What is Congenital Pulmonary Airway Malformation (CPAM)?
Congenital Pulmonary Airway Malformation (CPAM) is a rare developmental anomaly of the lung. It is characterized by the presence of cystic and adenomatous malformations that can disrupt normal lung architecture. CPAM was previously known as congenital cystic adenomatoid malformation (CCAM).
Type 0: Extremely rare, involves tracheal or bronchial tissue.
Type 1: The most common type, characterized by large cysts (more than 2 cm in diameter).
Type 2: Composed of smaller cysts (less than 2 cm), often associated with other congenital anomalies.
Type 3: Solid, bulky lesions that can cause significant mediastinal shift.
Type 4: Characterized by large peripheral cysts, similar to Type 1 but located at the periphery of the lung.
How is CPAM Diagnosed?
CPAM is often diagnosed prenatally through routine
ultrasound scans. The presence of cystic or solid lung lesions can be identified as early as the second trimester. Postnatal diagnosis may involve additional imaging techniques such as
chest X-ray,
CT scan, or
MRI. In some cases, a biopsy may be required for definitive diagnosis.
What are the Symptoms of CPAM?
The clinical presentation of CPAM varies widely. In some infants, CPAM may be asymptomatic and discovered incidentally. In others, symptoms can include:
Respiratory distress
Recurrent respiratory infections
Chronic cough
Failure to thrive
Observation: For asymptomatic patients with small lesions, regular monitoring may be sufficient.
Surgical Resection: Often recommended for symptomatic patients or those with large lesions. Surgery usually involves lobectomy or segmentectomy.
Prenatal Intervention: In severe cases, fetal surgery may be considered to prevent hydrops or other complications.
What are the Prognosis and Long-term Outcomes?
The prognosis for children with CPAM is generally good, especially when diagnosed early and managed appropriately. Most children lead normal lives post-surgery, although long-term follow-up is often recommended to monitor for potential complications such as recurrent infections or malignancy.
Can CPAM be Prevented?
Currently, there are no known preventive measures for CPAM, as its exact cause is not fully understood. Research suggests that it may involve disruptions in the normal development of the fetal lung, possibly due to genetic factors or environmental influences.
