Treatment for children with congenital lung malformations (CCAMs or PSs) without hydrops will need additional monitoring and may need surgery to remove the malformations.
If CCAMs or PSs are diagnosed before birth but are not causing hydrops or any other serious problems, 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 and 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 between 3 months and 12 months old. There is less risk using anesthesia (medicine to make your baby sleep without pain) 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. Malformations (both CCAMs and PSs) left in the lungs can get infected and cause pneumonia. There is also a small risk that they will become malignant. PS malformations 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 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.
The doctors at Seattle Children’s who give your child anesthesia are board certified in pediatric anesthesiology. This means they have extra years of training in how to give anesthesia to children.