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Congenital pulmonary airway malformation (CPAM)

What is CPAM?

CPAM was formerly known as congenital cystic adenomatoid malformation (CCAM). In this condition, a non-working cystic piece of abnormal lung tissue replaces an entire lobe of functional lung. Most patients with CPAM show no symptoms. However, 25 percent of patients develop cyanosis (a bluish tint to the skin) or pneumothorax (air or gas between the lungs and chest wall) and may have breathing difficulties including tachypnea (very fast breathing) and intercostal retractions (collapsing of the muscles between the ribs).

What causes CPAM?

This is a congenital lung disorder; a child is born with it. The underlying cause is unknown. CPAM occurs in approximately 1 in every 30,000 pregnancies.Thousands of people may be living with CPAM without knowing it.

Through ultrasound testing, many more patients are now aware they live with this condition. Rarely, long-standing CPAMs have been reported to become cancerous.

How is it diagnosed?

We can often identify CPAM during a routine prenatal ultrasound.

How we treat Congenital pulmonary airway malformation (CPAM)

If found prenatally, we closely monitor CPAM during pregnancy. Most babies with a CPAM are born without complication and are monitored during the first few months.

Surgical removal usually occurs before the child’s first birthday because of the risk of lung infections associated with CPAMs. Very large CPAMs that might pose a danger during birth because of airway compression can be treated with a special type of surgical delivery called the EXIT procedure. In rare and extreme cases where the fetus's heart is in danger, we may perform fetal surgery to remove the CPAM.

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