• T C Wu, Y F Lai, T Y Chao, M J Hsieh, and W J Chen.
• Division of Thoracic Medicine, Department of Internal Medicine, Chang Gung Memorial Hospital, Kaohsiung.
• Chang Gung Med J. 2000 Mar 1; 23 (3): 164-8.

AbstractPulmonary 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.

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