Congenital Pulmonary Airway Malformation (CPAM) is a rare developmental lung abnormality present at birth characterized by cystic masses or lesions in the lung tissue. 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 pregnancy. These lesions can be solid or filled with fluid. A baby with CPAM can have one lesion or many. There is no known cause for CPAM. The condition is relatively rare, affecting about 1 in 25,000 pregnancies. It is slightly more common in males than in females. CPAM is not hereditary, so it usually does not recur in families. These malformations can result in respiratory distress, infection, or other complications if not treated.
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:
Babies born with congenital pulmonary airway malformation are at high risk for complications during and after delivery. Managing these problems is the first and most important step in caring for babies with CPAM after birth. Lesions should be removed surgically since they can become infected or, in rare cases, become cancerous. The timing of surgery depends on the baby's symptoms after birth. In some cases, the lesions are very large and interfere with normal breathing, so surgery must take place immediately after birth. If the baby has no breathing problems, they may be discharged home and scheduled for surgery sometime within the first year of life.
Even if immediate surgery is not necessary, some babies with CPAM will experience breathing problems at birth. They may need to be placed on a ventilator or have extracorporeal membrane oxygenation (ECMO), a type of heart-lung bypass machine.
Most babies with CPAM do well after the lesion is removed. In rare situations, patients who had large lesions may have small lungs and subsequent chronic lung disease.
Pregnant patients can expect to have many different appointments in the weeks leading up to their baby’s birth. While each patient has their own unique needs, there are some screenings that everyone will receive.
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