Congenital Cystic Adenomatoid Malformation
What is a congenital cystic adenomatoid malformation (CCAM)?
Fetal Lung Lesion Prenatal Care Algorithm
A CCAM is a benign mass of abnormal lung tissue that replaces part of one lobe of a fetal lung. CCAMs can occur in either lung and are classified into three different types based on the size of the cyst or cysts.
The prognosis for most fetuses diagnosed with CCAMs is excellent, though the condition must be closely monitored. In a small percentage (approximately 10%) of cases, the CCAM can grow large enough to become life threatening during pregnancy. In those cases, the fetus may develop hydrops, a condition in which fluid is collected in the chest cavity signaling heart failure.
What will happen during pregnancy?
A CCAM is typically diagnosed by ultrasound. If one is detected, serial ultrasounds will be scheduled to monitor the size of the mass and to determine if the fetus is any danger. In addition, an amniocentesis may be recommended in some cases to look for chromosomal abnormalities.
Will a fetal treatment be required?
Most CCAMs do not require fetal intervention, but it may become necessary if the fetus develops hydrops. If a large dominant cyst causes compromise, the fluid may be drained with a needle similar to an amniocentesis procedure. Fetal surgery is occasional performed to remove the mass during pregnancy in severe cases where the fetus appears to be in distress from the mass. The decision to proceed with fetal surgery requires an extensive discussion by your doctors at the Center weighing the risks and benefits of the procedure. Your doctors will consider factors that affect mother as well as baby.
What special considerations should be made for delivery?
Mode of delivery - Typically, CCAMs do not require cesarean delivery. The delivery plan should be carefully discussed between the mother and her obstetrician.
Place of delivery - The baby should be delivered at a hospital that is well prepared to handle the potential intensive care and surgery required of infants with a CCAM. There should be a neonatal intensive care unit with the capability to provide specialized care and pediatric surgery services.
Time of delivery - There is no reason to intentionally induce early delivery. Most babies will benefit from reaching full gestational maturity.
What will happen at birth?
Immediately following delivery, doctors will carefully evaluate the baby. Some babies born with a CCAM will experience respiratory difficulties and may require oxygen therapy.
What will happen after delivery?
Babies born with a CCAM may eventually require surgery to remove the mass. In severe cases in which the CCAM is threatening the baby's health, surgical intervention may happen within days of birth. In less serious cases, the child will be monitored for several months and the decision on whether or not to perform surgery will be determined later. If the mass remains small at three to six months of age, surgery may be deemed unnecessary. If the CCAM is deemed to be large enough to threaten the child's health, though, doctors will proceed with the surgical removal of the mass.
What are the long-term outcomes and considerations?
The long-term outcomes for CCAM are excellent and most babies will recover to live a normal life.
Contact The Fetal Center to make an appointment with a high-risk pregnancy doctor.
Note: This information regarding your condition is meant to be a helpful, informative introduction. Because every newborn is different, this may not be applicable to all cases, especially if there are additional defects. Your team of experts at the Center will provide you with information specific to your pregnancy.