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Chylopericardium due to generalized lymphangiomatosis is a rare clinical entity. Its aetiology and treatment remain unclear. We report one case of a 51-year-old man who was diagnosed with generalized lymphangiomatosis with idiopathic chylopericardium by bilateral inguinal intranodal contrast-enhanced magnetic resonance lymphangiography. Magnetic resonance lymphangiography demonstrated abnormal communications between the pericardial sac and the thoracic duct. The patient with idiopathic chylopericardium was therefore successfully treated
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