Association between lymphatic abnormalities in the neck and thorax in primary chylopericardium and surgical outcomes evaluated by non-enhanced magnetic resonance (MR) lymphangiography.

Background: Chylopericardium refers to the accumulation of chylous fluid in the pericardial cavity. Non-enhanced magnetic resonance lymphangiography (MRL) can show neck and thoracic lymphatic abnormalities in the primary chylopericardium. It is not clear whether there is a relationship between neck and thoracic lymphatic abnormalities in primary chylopericardium and thoracic duct terminal release surgery. This study aimed to explore the correlation between the severity of neck and thoracic lymphatic abnormalities observed in non-enhanced MRL and the surgical outcomes in primary chylopericardium. Methods: This is a retrospective cohort study. A retrospective analysis was conducted on fifty-six patients diagnosed with primary chylopericardium between January 2016 and December 2021, all of whom underwent thoracic duct terminal release surgery. Ultrasonography, chest computed tomography (CT) and non-enhanced MRL were performed prior to the surgical intervention. Patients were categorized into four types based on the severity of neck and thoracic lymphatic abnormalities observed in the non-enhanced MRL. Clinical and laboratory examinations and surgical outcomes were compared across different types using χ 2-test or Fisher's exact test, t-test, and Kruskal-Wallis H-test. Additionally, independent factors influencing surgical outcomes were analyzed. Results: Among primary chylopericardium cases (n=56), 22 (39.2%) were classified as type I or II, 17 (30.4%) as type III, and 17 (30.4%) as type IV. Surgical outcomes were more favorable for type I or II patients than those with type III or IV, accompanied by a reduction in postoperative primary chylopericardium volume (P=0.002). Postoperative chest CT scans indicated that type I or II patients had fewer instances of large grid shadows, small grid shadows, and bronchovascular bundle thickening compared to preoperative scans (P=0.001, P=0.02, P=0.03). Age and bronchomediastinal trunk dilation emerged as independent factors influencing surgical outcomes [odds ratio (OR) 0.03, 95% confidence interval (CI): 0.003-0.220, P=0.001; OR 11.10, 95% CI: 1.70-72.39, P=0.01, respectively]. Conclusions: A more severe degree of neck and thoracic lymphatic abnormalities is associated with worse surgical outcomes. Moreover, age and bronchomediastinal trunk dilatation are independent predictors of surgical outcomes. Preoperative utilization of non-enhanced MRL for severity of lymphatic abnormalities classification in primary chylopericardium patients offers a noninvasive means of assessing surgical risk.

Diagnostic value of combined CT lymphangiography and 99Tcm-DX lymphoscintigraphy in primary chylopericardium.

OBJECTIVE: To investigate the diagnostic value of combined 99Tcm-DX lymphoscintigraphy and CT lymphangiography (CTL) in primary chylopericardium. METHODS: Fifty-five patients diagnosed with primary chylopericardium clinically were retrospectively analyzed. 99Tcm-DX lymphoscintigraphy and CTL were performed in all patients. Primary chylopericardium was classified into three types, according to the 99Tcm-DX lymphoscintigraphy results. The evaluation indexes of CTL include: (1) abnormal contrast distribution in the neck, (2) abnormal contrast distribution in the chest, (3) dilated thoracic duct was defined as when the widest diameter of thoracic duct was > 3 mm, (4) abnormal contrast distribution in abdominal. CTL characteristics were analyzed between different groups, and P < 0.05 was considered a statistically significant difference. RESULTS: Primary chylopericardium showed 12 patients with type I, 14 patients with type II, and 22 patients with type III. The incidence of abnormal contrast distribution in the posterior mediastinum was greater in type I than type III (P = 0.003). The incidence of abnormal contrast distribution in the pericardial and aortopulmonary windows, type I was greater than type III (P = 0.008). And the incidence of abnormal distribution of contrast agent in the bilateral cervical or subclavian region was greater in type II than type III (P = 0.002). CONCLUSION: The combined application of the 99Tcm-DX lymphoscintigraphy and CTL is of great value for the localized and qualitative diagnosis of primary chylopericardium and explore the pathogenesis of lesions.

