EJMCR |

Case Report

Volume: 7 | Issue: 2 | Published: Jan 01, 2023 | Pages: 46 - 51 | DOI: 10.24911/ejmcr/173-1664549057

Chylopericardium causing cardiac tamponade in a patient with lymphangioleiomyomatosis

Authors: Isaac Jun Jie Fong , Xu Cong Ruan , Yann Shan Keh , Yi-Hern Tan

Article Info

Authors

Isaac Jun Jie Fong

Department of Respiratory & Critical Care Medicine, Singapore General Hospital, Singapore

ORCID

Xu Cong Ruan

Department of Cardiology, National Heart Centre, Singapore

Yann Shan Keh

Department of Cardiology, National Heart Centre, Singapore

Yi-Hern Tan

Department of Respiratory & Critical Care Medicine, Singapore General Hospital, Singapore.

Publication History

Received: October 15, 2022

Revised: December 15, 2022

Accepted: December 15, 2022

Published: January 01, 2023

Abstract

Background: Chylopericardium is a rare entity characterized by the accumulation of chylous fluid in the pericardial cavity. Lymphangioleiomyomatosis (LAM) is a rare, progressive systemic disease with prominent features of lung cysts, abdominal tumors, and the development of chylous effusions, usually in the thorax and/or abdomen. Chylopericardium in association with LAM is exceedingly rare, with only a few cases reported in the literature. Case Presentation: We report a case of a 61-year-old woman with chronic dyspnea and bilateral cystic lung disease who presented with a 1-week history of acutely worsening dyspnea. At presentation, she was tachycardiac and hypoxemic, an electrocardiogram detected sinus tachycardia, and a chest radiograph was indeterminate for a small right apical pneumothorax or an apical bulla. An urgent echocardiogram revealed a large pericardial effusion with features of cardiac tamponade, necessitating urgent pericardiocentesis. Analysis of the pericardial fluid confirmed the presence of chylopericardium and a diagnosis of LAM was made. Oral Sirolimus was started and at 1 year of follow-up, the patient has successfully avoided repeat pericardiocentesis. Conclusion: This case describes a rare presentation of a rare disease and suggests that treatment with sirolimus may potentially be effective in reducing the accumulation of chylopericardium in LAM.

Keywords: Lymphangioleiomyomatosis, lung cysts, cardiac tamponade, chylopericardium, dyspnea, case report, green

Pubmed Style

Isaac Jun Jie Fong, Xu Cong Ruan, Yann Shan Keh, Yi-Hern Tan. Chylopericardium causing cardiac tamponade in a patient with lymphangioleiomyomatosis. EJMCR. 2023; 01 (January 2023): 46-51. doi:10.24911/ejmcr/173-1664549057

Web Style

Isaac Jun Jie Fong, Xu Cong Ruan, Yann Shan Keh, Yi-Hern Tan. Chylopericardium causing cardiac tamponade in a patient with lymphangioleiomyomatosis. https://ejmcr.com/articles/1276 [Access: December 04, 2024]. doi:10.24911/ejmcr/173-1664549057

AMA (American Medical Association) Style

Vancouver/ICMJE Style

Isaac Jun Jie Fong, Xu Cong Ruan, Yann Shan Keh, Yi-Hern Tan. Chylopericardium causing cardiac tamponade in a patient with lymphangioleiomyomatosis. EJMCR. (2023), [cited December 04, 2024]; 01 (January 2023): 46-51. doi:10.24911/ejmcr/173-1664549057

Harvard Style

Isaac Jun Jie Fong, Xu Cong Ruan, Yann Shan Keh, Yi-Hern Tan (2023) Chylopericardium causing cardiac tamponade in a patient with lymphangioleiomyomatosis. EJMCR, 01 (January 2023): 46-51. doi:10.24911/ejmcr/173-1664549057

Chicago Style

Isaac Jun Jie Fong, Xu Cong Ruan, Yann Shan Keh, Yi-Hern Tan. "Chylopericardium causing cardiac tamponade in a patient with lymphangioleiomyomatosis." 01 (2023), 46-51. doi:10.24911/ejmcr/173-1664549057

MLA (The Modern Language Association) Style

Isaac Jun Jie Fong, Xu Cong Ruan, Yann Shan Keh, Yi-Hern Tan. "Chylopericardium causing cardiac tamponade in a patient with lymphangioleiomyomatosis." 01.January 2023 (2023), 46-51. Print. doi:10.24911/ejmcr/173-1664549057

APA (American Psychological Association) Style

Isaac Jun Jie Fong, Xu Cong Ruan, Yann Shan Keh, Yi-Hern Tan (2023) Chylopericardium causing cardiac tamponade in a patient with lymphangioleiomyomatosis. , 01 (January 2023), 46-51. doi:10.24911/ejmcr/173-1664549057