Bronchopulmonary Sequestration

How is bronchopulmonary sequestration diagnosed?

Bronchopulmonary sequestration will appear as a bright, white mass in the vicinity of a baby's lungs during routine ultrasound. There will be a clearly defined blood vessel feeding the lesion, which usually confirms the diagnosis of BPS. Usually, however, an ultrasound cannot determine if the BPS is intralobar or extralobar. Sometimes, the ultrasound will show fluid in the lungs, extra amniotic fluid or fluid in two more areas of the body, such as the skin, lungs, heart and/or abdomen (referred to as hydrops).

At least 75% of prenatally diagnosed cases of BPS become smaller spontaneously, while those associated with hydrops (fluid accumulation), pleural effusions (fluid in lung tissue) or mediastinal shift (organ movement) will require intervention during pregnancy or after delivery.

How is bronchopulmonary sequestration treated?

When a chest mass is identified, a very detailed ultrasound is needed to evaluate other potential diagnoses. An amniocentesis (a needle inserted into the amniotic sac under ultrasound guidance to remove some amniotic fluid) may be recommended to test for a genetic/chromosomal abnormality. In severe cases, a family may decide to end the pregnancy if the diagnosis is made prior to 24 weeks of pregnancy.

If the family decides to continue with the pregnancy, arrangements will be made with the Colorado Fetal Care Center team to deliver the baby at our facility. This way, both mother and baby will have access to experts in the field of fetal medicine during and after delivery. Our fetal care team will also manage any complications that may arise.

On top of delivery at our state-of-the-art facility, we also offer different options to treat a fetal BPS, depending on the severity. These treatment options include:

Fetal intervention

Fetuses who develop hydrops prior to 30 weeks of pregnancy may be at significant risk of stillbirth. In those fetuses, fetal intervention (surgical procedures performed while the fetus is still in the uterus) may be necessary. Sometimes, a tube (called a shunt) may be placed to correct hydrops and excessive amniotic fluid (polyhydramnios).

Alternatively, a laser fiber can be inserted under ultrasound guidance to close off the blood supply to a mass. Open fetal surgery may be necessary in extreme cases.

Early delivery

Fetuses beyond 30 weeks of pregnancy may be considered for early delivery followed by removal of the lesion after birth.

What to expect after delivery

Newborn babies who have been diagnosed with BPS may need vigorous stimulation and resuscitation in the minutes after birth. The greatest concern at delivery is the amount of lung development and the newborn's ability to breathe once the umbilical cord is cut. In severe cases, the baby may need to be placed on a breathing machine (ventilator) or a very specialized heart-lung machine, known as ECMO, to uniquely provide oxygen to the blood.

If there is fluid in the chest or abdomen, a tube known as a shunt may need to be placed to drain it. In many cases, babies will need surgery to remove the chest mass, but as many as 75% of cases spontaneously shrink after birth. Monitoring the mass after birth is critical to decide if removal is needed. If the mass grows, the fetal surgery specialists at the Colorado Fetal Care Center will remove it in our specifically-designed fetal surgery suite. After the baby recovers, remaining lung tissue should enlarge to fill the space left from the mass.