Congenital Pulmonary Airway Malformations (CPAM) are rare congenital lesions involving a part of the lung that impair ventilation and lead to accumulation of fluid and secretions within the lung. These malformations occur in approximately 1 in 8,000 to 10,000 births. They are generally diagnosed during pregnancy or early infancy, though some cases may remain asymptomatic until childhood. Lesions can affect any part of either the right or left lung.

Similarly, pulmonary sequestration is a condition where non-functional lung tissue is located in an abnormal area within the chest cavity. Both conditions require follow-up and treatment by pediatric surgeons.

Symptoms

The symptoms of congenital pulmonary airway malformations include:

• Shortness of breath
• Cough
• Chest pain
• Cyanosis (bluish discoloration)
• Recurrent lung infections

These lesions are classified based on their structure and function. They may appear as large cystic lesions or as multiple small cystic or non-cystic lesions. Symptoms vary according to the type and location of the lesion.

Treatment

While some congenital pulmonary airway malformations may remain undetected for years without causing symptoms, others cause issues such as shortness of breath and recurrent lung infections. Diagnosis is often incidental during prenatal ultrasounds or through chest X-rays or CT scans taken after birth.

Asymptomatic cases may be monitored without surgery, but lesions causing significant symptoms usually require surgical removal.

Surgery can be performed via open or thoracoscopic (minimally invasive) approaches. Minimally invasive surgery reduces the risk of complications during and after the procedure, causes less pain, and shortens recovery time.

If the lesion affects a large part of the lung, surgeons may need to remove an entire lobe of the affected lung (lobectomy). Thoracoscopic lobectomy is performed in specialized centers worldwide, including in Turkey. The main goal is to remove the diseased tissue while preserving as much lung function as possible.

With correct diagnosis and timely treatment, most infants with congenital pulmonary airway malformations have a good prognosis. Early and appropriate intervention reduces the risk of complications and supports healthy development. Non-surgical management may be an option but should only be decided jointly by pediatric pulmonology and pediatric surgery specialists.

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