We report a case of pulmonary lymphangiomyomatosis in a young female. CT--and especially high resolution CT--is more specific and sensitive than chest radiography in showing the lung abnormalities. Pathognomonic CT findings are the presence of thin walled cystic air spaces spread throughout the lung parenchyma without nodules and without regional predominance and mostly surrounded by normal parenchyma. The superposition of the multiple thin walls of the cysts is responsible for the reticular interstitial pattern observed at chest radiography.
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