Lymphangiomyomatosis--newer concepts in pathogenesis and management--case reports.

Lymphangiomyomatosis was first reported more than forty years ago. Although its incidence is rare, its occurrence is being increasingly recognized and has been the subject of a growing number of cases reports in recent years. This study adds 2 more cases to the file. Both cases involved young women with the characteristic symptoms 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, pulmonary function was affected in a similar way by the presence of obstructive and restrictive defects and a decrease in diffusing capacity. Underlying abnormalities were dilated lymphatics, thickened lymphatic walls, and muscular proliferation 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. Pneumothorax, 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.

Immature angiomas: the importance of the Doppler exam in diagnosis and prognosis.

Immature angiomas are vascular hamartomatous malformations but clearly different from all other vascular, capillary, venous, arteriovenous, and lymphatic malformations, which, normally present at birth and established from a cellular viewpoint, grow because of the effect of hemodynamic stimuli and present no tendency toward spontaneous regression. The difference between immature angiomas and other hemangiomas has been demonstrated by investigations into cell dynamics. To facilitate a differentiated diagnosis and prognosis, the hemodynamics of the angiomas were studied with the Doppler flowmeter. The use of noninvasive techniques, such as those introduced by the Doppler flowmeter, permit a technical alternative and/or integration, giving results of equal relevance and importance, as well as the advantages of a more accessible and less expensive methodology.