Idiopathic giant bullous emphysema

Idiopathic giant bullous emphysema (vanishing lung syndrome, type 1 bullous disease, or primary bullous disease of the lung) is a progressive condition characterized by extensive paraseptal emphysema coalescing into giant bullae. It usually occurs in young men, most of whom are smokers, but can also occur in nonsmokers.

Radiographs will reveal giant bullae in one or both upper lobes that occupy at least one third of the hemithorax and compress surrounding normal lung parenchyma. On HRCT, multiple large bullae may be seen without a single dominant giant bulla. There may also be subtle parenchymal paraseptal and centrilobular emphysema in the apparently normal lung.

References

Stern EJ, Webb WR, Weinacker A, Müller NL. Idiopathic giant bullous emphysema (vanishing lung syndrome): imaging findings in nine patients. AJR Am J Roentgenol. 1994 Feb;162(2):279-82.