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What is bronchopulmonary 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 Extralobar sequestration?

Extrapulmonary sequestration. Computed tomography. Pulmonary sequestration is a rare congenital malformation in which a segment of nonfunctional lung lacks a bronchial connection and possesses an aberrant blood supply. Most often, blood supply is from the infradiaphragmatic aorta.

What is the most common form of pulmonary sequestration?

Pulmonary sequestration represents approximately 6% of all congenital pulmonary malformations. Intrapulmonary sequestrations are the most common form, and 60% of these are found in the posterior basal segment of the left lower lobe. Overall, 98% occur in the lower lobes.

How common is bronchopulmonary sequestration?

It is defined as functionless lung parenchyma without connection or with abnormal connection to the tracheobronchial system and has an abnormal blood supply. Lung sequestration is very rare and only represents 0.15–6.4% of congenital pulmonary abnormalities [2].

Is pulmonary sequestration hereditary?

While the vast majority of pulmonary sequestration cases occur in isolation (without any family history ), rare familial cases have been reported.

What causes pulmonary sequestration?

Pulmonary sequestration appears to result from abnormal budding of the primitive foregut. The tissue in this accessory lung bud migrates with the developing lung, but does not communicate with it. It receives its blood supply from vessels that connect to the aorta or one of its side branches.

What is lung 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 the IOC for sequestration lung?

A pulmonary sequestration is a medical condition wherein a piece of tissue that ultimately develops into lung tissue is not attached to the pulmonary arterial blood supply, as is the case in normally developing lung….

Pulmonary sequestration
Specialty Pulmonology

Is Pulmonary sequestration congenital?

Does CPAM require surgery?

Newborns with very large lesions may need to have surgery immediately after birth to remove the CPAM and prevent breathing problems. In some cases, the CPAM can be removed at birth with a surgery called the EXIT procedure.