Primary chylopericardium with pulmonary lymphedema

Primary chylopericardium is an uncommon entity, and its association with pulmonary lymphedema has been rarely reported. We describe a case of primary chylopericardium with pulmonary lymphedema developing into hypoxemia. The pulmonary lesions were histologically diagnosed as pulmonary lymphangiectasis and lymphedema on lung biopsies. Lymphedema seems to suggest the existence of chylous reflux with pulmonary lymphangiectasis. The patient underwent pericardial fenestration and resection of the thoracic duct. After the operation, the chylous accumulation in the pericardial cavity had disappeared, and hypoxemia improved following the disappearance of the pulmonary lesions.