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Congenital Lung Conditions

What is a Congenital Pulmonary Airway Malformation (CPAM)?

 

Congenital Pulmonary Airway Malformation (CPAM) is an unusual growth of tissue in or around your baby’s lungs. It can be solid, cystic (made up of sacs of fluid) or a combination of both. There can be one growth or several, but they usually only affect one lobe (section) of the lung.

CPAM is sometimes also called Congenital Cystic Adenomatoid Malformation (CCAM) or Cystic Lung Disease.

Types of CPAM


There are three types of CPAM:

  • Type 1 - Large, fluid-filled cysts, often with one cyst that is dominant (biggest)

  • Type 2 - Cysts of many different sizes

  • Type 3 - Many, many tiny cysts, often so close together they appear solid

Causes of CPAM


Doctors are not sure what causes this lung condition, which happens early in a baby's development. What we do know is that it is not related to anything the mother did or did not do during pregnancy, and it does not appear to have any genetic causes or implications for other family members.

Diagnosis


This condition is usually diagnosed by a prenatal ultrasound at around 20 weeks gestation. If your doctor sees a CPAM at your initial ultrasound, he or she will monitor you with regularly scheduled ultrasounds to check for any changes.

It's important to know whether the CPAM is growing, shrinking, or staying the same size. Occasionally, the CPAM disappears on its own as your baby grows and no longer causes concern.

However, if the CPAM grows larger, or interferes with the development and function of other organs, such as the heart, your doctor may recommend intervention before or shortly after your baby is born. Large CPAM growths can sometimes lead to hydrops, a very serious condition for your baby.

Treatment options

 

Fetal interventions

 

Children's Mercy is one of only a few centers in the region to offer fetal treatment for babies before they are born. Although most interventions for CPAM are performed after birth, fetal treatment may include:

  • Cyst aspiration, to puncture and drain a large CPAM cyst

  • Thoracoamniotic shunt placement - this procedure uses ultrasound guidance to place a straw-like tube into the baby's chest. This allows the CPAM cyst's fluid to drain out of the chest and into the amniotic fluid surrounding the baby

  • Removal of the cyst - this is only done prenatally in the rare case of a baby experiencing heart failure (hydrops) due to a very large CPAM

After birth


If it is not possible or necessary to provide treatment before birth, your care team will evaluate your baby as soon as possible after they are born and stable. Typically, doctors will schedule a surgery to remove the affected part of the lung. Removal helps eliminate the risk of infection and malignancy (cancer) in the future. Most babies have a very good prognosis once the CPAM is removed.

The timing of surgery depends on whether your baby's overall health is impacted by the CPAM. If the growth is relatively small and there are no problems with breathing or other concerns, you may be able to go home with your baby and schedule surgery for when they are 3-6 months old.

If the CPAM is causing problems for your baby, your doctors may recommend surgery shortly after birth in order to give your baby the best possible outcome. If this is the case, you may consider delivering your baby in the Special Care Delivery unit at the Fetal Health Center to ensure your baby receives immediate medical attention while still staying close by to you.

The surgical procedure consists of making either one incision (open technique) or a few smaller incisions (minimally invasive technique) in the side of the chest. This allows your surgeon to remove the affected lobe. Babies are able to do very well with the remainder of their healthy lung tissue after the CPAM is removed.

During the procedure, your baby will need general anesthesia and will have a tube in their chest after surgery for a few days. A follow-up chest X-ray helps doctors check for full expansion of the remaining lung tissue.

Most of the time, you can go home within a week after surgery. However, the length of stay in the NICU for an infant with this condition varies. Your baby will be able to go home when they are gaining weight and can tolerate full feedings. After discharge, a pediatrician will monitor your baby. Your baby will also come back to Children’s Mercy to see the pediatric surgeon or any other necessary specialists.

Outcomes and prognosis for CPAM


The outcomes for babies with CPAM are generally very good. In most cases, infants recover well and usually have no immediate issues after discharge from the hospital. It's important to follow up with your pediatrician or primary care provider for routine care during infancy and childhood.

Choosing the best home for your child's care


At Children’s Mercy, our skilled professionals can care for you and your baby before, during and after delivery. Our Fetal Health Center works with families like yours every day to help correct and prevent problems that happen during prenatal development.

Our team of pediatric surgeons was one of the first to apply minimally invasive surgical techniques (surgery through very small incisions) to infants and children. Our expertise in minimally invasive thoracic surgery means we are able to remove most CPAMs using these specialized techniques to minimize pain, scars, and days in the hospital for your child.

Children's Mercy is an American College of Surgeons Verified Center

Highest Level of Surgical Care

Children’s Mercy is one of only 10 centers in the nation to be verified as a Level 1 Children’s Surgery Center — the highest possible rating — by the American College of Surgeons. 

Special Care Clinic: Care for your growing child

We recognize that many of our NICU graduates have special health care needs related to growth and development. The Special Care Clinic provides follow-up services for your babies after they're home from the NICU.