Birth Defects Respiratory Adenomatoid Malformation

Congenital cystic adenomatoid malformation (CCAM), also called congenital pulmonary airway malformation (CPAM), is one of several respiratory system defects affecting the lungs. Doctors aren’t sure what causes CCAM, but the condition involves abnormal development of lung tissue. Infants born with cystic adenomatoid malformation need surgery, and often require special respiratory care after birth.

CCAM and Lung Tissue

CCAM is a growth of abnormal, cystic tissue in the lungs of a fetus. It can affect any part of the lung, and often replaces one part, or lobe, of the lung. This tissue can consist of many small cysts, or fewer large cysts. These birth defects may affect organ development in the fetus.

The size and extent of CCAM tissue varies. Some cases affect only the lungs, and can be monitored until birth with a low level of risk. In rare cases, very large masses threaten the life of the unborn child. The abnormal lung tissue can impede lung development (pulmonary hypoplasia), and can even affect the heart. Heart failure can cause an accumulation of fluid in the chest or abdominal cavity (known as hydrops fetalis), which is often fatal.

Monitoring Cystic Adenomatoid Malformation

CCAM can be diagnosed prenatally. An ultrasound can reveal cystic tissue, or a problem may be inferred by the displacement of other structures, such as the heart. Growth of the cystic mass can affect the health of the baby. The mass may grow with the fetus, or appear smaller as the fetus grows and the mass does not.

According to the Fetal Treatment Center at the University of California, San Francisco, 90 percent of cases of CCAM have a positive prognosis. However, in some cases of CCAM, organs are compromised, which is more dangerous to the fetus. In addition, fetal distress can affect the mother’s health, a condition called maternal mirror syndrome. Because of the possibility of complications, any pregnancy in which CCAM is diagnosed must be closely monitored to ensure that both the baby and the mother are healthy.

CCAM Treatment and Respiratory Care

Many cases of CCAM require no treatment other than frequent monitoring. In some cases, however, fetal surgery can be performed before birth to treat CCAM. Minimally invasive procedures can drain fluid from the hydrops and large cysts, reducing the size of the mass. Other cases may require open fetal surgery.

Some cases of CCAM are treated at birth. Infants undergo a special procedure called ex utero intrapartum treatment (EXIT), in which surgery is performed while the infant is still attached to the placenta.

All babies with CCAM should be delivered at a hospital that has a neonatal intensive care unit. That’s because many babies with cystic adenomatoid malformation need oxygen and help breathing, even if they had prenatal surgery.

Children who have CCAM that was not treated at or before birth are generally scheduled for surgery between the ages of three and five months. Successful treatment of CCAM usually yields good prognosis, as healthy lung tissue continues to grow until late childhood.

Resources

Children’s Hospital Boston (n.d.). Congenital cystic adenomatoid malformation.Retrieved April 22, 2010, from: http://www.childrenshospital.org/az/Site2192/mainpageS2192P0.html.

Fetal Care Center of Cincinnati (n.d.). Congenital cystic adenomatoid malformation/CCAM/CPAM.Retrieved April 22, 2010, from: http://www.fetalcarecenter.org/conditions/ccam/default.htm.

University of California San Francisco Fetal Treatment Center (n.d.). Congenital cystic adenomatoid malformation.Retrieved April 22, 2010, from: http://fetus.ucsfmedicalcenter.org/ccam/.