Lymphangiomyomatosis: Radiological and measured lung function deterioration after contrast‐enhanced computed tomography

Abstract

Pulmonary lymphangiomyomatosis (LAM) is a rare disease of unknown aetiology which occurs exclusively in women, usually of reproductive age. The findings on CT and high-resolution CT (HRCT) are well described and characteristic, and in a young woman they are virtually pathognomonic. A case of symptomatic, radiological and measured lung function deterioration following contrast-enhanced CT in a patient with LAM are reported here. These observations, to the authors' knowledge, have never been reported before. The authors attempt to explain these observations based on the known pathology of LAM.

Lymphangioleiomyomatosis with a Giant Bulla: Report of a Case

“…The problem of increased extracellular fluid can be compounded by impaired lymphatic drainage. 7 Our patient's hypervolemia during and after surgery probably caused her pulmonary edema; therefore, the likelihood of hypervolemia and pulmonary edema should be borne in mind perioperatively in such patients.…”

“…Teo et al 7 reported that the proliferation of smooth muscle around the veins causes venular congestion in LAM, and the resulting hypervolemia may lead to greater leakage of fluid into the lung parenchyma. The problem of increased extracellular fluid can be compounded by impaired lymphatic drainage.…”