Congenital pulmonary airway malformation (CPAM) is a mass of fetal lung tissue that can cause problems for you and your baby. It develops during pregnancy, and doctors usually find it during a routine ultrasound.

Parents who come through the doors of the Fetal Care Center at Connecticut Children’s are often full of fear. Whether you’ve been referred here or you found us on your own, know that we’re a leader in treating CPAM.

Connecticut Children’s is the only fetal care center of its kind in the Northeast. A national destination for specialized fetal care, we offer:

Prompt diagnosis and same-day answers, all under one roof.
The best care possible for you and your baby, before and after delivery.
CPAM treatment outcomes that are among the best in the country.

What is Congenital Pulmonary Airway Malformation (CPAM)?

A congenital pulmonary airway malformation (CPAM) is a type of non-cancerous lung tumor that forms in-utero.

We’re not sure why these masses, or lesions, form. It’s slightly more common in males than females. You didn’t do anything to cause it and it isn’t hereditary.

A baby might have several lesions, but they are usually only in one lung.

Sometimes the masses stay small or disappear. Masses that grow too large can cause problems for both you and your baby.

One of the most serious problems is fetal hydrops. Hydrops is a buildup of fluid in the baby’s tissues. It leads to severe swelling and can cause problems for the pregnant person as well.

How is CPAM Diagnosed?

CPAMs are usually diagnosed on a routine prenatal ultrasound. We usually can’t see the masses before about 20 weeks.

Often, you may need more imaging tests to confirm the diagnosis.

There are different types of CPAM. Connecticut Children’s is an expert at diagnosing your correct type.

Because the masses can grow rapidly after 20 weeks, it’s important to get the right diagnosis as soon as possible.

What Happens When I Come to Connecticut Children’s for CPAM?

At the Fetal Care Center at Connecticut Children’s, we’ll welcome you into a warm, family-friendly space. Here, we do everything under one roof, from diagnostic tests to surgical procedures.

During one, day-long visit, you’ll meet your specialists and have the chance to ask questions. We’ll do imaging tests to help us learn more about the lung masses and how they are affecting you and your baby.

A fetal MRI helps us see where the lesions are and how your baby’s lungs are growing.
A fetal echocardiogram lets us see the structures of your baby’s heart.

Some CPAM types are lower risk than others. Through measuring the CPAMs, we can identify which types are at a higher risk for hydrops.

We’ll talk through risks and treatment options. We have very good ways to treat CPAM during pregnancy and after delivery.

CPAM Treatment at Connecticut Children’s

At Connecticut Children’s, we treat all types of CPAM, including the most complex. Our outcomes for fetal surgery to remove the lesions are among the best in the country.

How We Treat CPAM in Utero

We use a size measurement called the CVR to figure out how serious the CPAM is and how best to treat it.

Low-Risk (CVR less than 1.6)

What it means: The mass is small and rarely causes complications.
What to expect: Weekly ultrasounds to monitor its growth.
Outlook: The mass usually stops growing around 26 weeks into the pregnancy.

High-Risk (CVR greater than 1.6)

What it means: The mass is larger, which increases the risk of hydrops (severe fluid buildup in the baby).
What to expect: We often start by giving the pregnant person two steroid shots to slow or stop the mass from growing.
Outlook: If there is no fluid buildup, steroid treatment is very successful. If there is fluid buildup, we’ll likely give you a second round of steroid shots if the fluid does not go away.

If the mass continues to cause problems, we may do a procedure called a thoracentesis. We place a tiny tube called a shunt into the baby's chest to drain the fluid and relieve pressure.

For high-risk cases, with severe hydrops and large CPAMs, we may recommend surgery to remove the masses. These are among the most serious cases. Your team will talk to you about all options, which may also include early delivery.

How We Treat CPAM After Delivery

If your baby is born with CPAM, they’ll likely be in neonatal intensive care (NICU). This can be scary for parents. But Connecticut Children’s cares for more than half of all NICU babies in the region.

First, we’ll want to make sure to monitor your baby and diagnose any complications. Babies with CPAM can have breathing and heart issues.

Some babies need immediate surgery to be able to breathe normally. Other babies may need to be stronger before surgery. We have ways to help their heart and lungs rest before surgery.

If your baby doesn’t have any symptoms, we may still recommend surgery to remove the lung masses within their first year.

Is Surgery Always the Best Answer for CPAM?

At Connecticut Children’s, we look closely at your case.

Doctors all agree that if a lung mass causes symptoms like breathing trouble, it should be surgically removed. What about if the mass is not causing symptoms?

We’ll talk to you about risks versus benefits. Reasons why we might recommend surgery to remove the mass include:

Infection risk: CPAM tissue cannot clear out germs like normal lung tissue does. About 5% to 10% of these masses get infected over time.
Cancer risk: While rare, cancer can eventually develop inside the mass. Though this usually happens decades later, it can occur in young children. Removing the mass removes this risk.

Safety, growth and peace of mind after surgery.

Safer surgery now: Operating early, before infections or complications happen, is generally safer. It carries fewer risks than operating after a problem arises.
Room for healthy lungs to grow: Removing the mass gives the remaining healthy lung room to expand and grow normally.
No need for lifelong monitoring: Once the mass is removed, your child will not need ongoing tests or imaging scans to keep checking on it throughout their life.

Dr. Crombleholme has performed more than 1,000 of these CPAM surgeries to remove lung masses, with no mortality. His patients have an average post-operative stay of just 48 hours. Babies treated for CPAM after delivery can go on to live healthy lives with strong lung function.