Imaging description

Lymphangioleiomyomatosis (LAM) is characterized on CT imaging by diffuse bilateral thin-walled cysts without a zonal predominance (Figures 11.1 and 11.2) [1–3]. LAM can affect just the lungs, but it can also be a systemic disease. Findings of pulmonary LAM are also seen associated with tuberous sclerosis. When LAM is systemic or associated with tuberous sclerosis additional findings can be present, the most common of which are angiomyolipomas of the kidneys (Figure 11.1) [2]. Adenopathy and dilated cystic masses can be seen in the abdomen and pelvis from obstructed lymphatics (lymphangioleiomyomas) [2]. Lymphatic obstruction can also result in chylous pleural effusions (Figure 11.3). Spontaneous pneumothorax occurs in 20%.

Importance

LAM is a progressive lung disease with a poor prognosis that typically affects women of childbearing age. In the appropriate clinical setting the CT findings are diagnostic.