What is congenital cystic adenomatoid malformation (CCAM) / congenital pulmonary airway malformation (CPAM) of the lung?
The normal pair of lungs is made up of five individual lobes - three lobes form the right lung and two lobes form the left lung. When one of these lobes forms incorrectly in a developing fetus, it is called a congenital cystic adenomatoid malformation (CCAM) or congenital pulmonary airway malformation (CPAM) of the lung. This malformation is a fluid-filled, or cystic, mass of abnormal lung tissue that doesn't work like normal lung tissue. In some patients, the cysts are so small that they appear solid on ultrasound. It is a tumor, an abnormal growth, but not a cancer or malignant growth. It is simply a growth of abnormal cells within a lobe of your baby's lung. They look spongy but are not connected with the bronchi (tubes through which the air passes). Most of the time they are asymptomatic but are sometimes seen incidentally on prenatal ultrasound. Although it is not known why these form, the condition usually isn't associated with other birth defects or with abnormal chromosomes. It is also not known whether the condition is passed on from parents to children.
What is a pulmonary sequestration of the lung?
Pulmonary sequestration is another type of lung mass, that is comprised of lung tissue that forms separately, and is not in continuity with the normal lung. They may contain bronchi and alveoli but are not connected with the normal lung. The pulmonary sequestration does not function like the normal lung and may be within a lobe of the lung or outside of the lung. It may be found above or below, and very rarely within the diaphragm muscle.
What is Congenital Lobar Emphysema?
CLE is another type of lung mass. This involves a whole lobe that does not function normally. The bronchi are abnormal hence the gross appearance is that of emphysema (abnormally dilated alveoli).
What are true Congenital Lung Cysts?
CLC is another type of lung mass. It may be single or multiple. It/they are connected to the bronchi so they can get infected. If infected, they may be mistaken for an empyema, or collection of pus.
Prenatal diagnosis and Fetal treatment of Lung masses
Lung masses may be diagnosed during pregnancy. In the case of a CCAM, prenatal ultrasound may show a bright or "echogenic" mass in the baby's chest. The size of the mass varies with each fetus and may even vary with each month of gestation in the same fetus. With relatively large lung masses, ultrasound findings may show that the mass has caused the heart to move from its normal position in the midline and away from the mass and that it has flattened the diaphragm, which should normally be bow-shaped. Normal lung tissue may be difficult to see on the ultrasound if it is "hidden" or behind the mass.
A sequestration may appear as a mass in the chest (above the diaphragm) or abdomen (below the diaphragm) or in the diaphragm.
Your obstetrician may request frequent ultrasounds to measure the size of the mass. The time interval between ultrasound studies depends on your individual case. When a fetus has a CCAM, usually one of two things may happen. The mass in the lung may actually start to shrink and seem to "disappear." This is because the mass has stopped growing while the baby's chest and normal lung continues to develop. Conversely, the mass may continue to grow during your pregnancy. At the end of your pregnancy, your obstetrician (perinatologist) - in consultation with neonatologists (doctors who specialize in treating newborns) and pediatric surgeons - will advise you on a delivery plan.
For more information about prenatal diagnosis and fetal treatment visit Fetal Treatment Center: CCAM
What will happen after my baby is born?
Most babies have no symptoms at birth. Rarely, some babies have difficulty breathing after birth and may require extra oxygen or an immediate operation to remove the mass. If this happens, your baby may stay in the hospital for one to two weeks. Most infants go home with their parents two or three days after birth if asymptomatic.
For these babies, we recommend a special X-ray called a CT scan performed at 3 to 6 months of age or earlier if your baby has respiratory symptoms. You will need to contact the pediatric surgical office at (415) 476-2538 or your pediatrician's office to schedule this procedure.
After the CT scan, the pediatric surgeons will meet with you to discuss the results of the CT scan. The clinical condition of your child is an important factor in deciding whether surgery is needed or not; and if needed, how soon. If surgery is needed, the CT scan will also help determine how the operation will be done. The preferred approach is thoracoscopic, using a small telescope, small instruments and several tiny incisions. Our group has pioneered minimally invasive surgery for CCAM, CPAM, and sequestration. Our surgeon will discuss with you the most appropriate surgery for your child, if it is necessary.
What will happen if my child has the operation?
This requires a short hospital stay. The abnormal mass of tissue and if necessary, some part of the normal lung may be removed. Sometimes, one whole lobe is removed. A chest tube is placed in the operating room to help drain air and fluid, while the rest of the lung heals. Children are usually in the hospital for 2-3 days. The chest tube is removed before discharge.
How do I care for my child at home, after surgery?
The plastic dressings over the chest incision(s) and the chest tube site may be removed in 48 hours or as your child's surgeon instructs you. A full bath may be given at 5 days and the incisions/tape strips may be gently cleaned with soap and water and patted dry. The tape strips will fall off on their own. There are no diet restrictions. Your child may be given Tylenol or Advil for pain. If this is not adequate please contact our office.
Contact our office for any of the following:
- If your child develops a cough or difficulty of breathing
- If your child has a fever > 101 Fº
- If your child has an incision that is red, swollen, tender or draining fluid or blood
- If you have any other questions or concerns.
The office number is 415-476-2538. After hours press option #1 for the answering service.
When does my child return to the office for an appointment?
If your child is doing well, we will schedule a follow up visit in 2-3 weeks. A chest xray may be requested before the visit. The surgeon will evaluate the incisions and review the results of the chest xray, if done, and pathology report with you. Please contact us sooner, if you are concerned about your child or have questions before the scheduled visit.
What is long term follow up?
Children who have had a CCAM/CPAM removed are included in our annual Long term Infant to adult Follow -up and Evaluation or LIFE program. We see children once a year with an office visit and a chest x-ray to evaluate their respiratory health. Children who have not undergone surgical removal are also welcome.