Congenital Cystic Adenomatoid Malformation / CCAM / CPAM
Overview of Congenital Cystic Adenomatoid Malformation
Definition | Cause(s) | Incidence of CCAM | Diagnosis | Management of Pregnancy | Fetal Surgery and Intervention | Contact Us / Request an Appointment
Congenital Cystic Adenomatoid Malformation / CCAM of the lung is a relatively rare abnormality that appears on an ultrasound as a solid or cystic mass (tumor) of pulmonary tissue that has an abundance of bronchial structures (breathing channels).
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The cause of Congenital Cystic Adenomatoid Malformation is unknown.One theory holds that it is the result of bronchial structures failing to mature properly at approximately the fifth or sixth week of gestation, about the time the lungs begin to form.Another theory holds that Congenital Cystic Adenomatoid Malformation is due to an abnormal growth pattern of lung tissue that occurs as a result of bronchial obstruction.There is no known genetic cause for Congenital Cystic Adenomatoid Malformation, and no cases of recurrence in a sibling or offspring have been reported.
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Although Congenital Cystic Adenomatoid Malformation is increasingly being diagnosed in utero, the true incidence of this lesion is not known. A Congenital Cystic Adenomatoid Malformation lesion is slightly more common in males than females.
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How is Congenital Cystic Adenomatoid Malformation / CCAM Diagnosed?
Congenital Cystic Adenomatoid Malformation can be detected by a detailed ultrasound using color flow Doppler and by ultrafast fetal MRI to confirm the diagnosis and document size and location of the Congenital Cystic Adenomatoid Malformation. A fetal echocardiogram can rule out any associated structural heart abnormalities and assess the effect of the Congenital Cystic Adenomatoid Malformation on heart function.
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Congenital Cystic Adenomatoid Malformation should be followed at least weekly with ultrasound scans for measurement of CAM volume. The CAM volume has been shown to be predictive of outcome in fetuses with these malformations. Weekly ultrasound scans also are recommended to detect signs of hydrops (in utero heart failure) caused by the CCAM. The development of hydrops in a fetus with a Congenital Cystic Adenomatoid Malformation is usually an indication for fetal surgery. Another sign of hydrops is placental swelling. If placental swelling develops as a result of the CCAM, the mother is at risk to develop the "mirror syndrome," an unusual condition in which the mother’s health "mirrors" that of the sick fetus by developing swelling and high blood pressure. This condition calls for immediate delivery of the fetus because "mirror syndrome" is potentially life threatening for the mother.
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Fetal Interventions for CCAM |
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The indications for fetal intervention depend on the type of lesion, whether solid or cystic, and the development of hydrops. Malformations with a large cyst can be treated by aspirating with a needle or by placing a specially designed catheter (called a thoracoamniotic shunt) to drain fluid from the cyst to outside the fetal chest.
In patients with large, solid Congenital Cystic Adenomatoid Malformation with associated hydrops, open fetal surgery is indicated to remove the Congenital Cystic Adenomatoid Malformation. Cases in which the malformations are large but have not resulted in hydrops may require removal during an EXIT (ex utero intrapartum treatment) procedure.
The EXIT procedure is a variation of Cesarean section which allows surgery to be performed on the baby while still connected to the placenta. The EXIT procedure is reserved for cases in which the Congenital Cystic Adenomatoid Malformation would interfere with the baby’s breathing after birth.
To request an appointment at the Fetal Care Center of Cincinnati or contact us, please use our secure, online appointment form or call 1-888-338-2559 (1-888-FETAL59).
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Revised 1/05
