A patient with pulmonary lymphangioleiomyomatosis may present with recurrent spontaneous pneumothorax. The pulmonary disease may be primary ot spread from an extrapulmonary focus.
Patient selection: recurrent spontaneous pneumothorax
When to suspect the possibility of pulmonary lymphangioleiomyomatosis:
(1) evidence of tuberous sclerosis complex
(2) premenopausal woman
(3) chylous ascites, chyluria, chyloptysis, and/or chylous pleural effusion
(4) hemoptysis
(5) dyspnea with chronic cough
(6) history of "benign" smooth muscle or spindle cell proliferation (cytology or biopsy)
(7) multiple lung nodules on imaging studies
(8) multiple lung cysts or honeycombing on imaging studies
(9) exclusion of other causes of pneumothorax including endometriosis
Continue Reading
