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Vanishing Lung Syndrome
Idiopathic Giant Bullous Emphysema

     

     

    • 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
    • Usually seen in association with paraseptal and centrilobular emphysema
    • Most common in young men, mostly in smokers
    • Bullous disease asymmetrically involved the upper lobes predominantly
    • Bullae compress adjacent lung and can predispose to pneumothorax
    • On high-resolution CT, bullae range from 1 to 20 cm in diameter, mostly in the range of  2-8 cm in diameter
    • Paraseptal emphysema and subpleural bullae are seen in virtually all patients
    • Most also have separate centrilobular emphysema
    • Treatment
      • Volume reduction surgery
      • The extent of the centrilobular emphysema, seen in cigarette smokers, is the important variable finding for determining the preoperative assessment of giant bullous lung disease


    Vanishing Lung Syndrome. Left hemithorax is hyperlucent compared to right because of the presence
    of giant bullae occupying the left hemithorax and rendering whatever normal lung remains nearly invisible.
    This is an example of Vanishing Lung Syndrome.



American Journal of Roentgenology, Vol 162, 279-282, Copyright © 1994 by American Roentgen Ray Society Idiopathic giant bullous emphysema (vanishing lung syndrome): imaging findings in nine patients EJ Stern, WR Webb, A Weinacker and NL Muller