Congenital Pulmonary Airway Malformation (CPAM)

What is CPAM?

CPAM is an abnormal area of tissue in the lung that is present from birth. They happen when tissues in the lungs or airways haven’t developed properly during pregnancy. What causes them isn’t known.

CPAM is the most common type of congenital lung abnormality and is usually detected before your baby is born, at the 20-week prenatal scan.  

There are different types of CPAM.  Doctors will not know for certain exactly which type of CPAM your baby is affected by until imaging is performed after birth.  Different types of CPAM that may show up on a chest CT scan include: Bronchogenic cysts, CCAM and bronchopulmonary sequestration CPAM is reported to occur in 1/6,000 births

CPAM is, however, the most commonly diagnosed lung abnormality in fetal life.

How is it diagnosed?

CPAM is usually found during  routine pregnancy ultrasound. The scan may show a bright or cystic area in the chest. A special Doppler scan may be used to look at blood flow, and sometimes an MRI scan is done.

What causes CPAM?

CPAM happens when part of the lung develops abnormally. The cause is not fully understood, and it is usually not inherited.

What does it mean for my baby?

You will be cared for by the fetal medicine team and have regular scans in pregnancy.

This condition is not usually associated with genetic or chromosome differences.

In most cases, CPAM does not cause problems. Some may shrink or disappear before birth. Occasionally the lesion can grow bigger and cause strain on the heart and pressure on the normal lung tissue. In this situation we can consider giving steroids or performing a needle procedure to drain a large cyst.

In such cases early birth may be indicated.

What happens after birth?

Your baby may need breathing support after birth, some babies can have significant breathing difficulties and may need ventilation (a breathing machine) or taking oxygen through a face mask or tubes in their nose.

They will need more scans and often a CT scan is needed. Surgery is sometimes needed to remove the abnormal area, especially if it causes symptoms or infections.

What are the symptoms in older children and adults?

Most children with CPAM will not experience symptoms.

In older children and adults, symptoms of congenital lung abnormalities can include:

  •  infections that keep coming back in the affected area of the lung
  • bronchiectasis
  •  asthma-like symptoms (coughing, wheezing, breathlessness) that do not respond to asthma treatment.

Will it happen again?

CPAM usually happens once and is not inherited. The chance of it happening again in future pregnancies is very low.

What is the long-term outcome?

The long-term outlook and any long-term complications for CPAM and other congenital lung abnormalities will depend on the type of condition your child has. Generally, when CPAM is diagnosed prenatally (before your child is born), the long-term outlook is good. However, a few types of CPAM (around 5%) can develop complications and it is not known which.