Chest
Selected ReportsChylothorax in Lymphangioleiomyomatosis*
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)
Lymphangioleiomyomatosis: a review
Chest
(1998)- et al.
High frequency of pulmonary lymphangioleiomyomatosis in adult women with tuberous sclerosis complex
Mayo Clin Proc
(2000) - et al.
Comprehensive evaluation of 35 patients with lymphangioleiomyomatosis
Chest
(1999) - et al.
Pulmonary lymphangioleiomyomatosis: quantitative analysis of lesions producing airflow limitation
Hum Pathol
(1985) - et al.
Pulmonary lymphangioleiomyomatosis associated with tuberous sclerosis: treatment with tamoxifen and tetracycline-pleurodesis
Chest
(1985) - et al.
Diffuse lung disease due to lymphangiomyoma
Am J Med
(1969) Lymphangioleiomyomatosis: clinical features, management and basic mechanisms
Thorax
(1999)- et al.
Mutational and radiographic analysis of pulmonary disease consistent with lymphangioleiomyomatosis and micronodular pneumocyte hyperplasia in women with tuberous sclerosis
Am J Respir Crit Care Med
(2001) - et al.
Prevalence and clinical characteristics of lymphangioleiomyomatosis (LAM) in patients with tuberous sclerosis complex
Am J Respir Crit Care Med
(2001) - et al.
Pulmonary lymphangioleiomyomatosis and micronodular pneumocyte hyperplasia in a male patient with tuberous sclerosis complex
Am J Respir Crit Care Med
(2000)
Lymphangioleiomyomatosis: clinical course in 32 patients
N Engl J Med
Pulmonary lymphangioleiomyomatosis: a report of 46 patients including a clinicopathologic study of prognostic factors
Am J Respir Crit Care Med
Pulmonary lymphangioleiomyomatosis: a study of 69 patients
Medicine
Clinical experience of lymphangioleiomyomatosis in the UK
Thorax
Pulmonary lymphangiomyomatosis: a review
Am J Pathol
Chylothorax
Semin Respir Crit Care Med
Lymphangiomyomatosis: a clinical-roentgenologic-pathologic syndrome
Am J Roentgenol Radium Ther Nucl Med
Cited by (117)
Lymphangioleiomyomatosis
2023, Presse MedicaleFrench recommendations for the diagnosis and management of lymphangioleiomyomatosis
2023, Respiratory Medicine and ResearchChylothorax and Pseudochylothorax
2021, Clinics in Chest MedicineCitation Excerpt :Not all patients with chylothorax in LAM require therapeutic intervention. Management is tailored to the size and clinical effects of the chylous pleural effusion and has included low-fat diet, therapeutic thoracentesis, pleurodesis, pleurectomy, and thoracic duct ligation.38,41,42 More recently, the discovery of abnormalities in the TSC1/2 genes, which results in activation of the kinase mammalian target of rapamycin (mTOR), has led to trials using mTOR inhibitors like sirolimus.40
Lymphangioleiomyomatosis
2021, Encyclopedia of Respiratory Medicine, Second EditionRole of thoracic imaging in the management of lymphangioleiomyomatosis
2019, Respiratory MedicineCitation Excerpt :Pneumothorax occurs in ~40–70% [2,17,21,22,32], with a recurrence rate >70% [33,34] - (Fig. 6). Unilateral or bilateral chylothorax can occur in about one third of patients [2,35]. Treatments of chylo- and pneumothorax include pleurodesis, whose effectiveness depends upon the pleuritis caused by chemical irritation or mechanical abrasion [5,34].
Clinical approach and review of causes of a chylothorax
2019, Respiratory Medicine
Funding was provided by Mayo Institutional funds and Robert N. Brewer Family Foundation.