Dynamic Contrast-Enhanced Magnetic Resonance Lymphangiography for Diagnosis and Treatment of Chylopericardium After Cardiac Transplantation.

Chylopericardium is a rare complication after cardiac transplantation. We report a case of a 69-year-old woman with persistent chylopericardium after a heart transplantation due to Chagas disease. Failure of conservative treatment led to dynamic contrast-enhanced magnetic resonance lymphangiography and percutaneous radiologic intervention of the lymphatic leakage and symptoms resolution.

A Rare Occurrence of Chylopericardium in a Case of Anterior Mediastinal Mass.

A middle-aged gentleman presented with dyspnea and hypotension, accompanied by an anterior mediastinal mass infiltrating the anterior chest wall and a moderate to large pericardial effusion, which upon evaluation revealed chylous fluid. Further investigation diagnosed him with right lung adenocarcinoma infiltrating the chest wall, staged at T4N3M1. The patient subsequently developed cardiac tamponade, necessitating immediate medical intervention. Management of the patient's cardiac tamponade involved pericardiocentesis via an indwelling pericardial catheter, allowing continuous drainage of the chylous fluid. Additionally, the patient was placed on a medium-chain triglyceride diet (MCTD) to reduce chyle production. These interventions resulted in significant symptomatic improvement, stabilizing the patient's hemodynamic status, and alleviating the immediate life-threatening condition. This case highlights the clinical challenges posed by rare presentations such as chylopericardium secondary to malignancy and emphasizes the importance of comprehensive diagnostic evaluation and prompt therapeutic management. The successful outcome, achieved through a combination of pericardial drainage and dietary modifications, underscores the critical role of a conservative approach in managing complex oncological cases with acute complications.

Pediatric Idiopathic Chylopericardium: A Case Managed with Thoracoscopic Window Pericardiotomy.

Idiopathic chylopericardium (CP) in the pediatric population is a rare entity with very few reported cases and is characterized by the accumulation of chyle in the pericardial cavity. There are no guidelines for the management of this rare entity. The present study reports a case of idiopathic CP in an infant and our experience of managing it by pericardial window creation using VATS and a multidisciplinary approach providing the optimum care for the child.

Chylopericardium following esophagectomy: a case report and systematic review.

BACKGROUND: Chylopericardium is a rare condition characterized by the accumulation of chyle in the pericardial space. It is most commonly caused by thoracic duct injury. Chylopericardium following esophagectomy is extremely rare but can cause life-threatening complications. This report presents a case of chylopericardium post-esophagectomy, resulting in cardiac tamponade and cardiac arrest. A systematic literature review was also conducted to facilitate the understanding of this rare condition. CASE PRESENTATION: A 41-year-old male was admitted to our hospital with intermediate to highly differentiated squamous cell carcinoma of the mid-thoracic esophagus (clinical T4NxM0). He underwent thoracoscopic-laparoscopic esophagectomy with cervical anastomosis. On postoperative day 1, patient had a cardiac arrest secondary to cardiac tamponade, requiring emergency ultrasound-guided drainage. The drained fluid was initially serous but became chylous after the administration of enteral nutritional emulsion. As a result of significant daily pericardial drainage, patient subsequently underwent thoracic duct ligation. The amount of drainage was substantially reduced post-thoracic duct ligation. Over a period of 2 years and 7 months, patient recovered well and tolerated full oral diet. A comprehensive literature review was conducted and 4 reported cases were identified. Among these cases, three patients developed pericardial tamponade secondary to chylopericardium post-esophagectomy. CONCLUSION: Chylopericardium is a rare but serious complication post-esophagectomy. Prompt echocardiography and thorough pericardial fluid analysis are crucial for diagnosis. Thoracic duct ligation has been shown to be an effective management approach for this condition.

