Abstract

MARTINEZ-MARTINEZ, Blanca Estela et al. Pediatric generalized lymphangiomatosis. A case report and literature review. Neumol. cir. torax [online]. 2018, vol.77, n.4, pp.280-285. ISSN 0028-3746.

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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