What is it?

Congenital cystic adenomatoid malformation (CCAM), also known as congenital pulmonary airway malformation (CPAM), is an abnormal growth of lung tissue arising from various parts of the airway that can compress normal lung tissue and affect lung development.

How common is it?

CCAMs are the most common congenital lung lesions, although they are still very rare. They occur in approximately 1 in 8,000 to 1 in 35,000 live births.

How is it managed?

Pregnancies with CCAM are followed closely with ultrasound to monitor the size of the lesion and ensure continued fetal well-being. Magnetic resonance imaging (MRI) is also recommended to better define these lesions, and fetal echocardiography is also performed to exclude associated heart problems. Consultations with neonatology and pediatric surgery are recommended to discuss prognosis and management after birth.  While most CCAMs are well-tolerated during pregnancy, large CCAMs may lead to significant complications and must be watched closely.

Postnatal/Prognosis?

Newborns with CCAM must be evaluated by neonatologists to assess for respiratory distress.  Pediatric surgeons may choose to surgically remove the lesion if the CCAM is large enough and causes significant respiratory symptoms. Smaller lesions may be observed with imaging via CT scan, and may still require removal at a later age. For the majority of CCAMs, surgery is curative with an excellent prognosis.