Congenital Pulmonary Airway Malformation (Pediatric)

What is a congenital pulmonary airway malformation?

This condition is present at birth and affects lung development. Pulmonary airway malformation occurs when there is an abnormal growth of lung tissue arising from various parts of the airway that compresses normal lung tissue. 

What causes a congenital pulmonary airway malformation?

The underlying causes of this condition are unknown. 

What are the symptoms of a congenital pulmonary airway malformation?

Children born with this condition have respiratory problems characterized by rapid breathing and grunting. Other symptoms are frequent pulmonary infections, coughing (including coughing up blood), shortness of breath, chest pain, and fever.

How is a congenital pulmonary airway malformation diagnosed?

A prenatal ultrasound usually detects this condition. The mother’s pregnancy will be closely followed to monitor the size of the lung lesion. Magnetic resonance imaging (MRI) may be used to better define these lesions, and fetal echocardiography can be done to exclude associated heart problems. Our pediatric surgeons and neonatologists collaborate to discuss prognosis and management of this condition after birth.   

What is the treatment for congenital pulmonary airway malformation?

Our pediatric surgeons may choose to surgically remove the lesion at birth if the congenital pulmonary airway malformation is large enough and causes significant respiratory symptoms. Smaller lesions are removed when the infant is between 4-6 months using minimally invasive techniques.