Isolated primary chylopericardium: A rare case report and literature review

INTRODUCTION: Primary chylopericardium is an extremely rare disease with no previous reported case in Indonesia. It may be caused by an abnormality of lymphatic drainage in the thoracic region. CASE PRESENTATION: We report the case of a 19-year-old female who presented with a chief complaint of shortness of breath and fluid accumulation in her pericardial sac. Screening for autoimmune disease, tuberculosis, and malignancy all came back negative. Lymphoscintigraphy showed suspected leakage or obstruction of lymph at the supradiaphragmatic level. The patient was diagnosed with primary chylopericardium. In a span of 1 year, she had undergone 3 pericardiocentesis procedures and declined surgical treatment. On her fourth pericardiocentesis, the cardiologists were unable to remove fluid from the pericardial sac. Thoracic duct ligation and pericardial window were eventually performed and a total of 850 ml of chylous fluid was removed. At 6 months postoperation patient showed no signs and symptoms of pericardial effusion and was in good condition. DISCUSSION: Constrictive pericarditis can occur as a complication of chronic inflammation in the pericardium due to the presence of chylopericardium and a history of multiple pericardiocenteses. However, there are no reports regarding the exact duration of chylopericardium that will lead to constrictive pericarditis. Difficulty in pericardiocentesis might be an early sign of scarring in the pericardium. CONCLUSION: Delayed surgical treatment of chylopericardium increases the risk of constrictive pericarditis and significantly reduces the patient's quality of life due to recurrent symptoms and hospitalizations.