Lymphangiomatose − seltene Ursache eines Pleuraergusses

A 33-year-old man presented to us with slowly progressive dyspnea. At the physical examination we found pleural effusion on the right side, lymph edema of the left lower limb and foot and blotchy map-like skin changes. The pleural puncture revealed a chylous effusion. In whole body MRI we saw the pleural effusion on the right, an increase in lymph vessels in the pelvis and paraaortic, and prominent chylous vessels. There was no bone involvement. The abdominal thoracic duct was enlarged by up to 3 mm. We diagnosed a general lymphatic anomaly (GLA) - lymphangiomatosis. GLA is a rare disease of unknown origin with dysplasia of the lymphatic system. The thoracic manifestation is often the development of chylous pleural effusions. However, many other organs can also be involved. Therapy includes in most cases symptomatic relief and the attempt to slow the progression of the disease.