Chest
Volume 123, Issue 2, February 2003, Pages 623-627
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Selected Reports
Chylothorax in Lymphangioleiomyomatosis*

https://doi.org/10.1378/chest.123.2.623Get rights and content

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.

Section snippets

Materials and Methods

Patients with LAM seen at Mayo Clinic, Rochester, MN, from January 1, 1976, to December 31, 2000, were identified by a computer-assisted search of medical records. In addition, all patients with TSC were identified, and available medical records along with imaging studies were reviewed to identify additional patients with LAM.3 The diagnosis of LAM required the following: (1) diagnostic histopathologic findings on surgical or bronchoscopic lung biopsy, (2) characteristic high-resolution CT

Results

Seventy-nine patients with LAM were seen at Mayo Clinic Rochester over the 25-year period from January 1, 1976, to December 31, 2000; 25 of these patients had TSC. All were women > 18 years old at the time of the LAM diagnosis, except for one 17-year-old patient. The presence of a pleural effusion had been documented in eight of these patients (10.1%); all of these pleural effusions were chylous. Clinical characteristics and course of these patients are described in Table 1. These patients with

Discussion

Chylothorax and pneumothorax are two major complications of LAM. Pneumothorax is more common and is noted in 39 to 53% of patients at presentation and in 60 to 81% of patients during the course of disease.127891011 The occurrence of chylothorax has been described in patients with sporadic LAM, as well as in patients with TSC-associated LAM.121415 Pleural effusions occurring in patients with LAM are nearly always chylous.1291416 Previous reports have suggested that LAM-associated chylous

REFERENCES (35)

  • JR Taylor et al.

    Lymphangioleiomyomatosis: clinical course in 32 patients

    N Engl J Med

    (1990)
  • M Kitaichi et al.

    Pulmonary lymphangioleiomyomatosis: a report of 46 patients including a clinicopathologic study of prognostic factors

    Am J Respir Crit Care Med

    (1995)
  • T Urban et al.

    Pulmonary lymphangioleiomyomatosis: a study of 69 patients

    Medicine

    (1999)
  • SR Johnson et al.

    Clinical experience of lymphangioleiomyomatosis in the UK

    Thorax

    (2000)
  • B Corrin et al.

    Pulmonary lymphangiomyomatosis: a review

    Am J Pathol

    (1975)
  • C Doerr et al.

    Chylothorax

    Semin Respir Crit Care Med

    (2001)
  • WT Miller et al.

    Lymphangiomyomatosis: a clinical-roentgenologic-pathologic syndrome

    Am J Roentgenol Radium Ther Nucl Med

    (1971)
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    Funding was provided by Mayo Institutional funds and Robert N. Brewer Family Foundation.

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