Children's Hospital Colorado
Colorado Fetal Care Center

Congenital Pulmonary Airway Malformation (CPAM)

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What is congenital pulmonary airway malformation?

Congenital pulmonary airway malformation (CPAM) is a relatively uncommon condition. It affects only 1 in approximately 4,000 babies born each year and involves lung lesions or masses that form in a baby's lower respiratory tract.

CPAM is the newer term for what was previously known as congenital cystic adenomatoid malformation (CCAM). It is part of a variety of conditions that involve cystic lung lesions or masses that form in the lower respiratory tract during a baby's development.

These are typically noncancerous masses that occur most commonly in one lung rather than both lungs, though any lobe of either lung may be affected.

As a parent, it can be hard to discover your child has this condition. Fortunately, the Colorado Fetal Care Center has a large team of multidisciplinary fetal care specialists who are here for you from the initial diagnosis to continuing care after delivery.

What causes congenital pulmonary airway malformation?

The condition is found more commonly in males than females. It occurs because of a change in lung development very early in pregnancy (about 8-9 weeks), though the exact cause is not known. It is not thought to be genetic, as no cases of recurrence of congenital pulmonary airway malformation in a sibling or offspring have been reported.

What are the risks of CPAM?

Depending on the size, the malformation can grow rapidly, causing a shift in structures within the chest, (e.g., heart, lung, esophagus) which may not allow the baby to develop normally. In other cases, the mass may also compress the esophagus, causing amniotic fluid to increase, which puts the mother at risk for preterm labor.

The impact a malformation has on a baby will differ greatly depending on the size of the mass, its location and whether it impacts other organs in the chest cavity. Larger lesions can cause fetal hydrops (excessive fluid buildup) due to shifting in the chest and compression of the large vein (vena cava) that returns blood to the right side of the heart.

Maternal "mirror syndrome" can also develop in cases of fetal CPAM. In these cases, the mother accumulates excessive fluid similar to the fetus, as well as very high blood pressure (preeclampsia). Though quite rare, this condition would require immediate delivery.

The worst risk with congenital pulmonary airway malformation, however, is the immediate period of time before birth. If air becomes trapped within the cystic tumor, it can cause serious respiratory distress in the unborn baby. After birth, ongoing cysts can result in recurrent bouts of pneumonia and other respiratory infections. Collapse of the lung (pneumothorax) is also an additional concern. In rare cases, some CPAM lesions can become cancerous.

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Get to know our pediatric experts.

Adel Younoszai, MD

Adel Younoszai, MD

Cardiology - Pediatric

Nicholas Behrendt, MD

Nicholas Behrendt, MD

Maternal-Fetal Medicine, Ob/Gyn Obstetrics & Gynecology

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Mariana Meyers, MD

Mariana Meyers, MD

Radiology, Radiology - Pediatric

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Kenneth Liechty, MD

Kenneth Liechty, MD

Surgery - Pediatric, Surgery