Congenital Pulmonary Airway Malformation (CPAM)

Also known as: CCAM (Congenital Cystic Adenomatoid Malformation).

What is congenital cystic adenomatoid malformation?

CPAM is one or more benign (non-cancerous) lumps (masses) or cysts of abnormal lung tissue usually prenatally diagnosed with US during pregnancy. They can range from small to very large in size where they may (in 10% of children) cause significant complications during fetal intrauterine life.


What causes congenital pulmonary airways malformation?

The exact etiology is unknown, but it is secondary to abnormal development of part of the lung tissue which will never function normally.


What are the symptoms of CPAM?

Most infants with CPAM are diagnosed by prenatal maternal ultrasound, and many do not require any intervention. At Nicklaus Children’s Hospital Fetal Care Center, we will be able to assess the lesion based on the US. Often, in addition, we perform state of the art maternal MRI which allows for accurate assessment of the fetal lungs which allows us to predict the severity of the symptoms after birth. Fetal intervention is reserved for severe cases where fetal hydrops (excess fluid around the fetal organs) is present. After birth most lesions are asymptomatic and can be dealt with outside of the newborn period.


What are congenital cystic adenomatoid malformation care options?

Surgical removal of the part of the lung that contains the CPAM considered to be the standard of care by most centers in the United States. In our center at Nicklaus Children’s Hospital we employ the most advanced minimally invasive surgical techniques to remove the affected part of the lung (frequently around 3 to 6 months of age).


Recovery:

After the surgical intervention, the baby will be closely monitored and the duration of hospital stay, usually a couple of days, varies based on the extent of the resection and the surgical approach. Most children have no untoward outcomes and go on to lead normal lives.


Reviewed by: Fuad Alkhoury, MD

This page was last updated on: 6/21/2019 2:20:46 AM


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