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Congenital Cystic Adenomatoid Malformation

Prenatal Steroid Clinical Trial

A new treatment for fetuses with microcystic CCAMs that have hydrops has emerged here at UCSF. We are embarking on a trial to determine whether steroids might be effective in preventing fetuses from developing hydrops fetalis. To find out more and see if you are eligible for this trial visit:

Investigation of Prenatal Steroids for Treatment of Prenatally Diagnosed CCAMs

Our long experience with prenatal diagnosis and planning for optimum care allows us to offer the highest quality treatment whether intervention is indicated before or after birth.

Under the direction of Dr. Michael Harrison, the Fetal Treatment Center was the first institution to develop fetal surgery techniques. The first open fetal surgery in the world was performed at UCSF since the early 1980's. We presently have more experience with fetal surgery and endoscopic fetal intervention (FETENDO fetal surgery) than any other institution in the world. We are dedicated to congenital cystic adenomatoid malformation research and treatment innovation.

What is a Congenital Cystic Adenomatoid Malformation?

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A congenital cystic adenomatoid malformation (CCAM) is a cystic piece of abnormal lung tissue that does not work like normal lung tissue. It usually replaces one part (lobe) of the lung. CCAMs occur with equal frequency in both lungs. We do not know what causes one part of the lung to develop abnormally, but we know that cystic tissue involved will never function as normal lung tissue.

Learn more about Congenital Cystic Adenomatoid Malformation

What is the outcome for a fetus with CCAM?

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Congenital Cystic Adenomatoid Malformations vary in size at presentation and can change dramatically throughout the pregnancy. The diagnosis is made by prenatal ultrasonographic findings of an echogenic (bright) mass appearing in the chest of the fetus. Other ultrasound findings may include displacement of the heart from its normal position, a flat or everted (pushed downward) diaphragm, or the absence of visible lung tissue.

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How serious is my fetus’s condition?

watch videoIn order to determine the severity of your fetus's condition it is important to gather information from a variety of tests and determine if there are any additional problems. These tests along with expert guidance will determine what choices you have for your pregnancy and are important for you to make the best decision about the proper treatment.

This includes:

  1. The type of defect—distinguishing it from other similar appearing problems.
  2. The severity of the defect—is your fetus’s defect mild or severe.
  3. Associated defects—is there another problem or a cluster of problems (syndrome).

Learn more about the severity of CCAM

What are my choices during this pregnancy?

watch video 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.

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Prenatal Steroid Clinical Trial

watch videoA new treatment for fetuses with microcystic CCAMs that have hydrops has emerged here at UCSF. Our group has discovered that steroids, which are commonly given in pregnancy, may help fetuses with large CCAMs to either prevent or reverse hydrops fetalis. Our study published in 2003 showed the potential efficacy of steroids for treatment in these situations.

We are embarking on a trial to determine whether steroids might be effective in preventing fetuses with large microcystic CCAM's from developing hydrops fetalis. 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?

watch video 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.

Learn more about what will happen after birth

Last Updated: 1/26/2012
Angela and Guy

Angela and Guy

The innovative procedures at FTC saved the lives of both Angela and Guy, whom were both diagnosed with severe CCAMs.

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Rae Gerking

We also discovered our baby girl had Congenital Cystic Adenomatoid Malformation (CCAM), of the lung...

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Ethne Nerrmana
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