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- W4313441009 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." @default.
- W4313441009 created "2023-01-06" @default.
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- W4313441009 date "2023-01-01" @default.
- W4313441009 modified "2023-09-24" @default.
- W4313441009 title "Chylopericardium causing cardiac tamponade in a patient with lymphangioleiomyomatosis" @default.
- W4313441009 doi "https://doi.org/10.24911/ejmcr/173-1664549057" @default.
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