Congenital lobar emphysema is a rare respiratory condition that can be diagnosed in newborns and infants. A mild form of this condition is also found in adults. emphysema is an acquired condition associated with smoking.

Both emphysema and CLE affect the lungs. These conditions are different from one another. They are not different versions of the same disease.

There are outcomes and treatments for people with this condition.

Congenital lobar emphysema is a respiratory disorder that occurs in around 1 out of every 20,000 to 30,000 live births. It causes a malformation of the lungs to occur. This may present in a variety of ways.

Doctor looking at chest x-ray images

CLE can cause a defect in bronchial cartilage, which results in airway obstruction or collapse. It may also reduce the amount of bronchial cartilage in the lung. Bronchi are the lung’s main airways. Their job is to carry air in and out.

CLE may be earmarked by alveoli that are abnormally small. Alveoli are air sacs located at the ends of bronchioles (air tubes) in the lungs. Alveoli are where blood and lungs exchange oxygen and carbon dioxide during respiration.

Babies born with CLE can breathe air in, but they can’t breathe air out. This causes the lobes of the lung to overinflate (hyperinflation). Unlike emphysema acquired in adulthood, CLE doesn’t cause tissue damage or alveoli damage.

There are two forms of the same thing.

  • Hypoalveolar: Lungs contain fewer alveoli than is typical.
  • Polyalveolar: Lungs contain more alveoli than is typical.

Around 50% of infants with CLE are diagnosed as newborns. Nearly 100% of babies with this condition will receive a diagnosis by the time they are six months old. Adults with mild CLE are occasionally diagnosed later in life.

People assigned male at birth are more likely to have CLE than people assigned female at birth.

In some cases, a faulty gene may be the cause of CLE. Only one parent needs the gene to be passed down. This is a dominant gene.

People with the gene can pass it on to their offspring at 50%.

Even though this condition is more common in boys than girls, the mutated gene at cause is autosomal. Autosomal genes are located on a nonsex chromosome. This means that the gene mutation can be passed onto a child of any gender.

A baby born with this condition may have a gene abnormality that causes it.

Genetics aren’t always the cause of CLE. An underlying cause can’t be determined in around half of all babies born with this condition.

CLE symptoms in infants can be severe. In some instances, CLE symptoms may be mistaken for pneumonia.

Symptoms of the disease include:

  • breathing problems
  • Rapid breathing.
  • trouble feeding
  • wheezing
  • The skin is covered with a substance.
  • Respiratory retractions are when muscles are pulled between the ribs.
  • Overinflation of at least one lung lobe causes an enlarged chest.
  • The lung tissue is compressed.

In adults, CLE is not diagnosed. Adults with this congenital condition may have symptoms such as coughing and recurrent pneumonia.

In adults, acquired emphysema takes years to develop and worsens. Smoking cigarettes is the main cause of emphysema.

There are symptoms of emphysema.

  • wheezing
  • The breath was very thin.
  • The chest is tight.
  • coughing with mucus

Fetal scans may show CLE in your baby. Preparedness and symptom control can be improved by a pre-Natal diagnosis.

A third of babies with CLE are diagnosed after birth.

Adults with this condition often find out they have it when they are testing.

If your baby presents with symptoms within the first few months of life, you should get medical support immediately.

Your baby may have some or all of these tests done to confirm a diagnosis.

An infant pulmonary function test (lung function test) may also be done. This test measures breathing and the amount of air in the lungs. Sedation is required for this test.

The type of treatment your baby needs will be determined by symptom severity. If your baby has mild or moderate CLE, conservative treatments may be enough to reduce respiratory symptoms.

When the CLE is severe, the entire lung is needed for surgery. This can sound frightening. Lung surgery is beneficial for eliminating or reducing respiratory symptoms.

The lung of a child will grow rapidly. This results in a normal or near-normal lung size for many children who have lung surgery as babies.

Monitoring will be needed even if your child has a different type of treatment.

Diagnostic testing and treatments for CLE have improved over the years. Early diagnosis is crucial. This condition is potentially life threatening if it is not monitored and treated correctly. Excellent outcomes can be achieved by timely treatment of CLE. Babies with this condition can have a normal life span.

“Congenital lobar emphysema is a rare respiratory disorder that is sometimes genetic in origin. Babies can breathe in air but can’t exhale.”

Conservative treatments are available for CLE. The outlook for babies born with CLE is excellent if diagnosis and treatment are provided in a timely manner.