Congenital Lobar Emphysema

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Congenital Lobar Emphysema
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Causes

• Autosomal Dominant Iheritance
• Sporadic
• Airway obstructed at birth

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Autosomal Dominant Inheritance
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First Signs and Symptoms

• CLE can be detected as early as utero
• CLE can most often be detected in newborns by MRI
  or CT scan

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Physical Symptoms

• Wheezing/Shortness of breath
• Chest pain
• Bluish tint to lips and fingernails
• Frequent coughing and colds
• Decreased breathing sounds by stethoscope
• Often misdiagnosed as Asthma

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CT scan of normal lungs vs CLE lungs
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Frequency

• Right lung has 3 lobes and the left lung has 2
  lobes
• CLE occurs most often in the left upper lobe (41
  of cases), second most frequently in the right
  middle lobe (34) and then 21 in the right upper.

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Adults vs Children

• ½ cases of CLE occur in the first 4 weeks of life
• ¾ of cases occur in infants less than 6 months

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Treatment

• Lung volume reduction
• Lobectomy
• Bullectomy
• Transplant
• Homeopathic

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Lung Transplant
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