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What is Congenital Lobar Emphysema?

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  • Written By: Tricia Ellis-Christensen
  • Edited By: O. Wallace
  • Last Modified Date: 25 November 2016
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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.

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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.

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.

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.

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