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

Treatment

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

Complications

• Bullae compress adjacent lung and can predispose to pneumothorax

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.