Airway, Breathing and Lung Conditions

Congenital Lung Malformations

What are congenital lung malformations?

The name “congenital lung malformations” covers a broad range of lung problems. Some are cysts (abnormal closed sacs inside the body that contain fluid or gas). Some are solid. Some are mixed (part cyst, part solid). Congenital lung problems form while a baby’s lungs are developing in the womb.

Two types of congenital lung malformations are:

  • Congenital cystic adenomatoid (pronounced add-en-OH-muh-toyd) malformations (CCAMs): These form when some of a baby’s lung tissue grows more than it should. The growth may cause cysts filled with fluid, ranging in size from very small to very large. CCAMs may prevent some of the tiny air sacs (alveoli) in the lungs from developing normally. A baby may have one or more CCAMs. Most babies with CCAMs have them in only one section (lobe) of a lung.
  • Pulmonary sequestrations (pronounced PULL-muh-nair-ee see-ques-TRAY-shuns) (PSs): These are solid masses of lung tissue. Usually they are closed off from the rest of the lung. They don’t connect to the normal airways or normal blood supply for the lungs. They may be inside the lungs (intralobular) or outside the lungs (extralobular) in the chest or belly. (PSs are also called bronchopulmonary sequestrations, or BPSs.)

CCAMs and PSs form in similar ways. Some children have cysts or masses that seem to be a cross between the two. Sometimes these two conditions are grouped under one name – congenital pulmonary adenomatoid malformations (CPAMs).

Another lung problem sometimes grouped with lung cysts is congenital lobular emphysema (pronounced em-fizz-EE-muh). In this condition, the airway to one lobe of the lung is blocked. Air becomes trapped in this lobe, which makes it expand. It may expand so much that it presses on the healthy lobes or the other lung.

Congenital Lung Malformations in Children

The effects of congenital lung malformations depend on where they are and how large they are. Some lung malformations, mainly small ones, have no effects at all. Others have such serious effects that they threaten the life of a baby even before birth. They may need to be treated while the baby is still in the womb.

One main concern is that a large malformation takes up space in your baby’s chest. It may keep the lung on the same side from reaching a normal size (pulmonary hypoplasia). It may even push on the lung on the other side or on structures in the space between the lungs (mediastinum).

These structures include the airways, the heart and the swallowing tube to the stomach (esophagus).

Another concern is that too much blood may flow to your baby’s CCAM. This can cause congenital heart failure and lead to hydrops, which can be fatal for a baby. If doctors detect hydrops at a certain stage of development, they may be able to treat it before birth with medicines called corticosteroids.

Congenital Lung Malformations at Seattle Children’s

We have treated many children with congenital lung malformations at Seattle Children’s. The team from our Prenatal Diagnosis and Treatment Program has the expertise to monitor babies with malformations that are found before birth and to provide prenatal treatment.

Our doctors are experienced in performing the surgeries these babies need to remove CCAMs or PSs after birth. For patients with severe breathing problems, we have an Extracorporeal Life Support Program. Our Surgery Pulmonary Follow-Up Clinic helps your child be as healthy as possible after surgery.

Read more about expert monitoring and treatment for congenital lung malformations at Seattle Children’s.

Contact Us

If you have questions about congenital lung malformation treatment, call our General and Thoracic Surgery Department at 206-987-2794.