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Congenital Cystic Adenomatoid Malformation Treatments

• What are my choices during this pregnancy?
• CCAM Prenatal Steroid Clinical Trial
• What will happen after birth?

What are my choices during this pregnancy?

Most fetuses with Congenital Cystic Adenomatoid Malformation do not require intervention during the pregnancy and will do well with management after birth (usually surgery to remove the abnormal piece of lung). A few fetuses with the type of CCAM that has a few large cystic fluid collections can be treated by draining the cysts into the amniotic fluid using a Harrison pleuro-amniotic shunt catheter. A few fetuses with very large lesions that will make resuscitation after delivery dangerous will need special management at birth: a specialized delivery called the EXIT (ex utero intrapartum treatment) procedure. We developed the EXIT procedure for management of fetal airway emergencies, but it can be adapted to allow surgical resection of the CCAM while the baby remains attached to the placenta. The EXIT procedure can also be used to facilitate immediate support using extracorporeal membrane oxygenation (ECMO).

But the most effective treatment of very large CCAMs causing hydrops is fetal surgical resection of the CCAM. Only the most severely affected fetuses are candidates for this intervention. Fetal surgery is only offered when there is evidence of hydrops (heart failure) in the fetus. The hydrops is caused by the large mass pushing on the heart. Because the heart has to work harder, the fetus develops signs of heart failure. These signs are abdominal ascites (fluid in the abdomen), pleural or pericardial effusions (excess fluid around the lung or heart), skin or scalp edema (excess fluid under the skin or scalp), polyhydramnios (too much amniotic fluid), or a thickened placenta (placentomegaly). Women with signs of pre-eclampsia (high blood pressure, protein in the urine) have the mirror syndrome and are not candidates for fetal intervention

Fetal hydrops will lead to death unless treated. The hydrops can be reversed by removing the large CCAM causing the heart failure. The operation to remove the abnormal lobe of the lung (lobectomy) is the same that is done after birth. The mother’s uterus is surgically opened, like a Cesarean section, and the fetus’s chest is opened and the abnormal piece of that lung is removed. This procedure was developed at the UCSF Fetal Treatment Center and has proved quite successful in over 20 cases (read Rae Ellen Gerking's story). However, this is a major operation. The mother will need to be carefully monitored and take medications to control preterm labor. Delivery is by Cesarean section, usually before 36 weeks’ gestation. 

CCAM Prenatal Steroid Clinical Trial

In a few cases where fetal surgery to remove the CCAM is not possible and the fetus is still too immature to delivery, we give steroids to the mother. We have seen hydrops resolve after steroid treatment in a few cases, and we are currently studying the biology of this potential treatment to see if it will help shrink the CCAM.

You may be eligible to be considered for this clinical trial. For more information please visit Investigation of Prenatal Steroids for Treatment of Prenatally Diagnosed CCAMs

What will happen after birth?

Fetuses with small or moderate-sized Congenital Cystic Adenomatoid Malformations that do not change much during pregnancy can be successfully managed after a normal birth. They usually do not have any difficulty during pregnancy or any trouble breathing at the time of birth. If imaging studies (X-ray or CT scan) show a significant CCAM mass, or if the baby has any difficulty breathing, the CCAM should be surgically removed to prevent the development of infection or possible cancer later in life. This surgery is quite safe even in the first year of life and does not compromise lung function or the normal development of the baby.  These children will have perfectly normal lung function. 

If the mass is very small and the baby has no difficulty breathing, the baby can go home. If the mass remains very small on CT scan at 3-6 months, surgery is not necessary. However, if the CCAM is larger or the baby develops any symptoms, then the CCAM should be removed.