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Lungs are normally divided into sections called lobes; three on the right and two on the left. A congenital cystic adenomatoid malformation (CCAM) is an abnormality of one or more lobes in which the lobe appears as a fluid-filled sac -- called a cyst -- which does not function as normal lung tissue. CCAMs develop with equal frequency on either side of the lung, but rarely occur on both sides.

Most CCAMs either shrink or are small enough not to cause a problem. They are almost always benign, although in rare cases, they become cancerous later in life. The cyst also can become infected and cause pneumonia.

But extra large CCAMs can result in serious and potentially fatal problems in fetuses. First, a large CCAM can cause a condition called "hydrops" or heart failure as it presses against the heart and makes it work harder to circulate blood. About 10 percent of all fetuses with CCAM develop hydrops. Untreated, a fetus with hydrops and CCAM usually will not survive.

The mass can be so large that it limits lung development, resulting in "pulmonary hypoplasia" or small lungs. The CCAM also can push on the heart and the esophagus of the fetus, preventing the fetus from swallowing amniotic fluid. This can result in the mother suffering from "polyhydramnios," or too much amniotic fluid.

 

Reviewed by health care specialists at UCSF Children's Hospital.
Last updated January 15, 2008

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