Congenital Pulmonary Airway Malformation
Overview
A congenital pulmonary airway malformation (CPAM) is when a baby is born with a piece of unusual lung tissue involving one of their lungs. This unusual piece of lung tissue, which contains a blood vessel, is made of small cysts (sacs of fluid or air). A baby can have one CPAM or multiple CPAMs, and they often change in size or shape throughout your pregnancy.
CPAMs are similar to bronchopulmonary sequestrations. CPAMs are different in that a blood vessel runs directly from the aorta (major artery that brings blood from the heart to the rest of the body) to the unusual piece of lung tissue. The heart will need to work harder than usual to pump blood to that unneeded, excess lung tissue. The extra piece of tissue is not needed for the lung to function properly and will need to be removed. If not removed, excess blood flow to the CPAM can lead to health problems, including difficulty breathing, infection or heart failure
Symptoms of Congenital Pulmonary Airway Malformation
A prenatal (pregnancy) ultrasound will reveal the CPAM in your baby’s lung. If the CPAM is large, it may press on the heart and move it out of its usual location. This unusual positioning can also be seen on a prenatal ultrasound.
Babies with CPAM may also experience a buildup of extra fluid during development in their mother’s womb. This excess fluid buildup, called fetal hydrops, can occur in the baby’s abdomen (belly), lung, scalp or skin. If your baby has fetal hydrops, you may need to visit your obstetrician (pregnancy doctor) more often for frequent prenatal (pregnancy) ultrasounds.
When to see a doctor
If your OB/GYN notices anything out of the ordinary, such as unusual tissue involving your baby’s lung during your prenatal ultrasound, they will refer you to a high-risk pregnancy doctor. This doctor will give you more information about your baby’s health and provide additional testing.
Causes of Congenital Pulmonary Airway Malformation
This condition occurs during a baby’s development in their mother’s womb, and experts don’t know what causes CPAMs. This condition does not appear to run in families.
Who’s at risk
For unknown reasons, male babies are at a slightly higher risk of being born with CPAM than female babies.
Diagnosis of Congenital Pulmonary Airway Malformation
A routine prenatal (pregnancy) ultrasound will show unusual tissue in your baby’s lung tissue before the baby is born. If your OB/GYN thinks your baby has CPAM, you may need the following prenatal tests:
Fetal echocardiogram (ECG). This test for your unborn baby uses sound waves to determine if the CPAM affects their heart structure or function.
Magnetic resonance imaging (MRI). This test uses magnetic fields and radio waves to create detailed images of your baby’s lungs and determine the abnormal lung tissue’s exact location.
Weekly ultrasound tests. You may need weekly ultrasound tests to see if your baby’s CPAM changes in size or shape during pregnancy.
During your pregnancy, the extra lung tissue may grow, shrink or remain the same size. If your baby’s CPAM grows, your doctor will need to closely monitor the condition and related complications (such as chronic lung disease).
Treatments for Congenital Pulmonary Airway Malformation
If your baby has a CPAM, your OB/GYN may recommend one or more of the following prenatal (before birth) treatments:
Early delivery. Your doctor may recommend delivering your baby early to avoid further complications.
Steroid treatment. Steroid treatment may help shrink the CPAM.
Thoracentesis. For large CPAMs filled with fluid, your doctor may recommend thoracentesis, a procedure to drain the fluid. Your doctor uses ultrasound guidance for this procedure. Your doctor inserts a small needle through the uterus and into the chest of your baby. The doctor drains the excess fluid from the baby’s chest.
In many cases, doctors can treat your baby for CPAM after birth using a lobectomy surgery. This procedure removes the lobe (section) of the lung that contains the abnormal tissue through an incision in the chest. If your baby has breathing issues due to the CPAM, they will generally need surgery soon after birth. If the CPAM is smaller and doesn’t affect your baby’s breathing, the doctor may delay surgery until your baby is about one year old.
Prior to planning surgery, your child will have a computerized (CT) angiogram of the chest. This test uses intravenous (IV) contrast to take detailed images of the inside of the chest and to better identify the lung lesion.
If the lobectomy is performed soon after birth, your baby will likely spend time in the neonatal intensive care unit (NICU) so they can be monitored. During surgery, your baby will be under general anesthesia (fully asleep). Your baby’s surgeon will make an incision (cut) in your baby’s chest to remove the extra lung tissue. The cut will then be closed with stitches and covered with a bandage until it heals. Your baby will typically stay in the hospital for two to three days following the surgery for close monitoring.
Babies who undergo this surgery may need to be on a ventilator (breathing machine). Or they might need a heart-lung bypass machine to temporarily take over heart and lung function to allow those organs to rest and heal.
Once your baby recovers from the surgery and if he or she has no other health conditions, your baby will likely go on to be healthy and have no further complications. If your baby has other related health conditions, such as chronic lung disease, he or she will need ongoing monitoring and care.
Contact Us
If you suspect that your child may have Congenital Pulmonary Airway Malformation, make an appointment with an Orlando Health pediatrician today so your child can start on the road to improved health.
