it is not in itself a life-threatening condition, a pulmonary sequestration can cause health complications including cardiovascular problems, long-term infections like tuberculosis, and bronchial cancer. It could be fatal if blood vessels in the lung begin to hemorrhage.

What is the most common form of pulmonary sequestration?

The PS is divided into two types, intralobar sequestration (ILS) which is the more common type, where the lesion lies within pleural layer surrounding the lobar lung and extralobar sequestration (ELS) which has its own pleural covering, maintaining complete anatomic separation from adjacent normal lung [5].

Is pulmonary sequestration serious?

What is Extralobar sequestration?

Intralobar sequestration (ILS) is abnormal lung tissue surrounded by normal lung pleura, while extralobar sequestration has its own separate pleura. Extralobar sequestration is less common than its intralobar counterpart and comprises approximately 25% of all sequestrations.

Is pulmonary sequestration life threatening?

How is pulmonary sequestration treated?

Surgical resection is the treatment of choice and ILS often requires lobectomy. Open thoracotomy remains the best approach with safe isolation and division of anomalous systemic feeding arteries. However, complete thoracoscopic resection has been reported with low morbidity and mortality [7].

Is Pulmonary sequestration serious?

How common is pulmonary sequestration?

Pulmonary sequestration, both intralobar or extralobar, is a rare clinical phenomenon with less than 6% incidence among all congenital lung malformations.