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Congenital Cystic Pulmonary Malformation (CPAM or CCAM)

What is Congenital Pulmonary Airway Malformation (CPAM)?

A congenital cystic pulmonary malformation (CPAM) is a lesion on a fetus’ lung. It is benign (non-cancerous) and can appear as a cyst or a lump in the chest. The cause of a CPAM is unknown, and it is not related to anything the mother did or did not do during the pregnancy. CPAM is sometimes referred to as congenital cystic adenomatoid malformation (CCAM).

CPAM Diagnosis, Causes, & Treatments

How is CPAM diagnosed?

With improvement in prenatal imaging technology, most CPAMs are detected during a routine prenatal ultrasound. A fetal MRI or fetal echocardiogram may also be used to confirm the diagnosis. Learn more about fetal MRIs and fetal echocardiograms at CHOC.

During pregnancy, CPAMs typically increase in size throughout the second trimester. Then, in the third trimester, they usually start to shrink in size. Your baby’s doctor will pay close attention to see if this happens. Most babies will be carried to term and have normal development and lung function at birth. In rare cases where a CPAM grows so large it cause distress to your unborn baby, the specialists at The Fetal Care of Southern California can help.

If your baby has been diagnosed with CPAM in the prenatal period, we would be happy to schedule a consultation with your family and one of our top pediatric surgeons, as well as a CHOC neonatologist and your perinatologist, to prepare for the birth and subsequent care of your baby. If your child has been diagnosed with CPAM after delivery, we would be happy to schedule a consultation with your family and one of our top pediatric surgeons.

What causes a Congenital Pulmonary Airway Malformation (CPAM)?

The cause of a CPAM is unknown, and it is not related to anything the mother did or did not do during the pregnancy. CPAM used to be named congenital cystic adenomatoid malformation (CCAM).

What is the difference between a Congenital Pulmonary Airway Malformation (CPAM) and a Congenital Cystic Adenomatoid Malformation (CCAM)?

CPAM is the new term for abnormal cystic tissue in the lung. It has replaced the older them CCAM.

How is CPAM treated?

Most babies with a small CPAM are born with no symptoms and can go home after a few days in the hospital. Surgery to remove the CPAM is usually performed when a baby is two to six months old. Surgery is performed to prevent potentially severe infections related to the CPAM tissue. It is also done to prevent the rare lung cancer that can develop much later in life in CPAM tissue that has not been removed.

Although it is very rare, sometimes a CPAM can grow so large in the prenatal period as to cause heart failure and swelling of the body in a fetus. This condition is called hydrops. . When a CPAM is this large, it can also prevent the fetus’ lungs from fully developing, leading to pulmonary hypoplasia, or small lungs. In these very rare cases, the fetus may benefit from prenatal interventions which can include treatment with steroids, drainage of a fluid filled CPAM cyst, or even surgery in the womb to remove the CPAM tissue.

Your perinatologist, a specialist in fetal and maternal medicine, will discuss your delivery plans. Your baby should be born at a hospital with close access to a neonatal intensive care unit (NICU) that has extracorporeal membrane oxygenation (ECMO) available, which will help your baby’s heart and lungs function if the condition is severe. The CHOC NICU is designated a Level 4 NICU—the highest level available because of the complex conditions we treat—and we are the only hospital in Orange County to provide ECMO for children. Learn more about our NICU.

Congenital Pulmonary Airway Malformation Repair Surgery

CPAM/CCAM surgery is a procedure that removes a malformation in the lung of a baby. The surgery is performed by a pediatric surgeon.

How does CPAM/CCAM surgery work?

The type of surgery performed depends on the size and location of the CPAM or CCAM. Most commonly, it requires removal of a lobe of the lung. In some cases, the surgeon may be able to remove the malformation through a three small incisions in the chest (minimally invasive surgery, also known as “thoracoscopic lobectomy”). In other cases, the surgeon may need to make a larger incision (open surgery, also known as “thoracotomy”) to access the malformation.

