What causes bronchopulmonary sequestration?
The cause of bronchopulmonary sequestration remains unknown. It has not been linked to a genetic or chromosomal anomaly, and does not appear to run in families (is not hereditary). Most clinicians believe the condition begins during prenatal development when an extra lung bud forms and migrates with the esophagus.
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].
What is sequestration lungs?
Pulmonary sequestration is a condition in which a segment or lobe of dysplastic lung tissue exists with no communication with the rest of the tracheobronchial tree and receives an anomalous systemic vascular supply, separate from the rest of the lung. It is, therefore, a nonfunctional tissue.
Is pulmonary sequestration serious?
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 are symptoms of pulmonary sequestration?
Pulmonary sequestrations may be defined as intralobular or extralobular, depending on their location. Symptoms may include a chronic or recurrent cough, respiratory distress or lung infection.
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.
Is pulmonary sequestration rare?
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 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.
What are the symptoms of pulmonary sequestration?
How is lung sequestration diagnosed?
The diagnosis of pulmonary sequestration traditionally requires arteriography to identify abnormal systemic vessels feeding the abnormal portion of the lung. Non-invasive imaging techniques have recently been used to replace arteriography.
What is considered the gold standard for diagnosis of pulmonary sequestration?
However, the gold standard for identifying the pulmonary sequestration recently is CT/MR angiography as it confirms the anatomy, identifies the anomalous systemic arterial supply, and shows the venous drainage [5].
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.
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