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Prenatal diagnosis and management of bronchopulmonary sequestration

Dorothy I Bulas, MD
Alexia Egloff, MD
Section Editors
Deborah Levine, MD
Louise Wilkins-Haug, MD, PhD
Deputy Editor
Vanessa A Barss, MD, FACOG


Bronchopulmonary sequestration (BPS) is a congenital anomaly consisting of a nonfunctioning mass of lung tissue that lacks normal communication with the tracheobronchial tree and receives its blood supply from the systemic (rather than the pulmonary) circulation [1]. The feeding vessel most commonly originates from the aorta, although other origins, such as the splenic artery or gastric artery, have been described [2]. BPS accounts for only 0.15 to 6.4 percent of congenital lung malformations [3]. Eighty percent of cases occur on the left [4].

BPS is classified anatomically as intralobar or extralobar:

In intralobar pulmonary sequestration (ILS, also known as intrapulmonary sequestration), the mass is located within the normal lung and is covered by the visceral pleura of the lung. Postnatally, ILS accounts for about 75 percent of BPS and is almost always located in the lower lobes, most commonly in the left medial or posterior segment [5,6]. Venous drainage is usually via the pulmonary vein to the left atrium, although connections to the vena cava, azygous vein, or right atrium also occur. ILS is distributed equally between sexes.

In extralobar pulmonary sequestration (ELS, also known as extrapulmonary sequestration), the mass is located outside the normal lung and has its own visceral pleura. Postnatally, about 25 percent of BPS is extralobar [7]. An extralobar lesion can occur between the neck and diaphragm, within the diaphragm, or infradiaphragmatically, where it can mimic a suprarenal neuroblastoma [5,7,8]. The most common location is between the left lower lobe and hemidiaphragm (80 percent) [6,9]. Ten to 15 percent are subdiaphragmatic, usually in the suprarenal space and left-sided (90 percent) [10]. Most ELS drains to the systemic circulation through the azygous or hemiazygous vein or vena cava [3]. ELS is more likely to affect males and to be associated with anomalies, including congenital diaphragmatic hernia or eventration, cardiac defects, and foregut anomalies.

Hybrid combinations of congenital pulmonary airway malformation (CPAM) and BPS are relatively common, and have connections with both the pulmonary and systemic arterial supply.


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Literature review current through: Sep 2016. | This topic last updated: Dec 2, 2015.
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