Chylothorax in lymphangioleiomyomatosis.
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.


METHODS
Identification and retrospective review of available medical records on patients with LAM and chylothorax.


METHODS
Tertiary-referral medical center.


METHODS
All patients with LAM seen at Mayo Clinic, Rochester, MN, from January 1, 1976, to December 31, 2000.


METHODS
None.


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.
Ryu
Jay H
JH
Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN 55905, USA.
Doerr
Clint H
CH
Fisher
Susan D
SD
Olson
Eric J
EJ
Sahn
Steven A
SA
D000328:Adult; D002916:Chylothorax / Q000175:diagnosis* / Q000601:surgery; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008171:Lung Diseases / Q000175:diagnosis* / Q000601:surgery; D018192:Lymphangioleiomyomatosis / Q000175:diagnosis* / Q000601:surgery; D008875:Middle Aged; D010994:Pleura / Q000601:surgery; D012189:Retrospective Studies; D013897:Thoracic Duct / Q000601:surgery; D013907:Thoracostomy; D013908:Thoracotomy; D014057:Tomography, X-Ray Computed