Children's Hospital Colorado
Colorado Fetal Care Center

Bronchopulmonary Sequestration

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What is bronchopulmonary sequestration (BPS)?

Bronchopulmonary sequestration (BPS) is a mass of nonfunctioning lung tissue that does not communicate with the bronchioles, the passages that move air and in out of the lungs.

There are two types of BPS: intralobar (inside a lung lobe) or extralobar (outside of the lung with its own pleural cover). Intralobar BPS is more common, accounting for 75% of cases, and it is located in the lower lobe of the lung in 98% of cases. Extralobar BPS is usually located in the lower part of the chest, closer to the back, with about 90% of masses occurring on the left side.

Bronchopulmonary sequestration can also be intrathoracic (inside the chest) or extrathoracic (outside the chest). These masses receive blood supply from a systematic "feeding" vessel, such as the pulmonary artery, which help them grow. As they increase in size, these masses may cause amniotic fluid to accumulate in the chest or abdomen of the baby, making intervention necessary.

Who gets bronchopulmonary sequestration?

While the cause of BPS is not entirely known, there is a slightly higher chance for male babies to be diagnosed with this condition. It is not believed to be genetic, as there is no familial predisposition. Extralobar BPS is much more common in the fetus and newborn than intralobar BPS.

Bronchopulmonary sequestration may compress lung tissue or push the heart into an abnormal position. Extralobar BPS can also twist, causing surrounding blood vessels to twist as well (called "torsion"). This could cause hydrops, or fluid accumulation, to develop. The BPS may cause extra fluid to build up around the fetus, as well, which is called polyhydramnios.

In some cases, other abnormalities seen in and around the chest and upper abdomen may be associated with BPS. It is less common to see associated abnormalities in the intralobar type of BPS (only 10% of cases), but abnormalities are seen in 60% of cases of extralobar BPS. Because of its size, a BPS mass might result in incomplete development or underdevelopment of a baby's lungs, which could significantly impact the ability to breath after delivery.

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