What is Congenital Lobar Emphysema?
Congenital lobar emphysema is a condition that is diagnosed in children and in milder form in adults.
Congenital lobar emphysema is different from emphysema which is associated with smoking. Both the condition affect the lungs but are distinct from one another.
Congenital lobar emphysema causes a defect in the bronchial cartilage that results in airway obstruction or collapse. It reduces the bronchial cartilage in the lungs which is the main airway playing a role in carrying air in and out of the lungs.
In this condition, the alveoli are abnormally small. Alveoli are the air sacs that are present at the end of the bronchioles. The blood and air exchange takes place in the alveoli.
In children with congenital lobar emphysema, the air can go into the lungs but cannot be breathed out. This causes hyperinflation of the lungs.
Congenital lobar emphysema is of 2 types:
- Hypoalveolar: In this, there are fewer alveoli in the lungs than normally present
- Polyalveolar: In this, there are more alveoli in the lungs than normally present
About 50% of infants are diagnosed with congenital lobar emphysema as newborns and 100% by the age of 6 months(1).
Causes of Congenital Lobar Emphysema
Congenital lobar emphysema is known to occur more in males than females. It occurs due to the mutated gene inherited from one of the parents.
It can also occur due to a gene mutation without being inherited, in children born with this condition.
However, genetics is not always the cause. The cause of the condition remains undetected in half of the babies born with congenital lobar emphysema(1).
Symptoms of Congenital Lobar Emphysema
Sometimes the symptoms of congenital lobar emphysema can be severe and are mistaken for pneumonia.
The symptoms of congenital emphysema include:
- Difficulty in breathing
- Wheezing
- Trouble in feeding
- Compressed lung tissue
- Cyanosis
- Respiratory retractions
- Enlarged chest
- Rapid breathing
Congenital lobar emphysema is rarely diagnosed in adults and they may have symptoms of coughing and recurrent pneumonia.
Diagnosis of Congenital Lobar Emphysema
During pregnancy, congenital lobar emphysema can be detected with the prenatal imaging test. This can be helpful for preparing to control symptoms at the time of birth. Most babies get diagnosed with congenital lobar emphysema if present immediately after birth.
Adults are diagnosed when they are doing tests for some other condition.
A child diagnosed with congenital lobar emphysema should immediately get medical help. There are a few imaging that can be helpful in confirming the diagnosis of congenital emphysema. These include:
- X-ray of the chest
- Magnetic resonance imaging
- CT scan imaging
A pulmonary function test may also be done on an infant. It measures breathing and the amount of air present in the lungs.
Treatment of Congenital Lobar Emphysema
The treatment needs of the baby depend on the severity of congenital lobar emphysema. Babies with mild symptoms of congenital lobar emphysema get relief from conservative treatments. In case of severe congenital lobar emphysema removal of a part of the lung or entire lung might be needed. This enables the lungs to thereby function normally and reduces respiratory symptoms.
As the child grows the lung size also increases, which results in the near-normal size of the lungs.
Whatever treatment is given to the child, monitoring of symptoms is very important.
Outlook for Children with Congenital Lobar Emphysema
Early diagnosis and treatment of congenital lobar emphysema are very important. It is a life-threatening condition if not monitored and treated properly.
Treatment on time has an excellent outcome for children.
Congenital lobar emphysema is a rare respiratory disorder that occurs in children. It allows children to breathe in the air but not breathe it out. There are treatments for this condition that range from conservative to surgical. Whatever treatment is given, monitoring a child plays a very important role.
