Introduction: Chylopericardium is the presence of chylous fluid in the pericardial space. The most frequent causes of this rare entity are secondary to surgery and other invasive techniques on the thorax, followed by idiopathic ones, although they have also been associated with congenital anomalies of the lymphatic system, lymphangiomas and other mediastinal tumors, tuberculosis, etc. Its appearance is more common in adults, but lately cases have been described equally between the pediatric population and adults. Cases Reports: The presence of two patients of 8 and 11 months of age who present with significant amount of fluid at the level of the pericardium with involvement of right cardiac chambers in whom the pericardiocentesis performed reveals chyle without a frank pathological association is reported. Different managements with a single purpose, to achieve the ideal treatments for each case. Results: Two patients were identified from our surgical registry in the period described. The first corresponded to a male, of 11 months of age, without significant antecedents, with evidence of chylous pericardial effusion of spontaneous appearance that did not yield to the established conservative measures and later went to surgery for ligation of the thoracic duct and partial pericardiectomy. The second is a patient recently evaluated in the area of pediatrics, male, of 8 months, with Down Syndrome who comes to emergency of our hospital due to the presence of dyspnea, general malaise and food intolerance. The chest X-ray revealed the presence of cardiomegaly and a complementary transthoracic echocardiogram revealed severe pericardial effusion with signs of cardiac tamponade. Diagnostic and evacuation pericardiocentesis was performed with a pediatric central catheter, obtaining serous fluid and draining 35 cc, but three days after the puncture, the exit of chylous fluid through the catheter maintained in the pericardial space was observed. Conservative management is conditioned for a period of 7 days. The rest of the literature review identifies 18 pediatric patients with the presence of a spontaneously occurring pericardial effusion with different treatments offered. Discussion and Conclusions: Chylopericardium is a pathology of rare appearance, the main etiologies define patients who have been taken to cardiac or thoracic surgery. The most categorical description is given for adulthood but in the pediatric age (under 18 years) has not been described a consensus management yet for the variability of its diagnosis, however management remains uncertain after its appearance in those who debuted with primary chylopericardium or spontaneous origin, but despite this the implementation of conservative measures such as enteral nutrition with fatty acids of each media and use of parenteral nutrition between 7 to 15 days; and after the failure of these measures, surgery with thoracic duct ligation by right thoracotomy plus pleuropericardial window confection or partial pericardiectomy are the most successful management options that are known to date with good results in the short and medium term.
Unusual Case of Recurrent Pericardial Effusion ― Erdheim-Chester Disease ―
