This leaflet is to help you understand what an Intraabdominal Bronchopulmonary Sequestration is, what tests you need and the implication of being diagnosed are for you and your baby.
What is an Intraabdominal Bronchopulmonary Sequestration?
A Bronchopulmonary Sequestration (BPS) is an abnormal mass consists of non-functioning lung tissue which is not normally connected to the airway. A BPS can be found in either the chest or abdomen of the baby. When it is found in the abdomen (baby’s belly) it is called an intraabdominal BPS. This mass does not function normally and receives its main blood supply from the body’s main artery (aorta), not from the lung arteries.
How is an Intraabdominal BPS detected?
An intraabdominal BPS is usually detected during the routine scan done between 18 and 22 weeks of gestation. At this scan, many of the structures inside of the baby are evaluated. An intraabdominal BPS appears as a solid white mass in the abdomen. The exact reason why this happens is not clearly understood, but it is thought that this condition happens early in pregnancy when an extra lung bud develops and migrates with the feeding tube into the abdomen. Intraabdominal BPS are rare and occur in 1 of 10,000 babies.
Should I have more tests done?
All babies with a mass in the abdomen should have a detailed ultrasound to look for additional structural defects. A series of ultrasounds are usually suggested to monitor the mass and the baby. Sometimes your doctor may recommend a fetal MRI to obtain a more detailed view of the mass if it is not clear what the mass is. Your doctor may discuss amniocentesis to evaluate the baby’s chromosomes when there are additional malformations present.
You may wish to have a consultation with a neonatologist and a pediatric surgeon. They will help you understand how the baby will be evaluated and followed after birth.
What are the things to watch for during the pregnancy?
Often, the mass may grow until 26-28 weeks of gestation, and then remain the same size or decrease in size until birth. Occasionally the mass grows in size. Very rarely, the baby will develop extra fluid in the skin around the baby or fluid in the chest, abdomen or around the heart. Your doctor will recommend re-evaluation of the baby to check the baby’s size and to look for extra fluid inside or around the baby.
What does it mean for my baby after it is born?
After birth, most babies have additional examinations to evaluate the mass. The type of examinations done may depend on where you live. The treatment for an intraabdominal BPS varies according to the size of the lesion. Most of the time the baby will have surgery to remove the mass, but this is typically not done as an emergency surgery. Sometimes, very small masses are followed with a “wait-and-see” approach. The good news is that most babies with an intraabdominal BPS have good prognosis.
Will it happen again?
It is very unlikely that an intraabdominal BPS would happen again in the next pregnancy.
What other questions should I ask?
- Are there any other abnormalities on the ultrasound?
- How sure are you that this is an intraabdominal BPS?
- Are there any other tests that are suggested?
- How often will I have ultrasound examinations done?
- Will my baby need surgery after delivery?
- Where should I deliver?
- Where will the baby receive the best care after it is born?
- Prior to delivery, can I meet the team of doctors that will be assisting my baby when it is born.