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.
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