title: Lung transplantation for lymphangioleiomyomatosis: the French experience.
abstract: BACKGROUND
  Lymphangioleiomyomatosis (LAM) is a rare disease, leading in some cases to end-stage respiratory failure. Lung transplantation (LT) represents a therapeutic option in advanced pulmonary LAM.
  
  
  METHODS
  We conducted a retrospective multicenter study of 44 patients who underwent LT for LAM at 9 centers in France between 1988 and 2006.
  
  
  RESULTS
  All patients were women with a mean age of 41+/-10 years at LT. There were 34 single-lung transplants and 11 bilateral transplants (one retransplantation). Prior clinical events related to LAM were present in 75% of the patients and previous thoracic surgical procedures were noted in 86.6% of cases. At the latest preoperative evaluation, 30 patients had an obstructive pattern (mean forced expiratory volume in 1 second: 26%+/-14% of predicted) and 15 had a combined restrictive and obstructive pattern, with a mean KCO=27%+/-8.8% of predicted, PaO2=52.8+/-10.4 and PaCO2=42.6+/-9.8 mm Hg. Intraoperative cardiopulmonary bypass was required in 13 cases. The length of mechanical ventilation was 7.5+/-12.8 days. The median duration of follow-up was 37 months. The 1, 2, 5, and 10 years survival rates were 79.6%, 74.4%, 64.7%, and 52.4%, respectively. Extensive pleural adhesions were found in 21 patients leading to severe intraoperative hemorrhage. Postoperative LAM-related complications were pneumothorax in the native lung in five patients, chylothorax in six, bronchial dehiscence or stenosis in seven. There were two cases of recurrence of LAM.
  
  
  CONCLUSIONS
  Despite a high morbidity mainly caused by previous surgical interventions and disease-related complications, LT is a satisfactory therapeutic option for end-stage respiratory failure in LAM.
authors: The Divisions of Pulmonary Medicine and Thoracic Surgery, Hôpital Ste Marguerite, Marseille, France. martine.reynaud@ap-hm.fr
authors: Martine
authors: M
authors: Reynaud-Gaubert
authors: Jean-François
authors: JF
authors: Mornex
authors: Hervé
authors: H
authors: Mal
authors: Michèle
authors: M
authors: Treilhaud
authors: Claire
authors: C
authors: Dromer
authors: Sébastien
authors: S
authors: Quétant
authors: François
authors: F
authors: Leroy-Ladurie
authors: Romain
authors: R
authors: Guillemain
authors: François
authors: F
authors: Philit
authors: Gaëlle
authors: G
authors: Dauriat
authors: Dominique
authors: D
authors: Grenet
authors: Marc
authors: M
authors: Stern
mesh_terms: D000328:Adult; D001706:Biopsy; D005260:Female; D005602:France; D007839:Functional Laterality; D006650:Histocompatibility Testing; D006801:Humans; D007431:Intraoperative Complications / Q000453:epidemiology; D008175:Lung Neoplasms / Q000601:surgery*; D016040:Lung Transplantation / Q000706:statistics & numerical data*; D018192:Lymphangioleiomyomatosis / Q000503:physiopathology / Q000601:surgery*; D008875:Middle Aged; D010101:Oxygen Consumption; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D012907:Smoking / Q000453:epidemiology; D011795:Surveys and Questionnaires; D017211:Treatment Failure; D016896:Treatment Outcome