Pulmonary lymphangioleiomyomatosis: CT and pathologic findings

We present two cases that illustrate the appearance of pulmonary lymphangioleiomyomatosis on high resolution CT. Both patients had numerous thin-walled cystic airspaces of varying sizes distributed diffusely throughout the lungs. Most of the lung parenchyma surrounding the cystic spaces was normal. This pattern correlated closely with the surgical and pathologic findings and is distinct from other diffuse lung diseases.