What Is Bronchopulmonary Sequestration?

“We’ve discovered something abnormal on your ultrasound. It’s called bronchopulmonary sequestration.” 

It’s something no expecting parents want to hear — and if they do, they’re likely reeling from the deluge of medical jargon, extra appointments and the seemingly endless swirl of unanswered questions. 

If your baby has received the Bronchopulmonary Sequestration diagnosis, or you just want to know more about it, this article can help. We will give you a layperson’s definition of bronchopulmonary sequestration and how it’s diagnosed, and help you understand what this diagnosis means for you and your baby. 

What Is Bronchopulmonary Sequestration?

Bronchopulmonary sequestration (BPS) is a mass of tissue that develops alongside or inside fetal lungs. It affects one in 15,000 births, and 68% of bronchopulmonary sequestration cases get smaller or disappear on their own before the baby is delivered. 

This mass most likely develops as a small piece of extra tissue called a lung bud and grows alongside the esophagus. A BPS mass gets its blood supply from the baby’s pulmonary artery. This connection to the pulmonary artery is what distinguishes BPS from a similar condition called CPAM.

It’s not clear what causes BPS. It’s simply an extra piece of tissue that may shrink, stay the same size or expand as your baby grows.

How Bronchopulmonary Sequestration Is Diagnosed

A BPS diagnosis typically starts with a routine ultrasound. The mass is usually visible at around 20 weeks gestation and appears as a bright white spot on or near the baby’s lungs. 

When your ultrasound technician and provider see this abnormality, they may order an MRI or a fetal echocardiogram to find out more about the tissue’s size and location. 

Most BPS masses are found inside a lung (intralobar). If intralobar BPS isn’t detected during pregnancy, it can sometimes show up in childhood as recurring respiratory issues. One-fourth of BPS cases are extralobar, meaning they’re located outside the lungs. Extralobar BPS can move or twist, displacing vessels or other organs like the lungs or heart. In some rare cases, extralobar BPS can travel down to the diaphragm. 

If a BPS mass continues to grow, it could affect breathing and heart functions in unborn babies, infants and children. It could also interfere with fetal health, growth and development. In extreme cases, it can cause fluid in the chest, abdomen, lungs and even fetal heart failure.

What Causes Bronchopulmonary Sequestration?

The cause of BPS is currently unknown. There are no known risk factors, and it’s not inherited from either parent. BPS is not a result of inadequate prenatal care or any other external factors. Some studies have found more male than female-born infants have extralobar BPS, but the reason for that is also unknown. 

What Happens After a Diagnosis?

If your baby is diagnosed with a BPS, you will probably be referred to a maternal-fetal medicine specialist. Your specialist can order regular ultrasound imaging throughout your pregnancy to keep an eye on the mass. 

Once you’ve met with your new medical team, you can discuss treatment options together. You may decide to monitor a BPS mass to see if it changes. You may also decide on some kind of intervention. Your health care team may recommend surgically removing BPS if:

• It’s growing
• Fluid is detected in the chest (hydrops)
• It’s pushing on the baby’s heart, affecting blood flow
• It’s interfering with lung function and development

Post-Birth Outlook 

If BPS tissue doesn’t change or grow, there’s a good chance your baby won’t need intervention before birth. Your child will likely undergo monitoring after they are born to determine if BPS removal is necessary.

Many cases of BPS have positive outcomes with typical deliveries and healthy babies. 

Because each case of BPS is so unique, there are no one-size-fits-all treatments. Your specialized health care team can provide BPS treatment options that are tailored to your situation.  

BPS only has an effect on the baby, not the mother. One instance of BPS doesn’t mean you are more likely to have another baby with the same diagnosis in the future. 

Bronchopulmonary Sequestration Treatment Options

Many unique factors dictate the type and timing of treatment (if any). With guidance from your health care team, you can determine the best course of action for your situation. If your baby’s BPS needs to be removed, it may be done in one of the following ways:

In-Utero Procedures

In-utero procedures are done while the baby is still inside the womb. Most babies with BPS do not require in-utero interventions unless they have other unrelated complications or the mass is causing health problems. Some common in-utero procedures in BPS cases are:

• Interstitial Laser Devascularization. This is an ultrasound-guided technique where a needle is inserted through the mother’s abdomen to drain fluid or administer other interventions. 
• Shunt Placement. Inserting a shunt can guide recurring fluid into the amniotic sac and away from the baby’s lungs. 
• Ex-Utero Intrapartum Treatment (EXIT). This procedure keeps an unborn child connected to the placenta while undergoing corrective treatment(s). The mother is placed under general anesthetic during an EXIT procedure. 

Post-delivery removal

Surgical BPS removal after birth has a high success rate. Your child’s medical team can determine the best method and timing of BPS removal. If a child is born without breathing complications, their pediatrician will monitor the mass as they grow and plan for surgery within the first one to four years of life. If a child is born with complications caused by BPS, emergency surgery may be necessary after birth. 

BPS Terms to Know

Navigating a new world of unfamiliar terminology can be confusing. Here are some terms you may frequently hear during your journey to diagnose and treat BPS:

• Congenital Lung Lesions/Fetal Lung Lesions. These terms refer to several lung issues, including BPS. BPS is considered one type of congenital lung lesion.
• Mass/Tumor. Many people equate these terms exclusively with cancer. While a mass or tumor can certainly be cancerous, that’s not always the case. In BPS, a mass or tumor refers to the tissue itself (the BPS). BPS and cancer can exist together. However, the terms mass and tumor don’t refer to a cancer diagnosis in BPS. Cancer (or a malignant tumor) would be considered a separate diagnosis. 
• Hydrops. A condition where fluid builds up in one or two areas of the body. BPS mass location and size can sometimes cause hydrops, but it is also a separate diagnosis. 
• Pulmonary Sequestration. Another name for bronchopulmonary sequestration.
• Plural Effusion. Fluid around the lungs.

You’re Not Alone

A BPS diagnosis in your pregnancy — as with any unexpected compilation — can feel overwhelming. It’s important for you to take care of your physical and mental health. Here are a few ways to help yourself stay well during this uncertain time:

• Find a health care team that will communicate with and support you.
• Talk about your concerns with friends, loved ones or a mental health professional.
• Allow members of your support system to help you with day-to-day tasks and meals.
• Keep a journal to process what you’re going through.
• Ask coworkers and loved ones to help you manage a reasonable workload/reduce stress.
• Get plenty of sleep.
• Consult your health care team about physical activity and follow their guidelines for exercise.
• Ask your medical provider about any dietary restrictions or guidelines and make a plan about how you can fit those into your day-to-day life.

Navigating a BPS diagnosis and treatment can feel like uncharted waters. Rely on your care team and support system as you move toward the big day.