Congenital Lung Malformations

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What are congenital lung malformations?

A congenital lung malformation forms while a baby’s lungs are developing in the womb. “Congenital” means that the condition is present at birth. These lung problems include:

Cysts (abnormal closed sacs inside the body that contain fluid or gas).
Solid growths.
Mixed (part cyst, part solid).
Congenital lobular emphysema (pronounced em-fizz-EE-muh). The airway to 1 section (lobe) of the lung is blocked. Air gets trapped, making the lobe expand. It may expand so much that it presses on the healthy lobes or the other lung.

Types of congenital lung malformations are:
There are 2 types of malformations. They form in similar ways. Some children have cysts or masses that seem to be a cross between the 2 types. Sometimes these conditions are grouped under 1 name – congenital pulmonary airway malformations (CPAMs).
- Congenital cystic adenomatoid (pronounced add-en-OH-muh-toyd) malformations (CCAMs): These malformations form when some of a baby’s lung tissue grows more than it should. The growth may cause cysts, which are filled with fluid or air. They range 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 1 or more CCAMs. Most babies with CCAMs have them in only 1 section (lobe) of a lung.
- Pulmonary sequestrations (pronounced PULL-muh-nair-ee see-ques-TRAY-shuns) (PSs): These are solid masses of lung tissue. They are usually closed off from the rest of the lung and don’t connect to the normal airways or normal blood supply. 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.)
How serious is this condition?

The effects of congenital lung malformations depend on their size and location. Some lung malformations cause few problems, especially small ones. Others may threaten the life of a baby even before birth and may need to be treated while the baby is still in the womb.

If a malformation is large, it may keep the lung on the same side from reaching a normal size. This is called pulmonary hypoplasia. It may even push on the lung on the other side or on structures in the space between the lungs. This is called mediastinum. These structures include the airways, the heart and the swallowing tube to the stomach (esophagus).

With CCAMs, too much blood may flow to your baby’s malformation. This can cause congenital heart failure and lead to hydrops, which can be fatal for a baby. Hydrops is a buildup of fluid in a baby’s body that can cause swelling, heart problems, breathing problems and other symptoms. If doctors find hydrops at a certain stage of development, they may be able to treat it before birth by giving the mother medicines called corticosteroids.

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Symptoms of Congenital Lung Malformations

Children with congenital lung malformations are usually diagnosed before birth during a fetal ultrasound examination. Children who are diagnosed after birth may have these symptoms:

Trouble breathing
Frequent or long-lasting chest infections (pneumonia)
Wheezing
Shortness of breath
Pain with breathing
Trouble eating and gaining weight as babies

Diagnosing Congenital Lung Malformations

Diagnosis before birth
Often, lung malformations are found on an ultrasound during pregnancy. Our Fetal Care and Treatment Center can monitor your baby’s condition, provide any needed treatments and get ready for your baby’s delivery and care they may need after birth. These types of malformations rarely shrink before birth.

You may need imaging studies during pregnancy. To check the size of the malformations and watch for problems, the team may use:
- Ultrasound
- Fetal MRI (magnetic resonance imaging)
- Fetal echocardiogram
Before your baby’s birth, doctors may use a measurement called CVR to help tell whether your baby is at risk for heart failure and hydrops. CVR stands for CCAM volume–to–head circumference ratio. It is done by dividing the size of the CCAM by the size of your baby’s head. A CVR of 1.6 or greater means higher risk.

All babies with CCAM and PS lung malformations will have imaging studies after they are born to evaluate their malformations. Usually, this means having a chest X-ray and a CT (computed tomography) scan (also called a CAT scan). Sometimes the lesion will not be seen on a chest X-ray. A CAT scan is still needed to evaluate the lungs.
Diagnosis after birth
Some lung malformations, mainly small ones, have no effects at all. These are usually diagnosed by a CT scan. Such small malformations usually cannot be seen on a chest X-ray.

If malformations were not diagnosed before birth and your child develops symptoms, your child’s doctor will ask about your child’s health and do a thorough exam.

Your child will likely need imaging studies to check for lung problems. Most children need a chest X-ray and a CT scan of the chest. The doctor may also use other studies, including:
- Bronchoscopy (threading a thin tube with a camera through your child’s mouth or nose to their airways)
- Echocardiogram (an ultrasound image of your baby’s heart)

Treating Congenital Lung Malformations

The best treatment option for your child depends on how the malformation affects their health.

Surgery is almost always needed to remove the malformation. This helps to correct problems your baby may have now, such as not having enough room for their lungs to develop fully. It also helps your child avoid future problems, such as repeated chest infections or having the malformation become cancerous.

