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What is CPAM in pregnancy?

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What is CCAM/CPAM?

Congenital cystic adenomatoid malformation (CCAM) is a benign lung lesion that appears before birth as a cyst or mass in the chest. It is made up of abnormal lung tissue that does not function properly, but continues to grow. CCAM is also frequently referred to as a congenital pulmonary airway malformation (CPAM).

How do I use the patient portal?

A congenital pulmonary airway malformation (CPAM) is a mass of abnormal fetal lung tissue that forms during pregnancy. This mass, or lesion, is usually located in one lung and it does not function as normal lung tissue. Lesions vary in size and appearance and can change significantly during the pregnancy.

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What causes CCAM/CPAM?

Causes of CCAM/CPAM. The causes of CCAM/CPAM are unknown. It’s not linked to a gene or to a chromosomal abnormality, and does not run in families (is not hereditary). The malformation results from abnormal lung tissue that grows, usually in one lobe of the lung.

How to diagnose CCAM?

Evaluation and diagnosis of CCAM/CPAM 1 You will have a high-resolution fetal ultrasound to evaluate fetal anatomy as well as the lung lesion. 2 A dedicated fetal cardiologist will perform a fetal echocardiogram to evaluate heart function and structure. 3 If indicated, you may also have an ultrafast fetal MRI, pioneered at CHOP, to provide additional anatomic detail about the lung lesion.

What is a CCAM lung lesion?

CCAM/CPAM is the most common type of fetal lung lesion. It develops before a baby is born, and can vary in size and be either fluid-filled or solid. A large cyst is called a macrocystic lesion, and a small cyst or solid appearing lesion is called microcystic. Some CCAM/CPAMs can be life-threatening if they are not treated, ...

What is a CCAM?

What is a congenital cystic adenomatoid malformation (CCAM/CPAM)? Congenital cystic adenomatoid malformation (CCAM) is a benign lung lesion that appears before birth as a cyst or mass in the chest. It is made up of abnormal lung tissue that does not function properly, but continues to grow.

How to tell if a baby has a CCAM?

Signs and symptoms of CCAM/CPAM. Congenital cystic adenomatoid malformations are typically discovered during a routine prenatal ultrasound. The ultrasound technician may notice a bright spot in your baby’s lung that indicates a cyst or mass. CCAM/CPAMs typically increase in size until 26 – 28 weeks’ gestation, after which the size ...

Why is open fetal surgery necessary?

The goal of these procedures is to decrease the size of the mass to ward off heart failure (fetal hydrops). Open fetal surgery to remove CCAM/CPAM: We have learned that open fetal surgery is now rarely necessary due to advances in care before birth.

What is the purpose of fetal echocardiogram?

A dedicated fetal cardiologist will perform a fetal echocardiogram to evaluate heart function and structure. If indicated, you may also have an ultrafast fetal MRI, pioneered at CHOP, to provide additional anatomic detail about the lung lesion.

What does a CVR of 1.6 mean?

CVR is the ratio of lesion volume to the baby’s head circumference. A CVR of less than 1.6 indicates a favorable prognosis; 1.6 or greater indicates an increased risk of hydrops and the possibility of fetal surgery. Another indicator of serious problems is swelling in the placenta.

What does it mean when a placenta swells?

Placental swelling is associated with hydrops and is a sign of “mirror syndrome,” which means that the mother’s life could be at risk. In this case, our team will meet with the patient to discuss the option of immediate delivery. In most cases, the growth of the lesion stops at about the 26th week of pregnancy.

What are the factors that influence fetal therapy?

Many factors influence decisions about which fetal therapy to pursue for a baby with CPAM, if any. These include the size and number of lesions, whether the lesions are solid or filled with fluid, and whether hydrops or mirror syndrome has developed. Steroid Treatment.

Can a baby with CPAM survive?

Babies who have CPAM and hydrops typically will not survive unless they can respond quickly to medicine or undergo fetal surgery to remove the lesions. Hydrops can cause “mirror syndrome,” in which the mother “mirrors” the baby’s illness. The mother can develop high blood pressure and fluid retention.

Can a CPAM be detected during pregnancy?

Congenital pulmonary airway malformation is often detected during a routine ultrasound during pregnancy. Additional tests can confirm the diagnosis, evaluate how severe the CPAM is, and identify any other problems. These tests include:

Can a congenital lung malformation cause a baby to die?

For most babies with a congenital pulmonary airway malformation, the condition is not life threatening. Sometimes, the lesions decrease in size or can no longer be seen during the pregnancy. Other times, the lungs develop normally despite the presence of these lesions. In about 10 percent of cases, CPAM can cause serious problems for ...

Can CPAM cause heart failure?

In about 10 percent of cases, CPAM can cause serious problems for the baby and sometimes, for the mother as well. The most serious problems occur when babies develop hydrops, a prenatal form of heart failure characterized by fluid build-up in the skin, chest or abdomen.

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