Bronchopulmonary Sequestration

Also known as: BPS, pulmonary sequestration, accessory lung.

What is bronchopulmonary sequestration?

When a fetus develops a cystic piece of nonfunctioning abnormal lung tissue either within a lung (intralobar sequestration) or next to it (extralobar- more common in boys) that is not connected to the body’s airways, its known as bronchopulmonary sequestration.

What causes bronchopulmonary sequestration?

The cause of the rare congenital abnormality of lung development that occurs before birth is unknown. It does not appear to be inherited from parents and does not run in families. Other congenital malformations may be present (10%).

What are the symptoms of bronchopulmonary sequestration?

Bronchopulmonary sequestration may be diagnosed prenatally and have no symptoms during the neonatal period. Later in early childhood children may present with multiple episodes of pneumonia and a chronic cough. If the sequestered cystic piece enlarges it can compress normal lung resulting in breathing and heart problems. Extralobar sequestration may present with gastrointestinal symptoms i.e feeding difficulties.

What are bronchopulmonary sequestration care options?

A bronchopulmonary sequestration is typically removed surgically a short time after the baby is born. Outcomes from the surgery are typically quite good.

Reviewed by: Jack Wolfsdorf, MD, FAAP

This page was last updated on: September 09, 2019 03:22 PM