title: [Pulmonary lymphangioleiomyomatosis as a rare cause of recurrent spontaneous pneumothorax].
abstract: BACKGROUND
  Pulmonary 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.
  
  
  METHODS
  A 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.
  
  
  CONCLUSIONS
  Pulmonary 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.
authors.affiliation: Zentrum für Pneumologie und Thoraxchirurgie, Asklepios-Fachkliniken, München-Gauting, Germany. khaeussinger@asklepios.com
authors.forename: Stefan
authors.initials: S
authors.lastname: Krüger
authors.forename: Claudia
authors.initials: C
authors.lastname: Varwerk
authors.forename: Bernward
authors.initials: B
authors.lastname: Passlick
authors.forename: Alicia
authors.initials: A
authors.lastname: Morresi-Hauf
authors.forename: Olaf
authors.initials: O
authors.lastname: Thetter
authors.forename: Karl
authors.initials: K
authors.lastname: Häussinger
mesh_terms: D000328:Adult; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008168:Lung / Q000473:pathology; D008175:Lung Neoplasms / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D018192:Lymphangioleiomyomatosis / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D011013:Pneumonectomy; D011030:Pneumothorax / Q000209:etiology*