Pediatric Congenital Pulmonary Airway Malformations

• 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

• Rapid breathing (tachypnea): Tachypnea is the most common sign encountered in the newborn period, reflecting respiratory distress.
• Air trapping (pneumothorax): Air leaks into the space between the chest wall and outer tissues of the lungs resulting in increasing respiratory distress
• Cyanosis (turning blue)
• Accessory muscle use
• Grunting
• Failure to thrive, or poor growth

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.