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Congenital Pulmonary Airway Malformation

CPAM: Congenital Pulmonary Airway Malformation

Congenital Pulmonary Airway Malformation (CPAM), also known as Congenital Cystic Adenomatoid Malformation (CCAM), is a condition affecting the lungs of a fetus. The expert high risk obstetricians, pediatric surgeons and neonatologists of the Center for Maternal Fetal Health  at Rocky Mountain Hospital for Children, Denver are specially trained to diagnose and treat this rare condition. In addition, our staff and facility are uniquely equipped to handle the careful treatment of your baby’s health during pregnancy as well as during and after birth.

About CPAM/CCAM

CPAM/CCAM is a condition in which a benign mass grows on a fetus’ lung. The growth does not function as lung tissue and takes the place of normal, healthy tissue. There are three types of CPAM/CCAM:

  • Type I and Type II CPAM/CCAM: one or more fluid filled cysts
  • Type III CPAM/CCAM: a solid mass (and sometimes cysts as well)

This condition is similar to another fetal lung condition, Pulmonary Sequestration or BPS, but BPS is unique in that it has an artery directly connecting it to the aorta.

CPAM/CCAM Diagnosis

CPAM/CCAM typically is diagnosed during the 20-week ultrasound.  It is seen as a bright white mass on the lungs. Additionally, the heart may be displaced, the diaphragm may be flattened and healthy lung tissue is decreased.

Treatment of CPAM/CCAM

Babies diagnosed with CPAM/CCAM undergo regular ultrasounds from 20-30 weeks gestation, checking the size of CPAM/CCAM. It may get smaller, remain unchanged or even disappear entirely in the third trimester. During this time, doctors are looking for signs of fetal hydrops, a rare but serious complication of CPAM, defined by massive fluid retention. In some cases, the mother “mirrors” this condition and develops preeclampsia.

If fetal hydrops occurs, treatment is necessary during pregnancy.  The pediatric surgeons at Rocky Mountain Pediatric Surgery are uniquely qualified to perform such delicate procedures.

Prenatal CPAM/CCAM treatment may include: 

  • Drainage of the cyst
  • Steroid medications

Babies who do not develop fetal hydrops can be carried to term without treatment, but should be delivered at a hospital with a Level IV NICU  equipped to care for the immediate care of a high risk newborn and any potential complications that may arise. Typically, surgery to remove the growth is scheduled when the baby is several months old.  It is important to have the mass removed to prevent lung infections and lower the risk for other complications. Our highly experienced pediatric surgeons of Rocky Mountain Pediatric Surgery are pioneers in minimally invasive surgical techniques and the removal of these abnormal lung lesions using tiny incisions and instruments. They have the largest series of thoracoscopic lobectomies in world. These procedures decrease incision size, pain and recovery time. To learn more about the specialized, expert surgeons of Rocky Mountain Pediatric Surgery, call 303-839-6001.

Long-Term Outlook for CPAM/CCAM

Babies with CPAM/CCAM can go on to live healthy, normal lives. Lungs continue to develop until about the age of 9, so children have plenty of time to create normal, healthy lung tissue after the abnormality is removed. Additionally, the condition is not believed to be hereditary, so future pregnancies often are unaffected.

To learn more about CPAM/CCAM or to contact a high-risk obstetrician or pediatric surgeon, call our program care coordinator at 720-754-4902.