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Lymphangiomyomatosis Newer Concepts in Pathogenesis and Management—Case Reports

Medical Research Laboratory, London, England

Edward M. Cordasco

Departments of Nuclear Medicine and Pulmonary Medicine. The Cleveland Clinic Foundation, Cleveland, Ohio

G. Meden

Departments of Nuclear Medicine and Pulmonary Medicine. The Cleveland Clinic Foundation, Cleveland, Ohio

James K. O'Donnell

Departments of Nuclear Medicine and Pulmonary Medicine. The Cleveland Clinic Foundation, Cleveland, Ohio

William J. MacIntyre

Departments of Nuclear Medicine and Pulmonary Medicine. The Cleveland Clinic Foundation, Cleveland, Ohio

W. Dishner

Departments of Nuclear Medicine and Pulmonary Medicine. The Cleveland Clinic Foundation, Cleveland, Ohio

Lymphangiomyomatosis was first reported more than forty years ago. Al though its incidence is rare, its occurrence is being increasingly recognized and has been the subject of a growing number of case reports in recent years. This study adds 2 more cases to the file. Both cases involved young women with the characteristic symtoms of dyspnea, cough, abdominal discomfort and swelling, chest pain, and hemoptysis, with abundant formation of refractory chylous, serous ascites. Although the progression of the disease differed in each case, pulomonary function was affected in a similar way by the presence of obstructive and restrictive defects and a decrease in diffusing capacity. Underlying abnor malities were dilated lymphatics, thickened lymphatic walls, and muscular pro liferation of leiomyomatous origin, leading to bronchial restriction. The authors point to the subtlety required in arriving at a differentiated diagnosis of lymphangiomyomatosis lymphangioleiomyomatosis and the difference between the two conditions. They also make particular recommendations in respect to the importance of preliminary hormone receptor tests and to the wisdom to be exercised in ligating a main lymphatic duct in the chest to control the formation of ascites. Pheumothorax, a frequent manifestation of lymphangiomyomatosis, is found to be the result of chronic air trapping due to a combination of narrowing of conducting airways and disruption of normal lung parenchyma.

Angiology, Vol. 41, No. 11, 957-963 (1990)
DOI: 10.1177/000331979004101110


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