• Stefan Krüger, Claudia Varwerk, Bernward Passlick, Alicia Morresi-Hauf, Olaf Thetter, and Karl Häussinger.
• Zentrum für Pneumologie und Thoraxchirurgie, Asklepios-Fachkliniken, München-Gauting, Germany. khaeussinger@asklepios.com
• Med Klin. 2004 Jan 15; 99 (1): 43-6.

BackgroundPulmonary lymphangioleiomyomatosis is a rare disease that occurs mainly in women of reproductive age. The clinical characteristics include recurrent spontaneous pneumothorax and progressive dyspnea. The features of chest computed tomography are nearly pathognomonic with the detection of bilateral thin-walled cysts.Case ReportA 32-year-old female presented with sudden-onset right-sided chest pain, which aggravated during inspiration, dyspnea at exertion, and cough. She had a history of bilateral recurrent spontaneous pneumothorax. Physical examination showed reduced pulmonary sounds on the right side. Chest X-ray confirmed the diagnosis of recurrent right-sided pneumothorax. Computed tomography showed multiple bilateral bullae. Video-assisted pleurectomy, bulla resection, and bulla coagulation were performed. The diagnosis of pulmonary lymphangioleiomyomatosis was confirmed by pulmonary biopsy. After pleurectomy, the patient remained symptom-free without recurrent pneumothorax.ConclusionPulmonary lymphangioleiomyomatosis is a rare cause of recurrent pneumothorax and should be considered a differential diagnosis, especially in young women with diffuse bilateral bullous emphysema or tuberous sclerosis.

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