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Conditions We Treat

Congenital Lung Lesions

Congenital lung lesions are a group of conditions where part of the lung has an abnormal growth or mass. Congenital pulmonary airway malformation (CPAM), congenital cystic adenomatoid malformation (CCAM), bronchopulmonary sequestration and congenital lobar emphysema are the names of the most common types of congenital lung lesions.

What Are Congenital Lung Lesions?

Congenital lung lesions are a group of conditions where part of the lung has an abnormal growth or mass. The exact cause of congenital lung lesions is unknown. The most common types are:

  • Congenital pulmonary airway malformation (CPAM)
  • Congenital cystic adenomatoid malformation (CCAM)
  • Bronchopulmonary sequestration
  • Congenital lobar emphysema

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How Are Congenital Lung Lesions (CPAM, CCAM) Diagnosed?

A lung lesion may be found during routine prenatal ultrasound. If your doctor suspects a problem, additional tests may be ordered. These may include: 

  • Fetal echocardiogram: A specialized ultrasound to look at your baby’s heart and see any effect of the mass on the heart.
  • Fetal MRI (magnetic resonance imaging): This type of imaging gives more detailed pictures of your baby’s organs. It can provide additional information on the growth of the normal parts of the lungs and can help identify if there is any extra blood supply to the lesion as well as help to differentiate the different types of lung lesions.

How Are Congenital Lung Lesions (CPAM, CCAM) Treated During Pregnancy?

If a test finds you are carrying a baby with congenital lung lesion, you will keep getting care from your obstetrician (OB/GYN). Your OB/GYN may also refer you to the Fetal Center at Nationwide Children’s, where you will meet with a team including maternal-fetal medicine subspecialists, neonatologists (doctors who specialize in treating newborns), pediatric and fetal surgeons (doctors who perform surgery), and nurses to learn more about your baby’s care. They may suggest treatments such as:

  • Ultrasound: A type of imaging that uses sound waves to see inside the body. May be used to monitor the size of the lung lesion, and to check for the development of poor heart function or hydrops (extra fluid buildup in the baby).
  • Steroids: Studies suggest that giving the mother a short course of steroids in the second trimester may help to prevent hydrops in babies with large lung lesions. Your maternal fetal medicine specialist will determine if steroids are needed based on the size of the lung mass and your baby’s measured lung volume. 
  • Open fetal surgery (intrauterine surgery) or EXIT-to resection: Rarely, persistent large masses (that have failed steroid therapy) may need surgery before birth. When fetal intervention is required, procedures are performed at Nationwide Children’s in a specially equipped operating room. For large lung masses that persist until delivery, an EXIT procedure may be helpful.  The fetus is partially delivered, and the lung mass removed to allow room for the rest of the lungs to expand as the baby breathes. After the procedure, patients are transported to our fetal care unit with a dedicated team of experts for both mom and baby.

What Is My Baby’s Outcome (Prognosis)

Most babies with congenital lung malformations do very well. Fetuses with very large lesions will need to be followed closely during pregnancy. Your high-risk obstetrician will closely monitor your pregnancy for any signs of hydrops and other complications. Survival for babies who have no symptoms at birth is excellent. All the babies will follow up with a pediatric surgeon after birth to discuss if surgery is needed for the lung lesion.

Speak With Our Team

Our team is here to help as soon as you learn your baby may have a fetal concern or diagnosis. And a physician referral is not required. Our expert team evaluates and treats nearly every fetal complication, from the most common to the most complex conditions. With access to advanced diagnostic imaging, ultrasounds and fetal MRIs, you'll find the answers, and the treatment, here.

Looking for a second opinion? The Fetal Center team can help. Our team of specialists work together to provide comprehensive feedback on your diagnosis and treatment plan through either an in-person visit or via an online written report. Our team can help you decide which is right for you.

Complete the form below or call (614) 869-4609 and the Fetal Center team will be in touch with you.

 

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