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Bronchopulmonary Sequestration

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sequestration
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What is a bronchopulmonary sequestration (BPS or pulmonary sequestration)?

A BPS is a benign mass of non-functioning lung tissue that appears during early lung development. This lesion has no connection with the airway and receives its blood supply from the systemic circulation usually off the abdominal or thoracic aorta. The most common type of BPS is formed within the normal lung itself and is referred to as intralobar. The other type of BPS is known as extralobar, and is formed outside the normal lung. This type of lesion is usually found in the chest cavity although rarely it may be found in the abdomen. There is a higher incidence of associated anomalies in babies with extralobar BPS. Other anomalies often present with extralobar BPH include a congenital diaphragmatic hernia (CDH), pectus excavactum, tracheoesophageal fistula, esophageal duplication and congenital heart disease.

What testing is recommended during the pregnancy?

Serial ultrasounds will monitor the size of the baby's BPS and rule out other anomalies. Color flow Doppler ultrasound evaluation is used to demonstrate the abnormal blood supply. In some cases, the mass will get smaller and may disappear. However, in other cases the mass can grow. In large lesions, a fetal MRI may be helpful in confirming the diagnosis and determining lung volumes. A fetal echocardiogram is recommended.

Are there special considerations for the delivery?

The delivery should be planned at a hospital with pediatric surgical services and a neonatal intensive care unit. Although the majority of infants with small BPS are asymptomatic, large lesions may be associated with pulmonary hypoplasia and may require mechanical ventilation and ECMO.

Is there fetal treatment for BPS?

If the BPS is pressing on the esophagus, polyhydramnios may develop. The additional amniotic fluid may precipitate premature labor. Amnioreduction, or removal of the extra amniotic fluid, may be considered to reduce the risk of early labor.

Babies with a large BPS may develop increased fluid in the chest cavity which may precipitate hydrops. A thoracoamniotic shunt may be placed in the baby to drain this fluid and reverse the hydrops.

In rare cases, a giant BPS not associated with increased fluid in the chest may cause hydrops and require open fetal surgery to remove the lesion. In select cases, babies with giant BPS may be candidates for the EXIT procedure. Babies with BPS who have developed hydrops, and are over 30 weeks gestation, should be considered for early delivery.

What is the treatment for BPS?

Removal of the BPS is performed after birth. In symptomatic patients, this surgery is performed in the newborn period. In more severe cases, mechanical ventilation and ECMO are required.

Asymptomatic patients receive a chest X-ray usually in the newborn period and are discharged home. These patients will have a CT scan as an outpatient to better define the anatomy. Surgery is performed within the first year of life to prevent infection. Many of these lesions can be removed thoracoscopically (using a scope and tiny incisions). In asymptomatic patients, lung function after surgery is usually excellent.

Are there other health problems that are more common among babies with BPS?

The overall outcome for babies with BPS is excellent. However, babies with giant lesions often have pulmonary hypoplasia with subsequent breathing problems after surgery, including the need for prolonged ventilation and supplemental oxygen as an infant.

Can this happen again with another pregnancy?

BPS typically occurs as a sporadic anomaly and is not known to have a significantly increased risk of recurrence. There is no known genetic predisposition. Your doctor and a genetic counselor will review the risk in your family.