Cystic lymphangioma of pericardium presenting as isolated chylopericardium - a case report

Abstract

Isolated chylopericardium due to cystic lymphangioma of pericardium is a rare entity. We report a case of asymptomatic chylopericardium in a young male who presented with cardiomegaly. Echocardiography revealed massive pericardial effusion without tamponade. Pericardiocentesis yielded 1.25 L of tea-colored fluid which showed triglyceride level of 1723 mg/dL and cholesterol of 1021 mg/dL with a cholesterol to triglyceride ratio of <1, characteristic of chylous fluid. Lymphoscintigraphy using 99Tc demonstrated lymphatic leak around the heart region. Fusion of MRI images with lymphoscintigraphy was taken with a view of localizing the leak site; it demonstrated enhancement in the pericardial space. Surgery was done via right lateral thoracotomy. Thoracic duct was ligated above diaphragm and pericardial window created by anterior pericardiectomy. The patient had an uneventful recovery and was well after 6 months of follow up. Pericardial biopsy showed cystic lymphangioma of pericardium.

Keywords: Cystic lymphangioma; Isolated chylopericardium; Lymphoscintigraphy.

Fig. 1

(A) X-ray of the patient showing cardiomegaly, (B) Echocardiogram showing pericardial fluid, (C) Tea-colored pericardial aspirate.

Fig. 2

(A) Lymphoscintigraphy showing Tc₉₉ radioactivity around the heart, (B) Fusion MRI and scintigraphy image showing seepage of Tc₉₉ in the pericardial space, (C) Photomicrograph of the histology of lymphangioma pericardium showing lymphoid follicles (arrow) and cystic spaces.