[Primary chylopericardium--stepwise diagnosis and therapy of a differential diagnostically important illness]

Primary chylopericardium is a rare disease with a highly variable clinical course. We report on a 24-year old female with chylopericardium detected during a pulmonary infection. Despite successful treatment of the infectious disease, the chylopericardium persisted and led to cardiac tamponade. From this case, as well as from the literature, it is intriguing to postulate an inflammatory injury of preexisting anomalous lymphatic vessels leading to onset or aggravation of primary chylopericardium. The clinical hallmark of chylopericardium is a milky white, but odorless pericardial fluid at pericardiocentesis. For cases where conservative treatment and pericardiocentesis fail, we newly introduced the method of pericardio-peritoneal shunting by a pericardial window. With postoperative reaccumulation of pericardial fluid, total parenteral nutrition followed by medium chain triglyceride diet was successfully reinitiated. This combined surgical and conservative approach was performed for the first time and may have helped to avoid the more aggressive treatment of thoracic duct ligation and resection. During 2 years of follow-up the patient was asymptomatic and had no recurrence of pericardial effusion.