A case report of generalized lymphangiomatosis with chylopericardium: the crucial role of magnetic resonance lymphangiography

Background Chylopericardium due to generalized lymphangiomatosis is a rare clinical entity. Its aetiology and treatment remain unclear. Case summary 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 by exclusive surgical ligation of the abnormal communications and partial pericardiectomy by thoracotomy. The patient’s postoperative recovery was uneventful, and no recurrence of pericardial effusion occurred during the 13-month follow-up. Discussion Magnetic resonance lymphangiography showed a good capability for evaluating the extent of generalized lymphangiomatosis and therefore is helpful for delineating the anatomy of the thoracic duct and identifying chyle leakage and abnormal communications between the thoracic duct and the pericardial sac. This makes a simple ligature of the abnormal communications, instead of thoracic duct mass ligation possible in the treatment of chylopericardium.