Pulmonary lymphangioleiomyomatosis in a 29-year-old woman with bilateral spontaneous pneumothorax: case report.

T. C. Wu*, Y. F. Lai, T. Y. Chao, M. J. Hsieh, W. J. Chen

*Corresponding author for this work

Research output: Contribution to journalJournal Article peer-review


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. Pneumothorax is almost always found on the initial chest radiograph. We report a case of a 29-year-old woman who presented with abrupt right chest pain and progressive dyspnea. Physical examination in the emergency department was significantly diminished breath sounds over the right lung, and a chest X-ray revealed a right-sided pneumothorax. She required a chest tube for complete lung re-expansion. On the sixth day of admission, left-sided pneumothorax occurred and another chest tube was inserted. 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.

Original languageEnglish
Pages (from-to)164-168
Number of pages5
JournalChang Gung Medical Journal
Issue number3
StatePublished - 03 2000
Externally publishedYes


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