Lymphangioleiomyomatosis

Case contributed by Andrew Dixon
Diagnosis certain

Presentation

Initially presented with increasing shortness of breath over several months.

Patient Data

Age: 35 years
Gender: Female
x-ray

There is diffuse cystic lung disease. Bilateral pleural fluid collections which were chylous. A coil is seen in the thoracic duct from an attempt to reduce the recurrent lymphatic leak.

ct

There is diffuse cystic lung disease where the cysts are round, thin walled and there is minimal septal thickening.  Right pneumothorax and small bilateral chylothorax which are two complications of LAM.

Case Discussion

There is diffuse cystic lung disease where the cysts are round, thin walled and there is minimal septal thickening. The plain radiograph shows bilateral pleural fluid collections which were chylous. A coil is seen in the throcic duct from an attempt to reduce the recurrent lymphatic leak. These findings are consistent with lymphangioleimyomatosis (LAM). The CT also shows a right pneumothorax and small bilateral chylothorax two complications of LAM.

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