Congenital Cystic Adenomatoid Malformation - SSM Cardinal ...

Congenital Cystic Adenomatoid Malformation
If signs of heart failure develop, or the CVR rises to 1.6 or higher, then fetal intervention may be required.
Oftentimes, prenatal steroids are the first step in the intervention. If the steroids do not stop the growth of
the CCAM, then open fetal surgery to remove the mass can be a life saving option for the baby. If the CCAM
has a dominant cyst of fluid then a needle can be used to drain the fluid and relieve the compression on the
heart. When the CCAM is a solid mass, then open fetal surgery is necessary to remove the mass. If your
baby has reached 32 weeks in pregnancy, then early delivery may be used instead of fetal surgery.
How is CCAM treated after delivery
If fetal intervention is not necessary the infant will be evaluated and treated after delivery. Babies with a
relatively small CCAM can be born without any apparent complications. These babies typically go home and
are followed as an outpatient in 2 to 4 weeks. The pediatric surgeons manage what to do with the CCAM.
Often they recommend surgical removal to prevent future infection and possible malignancy. This is done
though an operation in the chest and the part or lobe of the lung that contains the CCAM is removed.
Babies with a moderately large CCAM’s may have some difficulty breathing after birth. Usually, these babies
breathe very quickly and sometimes they require oxygen. Eating is difficult when breathing fast. These
babies need to be in a neonatal intensive care unit (NICU) for stabilization and surgery is performed to
remove the CCAM and to allow for the remaining normal lung to function optimally. The baby will remain in
the NICU until breathing and eating improve.
Rarely, a CCAM is so large that we anticipate the baby will have problems breathing right at birth. We try to
predict this based on the size of the CCAM and lungs by MRI, and the degree that heart has been pushed out
of the way. To avoid a crisis in the baby’s breathing at birth, we recommend that a special delivery method
be used. When a baby is delivered using an EXIT procedure, the placenta and umbilical cord are maintained
while we evaluate the baby’s lung function. If the baby breathes well, then the baby can be completely
delivered and we can plan for removal of the CCAM later. If the baby’s breathing is compromised, then the
CCAM needs to be removed immediately. The chest operation is performed while the mother’s placenta
supports the baby. When the operation is finished, the cord is cut and the baby can breathe better without
the compression of the CCAM.
What will happen after surgery
The post-operative course varies depending on when the surgery is done, the size of the CCAM, and how
much lung was removed. If the CCAM is removed during the neonatal period, then commonly a breathing
tube and intravenous line is needed. The baby may also have a tube in the chest to drain any fluid and help
expand the lung into the chest space. The baby will not be able to eat until his or her condition has stabilized
but nourishment will be given through the intravenous fluids.
The baby will go home when he or she can breathe sufficiently and eat enough to maintain and gain weight.
The average stay for the newborn can range from 2-3 days for small CCAM’s to 4-8 weeks for much larger
ones. The long-term outcome for infants who have the cyst removed is excellent. These children usually
have no limitations on their activities and have no increased risk for respiratory complications.
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