Idiopathic Chylous Pericardial Effusion Due to Lymphatic Malformation of Mediastinum.

Chylopericardium can be due to a variety of secondary causes like trauma, radiation, tumors, following cardiac surgery, etc., or may be idiopathic due to abnormal lymphatic system and mediastinal lymphangiectasia, which is a rare entity. Here, we present a case of a 34-year-old previously healthy male presenting with idiopathic chylopericardium. 2D echocardiography revealed massive pericardial effusion without features of cardiac tamponade. Following pericardiocentesis, a CT scan of the thorax and MR lymphangiogram were done to arrive at a diagnosis of idiopathic chylopericardium. In addition to medical management, surgical treatment included partial pericardiectomy and sclerotherapy of the mediastinal lymphatic sac. The patient had an uneventful post-operative period.

Primary idiopathic chylopericardium managed by uniportal VATS.

Chylopericardium is very rarely encountered in clinical practice. The common causes are post cardiac or thoracic surgery and neoplasms of the mediastinum. Most of the time, no cause is attributed and it is labelled as primary idiopathic chylopericardium. Conservative management is usually not successful and definitive surgery is required. The recommended surgery is creation of a pericardio-pleural window and thoracic duct ligation. We demonstrate that this procedure can be easily accomplished by uniportal video-assisted thoracic surgery (U-VATS).

Delayed chylopericardium after radical surgery for esophageal cancer: a case report.

Background: Postoperative chylpericardium is a rare clinical disease that often manifests as chest tightness, shortness of breathdyspnea, and other symptoms of pericardial tamponade. The etiological spectrum of chylopericardium is complex, but the disease is mainly idiopathic. Chylopericardium caused by thoracic surgery is rarely reported, both at home and abroad. Case summary: We report a case of a 65-year-old male patient who developed chylopericardium after thoracoabdominal combined incision and partial esophagogastric anastomosis plus lymph node dissection for 1 month. After pericardiocentesis and drainage, low-fat enteral nutrition, and parenteral nutrition, the patient was cured. Based on this case, this article reviews the literature on the diagnosis and treatment of chylopericardium after thoracic surgery. Conclusion: In conclusion, thoracic surgery (excluding cardiac surgery) can cause delayed chylopericardium. This condition is rarely reported in China, and only a few cases have been reported abroad. Thus, the diagnosis is likely to be missed or misdiagnosed. Early diagnosis and treatment are important to reduce patient discomfort as much as possible.

Challenges to manage primary chylopericardium in children.

Primary chylopericardium (PC) is a rare entity in the pediatric population with very few reported cases. Most cases of chylopericardium manifest after trauma or following cardiac surgery. The other etiologies which may lead to chylopericardium are malignancy, tuberculosis, or congenital lymphangiomatosis. We report two cases of PC in the pediatric population with contrasting outcomes. Both failed conservative management with dietary modification and octreotide. Surgery with pleuropericardial and pleuroperitoneal windows was performed in both. The first case had a thoracic duct ligation. The first patient died, and the second survived.

Computed tomography values of pericardial effusion may predict chylopericardium: a case report.

