Chylothorax is defined by extravasation of the milky fluid to pleural cavity from the thoracic duct or it's main branches due to operative trauma, congenital lesions, diagnostic procedures, tumor, etc. Another rare cause is diffuse pulmonary
lymphangiomatosis which is uncommon and not well characterized. We experienced a case of the bilateral chylothorax caused by the diffuse pulmonary lymphangiomatosis.
The patient was a 3 years old girl with symptoms of coughing and febrile sensation, and the chest radiographs showed bilateral pleural effusion and interstitial infiltrates. The laboratory data of the pleural effusion was identified as chyle.
Uncontrollable with closed tube thoracostomy, division of the thoracic duct and biopsy were decided. Biopsy showed anastomosing endothelial lined spaces along the pulmonary lymphatic routes especially in pleural and interlobar septum, and smooth
muscle
at the proliferative interstitium of the lymphatic duct was observed.
Postoperatively, chylothorax was controlled with several trial of chemical pleurodesis.
After discharge from the hospital, she was well for ten months follow up.
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