Congenital Pulmonary Airway Malformation

Cystic masses, or defects, that form in the lung tissue of a baby during pregnancy. This mass is usually located in one lung, preventing it from growing normally. CPAM may also cause the heart to shift to the opposite side of the chest or push downward on a baby’s diaphragm.

At SSM Health Cardinal Glennon St. Louis Fetal Care Institute, our team treats the whole condition, both monitoring your baby’s lungs and heart, and providing a comprehensive medical solution.

If your baby has been diagnosed with CPAM, you can rely on our dedicated, multidisciplinary team of providers to care for your child’s every need. We coordinate and provide immediate care in our NICU (Neonatal Intensive Care Unit) as well as long-term follow-up care as your child’s lungs continue to develop.

How is CPAM Diagnosed?

A CPAM is detectable during a routine prenatal ultrasound. It actually appears as a bright mass in the area of the chest where only lung tissue should be seen.

We are able to confirm this diagnosis using advanced fetal MRI to better identify the size and location of the defect. We may also perform a fetal echocardiogram (echo) to rule out any structural heart defects.

How Does CPAM Affect My Baby?

In a small number of cases, CPAM may grow so rapidly that it becomes life-threatening before birth. This usually happens between 18 and 26 weeks gestation. The large mass causes compression, leading to heart and lung failure. To prevent this from happening, either fetal surgery or early delivery is necessary.

In the vast majority of cases however, babies do well and have normal development and lung function. That’s because during the pregnancy, the cyst usually starts to slow down in the second trimester. Sometimes, the mass becomes very small and undetectable. In this case, your child’s outlook for healthy lungs is excellent.

How is CPAM Managed During Pregnancy?

During the initial ultrasound, we will measure the volume of the CPAM relative to the size of your baby. If the CPAM remains small after 28 weeks, we’ll monitor it through an ultrasound every three to four weeks until delivery.

If the defect pushes the heart out of the normal position, we may perform a second MRI around 32-34 weeks of pregnancy and monitor the heart with an echocardiogram twice a week.

In severe cases, we may recommend one, or a combination of interventions:

  • Steroids to slow or stop the growth of the mass
  • Open fetal surgery to remove the mass
  • Drain the fluid-filled cysts using a special needle
  • Early delivery starting at 32 weeks of pregnancy

How is CPAM Treated After Delivery?

Depending on the severity of the CPAM, your newborn may have difficulty breathing at delivery. If this is the case, we’ll move them to our NICU where they will be cared for as if they were our own. For infants who have the cyst surgically removed, the long-term outlook is excellent. In most cases, infants with a relatively small CPAM will have fewer complications and can go home after delivery.

We understand that CPAM 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. Our team is here to develop a comprehensive treatment plan that puts you at ease and gives your baby the best start in life.

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