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: 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.
|
Authors | Jay H Ryu, Clint H Doerr, Susan D Fisher, Eric J Olson, Steven A Sahn |
Journal | Chest
(Chest)
Vol. 123
Issue 2
Pg. 623-7
(Feb 2003)
ISSN: 0012-3692 [Print] United States |
PMID | 12576391
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
|
Topics |
- Adult
- Chylothorax
(diagnosis, surgery)
- Female
- Follow-Up Studies
- Humans
- Lung Diseases
(diagnosis, surgery)
- Lymphangioleiomyomatosis
(diagnosis, surgery)
- Middle Aged
- Pleura
(surgery)
- Retrospective Studies
- Thoracic Duct
(surgery)
- Thoracostomy
- Thoracotomy
- Tomography, X-Ray Computed
|