Primary idiopathic chylopericardium: a retrospective case series

Background Primary idiopathic chylopericardium is a rare clinical entity characterized by the accumulation of chyle within the pericardial cavity without a definitive cause. The aim of this study was to assess the clinical presentation, etiology, diagnosis, treatment and follow-up of primary idiopathic chylopericardium. Methods We retrospectively reviewed 9 cases of patients who suffered from primary idiopathic chylopericardium at our hospital from January 1993 to November 2013. Results There were two males and seven females among our patients. Their ages ranged from 13 to 55 years. The most common clinical presentation was dyspnea. The etiology was idiopathic. All patients were diagnosed by pericardiocentesis, computed tomography of the chest and lymphoscintigraphy. Non-surgical therapy was adopted in all nine patients and failed in six, who underwent subsequent successful surgery. Thoracic duct ligation with the creation of a pericardial window was the most common surgical procedure. All patients were followed up from 3 months to 9 years, and no recurrence occurred. Conclusions In assessing patients with an enlarged cardiac silhouette, one should be aware of primary idiopathic chylopericardium. The most effective treatment is ligation of the thoracic duct and the creation of a pericardial window.