Pulmonary sequestration

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. The arterial supply is derived in most cases from the thoracic aorta (75%) or the abdominal aorta (20%). In some cases (15%), two different arteries supply the blood.

If the accessory lung bud develops early in embryonic development, the pulmonary sequestration occurs among the normal lung tissue, where it is encased within the pleural sac. This results in intralobular pulmonary sequestration. Venous drainage of intralobular pulmonary sequestration is usually through the pulmonary circulation. If the accessory lung bud develops later, extralobular pulmonary sequestration results. This type of pulmonary sequestration is separated from the normal lung tissue by its own visceral pleura and can occur above, within, or below the diaphragm. Venous drainage is usually through the systemic circulation.

Last updated on 05-01-20

Is pulmonary sequestration inherited?

While the vast majority of pulmonary sequestration cases occur in isolation (without any family history), rare familial cases have been reported. Therefore, a genetic component cannot be ruled out.

Last updated on 05-01-20

How might extralobular pulmonary sequestration be treated?

Traditional treatment of extralobular pulmonary sequestration involves surgical removal via mini-thoracotomy for patients that are experiencing symptoms. Less invasive surgery techniques may include thorascopic surgery and coil embolization.

Last updated on 05-01-20

How might intralobular pulmonary sequestration be treated?

Due to the risk for infection and bleeding, intralobar pulmonary sequestrations are usually removed, either by segmentectomy (removal of part of the lung) or lobectomy (removal of the full lobe). Angiography along with CT scan (with or without contrast) and MRI are generally used to visualize the origins of the blood supply before surgery is performed.

Last updated on 05-01-20

Intra-abdominal Extralobar Pulmonary Sequestration (EPS) University Center for Fetal Medicine. Reference Link Erin G. Brown, Clifford Marr, Diana Farmer. Extralobar pulmonary sequestration: The importance of intraoperative vigilance Journal of Pediatric Surgery Case Reports. April 2013; 1(4). 74-76. Reference Link

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The RareGuru disease database is regularly updated using data generously provided by GARD, the United States Genetic and Rare Disease Information Center.

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