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Primary idiopathic chylopericardium: a retrospective case series.

Han Z, Li S, Jing H, Liu H - BMC Surg (2015)

Bottom Line: Non-surgical therapy was adopted in all nine patients and failed in six, who underwent subsequent successful surgery.All patients were followed up from 3 months to 9 years, and no recurrence occurred.The most effective treatment is ligation of the thoracic duct and the creation of a pericardial window.

View Article: PubMed Central - PubMed

Affiliation: Department of Thoracic Surgery, Peking Union Medical College Hospital (PUMCH), Chinese Academy of Medical Sciences & Peking Union Medical College (CAMS & PUMC), 1 Shuai-Fu-Yuan, Beijing, 100730, China. pumchhan@163.com.

ABSTRACT

Background: 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.

Methods: We retrospectively reviewed 9 cases of patients who suffered from primary idiopathic chylopericardium at our hospital from January 1993 to November 2013.

Results: There 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.

Conclusions: In 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.

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Related in: MedlinePlus

Lymphoscintigraphy. The local images were collected after injecting Tc-99 m antimony sulfide colloid 20 minutes, 2 and 5.5 hours. Lymphoscintigraphy demonstrates the marked dilatation of the upper of alimentary duct and the increased uptake in the pericardium
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Fig2: Lymphoscintigraphy. The local images were collected after injecting Tc-99 m antimony sulfide colloid 20 minutes, 2 and 5.5 hours. Lymphoscintigraphy demonstrates the marked dilatation of the upper of alimentary duct and the increased uptake in the pericardium

Mentions: The patient group consisted of two male and seven female patients with an age range from 13 to 55 years (median age, 36 years). In our cases, the clinical manifestations were variable, including dyspnea (n = 6), a lack of symptoms (n = 2), and atrial fibrillation (n = 1). No cardiac tamponade occurred. The time from symptom onset to diagnosis was uncertain (ranging from several months to several years). All patients denied a history of trauma, congenital heart disease, thoracic surgery, tuberculosis or thoracic tumors. The patients underwent a physical examination, routine blood testing, standard chest radiography, electrocardiography and thoracic computed tomography (CT). Clinical examination showed elevated jugular venous pressure and distant heart sounds. The chest radiograph demonstrated enlargement of the cardiac silhouette, and echocardiography revealed pericardial effusion. Diagnostic pericardiocentesis was performed, and milky-colored pericardial fluid was aspirated from all patients. These findings, along with the high level of triglycerides in the pericardial fluid, sufficed to diagnose chylopericardium. All patients underwent extensive evaluation to find the cause of the chylopericardium. Routine laboratory tests demonstrated that there was no sign of a systemic inflammatory reaction. Cultures of the pericardial fluid for bacteria and tuberculosis were subsequently reported as negative, and cytologic examination showed no tumor cells. A chest CT scan did not reveal mediastinal neoplasm, mediastinal lymphadenopathy or other abnormalities that might have obstructed the thoracic duct. Lymphoscintigraphy was performed in all patients. Communication between the thoracic duct and the pericardial space was directly confirmed by lymphoscintigraphy in three patients. In three cases without communication, pericardial accumulation of technetium-99 m sulfur colloid and a possible site of leakage from the thoracic duct were confirmed. In the other 3 cases without communication, accumulation of technetium-99 m sulfur colloid alone was found (Figs. 1 and 2).Fig. 1


Primary idiopathic chylopericardium: a retrospective case series.

Han Z, Li S, Jing H, Liu H - BMC Surg (2015)

Lymphoscintigraphy. The local images were collected after injecting Tc-99 m antimony sulfide colloid 20 minutes, 2 and 5.5 hours. Lymphoscintigraphy demonstrates the marked dilatation of the upper of alimentary duct and the increased uptake in the pericardium
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4465719&req=5

Fig2: Lymphoscintigraphy. The local images were collected after injecting Tc-99 m antimony sulfide colloid 20 minutes, 2 and 5.5 hours. Lymphoscintigraphy demonstrates the marked dilatation of the upper of alimentary duct and the increased uptake in the pericardium
Mentions: The patient group consisted of two male and seven female patients with an age range from 13 to 55 years (median age, 36 years). In our cases, the clinical manifestations were variable, including dyspnea (n = 6), a lack of symptoms (n = 2), and atrial fibrillation (n = 1). No cardiac tamponade occurred. The time from symptom onset to diagnosis was uncertain (ranging from several months to several years). All patients denied a history of trauma, congenital heart disease, thoracic surgery, tuberculosis or thoracic tumors. The patients underwent a physical examination, routine blood testing, standard chest radiography, electrocardiography and thoracic computed tomography (CT). Clinical examination showed elevated jugular venous pressure and distant heart sounds. The chest radiograph demonstrated enlargement of the cardiac silhouette, and echocardiography revealed pericardial effusion. Diagnostic pericardiocentesis was performed, and milky-colored pericardial fluid was aspirated from all patients. These findings, along with the high level of triglycerides in the pericardial fluid, sufficed to diagnose chylopericardium. All patients underwent extensive evaluation to find the cause of the chylopericardium. Routine laboratory tests demonstrated that there was no sign of a systemic inflammatory reaction. Cultures of the pericardial fluid for bacteria and tuberculosis were subsequently reported as negative, and cytologic examination showed no tumor cells. A chest CT scan did not reveal mediastinal neoplasm, mediastinal lymphadenopathy or other abnormalities that might have obstructed the thoracic duct. Lymphoscintigraphy was performed in all patients. Communication between the thoracic duct and the pericardial space was directly confirmed by lymphoscintigraphy in three patients. In three cases without communication, pericardial accumulation of technetium-99 m sulfur colloid and a possible site of leakage from the thoracic duct were confirmed. In the other 3 cases without communication, accumulation of technetium-99 m sulfur colloid alone was found (Figs. 1 and 2).Fig. 1

Bottom Line: Non-surgical therapy was adopted in all nine patients and failed in six, who underwent subsequent successful surgery.All patients were followed up from 3 months to 9 years, and no recurrence occurred.The most effective treatment is ligation of the thoracic duct and the creation of a pericardial window.

View Article: PubMed Central - PubMed

Affiliation: Department of Thoracic Surgery, Peking Union Medical College Hospital (PUMCH), Chinese Academy of Medical Sciences & Peking Union Medical College (CAMS & PUMC), 1 Shuai-Fu-Yuan, Beijing, 100730, China. pumchhan@163.com.

ABSTRACT

Background: 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.

Methods: We retrospectively reviewed 9 cases of patients who suffered from primary idiopathic chylopericardium at our hospital from January 1993 to November 2013.

Results: There 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.

Conclusions: In 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.

Show MeSH
Related in: MedlinePlus