Rare Diseases Symptoms Automatic Extraction

Successful management of bilateral refractory chylothorax after double lung transplantation for lymphangioleiomyomatosis.

[lymphangioleiomyomatosis]

Lymphangioleiomyomatosis (LAM) is a rare disease that leads to airways and lymphatic channels obstruction due to abnormal smooth muscle proliferation. It presents with dyspnea, pneumothorax or chylothorax. Lung transplantation (LT) has emerged as a valuable therapeutic option with limited reports. We report a case of LAM that underwent double LT and complicated by refractory bilateral chylothorax which was managed successfully by povidone-iodine pleurodesis and the addition of sirolimus to the post-transplantation immunosuppressive therapy. The patient has no recurrence with 24 months follow-up.

Diseases presenting "lung transplantation" symptom

  • lymphangioleiomyomatosis

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