Airway, Breathing and Lung Conditions
Congenital Lung Malformation Treatment
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 that leads to hydrops, the situation is more serious. Hydrops can be fatal, either before birth or soon after birth.
Seattle Children’s Prenatal Diagnosis and Treatment Program provides care for babies with congenital lung malformations before birth, if any care is needed.
Treatment for Congenital Lung Malformations Without Hydrops
If congenital cystic adenomatoid malformations (CCAMs) or pulmonary sequestrations (PSs) are diagnosed before birth but are not causing hydrops or any other serious problem, 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, eating well and gaining weight), your baby will be able to go home and will come back for a clinic visit in a couple of weeks.
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 recommend surgery to remove the malformation when your baby is 3 months to 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.
Even if your baby seems to have no problems now from the malformation, doctors recommend removing it. CCAMs and PSs left in the lungs can get infected and cause pneumonia. There is also a small risk that they will become malignant. PSs outside the lungs (extralobular) may need to be removed even if they seem harmless now. Until these masses are taken out, it may be difficult for doctors to tell them apart from cancer called neuroblastoma.
If your baby cannot breathe and feed well after birth because of large malformations, the doctor may advise doing surgery soon after birth. If the problems are severe, your baby may need a breathing machine (ventilator) or extracorporeal membrane oxygenation (ECMO). ECMO is a form of life support that does the work of the lungs and heart for a baby having, 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 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.
The doctors at Seattle Children’s who give your child anesthesia are board certified in pediatric anesthesiology. They have extra years of training in how to take care of children.
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. Seattle Children’s is the first hospital in the United States to have a designated Level IV NICU — the highest level of care.
When they’re ready, children 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 from the recovery room to a hospital room.
The length of hospital stay varies. 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 stay of 2 to 5 days, sometimes longer. The length also depends on your child’s condition after surgery and how quickly your child gets back to eating.
If your child goes home quickly, 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. Most children are seen in clinic several months later. Some are 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 the care they need.
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, 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, surgeons may be able to perform surgery on the baby 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 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. Few hospitals do it. We refer patients who need fetal surgery to the University of California San Francisco Medical Center.
If you have questions about congenital lung malformation treatment, call our General and Thoracic Surgery Department at 206-987-2794, extension 4.