CPAM/CCAM Surgery Recovery

After surgery, babies will be monitored in the neonatal intensive care unit (NICU). Older children may be monitored in the PICU. If the patient is healthy and surgery can be performed using minimally invasive techniques (thoracoscopy), the hospital stay usually lasts only a few days. If the patient is sick or if open surgery (thoracotomy) is needed, the hospital stay will likely be longer. During this time, your child will be closely monitored for breathing issues and will be given pain medication as needed.

CPAM/CCAM Success Rate

The majority of babies who have surgery to remove a CPAM or CCAM have a good outcome. Most babies will be able to go home from the hospital within a few days after surgery. Most babies will go on to have normal lung function and development.

The Only Surgical NICU on the West Coast

When it comes to surgery, babies present a unique challenge because of their small size and complex conditions. Their health can change in an instant, requiring quick action and expertise.

No other hospital in the region is prepared to meet this challenge like CHOC. We proudly offer the only Surgical Neonatal Intensive Care Unit on the West Coast, providing highly specialized care for babies who need surgery.

The Surgical NICU brings every member of the care team together to follow a coordinated plan for babies, and provide a seamless experience for families. This coordinated effort is backed by national-level research and quality improvement efforts, all of which has led to better patient outcomes.

Frequently Asked Questions About Congenital Pulmonary Airway Malformations (CPAM)

Can CPAM/CCAM go away on its own?

In rare cases, CPAM/CCAM may regress or go away on its own. However, most commonly they do not. It is not uncommon for a CPAM to become difficult to detect on ultrasound at the end of pregnancy but for it to remain fairly sizeable when seen on postnatal CT scan.

How common is CPAM/CCAM in babies?

Congenital pulmonary airway malformation (CPAM) is a rare lung malformation. Doctors used to think that it occurs in about 1 in 25,000 to 1 in 35,000 births. However, with improvement in fetal imaging technology, doctors now believe it to be more common, perhaps as frequent as 1 in 5,000 births. The team at The Fetal Care Center of Southern California is experienced at diagnosing and managing CPAM in the prenatal period, and the surgeons at CHOC are experienced at operating on this condition.

What is the long-term outlook for babies with a CPAM/CCAM?

The outlook for babies who have a CPAM/CCAM successfully removed is usually very good. They often have no limitations on their activities and have no increased risk for respiratory problems.

Are Bronchial Atresia, Bronchopulmonary Sequestration (BPS) and CPAM the same?

Bronchial Atresia, Bronchopulmonary Sequestration (BPS), and Congenital Pulmonary Airway Malformation (CPAM) are not the same conditions. Though all three conditions involve malformations in the lung, they have distinct characteristics, causes, and implications for treatment and management.

Bronchial atresia is a rare congenital condition where one of the bronchi (the tubes that branch off from the trachea and carry air into the lungs) is either partially or completely blocked. This can lead to the accumulation of mucus and air in the affected segment of the lung, causing a condition known as mucocele. It is usually diagnosed incidentally, often as a result of imaging studies done for other reasons, and it may not always cause significant symptoms.

Bronchopulmonary sequestration (BPS) is a rare congenital malformation in which a segment of lung tissue develops without normal communication with the airways. Instead, it receives its blood supply from systemic circulation, typically from the aorta or its branches. This means that the sequestered lung tissue functions independently from the rest of the lungs, and it may be prone to recurrent infections or other complications.

If you or someone you know is concerned about any of these conditions, it is important to seek advice from a qualified healthcare professional for a proper evaluation and diagnosis.

The Fetal Care Center of Southern California

Pediatric Spine Specialist and patient’s mom at CHOC’s Pediatric Spine Center

If an abnormality is detected before your baby is born, our team of pediatric experts at the Fetal Care Center of Southern California can confirm your baby’s diagnosis, provide extensive condition education and counseling, and begin comprehensive treatment planning for after your baby’s birth.