BACKGROUND: Idiopathic chylopericardium is a rare disease characterized by filling of the pericardial cavity with chylous fluid and has no evident cause. Secondary chylopericardium usually results from injury or damage to the thoracic duct. The most common causes of secondary chylopericardium are trauma, thoracic or cardiac surgery, and congenital lymphangiomatosis. Conservative or surgical treatment can be pursued; however, surgical treatment is required if conservative treatment is unsuccessful. Pericardiocentesis plays a crucial role in the definitive diagnosis of chylopericardium. However, although a serious complication, its occurrence is infrequent. Non-invasive methods, such as computed tomography (CT), could be useful in predicting the color or characteristics of pericardial effusion. CASE PRESENTATION: A 37-year-old Japanese woman presented to our hospital with a cough that persisted for 1 week. Echocardiography revealed pericardial effusion, which was diagnosed as acute pericarditis and treated with loxoprofen. However, pericardial effusion increased, and the patient presented to the emergency room with cardiac tamponade 1 month later. Pericardiocentesis was performed, which confirmed that the pericardial effusion was chylopericardium. Lymphatic scintigraphy did not show any connection between the thoracic duct and pericardial cavity, and the patient was diagnosed with idiopathic chylopericardium. The patient underwent continuous drainage for 11 days. After completion of cardiac drainage, the patient was discharged from the hospital without any exacerbation. The CT attenuation value of the pericardial fluid was 11.00 Hounsfield units (HU). Compared with the other causes of pericardial effusions encountered at our hospital, the HU on CT scan of pericardial effusion was low in our study and similar to the values on CT scan of chylous ascites reported in previous studies. CONCLUSIONS: Although idiopathic chylopericardium is rare, it should be considered an important cause of pericardial effusion. Pericardiocentesis is necessary for definitive diagnosis; however, the CT findings of pericardial effusion may help predict the presence of chylous fluid.

Chylothorax and chylopericardial tamponade following lobectomy and lymphadenectomy: a rare presentation.

BACKGROUND: Although postoperative chylothorax following lung cancer surgery is rare, it is a recognized complication in 0.25-3% of patients. However, cases of cardiac tamponade caused by chylopericardium after lung cancer surgery are extremely rare. CASE PRESENTATION: We describe hitherto unreported sequelae of chyle leak following lobectomy and systematic mediastinal lymph node dissection (SLND) causing pericardial tamponade and cardiovascular compromise. The patient was successfully treated with minimally invasive surgical repair and ligation. We also discuss the development of chylopericardium as a potential complication of lobectomy and SLND. CONCLUSIONS: The anatomical characteristics of the thoracic duct warrant special attention in postoperative chyle leak management in patients who undergo definitive mediastinal lymph node dissection. Surgeons should be aware that chylopericardium is a rare but potential complication of lobectomy and SLND as it may help with early diagnosis, management, and prevention of cardiac tamponade.

Chylopericardium or contamination by injectable lipid emulsion? / « Mets la main sur mon cœur, Et vois comme il se trouble ¼ ­ un pseudo-chylopéricarde ?

Effusions can show some surprises. We document the case of a fourteen-month-old male patient with short-bowel syndrome, hospitalized in a cardiology unit, receiving a chronic parenteral nutrition by a Broviac® catheter. The patient presented several thrombosis following iterative catheter replacements. In parallel with superior vena cava plasty, a right intra-atrial Broviac® catheter was placed in the absence of other peripheral venous accesses. This device has a cutaneous exit site to allow for infusion of a hyperosmolar lipid emulsion. Seven days later, a milky liquid was secreted from pericardial/mediastinal redon. A gel lipoprotein electrophoresis of the fluid suggested a preliminary diagnosis of chylopericardium. However, biochemical testing of certain analytes evoked a parenteral nutrition-related pericardial effusion and a possible pseudochyloperitoneum caused by the shearing of a migrated Broviac® in pericardium. The patient, on a fat-free diet, was admitted to the ICU to drain the effusion and reposition the catheter, with success. In the light of new datas on the interference of parenteral lipid emulsions with the lipoproteins gel electrophoresis, we will try to determine whether the apparent presence of chylomicrons in the gel would be the sign of a lesion of the lymphatic system, or rather the result of a contamination by artificial chylomicron in the lipid emulsion, if not the sign of contaminated blood. In our article, we highlight several considerations in identifying and confirming cases of pericardial effusion, such as chylopericardium and parenteral nutrition-related one, as well as points concerning the use of lipid emulsions for pediatric patients with short-bowel syndrome.

