title: Lung transplantation for lymphangioleiomyomatosis: single-center Brazilian experience with no chylothorax.
abstract: BACKGROUND
  Lymphangioleiomyomatosis (LAM), a rare cystic disease characterized by proliferation of smooth muscle cells in the lung interstitium, almost exclusively affects females in their reproductive years. Lung transplantation has been established as effective therapy for end-stage pulmonary LAM.
  
  
  METHODS
  This retrospective study includes lung transplantation patients with LAM at a single institution between 1989 and 2009.
  
  
  RESULTS
  During the study period we performed 300 lung transplantations, and in 10 cases the recipients had LAM. All patients were females with a mean age of 43.8 years. The mean time from the diagnosis to lung transplantation was 5 years. Seven patients had experienced previous pneumothoraces, five of whom were treated with pleurodesis. In all patients we performed a single-lung transplantation (left-sided = 9 and right-sided = 1). In three cases, the pleurodesis was on the same side as the transplantation, with great intraoperative bleeding in one subject (left pleurectomy). There was one early death due to infective endocarditis at posttransplant day 19. The median length of mechanical ventilation was 13 hours, while the mean hospital stay was 16.75 days. There was no case of chylothorax. Late complications included one case of native lung pneumothorax, one diaphragmatic hernia, one posttransplant lymphoproliferative disease, one respiratory sepsis, and one mycobacterial infection. The 1- and 3-year survival rates were 90% and 80%, respectively.
  
  
  CONCLUSIONS
  Lung transplantation is a feasible therapeutic option for patients with LAM, despite previous ipsilateral pleurodesis. The left-sided predilection for our procedures may have been responsible for the absence of chylothorax in this series.
authors.affiliation: Lung Transplantation Group, Santa Casa de Porto Alegre, Porto Alegre, Brazil. machuca36@hotmail.com
authors.forename: T N
authors.initials: TN
authors.lastname: Machuca
authors.forename: M J
authors.initials: MJ
authors.lastname: Losso
authors.forename: S M
authors.initials: SM
authors.lastname: Camargo
authors.forename: S M
authors.initials: SM
authors.lastname: Schio
authors.forename: I A
authors.initials: IA
authors.lastname: Melo
authors.forename: B
authors.initials: B
authors.lastname: Hochhegger
authors.forename: J C
authors.initials: JC
authors.lastname: Felicetti
authors.forename: J J
authors.initials: JJ
authors.lastname: Camargo
mesh_terms: D000328:Adult; D001938:Brazil; D005260:Female; D006801:Humans; D016040:Lung Transplantation; D018192:Lymphangioleiomyomatosis / Q000601:surgery*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies