Abstract
BackgroundPrimary idiopathic chylopericardium is a rare clinical entity characterized by the accumulation of chyle within the pericardial cavity without a definitive cause. The aim of this study was to assess the clinical presentation, etiology, diagnosis, treatment and follow-up of primary idiopathic chylopericardium.MethodsWe retrospectively reviewed 9 cases of patients who suffered from primary idiopathic chylopericardium at our hospital from January 1993 to November 2013.ResultsThere were two males and seven females among our patients. Their ages ranged from 13 to 55 years. The most common clinical presentation was dyspnea. The etiology was idiopathic. All patients were diagnosed by pericardiocentesis, computed tomography of the chest and lymphoscintigraphy. Non-surgical therapy was adopted in all nine patients and failed in six, who underwent subsequent successful surgery. Thoracic duct ligation with the creation of a pericardial window was the most common surgical procedure. All patients were followed up from 3 months to 9 years, and no recurrence occurred.ConclusionsIn assessing patients with an enlarged cardiac silhouette, one should be aware of primary idiopathic chylopericardium. The most effective treatment is ligation of the thoracic duct and the creation of a pericardial window.
Highlights
Primary idiopathic chylopericardium is a rare clinical entity characterized by the accumulation of chyle within the pericardial cavity without a definitive cause
The characteristics of the nine patients with primary idiopathic chylopericardium are summarized in the Table 1
Hasebrock was the first to describe the presence of 22.6 ml of chyle in the pericardial cavity [4], detected during the autopsy of a man who had died from asphyxia secondary to constriction and tracheal ulceration
Summary
Primary idiopathic chylopericardium is a rare clinical entity characterized by the accumulation of chyle within the pericardial cavity without a definitive cause. The aim of this study was to assess the clinical presentation, etiology, diagnosis, treatment and follow-up of primary idiopathic chylopericardium. The accumulation of chyle in the pericardial space, or chylopericardium, is a condition that occurs most frequently after trauma, cardiothoracic surgery, or radiation therapy or in association with tumors or tuberculosis. Congenital lymphangiomatosis or lymphangiectasia may be a cause of chylopericardium [1,2]. If the precise etiology cannot be identified, the condition is called primary or idiopathic chylopericardium, which is a rare clinical entity. We report 9 cases of primary idiopathic chylopericardium and assess the clinical presentation, etiology, diagnosis, treatment and followup of this entity.
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