Learning Radiology xray montage

Vanishing Lung Syndrome
Idiopathic Giant Bullous Emphysema

General Considerations

  • Uncommon disorder
  • Characterized by very large bullae
    • Bullae are air-filled, thin-walled (<1mm) spaces in the lung resulting from destruction of alveolar tissue
    • In vanishing lung syndrome the bulla takes up more than a third of the occupied lung
  • Paraseptal emphysema and subpleural bullae are seen in virtually all patients
    • Most also have separate centrilobular emphysema
  • Most common in young men, mostly in smokers

Clinical Findings

  • Same as for emphysema

Imaging Findings

  • Bullous disease asymmetrically involved the upper lobes predominantly
  • On high-resolution CT, bullae range from 1 to 20 cm in diameter, mostly in the range of  2-8 cm in diameter
  • Bullous disease asymmetrically involved the upper lobes predominantly

Differential Diagnosis

  • Mistaking a large bulla for a pneumothorax and inserting a chest tube into it will lead to a pneumothorax
  • Find the visceral pleural white line to identify a pneumothorax and, when in doubt, do CT


  • Volume reduction surgery
  • Extent of centrilobular emphysema is key variable for determining preoperative assessment of bullectomy


  • Bullae compress adjacent lung and can predispose to pneumothorax

vanishing lung syndrome

vanishing lung syndrome

Vanishing Lung Syndrome. The chest radiograph shows a giant bulla occupying the entire left hemithorax (white arrow). There is no visceral pleural line visible to indicate this is a pneumothorax.The CT scan of the lower chest shows the large bulla compressing the lung medially and posteriorly (white arrows)
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Idiopathic giant bullous emphysema (vanishing lung syndrome): imaging findings in nine patients. EJ Stern, WR Webb, A Weinacker and NL Muller. AJR, Vol 162, 279-282, 1994.