Congenital lobar emphysema (lobäres Emphysem)

General information:

• Postnatal over-distension of one or more lobes of a histologically normal lung, as a result of a collapse of bronchi (a cartilaginous deficiency in the tracheobronchial tree; check valve obstruction with air trapping) or extrinsic pressure (anomalous pulmonary artery) on the airway or idiopathic causes.
  
• The normal lobes are compressed and the mediastinum is shifted away from the affected side.
  
• The upper lobes are more commonly involved (especially the left).
  
• Males more affected than females.
  
• In about 40% associated anomalies (heart, kidneys).

Symptoms:

• Respiratory failure with cyanosis.
  
• Sometimes dramatic presentation due to overdistension of the affected lobe (mimics a tension pneumothorax).

Diagnostic workout:

• Plain chest radiograph, CT scan
  
• Ventilatory perfusion lung scannings.
  

Indication for operation:

• Conservative therapy is useless in congenital forms. Clear indication for resection.

Treatment/Operation:

• Urgent lobe or segment resection. Placement of a chest tube into emphysematous lobe can have catastrophic results (air leak, bleeding).
  
• In rare cases, the cause of the bronchial obstruction can be operatively treated.

Postoperative management:

• Chest tubes may be removed if the lung is fully expanded and drainage volumes decrease below 20 to 50cc during a 24 hour period.
  

Prognosis:

• Good.