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Congenital Pulmonary Airway Malformation

Some unborn babies develop a lesion, or mass of lung tissue, inside one lung. This condition is known as congenital pulmonary airway malformation, or CPAM.

Congenital Pulmonary Airway Malformation Treatment: Why Choose Us?

Doctors at the Fetal Care Center are experts at performing a fetal surgery called shunting to treat life-threatening cases of CPAM. For less severe problems, we offer a drainage procedure called thoracentesis.

As part of the Women & Infants Center, our pediatric lung specialists at the pulmonary medicine program at St. Louis Children’s Hospital provide appropriate ongoing treatment during delivery and after childbirth.

About Congenital Pulmonary Airway Malformation

CPAM is a rare birth defect, affecting approximately 1 out of every 1,500 to 4,000 newborns.

The mass of lung tissue caused by CPAM takes up valuable space in your unborn baby’s chest. This can affect the heart and lungs. Some babies with CPAM experience:

  • Lung problems: If the lungs do not have enough room to grow, they may be underdeveloped and smaller than normal. As a result, your baby may have breathing problems after delivery.
  • Heart problems: Pressure from the mass weakens the heart. This strain can lead to fetal heart failure.

Causes of Congenital Pulmonary Airway Malformation

CPAM has no known cause, but it is more common in boys than girls. Our genetic counselors can help you understand your future risk of having another child with CPAM.

Diagnosing Congenital Pulmonary Airway Malformation

Our maternal-fetal medicine specialists and ultrasound technicians can detect CPAM in unborn babies with the aid of the latest 3-D ultrasound technology.

Your doctor may order additional tests, including:

  • Fetal magnetic resonance imaging (MRI): This safe imaging procedure uses a magnetic field (not radiation) to provide detailed information about your unborn baby’s organs, including the heart and lungs.
  • Fetal echocardiogram: This ultrasound imaging test assesses the structure and health of your unborn baby’s heart.

Learn more about high-risk pregnancy tests.

Treating Congenital Pulmonary Airway Malformation

Throughout pregnancy, you undergo regular ultrasounds to check the growth of the mass and monitor your baby’s lung and heart development. Treatment for CPAM depends on the size and number of lesions and whether the lesions are solid or filled with fluid. The maternal-fetal medicine specialists at the Fetal Care Center discuss the best treatment for your unborn baby’s specific condition.

If your unborn baby shows signs of heart failure, your doctor may recommend fetal surgery. Our skilled fetal care specialists perform a surgery known as shunting to drain excess fluid from the chest into the amniotic sac. Learn more about shunting.

Treatment options during pregnancy for less severe cases of CPAM include:

  • Medication: Steroid medicine may slow lesion growth.
  • Drainage: During a procedure called thoracentesis, your doctor inserts a needle through your abdomen and womb to drain a fluid-filled lesion.

We recommend that you deliver at a hospital equipped to care for a high-risk pregnancy, such as the Women & Infants Center. After delivery, your baby may receive advanced medical care in our Level IV newborn intensive care unit (NICU). Our focus is on keeping mom and baby together, which is why our NICU is connected to labor and delivery.

After delivery, some infants born with CPAM don’t require treatment. The lesions diminish on their own. If lesions persist, they may become cancerous. Your child should undergo surgery at some point during childhood to remove the lesions.

Contact Us

To make an appointment with a Washington University fetal care specialist at the Women & Infants Center, call 855.925.0631.