Congenital Pulmonary Anomalies

Congenital Pulmonary Anomalies

Congenital pulmonary anomalies include lumps made of abnormal lung tissue or improper tissue development inside the lungs. These conditions often are discovered before a baby is born by ultrasound exam.

In some cases, congenital pulmonary anomalies cause no symptoms or are so mild that no action other than regular monitoring is required. In most cases, pulmonary lesions may cause breathing problems that need to be addressed at birth or later in a child’s life.

The three most common congenital pulmonary anomalies are:

  • Congenital pulmonary airway malformation (CPAM). CPAM involves an abnormal piece of lung tissue that appears as a lump on imaging tests. CPAM can cause breathing problems.
  • Congenital lobar emphysema. This developmental defect allows air to enter the lungs, but then it cannot exit the lungs. Severe cases are marked by breathing problems and heart trouble.
  • Sequestration. An abnormal piece of lung tissue is present in the chest cavity but is not connected to the bronchial tree or pulmonary artery.

When anomalies are more prominent, symptoms can be more serious. Symptoms of congenital pulmonary anomalies may include:

  • Breathing trouble or respiratory distress
  • Bluish tint to the skin from lack of oxygen
  • Enlarged chest
  • Rapid breathing
  • Heart problems
  • Heart and lung compression

These rare problems are often found on maternal ultrasound before the baby is born. A multidisciplinary team of pediatric specialists in surgery, obstetrics, maternal-fetal health, neonatology and pulmonology monitors any developing baby with a congenital pulmonary anomaly. When these problems are found before the baby is born, frequent ultrasounds are done and fetal magnetic resonance imaging (MRI) scans may be needed. A care plan is created and discussed with the baby’s parents.

Most of the time no action is needed before birth as some lesions go away or get smaller. Others may grow larger and cause problems during development. If that happens, procedures can be performed before birth to prevent further damage to the growing baby.

Some babies born with a congenital pulmonary lesion require treatment the day they are born. In most cases, children born with lung lesions or lumps have no symptoms and no immediate treatment is required. Children with these conditions continue to be monitored as they grow.

Congenital pulmonary lesions may also be discovered during infancy or childhood when a chest X-ray is done due to breathing complaints.

Diagnosis of Congenital Pulmonary Anomalies

If your unborn baby or child shows signs of a congenital pulmonary anomaly, a maternal-fetal obstetrician, pediatric surgeon or neonatologist can perform the following Trexams and tests to make a diagnosis:

  • Ultrasound. This is a painless imaging test that uses sound waves to view lung tissue.
  • Magnetic resonance imaging (MRI). MRIs use a magnetic field and radio waves to create detailed images of internal body tissues such as the lungs and blood vessels.
  • Chest X-ray and computed tomography (CT). After your baby is born, a chest X-ray and CT scan are used to help evaluate the anomaly.

Treatments

The most common treatment for a congenital pulmonary anomaly is regular monitoring by a pediatric surgeon who checks the size of any lung lesions.

Children who require surgery often receive it within the first year of life. A pediatric surgeon can remove the lump or lesion in lung tissue to help restore function.

Key Points to Remember

  • Congenital pulmonary anomalies include lumps made of abnormal lung tissue or improper tissue development inside the lungs.
  • Most congenital pulmonary anomalies are discovered before birth during an ultrasound exam.
  • In some cases, congenital pulmonary anomalies cause no symptoms or are so mild that no action other than regular monitoring is required. In most cases, pulmonary lesions may cause breathing problems that need to be addressed at birth or later in a child’s life.
  • The most common treatment for a congenital pulmonary anomaly is regular monitoring by a pediatric surgeon who checks the size of any lung lesions.
  • Children who require surgery often receive it within the first year of life.

Support Services & Resources

Visit the link below to find support groups and services and learn more about congenital pulmonary anomalies.

Riley at IU Health offers a broad range of supportive services to make life better for families who choose us for their children's care.

Learn More About Riley Support Services

National Organization for Rare Disorders

This group provides resources and information for patients and families about congenital lobar emphysema, a type of congenital pulmonary anomaly.

Locations

Locations

In addition to our primary hospital location at the Academic Health Center in Indianapolis, IN, we have convenient locations to better serve our communities throughout the state.

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Departments Treating This Condition