Health
Fact-checked

At WiseGEEK, we're committed to delivering accurate, trustworthy information. Our expert-authored content is rigorously fact-checked and sourced from credible authorities. Discover how we uphold the highest standards in providing you with reliable knowledge.

Learn more...

What is Congenital Lobar Emphysema?

Tricia Christensen
Tricia Christensen
Tricia Christensen
Tricia Christensen

Congenital lobar emphysema (CLE) is a rare lung condition present at birth that can result in the lungs over-inflating when air becomes trapped in one of the lung’s lobes. Although most times, emphysema is thought of as an adult lifestyle disease frequently cause by smoking, CLE, also called infantile emphysema, is very different. It is congenital or present at birth, and in severe cases may be immediately apparent in the newborn or diagnosable prior to birth with extensive ultrasounds. The degree to which this disease will require treatment is variable because CLE can present in multiple levels of severity.

The lung’s lobes contain millions of air-filled sacs called alveoli. In congenital lobar emphysema, one of the diagnostic differences is that there are fewer or more than the normal number of sacs. This allows doctors to separate CLE into two types called hypoalveolar and polyalveolar, or "too few" and "too many" alveoli in the affected lobe. Infantile emphysema may also be further distinguished and complicated by the presence of other medical issues. In about 10-15% of cases, children also present with congenital heart defects, and the combination of respiratory problems and heart issues can be very serious.

CLE can result in the lungs over-inflating when air becomes trapped in one of the lung's lobes.
CLE can result in the lungs over-inflating when air becomes trapped in one of the lung's lobes.

In many instances, children with congenital lobar emphysema, if not diagnosed in utero, will show signs of respiratory distress in early infancy, and are usually identified quickly. Such distress could occur before newborns leave the hospital, or it might occur in the first few weeks of life. Though CLE is quite rare, affecting only about .005% of the population, the presence of an expanding lung and fluid in the lungs tends to be a good sign of it. It should be noted that the condition can have no symptoms, be unproblematic for years, and might not be caught until people reach adolescence or adulthood. In any case, trouble breathing is always indication to see a physician for diagnosis.

Children with congenital lobar emphysema typically show signs of respiratory distress shortly after birth.
Children with congenital lobar emphysema typically show signs of respiratory distress shortly after birth.

With the most severe cases of congenital lobar emphysema, pulmonary specialists may suggest removing the affected and expanded lung lobe, as this may immediately improve the situation. Such a surgery is not performed lightly since it may compromise breathing to some degree. When possible, moderate types of this condition are treated with medicines instead, and the condition could always be medically instead of surgically managed, or might respond well to medical management for a time before surgery is required.

Children born with congenital lobar emphysema may also present with congenital heart defects.
Children born with congenital lobar emphysema may also present with congenital heart defects.

Anyone with CLE is vulnerable particularly when respiratory infections occur. People with congenital lobar emphysema will also need to be followed by a pulmonologist, and length of time between doctor visits will depend on its severity. It is quite possible for some infants with this condition to do extremely well, while prognosis for more severe cases can vary based on complications.

Tricia Christensen
Tricia Christensen

Tricia has a Literature degree from Sonoma State University and has been a frequent WiseGEEK contributor for many years. She is especially passionate about reading and writing, although her other interests include medicine, art, film, history, politics, ethics, and religion. Tricia lives in Northern California and is currently working on her first novel.

Learn more...
Tricia Christensen
Tricia Christensen

Tricia has a Literature degree from Sonoma State University and has been a frequent WiseGEEK contributor for many years. She is especially passionate about reading and writing, although her other interests include medicine, art, film, history, politics, ethics, and religion. Tricia lives in Northern California and is currently working on her first novel.

Learn more...

Discuss this Article

Post your comments
Login:
Forgot password?
Register:
    • CLE can result in the lungs over-inflating when air becomes trapped in one of the lung's lobes.
      By: Alexandr Mitiuc
      CLE can result in the lungs over-inflating when air becomes trapped in one of the lung's lobes.
    • Children with congenital lobar emphysema typically show signs of respiratory distress shortly after birth.
      By: Patricia Marks
      Children with congenital lobar emphysema typically show signs of respiratory distress shortly after birth.
    • Children born with congenital lobar emphysema may also present with congenital heart defects.
      By: Adrin Shamsudin
      Children born with congenital lobar emphysema may also present with congenital heart defects.
    • Surgery may be necessary for serious cases of congenital lobar emphysema.
      By: astoria
      Surgery may be necessary for serious cases of congenital lobar emphysema.
    • The lung’s lobes contain millions of air-filled sacs called alveoli.
      By: snapgalleria
      The lung’s lobes contain millions of air-filled sacs called alveoli.
    • Neonatology focuses primarily on the medical needs of newborn babies.
      By: cantor pannatto
      Neonatology focuses primarily on the medical needs of newborn babies.