Congenital lung malformations may have a wide range of effects, from mild to serious. There are many treatment options. The best options for your child depend on the effects on 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 getting repeated chest infections or having the malformation turn into cancer (become malignant).
For most children with lung malformations, the outlook is excellent. For babies with congenital heart failure (hydrops), the situation is more serious. Hydrops can be fatal, either before birth or soon after birth.
Treatment for Congenital Lung Malformations Without Hydrops
If congenital cystic adenomatoid malformations (CCAMs) or pulmonary sequestrations (PSs) are not causing hydrops or any other serious problem for your baby before birth, then no treatment is needed until after the baby is born. Your baby’s medical team will monitor your baby’s condition for any changes. Most babies with CCAMs or PSs do not need treatment before birth.
After delivery, your baby will need a chest X-ray to check the malformation. If your baby is doing well (such as breathing well and eating well), your baby will be able to go home and will come back for a clinic visit in a couple of weeks.
Sometimes a lung malformation cannot be seen on a chest X-ray. So sometime during the first few months after birth, every baby diagnosed with a malformation before birth will have a CT (computed tomography) scan to check the malformation again.
If your baby continues to do well, the doctor will most likely advise surgery to remove (resect) the malformation when your baby is between 6 months and 12 months old. Anesthesia (medicine to make your baby sleep without pain) has less risk 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 and function.
Even if your baby seems to have no problems now from the malformation, doctors advise removing it. CCAMs, in particular, and PSs left in the lungs can get infected later and cause pneumonia. There is also a small risk that they will become malignant later in life. PSs outside the lungs (extralobular) also need to be removed even if they seem harmless now. Until these masses are taken out, it is difficult for doctors to tell them apart from a type of cancer called neuroblastoma.
If your baby is not able to breathe and feed well after birth because of malformations that are too big, the doctor may advise doing surgery soon after birth. If the problems are severe, your baby may need a breathing machine (ventilator) or may undergo ECMO treatment. ECMO stands for extracorporeal (outside the body) membrane oxygenation, a form of extracorporeal life support that does the work of the lungs and heart for an infant undergoing, or recovering from, urgent or emergency surgery.
Most babies have malformations in only one section (lobe) of their lung. So they need only one lobe removed (lobectomy) or only part of one lobe. Some babies need more than one lobe or, rarely, an entire lung removed. With PSs outside the lungs, surgeons remove the malformation but no lung tissue.
The surgery can be done by opening the chest (thoracotomy) or through small incisions (thorascopically). Your baby’s surgeon will talk with you about the risks and benefits and which option is best for your baby.
With either type of surgery, your baby will have a chest tube in place after the surgery. The tube lets out any air or fluid that collects in the chest after surgery. The tube usually has to stay in place for at least two days. Your baby may need the tube longer, depending on how much air or fluid comes out.
Malformations found later
Children whose malformations are not found until later in life have the same treatment options. Doctors will advise removing the malformation — either to clear up current problems, such as chest infections, or to prevent future risks, such as the risk of cancer.
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, where they get the high level of care they need. When they’re ready, they move to a regular hospital room and eventually to home. Many older children do not need to go to the ICU after surgery. They go first to the recovery room and then to the room in the hospital.
The length of hospital stay varies for children who have surgery for lung malformations. It depends partly on how your child is doing before surgery and how urgent the surgery is. If the surgery is elective (not urgent or emergent), you can expect a hospital stay of two to five days, sometimes longer. The length of the stay also depends on your child’s condition after surgery, how soon the chest tube comes out and how quickly your child gets back to eating.
If your child goes home quickly, the surgeon will schedule a follow-up visit two to three weeks after the surgery to make sure the incision is healing and the chest X-ray looks good. Most children who have surgery are seen in clinic several months later. Some also need to be followed in the pulmonary medicine clinic, depending on their condition. The team from our Surgery Pulmonary Follow-Up Clinic will work with your child's other healthcare providers to make sure your child gets other care they need going forward.
If your child stays in the hospital longer, the surgeon will stay closely involved with your child’s care while they are in the hospital.
Treatment for Congenital Lung Malformations with Hydrops
If a malformation is causing hydrops before birth (most likely due to a CCAM), doctors may give the mother medicines called corticosteroids. This treatment, which is being tested in clinical studies, helps to control or clear up hydrops in some babies.
Doctors may advise delivering your baby early so the baby can have surgery and any other needed treatment right away.
If your baby is too young to be delivered early, it may be possible to perform surgery on the baby before birth (fetal surgery). During fetal surgery, a tube may be placed that lets extra fluid flow out of the space around the baby’s lung into the amniotic fluid in the womb (thoracoamniotic shunting). This relieves pressure on the baby’s lung, heart and blood vessels. It may clear up hydrops. Another option with fetal surgery may be to remove the lobe (or lobes) that have CCAMs or PSs. Fetal surgery is rarely needed and requires special expertise. It is done at few hospitals. We refer patients who need fetal surgery to the University of California San Francisco Medical Center.