ABSTRACT

Generalized lymphangiomatosis is a rare entity that consists of difusse and multifocal proliferation of abnormal lymphatic vessels that cause system obstruction. Seven-year-old male who start 15 days before entering a hospital with referred pain in left shoulder that motivated a chest X-ray detecting cardiomegaly. A pericardiocentesis drained 500 ml of chocolate-like liquid and was referred to a third level of medical care due to the recurrence of pericardial effusion. Pericardial window was performed quantifying an additional 900 ml salmon-like liquid with chylothorax characteristics. Computed tomography was compatible with lymphangiomatosis, affecting neck, mediastinum and bone and bazo. He presented a high-expenditure bilateral pleural effusion despite receiving parenteral nutrition and intravenous octreotide; with loculations in pleural space for which pleural decortication was performed, pleurodesis and lung and pleura biopsy that reported pulmonar and pleural lymphangiomatosis.

KEYWORDS

Generalized lymphangiomatosis, chylothorax, pleural efussion, pericardial efussion.

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