Bronchopulmonary sequestration (BPS) is a rare malformation of the lung that occurs before a baby is born. With BPS, a part of the lung is separated from the main development of the lung. This sequestered mass of issue doesn’t function normally and receives its blood supply from the body’s main artery (aorta), rather than the arteries in the lung.
Most times, BPS does not cause a baby significant problems. In rare cases, however, this condition can lead to breathing problems and infection. It can also displace the heart from its normal position and push on the diaphragm. As a result, it’s important to monitor a baby with a BPS closely. While it’s not unusual for the mass to shrink in size before birth, or for the normal part of the lung to continue to grow, frequent evaluation of your baby’s lungs is the best way to ensure your baby is okay.
For this reason, many moms come to the SSM Health Cardinal Glennon St. Louis Fetal Care Institute. Here, our leading team of fetal specialists, surgeons and nurses closely follow your baby throughout your pregnancy. Working collaboratively with experts across our hospital, we provide you and your infant complete, timely care, including:
- Breathing support in our NICU (Neonatal Intensive Care Unit)
- Prenatal surgery to repair a defect
- Long-term, follow-up care as your baby’s lungs continue to heal and develop
How is a Bronchopulmonary Sequestration Diagnosed During Pregnancy?
A BPS is typically detected during a routine ultrasound around 20 weeks gestation. It appears as a bright mass on the lung. A series of follow-up ultrasounds will give your physician more information about whether the lung mass is a BPS and its level of severity. Your doctor may also perform a fetal MRI to obtain a more detailed view of the mass, and a fetal echocardiogram (echo) to test for any structural heart defects.
How is BPS Managed During Pregnancy?
During your pregnancy, we actively monitor you and your baby for any complications. One of the things we look for is fluid collection in the chest cavity during the prenatal phase. In most cases, fluid build-up can be easily treated prenatally by placing a shunt to drain the fluid.
If fetal intervention is not necessary, your baby will be evaluated and treated at delivery. Most babies with a small BPS can be delivered vaginally without any complications. Once you go home, we provide follow-up care for your baby as an outpatient in two to four weeks. During this time, one of our pediatric surgeons will help determine if and when the BPS should be removed.
How is Bronchopulmonary Sequestration Treated During and After Delivery?
Depending on the size and severity of the BPS, we’ll consider different options to treat the condition either prenatally, or after the birth of your child.
Babies with a moderately large BPS may have some difficulty breathing after birth and require oxygen. In this case, your baby will be cared for in our neonatal intensive care unit (NICU) for stabilization until surgery is performed to remove the BPS, and to allow for the remaining normal lung to function optimally.
We offer an extensive program for lung surgery, using the most advanced, minimally invasive surgical techniques. As a result, the infants we care for experience faster recovery, reduced risk of long-term complications, and smaller scars.
In severe cases of BPS, we may recommend a special delivery procedure called EXIT (Ex Utero Intrapartum Treatment). During EXIT, you will give birth by C-section while asleep under general anesthesia. This will allow the surgeon to evaluate your baby’s airway and lung function while your baby’s placenta and umbilical cord remain attached. During this procedure, a breathing tube and ventilator machine is used to assist your baby's breathing. Once stabilized, your baby is fully delivered by cutting the umbilical cord.
What Are The Available Surgical Options?
The Cardinal Glennon St. Louis Fetal Care Institute is one of the only places in the US to offer this groundbreaking treatment for severe BPS.
During this minimally invasive fetal intervention, a fetal surgeon inserts a small needle into the BPS, allowing a laser fiber to target the abnormal blood vessel supplying the BPS. The laser blocks the blood flow to the mass, causing it to stop growing. This in turn allows a baby’s heart and lungs to develop normally as the BPS shrinks in size.
Open Fetal Surgery
Open fetal surgery is also an option to treat this condition. During this procedure, a fetal surgeon opens the womb (uterus) to expose the baby’s chest. Once exposed, the baby’s chest is also opened to allow for the BPS to be removed. Mothers who select this option must give birth by C-section to minimize additional complications during labor.
In nearly all cases, babies who receive surgery for BPS go on to develop normally without breathing or heart problems.
If you’re facing a diagnosis, schedule an appointment with us at the Cardinal Glennon St. Louis Fetal Care Institute. We understand that BPS can be a scary diagnosis. That’s why we’re available to help 24 hours a day, 7 days a week. For more information or to schedule an appointment, call us at 314-268-4037 or toll free at 877-SSM-FETL (877-776-3385). You can also email us and one of our trusted representatives will get back to you as soon as possible.
While we can’t change the diagnosis, we can provide you expert care and support, helping your baby get the most out of treatment and life.