Les liquides d'épanchements peuvent renfermer quelques surprises. Nous documentons le cas d'un patient de quatorze mois, hospitalisé en cardiologie, présentant un syndrome de grêle court et recevant une nutrition parentérale au long cours par cathéter Broviac®. Le patient présentait de multiples occlusions veineuses consécutives aux changements itératifs du dispositif. En parallèle d'une plastie de la veine cave supérieure, un Broviac® a été posé en intra-atrial droit devant l'absence d'autres abords veineux périphériques. Ce dispositif comporte un orifice de sortie sous-cutané pour apporter une solution de nutrition hyperosmolaire de type émulsion lipidique. Le liquide recueilli dans les drains péricardiques en post-opératoire est lactescent, particulièrement à partir du septième jour. Le lipidogramme du liquide d'épanchement péricardique semble conclure à la présence de chylomicrons - un chylopéricarde. Cependant, le dosage de certains analytes penche en faveur d'un perfusopéricarde, probablement pseudochyleux, lié au cisaillement du Broviac® dont l'extrémité a migré de l'oreillette droite au péricarde. Le patient, sous régime sans graisses, sans nutrition parentérale, sera réopéré pour drainer l'épanchement et repositionner le cathéter, avec succès. À la lumière de données originales quant à l'interférence des émulsions lipidiques sur le lipidogramme, nous tâcherons de déterminer si l'apparente présence de chylomicrons sur le gel serait le témoin d'une réelle lésion du lymphatique, ou plutôt le fruit d'une contamination par l'émulsion, si ce n'est par le sang. Des considérations au sujet des épanchements péricardiques, dont les chylopéricarde et nutripéricarde, ainsi que sur les émulsions lipidiques pédiatriques dans le contexte du grêle court émailleront ce travail.

Case Report: Progressive central conducting lymphatic abnormalities in the RASopathies. Two case reports, including successful treatment by MEK inhibition.

The RASopathies are a group of genetic conditions resulting from mutations within the RAS/mitogen-activated protein kinase (RAS-MAPK) pathway. Lymphatic abnormalities are commonly associated with these conditions, however central conducting lymphatic abnormalities (CCLA) have only recently been described. CCLAs may be progressive and can result in devastating systemic sequelae, such as recurrent chylothoraces, chylopericardium and chylous ascites which can cause significant morbidity and even mortality. Improvements in imaging modalities of the central lymphatics have enhanced our understanding of these complex abnormalities. Management is challenging and have mainly consisted of diuretics and invasive mechanical drainages. We describe two adult males with Noonan syndrome with a severe and progressive CCLA. In one patient we report the therapeutic role of targeted molecular therapy with the MEK inhibitor 'Trametinib', which has resulted in dramatic, and sustained, clinical improvement. The successful use of MEK inhibition highlights the importance of understanding the molecular cause of lymphatic abnormalities and utilising targeted therapies to improve quality of life and potentially life expectancy.

Infected chylopericardium: An unexpected cause of cardiac tamponade.

A 22-year-old immunocompetent female with a history of small pericardial effusion while infant presented with fever and hemodynamic collapse 4 days after facial trauma. She was found to have cardiac tamponade secondary to infected chylopericardium from bacterial translocation. We report this very unusual case and review of the literature on chylopericardium infections.

Isolated primary chylopericardium: A rare case report and literature review.

INTRODUCTION: Primary chylopericardium is an extremely rare disease with no previous reported case in Indonesia. It may be caused by an abnormality of lymphatic drainage in the thoracic region. CASE PRESENTATION: We report the case of a 19-year-old female who presented with a chief complaint of shortness of breath and fluid accumulation in her pericardial sac. Screening for autoimmune disease, tuberculosis, and malignancy all came back negative. Lymphoscintigraphy showed suspected leakage or obstruction of lymph at the supradiaphragmatic level. The patient was diagnosed with primary chylopericardium. In a span of 1 year, she had undergone 3 pericardiocentesis procedures and declined surgical treatment. On her fourth pericardiocentesis, the cardiologists were unable to remove fluid from the pericardial sac. Thoracic duct ligation and pericardial window were eventually performed and a total of 850 ml of chylous fluid was removed. At 6 months postoperation patient showed no signs and symptoms of pericardial effusion and was in good condition. DISCUSSION: Constrictive pericarditis can occur as a complication of chronic inflammation in the pericardium due to the presence of chylopericardium and a history of multiple pericardiocenteses. However, there are no reports regarding the exact duration of chylopericardium that will lead to constrictive pericarditis. Difficulty in pericardiocentesis might be an early sign of scarring in the pericardium. CONCLUSION: Delayed surgical treatment of chylopericardium increases the risk of constrictive pericarditis and significantly reduces the patient's quality of life due to recurrent symptoms and hospitalizations.

