Cystic adenomatoid malformation of lung

Cystic Adenomatoid Malformation of Lung (CAM), also known as Congenital Pulmonary Airway Malformation (CPAM), is a developmental anomaly of the lung characterized by cystic overgrowth of pulmonary tissue leading to abnormal lung development. This condition is congenital, meaning it is present at birth, and falls under the category of bronchopulmonary foregut malformation.

Etiology and Pathogenesis

The exact cause of CAM is not well understood, but it is believed to result from a disruption in the development of the bronchial tree during fetal life. This disruption leads to the formation of cysts that can vary in size and can affect any part of the lung, although they are most commonly found in the lower lobes.

Classification

CAM is classified into several types based on the size and appearance of the cysts:

• Type I: Characterized by large cysts, more than 2 cm in diameter.
• Type II: Characterized by smaller cysts, less than 2 cm in diameter.
• Type III: Appears as a solid mass of tissue due to microcystic lesions that are too small to be seen without magnification.

Symptoms

Symptoms of CAM can vary widely depending on the size and location of the cysts. Some infants may present with respiratory distress shortly after birth, while others may remain asymptomatic and the condition is discovered incidentally during imaging for another reason. Common symptoms include:

• Respiratory distress
• Cough
• Recurrent pneumonia
• Failure to thrive

Diagnosis

Diagnosis of CAM typically involves imaging studies such as ultrasound during pregnancy or CT scan and MRI after birth. Prenatal ultrasound can sometimes detect CAM in the fetus, allowing for early diagnosis and planning of care.

Treatment

The treatment for CAM depends on the severity of the condition. Asymptomatic cases may be monitored for any changes in symptoms or cyst size. In symptomatic cases, or if there is a significant impact on lung function, surgery may be required to remove the affected portion of the lung. This is typically done through a procedure known as lobectomy, where one lobe of the lung is removed.

Prognosis

The prognosis for individuals with CAM is generally good, especially for those who undergo successful surgery to remove the affected lung tissue. Most children go on to live normal, healthy lives with minimal lung function impairment.

Epidemiology

CAM is a rare condition, with its exact incidence unknown. It is one of the more common congenital lung anomalies diagnosed prenatally.

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