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Congenital Pulmonary Airway Malformation (CPAM)

Even the smallest person can change the course of history.

J.R.R. Tolkien

What is Congenital Pulmonary Airway Malformation (CPAM)?

Congenital Pulmonary Airway Malformation (CPAM), also known as Congenital Cystic Adenomatoid Malformation, is a rare developmental lung disorder that occurs during fetal development. In CPAM, abnormal lung tissue forms cysts or air-filled sacs within the lungs, which can affect lung function and development.

What causes Congenital Pulmonary Airway Malformation (CPAM)?

The exact cause of CPAM is not fully understood. It is believed to result from abnormal development of the lung tissue during fetal growth. Some research suggests genetic factors may play a role, but the condition typically occurs sporadically and is not inherited.

What are the symptoms of Congenital Pulmonary Airway Malformation (CPAM)?

Symptoms of CPAM can vary widely and may include:


  • Respiratory distress at birth, including rapid breathing and cyanosis (bluish discoloration of the skin)

  • Recurrent respiratory infections

  • Chest pain

  • Wheezing

  • Coughing

Sometimes, CPAM may be asymptomatic and discovered incidentally on prenatal ultrasound or during evaluation for other respiratory issues.

How to treat Congenital Pulmonary Airway Malformation (CPAM)?

Treatment for CPAM depends on various factors, including the size and location of the lesion, the severity of symptoms, and the overall health of the individual.

In many cases, surgical intervention is recommended to remove the affected portion of the lung and prevent complications such as infection, pneumothorax (collapsed lung), or malignancy.

The timing of surgery may vary and can depend on factors such as the presence of symptoms, the stability of the patient, and the specific characteristics of the CPAM lesion.

In some cases, CPAM may be managed conservatively with close monitoring, especially if the lesion is small and asymptomatic. However, regular follow-up with a healthcare provider is essential to monitor for any changes or complications.

It's important for individuals with CPAM to receive comprehensive care from a multidisciplinary team of healthcare professionals, including peadiatric pulmonologists, peadiatric surgeons, and neonatologists, to ensure the best possible outcomes.

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