Successful surgical correction of recurrent primary chylopericardium due to mediastinal lymphangiomyoma by total pericardiectomy and diaphragmatic fenestration.

We present a rare case of hemodynamically significant recurrent primary chylopericardium due to a rare lymphatic malformation, mediastinal lymphangiomyoma, treated by complete surgical resection with total pericardiectomy. Recurrent chylopericardium or chylothorax, a common complication following the primary intervention, was successfully treated by diaphragmatic fenestration technique which is as good as thoracic duct ligation.

Review of chylopericardium.

Chylopericardium is a rare pathologic condition consisting of the accumulation of excess amounts of chylous fluid within the pericardial cavity. Most patients are asymptomatic at presentation; however, chest pressure, chest pain and lightheadedness have been reported, and the most common presenting symptom is shortness of breath. Patients are noted to have enlargement of the cardiac silhouette on routine chest radiograph, and evidence of a pericardial effusion on echocardiography. The diagnosis is only definitively confirmed with pericardiocentesis and fluid analysis. The fluid is typically turbid white or milky in appearance, with a triglyceride level in excess of 500 mg/dL. The mechanism by which chyle accumulates within the pericardium is believed to be secondary to abnormal or damaged lymphatics or due to elevated pressure within the thoracic duct that results in chyle reflux into the pericardium. Following drainage with a pericardiocentesis or pericardial drain, attempts at conservative therapy with nothing by mouth and parental nutrition can be made, but have a high rate of failure and subsequent reaccumulation of chyle. Surgical treatment provides the most definitive management and consists of ligation of the thoracic duct just above the level of the diaphragm and creation of a pericardial window. With this treatment, risk of recurrence is incredibly low (<5%).

Chylotamponade in Pediatric Primary Mediastinal Large B-Cell Lymphoma.

Chylotamponade involves rapid accumulation of chyle in the pericardium elevating the pericardial pressures above normal right heart filling pressures, and is extremely rare. A 12-y-old boy presented to the emergency with complaints of facial puffiness for 1 mo and breathing difficulty for 1 wk. The neck veins were distended, and the heart sounds were muffled. A chest CT demonstrated a large anterior mediastinal mass with pleural and pericardial effusions. Echocardiography confirmed cardiac tamponade. Pericardiocentesis revealed chylopericardium. He was placed on a chyle leak diet, and the drain was removed after 48 h. Biopsy of the mediastinal mass revealed a primary mediastinal B-cell lymphoma. He was successfully managed with chemotherapy. The index case demonstrates how prompt identification and management of chylotamponade and treatment of the underlying cause can lead to good clinical outcomes.

Successful Treatment of Chylothorax and Chylopericardium by Radiotherapy in Lung Cancer.

A 58-year-old man was diagnosed with stage IVB lung adenocarcinoma in the right upper lobe and underwent systemic chemotherapy. Seven months after the diagnosis, large left pleural and pericardial effusion was detected. The patient developed both chylothorax and chylopericardium following superior vena cava (SVC) obstruction with mediastinal lymphadenopathy caused by lung carcinoma. Since conservative treatment of the chyle leakage was ineffective, we administered radiotherapy to treat the SVC obstruction and mediastinal lymphadenopathy. After radiotherapy, the chylothorax and chylopericardium gradually resolved, and no further chyle leaks were identified on follow-up computed tomography. This case indicates that radiotherapy can be used to ameliorate lung cancer-related chylothorax and chylopericardium.