Primary idiopathic chylopericardium--a case report and review of the literature.

Primary idiopathic chylopericardium is a rare entity with obscure etiology. In recent years, a few reports have been published where the lymphatic leak and fistula were identified by combined lymphangiography and computed tomography or intraoperative thoracic ductogram. Here we report a case occurring in a previously healthy 51 year old woman, to our knowledge the first case described in Scandinavia. She presented with cough and dyspnea of about 1 year duration. Chest X-ray demonstrated enlargement of the cardiac silhouette. Echocardiography revealed pericardial effusion and at puncture pericardiocentecis 760 ml of chylous fluid was evacuated. There was no history of trauma, infection or neoplasm. She underwent extensive investigations but no apparent cause of the chylous effusion could be determined. Conservative treatment with pericardial drainage and low fat (medium chain triglyceride) diet failed. The patient was cured by surgical ligature of the thoracic duct and partial pericardectomy through a left anterolateral thoracotomy. At 12 months post operation she was doing well and echocardiography did not reveal any signs of recurrence. A brief review of the literature is presented.