Primary idiopathic chylopericardium associated with cervicomediastinal cystic hygroma.
Chylopericardium is a rare clinical entity in which chylous fluid accumulates in the pericardial cavity. We report a case of primary idiopathic chylopericardium associated with multiple, small cervicomediastinal cystic hygromas occurring in an asymptomatic 43-year-old woman with no history of trauma, thoracic surgery, malignancy, infection or tuberculosis. Echocardiography showed a large amount of pericardial effusions and pericardial fluid analysis revealed inappropriately elevated triglyceride. We did not demonstrate communication between the thoracic duct and the pericardial sac by lymphangiography and chest computed tomography. She successfully responded to 30 days of continuous pericardial drainage and 15 days of a medium-chain triglyceride diet after 30 days of total parenteral nutrition. Follow-up echocardiography 6 months after treatment commencement showed a minimal reaccumulation of pericardial fluid without symptom. We conclude that if a patient is asymptomatic and can well tolerate daily life, surgery including pericardiectomy or ligation of the thoracic duct is not necessarily required.
Cardiology Division, Cardiovascular Research Institute, Yonsei University College of Medicine, 134 Sinchon-dong, Seodaemun-gu, Seoul 120-752, Korea.
Byoung Chul
BC
Cho
Seok Min
SM
Kang
Seung Chul
SC
Lee
Jeong Geun
JG
Moon
Dong Hyung
DH
Lee
Sang Hyun
SH
Lim
D000328:Adult; D005260:Female; D006801:Humans; D018191:Lymphangioma, Cystic / Q000150:complications* / Q000473:pathology; D008476:Mediastinal Cyst / Q000150:complications* / Q000473:pathology; D009333:Neck / Q000473:pathology; D010490:Pericardial Effusion / Q000209:etiology* / Q000473:pathology / Q000628:therapy