Condition

Pediatric Congenital Pulmonary Airway Malformations

Congenital pulmonary airway malformation (CPAM) is a development that usually occurs in one lobe of the lung. Most of these developments are cysts formed from lung tissue that have a large amount of breathing channels and can be detected early on in fetal development.

With the increasing use of prenatal ultrasonography as well as improvement in technology and skill, most cases of congenital pulmonary airway malformations are prenatally diagnosed. Children with prenatally diagnosed lesions can have various symptoms.

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Frequently Asked Questions

What are some CPAM symptoms in children?

Respiratory distress
- This is the most common symptom in most newborns with a diagnosis of CPAM. It may range in severity from grunting, rapid breathing and an oxygen requirement to severe respiratory failure requiring aggressive ventilator support or extracorporeal membrane oxygenation (ECMO)
- Multiple mechanisms account for the onset of respiratory difficulty:
  - Pulmonary hypoplasia may arise due to a large CPAM
  - Mediastinal shift may compromise cardiac and respiratory function
  - Spontaneous pneumothoraces (collapsed lungs) may occur, and air trapping within the cyst, which leads to compression of functional pulmonary tissue
Recurrent infection: Children who have not had surgery to fix CPAM are at risk of recurrent pulmonary infections due to bronchial compression, air trapping and inability to clear secretions
Coughing up blood or hemoptysis may occur especially in older children
Shortness of breath (dyspnea) and chest pain: Dyspnea may be related to a collapsed lung
Cough, fever and failure to thrive may also occur

What are the physical signs of CPAM in children?

What is the treatment for CPAM in children?

The treatment of CPAM in infants and children involves surgical removal of the affected area of the lung (lobe or lobes). The goals of the operation are to:

Prevent infection in the abnormal area of lung within the CPAM
Expand the CPAM, which will compress normal lung tissue
Prevent the CPAM from possibly becoming concerns in the future

Removal of lung lesions can be achieved by open surgery (thoracotomy), which involves a large incision in the side of the chest. Another method is by a minimally invasive surgery approach in the chest (thoracoscopy), where small incisions are made in the side and a camera guides removal of the lung lobe. With minimally invasive surgery in the chest, infants have less pain, less scarring, shorter hospital stays and fewer problems with chest wall problems related to open thoracic surgery.

Children’s National Hospital offers both treatment options and assess with families to determine the best care plan for each individual patient.