Congenital Cystic Adenomatoid Malformation

A congenital cystic adenomatoid malformation (CCAM), more recently renamed a congenital pulmonary airway malformation (CPAM), is an anomaly of the lower respiratory tract in which a small section of the tubes which carry oxygen to and remove carbon dioxide from the body (bronchioles) do not develop properly. CCAMs are the most common congenital lung lesion and many can be detected prenatally by ultrasound. Not all CCAMs have the same appearance. Depending upon the nature of the malformation, they can be solid, cystic or mixed solid cystic. The most common type of CCAM is comprised of one or more large cysts, greater than 2 cm. Typically the process is confined to a single region (lobe) of the lung and does not involve the entire lung.

How is it treated?

Before Birth:
Once your baby is diagnosed with a lung lesion, a visit is coordinated for the mother to meet with the pediatric surgery team as well as neonatology to discuss the course of treatment and to provide support. Most CCAMs do not cause any trouble to your unborn baby. Rather, your obstetrician and the pediatric surgery team will simply follow your baby a little more closely by ultrasound to make certain the CCAM is not growing quickly. If the CCAM is large enough, it can compress the heart and blood vessels in the chest. Ultrasound is excellent for detecting signs of this compression. CCAMs with large cysts causing compression may require in utero cyst aspiration. CCAMs with predominantly solid components causing compression usually respond to the administration of maternal steroids.

After Birth:
We recommend that all babies diagnosed in utero with CCAMs are born at Yale-New Haven Children’s Hospital. A small percentage of babies will have difficulty breathing at the time of birth because of the presence of the CCAM. In those infants, we recommend emergent surgery shortly after birth through a small incision made just below the tip of the shoulder blade. Your baby will be admitted to the newborn intensive care unit (NBICU) for recovery. Most children, however, have no problems at the time of birth. Nonetheless we recommend surgical resection of the involved lung to all children between 3 and 6 months of age because CCAMs are prone to develop recurrent pneumonias and are associated with a small risk of turning into cancer. In preparation for the operation, a CT scan will be done to better define the extent of the CCAM. Most CCAMs can be removed through a minimally invasive approach called a thoracoscopic lobectomy. A thoracoscopic approach involves making small cuts, usually three to four, on one side of the chest (thoracotomy). Instruments are placed inside the chest to identify the lesion and excise it. A lobectomy is a surgical excision of the affected lobe of the lung. At the end of the operation, a chest tube is placed to drain excess fluid and help the lung to re-expand.

What will happen in the hospital?

  • After surgery, your child will be admitted to the pediatric surgery floor and remain there for approximately 2-3 days. The chest tube, placed in surgery, will remain in place for approximately 1-2 days. The output of fluid will be monitored. The chest tube will be removed once the output is minimal and there is no evidence of an air leak. 
  • A dressing will be placed over the chest tube site with gauze and a plastic dressing. This should remain in place for 24 to 48 hours before removing. 
  • Your child will be on around the clock pain medication. 
  • The RN and MD staff will be monitoring your child’s oxygen status.

When will my child be discharged?

Once your child is able to tolerate formula, breast-milk or food, the pain is well controlled, the chest tube is out, and is breathing well he/she will be discharged.

What will be my child's recovery?

  • There will be little white bandages (steri strips) in place over the surgical sites. These will remain in place for 7-10 days and usually fall off on their own. 
  • Once your child is home he/she will be able to resume baseline activities. Your child may require 1-2 days of Tylenol or Motrin, as needed. 
  • Your child will be able to have a sponge bath or shower, but cannot be submerged in water for 7-10 days after surgery.

What should I be looking for after the operation/hospitalization?

  • Respiratory distress/compromise 
  • Fevers 
  • Drainage, redness or swelling from the incisions 
  • Uncontrolled pain
Your child will have follow up with the pediatric surgery team 2 weeks after discharge.