Massive primary chylopericardium: a case report

A large pericardial effusion was discovered in an asymptomatic 12-year-old boy admitted for an elective orthopedic procedure. On physical examination, heart rate was 96 and blood pressure was 130/70 without paradox. The neck veins were not distended, but heart tones were distant. Chest roentgenogram (CXR) showed an enlarged cardiac silhouette. Echocardiogram showed a massive pericardial effusion compressing the right atrium, with depressed ventricular contractility. Pericardiocentesis yielded 450 mL of chylous fluid. A percutaneous pericardial drain was placed and drained another 400 mL of chyle. Pericardial fluid reaccumulated even though the patient was on a low-fat diet, and 1 week after admission left thoracotomy was performed with partial pericardiectomy and pericardial window. There was 1 L of chyle in the pericardial sac; frozen section of the pericardium showed lymphangiectasia. Chest tube drainage diminished rapidly and the patient was discharged. Follow-up CXR at 1 week showed fluid in both pleural spaces requiring bilateral tube thoracostomies again draining chyle. Even with total parenteral nutrition (TPN), 500 mL/d of chyle drained from the pleural tubes. Right thoracotomy with ligation of the thoracic duct was performed after 1 week of TPN. Pleural drainage abruptly dropped, and there has been no reaccumulation in either the pleural spaces or pericardium at 6-month follow-up. This case dramatically supports early thoracic duct ligation and partial pericardiectomy as the treatment of choice for primary massive chylopericardium.