Chylothorax in lymphangioleiomyomatosis

Jay H Ryu, Clint H. Doerr, Susan D. Fisher, Eric J. Olson, Steven A. Sahn

Research output: Contribution to journalArticle

95 Citations (Scopus)

Abstract

Study objective: Pulmonary lymphangioleiomyomatosis (LAM) is a rare, serious disorder characterized by proliferation of abnormal smooth-muscle cells and affects almost exclusively women of childbearing age. Optimal management of chylothorax, a well-recognized complication of LAM, in these patients has not been defined. This study was performed to characterize the clinical course and identify appropriate management options for chylothorax occurring in patients with LAM. Design: Identification and retrospective review of available medical records on patients with LAM and chylothorax. Setting: Tertiary-referral medical center. Patients: All patients with LAM seen at Mayo Clinic, Rochester, MN, from January 1, 1976, to December 31, 2000. Intervention: None. Measurement and results: Eight of 79 patients (10.1%) with LAM had chylothorax. All were women aged 33 to 51 years, and four patients had underlying tuberous sclerosis complex. These eight women represented 3.5% of the 229 patients with chylothorax seen over this 25-year period at Mayo Clinic Rochester. Six patients had unilateral pleural effusion and two patients had bilateral effusions at initial presentation. The size of the chylothorax varied and was not necessarily progressive. Management of chylothorax ranged from thoracentesis only to thoracotomy with thoracic duct ligation and parietal pleurectomy. When needed, pleurodesis by instillation of sclerosing agents or parietal pleurectomy appeared to be effective in controlling chylothorax. Conclusions: Chylothorax occurring in patients with LAM has a variable clinical course. Although pleurodesis with or without thoracic duct ligation appears to be effective in controlling intractable chylothorax, less invasive treatments such as thoracentesis or observation may suffice in some cases. Management of chylothorax in patients with LAM should be individualized depending on the size and clinical effects of the chylous pleural effusion, as well as comorbid factors and local expertise.

Original languageEnglish (US)
Pages (from-to)623-627
Number of pages5
JournalChest
Volume123
Issue number2
DOIs
StatePublished - Feb 1 2003

Fingerprint

Lymphangioleiomyomatosis
Chylothorax
Pleurodesis
Thoracic Duct
Pleural Effusion
Ligation
Sclerosing Solutions
Tuberous Sclerosis
Thoracotomy
Tertiary Care Centers
Smooth Muscle Myocytes
Medical Records

Keywords

  • Chylothorax
  • Lymphangioleiomyomatosis
  • Pleural effusion
  • Tuberous sclerosis complex

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

Cite this

Ryu, J. H., Doerr, C. H., Fisher, S. D., Olson, E. J., & Sahn, S. A. (2003). Chylothorax in lymphangioleiomyomatosis. Chest, 123(2), 623-627. https://doi.org/10.1378/chest.123.2.623

Chylothorax in lymphangioleiomyomatosis. / Ryu, Jay H; Doerr, Clint H.; Fisher, Susan D.; Olson, Eric J.; Sahn, Steven A.

In: Chest, Vol. 123, No. 2, 01.02.2003, p. 623-627.

Research output: Contribution to journalArticle

Ryu, JH, Doerr, CH, Fisher, SD, Olson, EJ & Sahn, SA 2003, 'Chylothorax in lymphangioleiomyomatosis', Chest, vol. 123, no. 2, pp. 623-627. https://doi.org/10.1378/chest.123.2.623
Ryu JH, Doerr CH, Fisher SD, Olson EJ, Sahn SA. Chylothorax in lymphangioleiomyomatosis. Chest. 2003 Feb 1;123(2):623-627. https://doi.org/10.1378/chest.123.2.623
Ryu, Jay H ; Doerr, Clint H. ; Fisher, Susan D. ; Olson, Eric J. ; Sahn, Steven A. / Chylothorax in lymphangioleiomyomatosis. In: Chest. 2003 ; Vol. 123, No. 2. pp. 623-627.
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