For most children with lung malformations, the outlook is excellent. For a fetus with congenital heart failure that leads to hydrops, the situation is more serious.

Treatment for congenital lung malformations with hydrops

If a malformation is causing hydrops before birth, doctors may give you medicines called corticosteroids. This treatment helps to control or clear up hydrops in some babies. It is being tested in clinical studies.

Doctors may recommend that you deliver your baby early so they can be treated right away. Our Fetal Care and Treatment Center coordinates with your birth hospital to ensure a smooth transfer. Seattle Children’s transport team of physicians, nurses and respiratory therapists are national leaders in safely moving fragile newborns.

If your baby is too young to be delivered, surgeons may be able to operate before birth (fetal surgery). They may place a tube that lets extra fluid flow out of the space around the baby’s lung into the womb (thoracoamniotic shunting). This relieves pressure on the baby’s lungs, heart and blood vessels. It may clear up hydrops.

Another fetal surgery option may be to remove the lobe (or lobes) that have CCAMs or PSs. Fetal surgery is rarely needed. Few hospitals do it. We refer patients who need fetal surgery to the University of California San Francisco Medical Center.
Treatment for congenital lung malformations without hydrops

If CCAMs or PSs are not causing hydrops or any other serious problems, treatment is not needed until after your baby is born. Your baby’s medical team will monitor your baby’s condition for any changes. Your baby will likely need surgery to remove the malformations.

Our board-certified pediatric anesthesiologists have extra years of training in how to give children medicine to block pain and make them sleep without pain during surgery.

Monitoring

After delivery, your baby will need a chest X-ray to check the malformation. If your baby is breathing and eating well and gaining weight, they will be able to go home. We will check their condition in a couple of weeks at a clinic visit.

During the first few months after birth, your baby will have a CT (computed tomography) scan to check the malformation again.

Surgery if malformations are not causing immediate problems

If your baby continues to do well, your doctor will most likely recommend surgery to remove the malformation when your baby is between 3 months and 12 months old. There is less risk with anesthesia at this age than right after birth. This still leaves plenty of time for your baby’s healthy lung to recover and grow to nearly normal size.

Even if your baby seems to have no problems now, doctors recommend removing the malformation. Both CCAM and PS malformations left in the lungs can get infected and cause pneumonia. There is also a small risk that they will become cancerous. Without removing PS malformations outside the lungs (extralobular), it may be difficult for doctors to tell them apart from cancer of the nerve cells (neuroblastoma).

Surgery if malformations are causing problems

If your baby cannot breathe and feed well after birth because of large malformations, the doctor may advise surgery soon after birth. For babies with heart or lungs that are not working well, we have an Extracorporeal Life Support Program(ECLS) for heart-lung bypass support.

Most babies have malformations in only 1 section (lobe) of their lung, so they only need 1 lobe removed (lobectomy) or only part of 1 lobe. Some babies need more than 1 lobe or, rarely, an entire lung removed. With PS malformations outside the lungs, surgeons remove the malformation but no normal lung tissue.

The surgery can be done by opening the chest (thoracotomy) or through small incisions (thoracoscopically). Your baby’s surgeon will talk with you about the risks and benefits and which option is best for your baby.
What happens after surgery?
After surgery, your child will be in the recovery room or the Intensive Care Unit (ICU) or Neonatal ICU (NICU), depending on their condition. Most newborns will stay in our ICU or NICU so they get the high level of care they need. Seattle Children’s was the first hospital in the United States to have a Level IV NICU – the highest level of care. When they are ready, your child can move to a regular hospital room and later go home.

Many older children do not need to go to the ICU after surgery. Instead, they go from the recovery room to a hospital room.

The length of your child’s hospital stay depends on:
- How your child is doing before surgery.
- Your child’s condition after surgery and how quickly they get back to eating.
- How urgent the surgery is. If the surgery is not urgent, the hospital stay is usually 2 to 5 days.
If your child goes home within a week, the surgeon will schedule a follow-up visit 2 to 3 weeks after the surgery to make sure the incision is healing and the chest X-ray looks good. If your child stays in the hospital longer, the surgeon will be closely involved in their hospital care.

Most children are seen in clinic several months later. Some are followed in the Pulmonary Medicine Clinic, depending on their condition.
What happens if malformations are found later in life?

Children whose malformations are not found until later in life have the same treatment options as babies. Doctors will advise removing the malformation. This can clear up current problems, such as chest infections, and prevent future risks, such as cancer.