Primary chylopericardium: report of a case.

A 14-year-old girl was admitted due to exertional dyspnea of one month's duration. A grade 3/6 holosystolic murmur with distant heart sounds was heard at the left sternal border. Chest roentgenograms and echocardiograms showed a large amount of pericardial and left pleural effusion and a small perimembranous ventricular septal defect. Subxyphoid pericardial drainage and left pleural intubation were performed. The fluid from both sites was defined as chyle by the milky white appearance, presence of microscopic fat droplets and the lymphocyte predominance in the white cell count. Lymphangiograms and a chest computed tomographic scan demonstrated thoracic duct obstruction and leakage of contrast to pericardial and pleural spaces. The patient was treated with a medium chain triglyceride diet. Unfortunately, massive reaccumulation of the effusion was later noted. Therefore, a median sternotomy with ligation of the thoracic duct, creation of a posterior pericardio-pleural window and implantation of a left pleuro-peritoneal shunt with a Denver peritoneo-venous shunt were performed. Follow-up for one year with two-dimensional echocardiograms showed no more accumulation of the pericardial effusion.