Chang Gung medical journal
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Case Reports
Pulmonary lymphangioleiomyomatosis in a 29-year-old woman with bilateral spontaneous pneumothorax: case report.
Pulmonary lymphangioleiomyomatosis is a rare, progressive and fatal interstitial lung disease that affects women of child-bearing age. It is characterized by the nonneoplastic proliferation of atypical smooth muscle cells around lymphatics, venules, and bronchioles. Patients with pulmonary lymphangioleiomyomatosis most commonly present with exertional dyspnea and a cough. ⋯ She underwent high-resolution computed tomography of the thorax with the findings of numerous cysts evenly distributed throughout the parenchyma of both lungs. Bilateral thoracotomy with lung biopsies and pleurodesis were performed and the surgical findings confirmed the presence of multiple, small, pink-colored cystic lesions on the surfaces of both lungs. Pathological examination showed the characteristic findings of pulmonary lymphangioleiomyomatosis.