How is congenital lobar emphysema (CLE) differentiated from Swyer-James syndrome?

Updated: Apr 08, 2019
  • Author: Beverly P Wood, MD, MSEd, PhD; Chief Editor: John Karani, MBBS, FRCR  more...
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Answer

Answer

Congenital lobar emphysema (ie, congenital lesion) should be differentiated from Swyer-James syndrome (ie, acquired pulmonary abnormality secondary to infection).

In Swyer-James syndrome, infection results in the following:

  • Vascular compromise

  • "Pruning" of peripheral pulmonary vasculature

  • Small but hyperlucent lung (as the opposite, normal lung grows, the involved lung does not grow and appears more radiolucent)

Swyer-James, like CLE, appears as hyperlucency localized to one or several lobes of the lung with the entire affected lung having markedly reduced parenchyma and vascular density. The key distinguishing factors that differentiate Swyer-James from CLE is an acquired form of segmental emphysema that arises as a sequela of bronchopulmonary infection in childhood resulting in bronchiolitis obliterans and distal airspace destruction. [7]


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