Presentation
Shortness of breath.
Patient Data
Hyperinflated lungs with mildly increased lung markings. No consolidation, pneumothorax or pleural effusion. Cardiac silhoutte normal in size.
Innumerable scattered bilateral thin walled cysts with relative sparing of the apices. No pneumothorax, pleural effusion or lymphadenopathy.
Case Discussion
The differential diagnosis of cystic lung disease includes lymphangioleiomyomatosis, emphysema, lymphocytic interstitial pneumonitis (LIP), pulmonary Langerhans cell histiocytosis, and Birt-Hogg-Dubé syndrome. Cyst morphology, number and distribution are helpful in differentiating among these possibilities: thin-walled rounded cysts are scattered throughout the lungs in lymphangioleiomyomatosis with relative apical sparing, while in the most common type of emphysema and pulmonary Langerhans cell histiocytosis, cysts or lucencies have an upper lobe predominance. In LIP, cysts are mainly in the lower lungs while in Birt-Hogg-Dubé syndrome, cysts are subpleural. This case shows the typical scattered cyst distribution in lymphangioleiomyomatosis.
Unable to process the form. Check for errors and try again.