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Lymphangioleiomyomatosis and tuberous sclerosis

Case contributed by Craig Hacking
Diagnosis certain

Presentation

Follow up of pneumothorax treated with pleural pigtail drain. Recurrent pneumothorax on a background of lymphangioleiomyomatosis and tuberous sclerosis.

Patient Data

Age: 35 years
Gender: Female

Left-sided pigtail drain noted. Diffuse bilateral reticular interstitial pattern and cystic lucencies are consistent with the known history of lymphangioleiomyomatosis. No residual pneumothorax detected. Blunting of the costophrenic angles most likely related to pleural thickening. The cardiothoracic ratio within normal limits. Mediastinal contours are unremarkable. No bony abnormality.

Background cystic lung disease in keeping with the history of lymphangioleiomyomatosis (LAM). A left anterior intercostal pigtail catheter is positioned in the left pleural space anteriorly. Small residual pneumothorax within the left lateral costophrenic angle.

The previously demonstrated 16 mm nodule within the left upper lobe remains similar in size and morphology to the previous examination from one year prior. Stable left-sided pleural thickening is also again noted. 

No destructive bony abnormality identified. Innumerable renal angiomyolipomas
also again noted in keeping with the history of tuberous sclerosis (TS).

Impression

  • tiny residual pneumothorax within the left costophrenic angle. The pleural catheter is well positioned

  • background changes of LAM and TS noted

Case Discussion

This case demonstrates the classic CXR and CT appearances of lymphangioleiomyomatosis, which in this patient was associated with tuberous sclerosis.

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