Quilopericardio espontáneo en edad pediátrica ¿hay un algoritmo de manejo idóneo? / Spontaneous chilopericard in pediatric age is there an ideal management algorithm?

Introducción: El quilopericardio es la presencia de líquido quiloso en el espacio pericárdico. Las causas más frecuentes de esta rara entidad son secundarias a cirugía y otras técnicas invasivas sobre el tórax, seguidas de las idiopáticas, aunque también se han asociado a anomalías congénitas del sistema linfático, linfangiomas y otros tumores mediastínicos, tuberculosis, etc. Su aparecimiento es más común en los adultos, pero últimamente se han descrito casos por igual entre la población pediátrica y los adultos. Casos Clínicos: Se reporta la presencia de dos pacientes de 8 y 11 meses de edad que se presentan con importante cantidad de líquido a nivel del pericardio con compromiso de cámaras de cardiacas derechas en quienes la pericardiocentesis realizada revela quilo sin una asociación patológica franca. Distintos manejos con un solo fin, alcanzar los tratamientos idóneos para cada caso. Resultados: Se lograron identificar de nuestro registro quirúrgico en el periodo descrito dos pacientes. El primero correspondía a un varón, de 11 meses de vida, sin antecedentes de importancia, con evidencia de derrame pericárdico quiloso de aparecimiento espontáneo que no cedió a las medidas conservadoras instauradas y posteriormente fue a cirugía para ligadura del conducto torácico y pericardiectomía parcial. El segundo es un paciente valorado recientemente en el área de Pediatría, varón, de 8 meses, con Síndrome de Down que acude a emergencia de nuestro hospital por presencia de disnea, malestar general e intolerancia alimentaria. La radiografía de tórax realizada reveló la presencia de cardiomegalia y al realizar un ecocardiograma transtorácico complementario se encuentra derrame pericárdico severo con signos de taponamiento cardiaco. Se realizó pericardiocentesis diagnóstica y evacuatoria con un catéter central pediátrico obteniéndose líquido seroso y drenando 35 cc, pero tres días luego de la punción se aprecia la salida de líquido quiloso por el catéter mantenido en el espacio pericárdico. Se condiciona manejo conservador por un lapso de 7 días. El resto de la revisión bibliográfica logra identificar 18 pacientes pediátricos con presencia de derrame pericárdico quiloso de origen espontáneo con diferentes manejos ofertados. Discusión y Conclusiones: El quilopericardio es una patología de raro aparecimiento, las principales etiologías definen a pacientes que han sido llevados a cirugía a nivel cardiaco o torácico. La descripción más categórica está dada para la edad adulta pero en la edad pediátrica (menores de 18 años) no se ha descrito un manejo consensuado aún por la variabilidad de su diagnóstico, sin embargo sigue incierto el manejo tras su aparecimiento en los que debutan con quilopericardio primario o de origen espontáneo, pero a pesar de ello la implementación de medidas conservadoras como la nutrición enteral con ácidos de grasos de cada media y uso de nutrición parenteral entre 7 a 15 días; y tras el fracaso de estas medidas, la cirugía con ligadura del conducto torácico por toracotomía derecha más confección de ventana pleuropericárdica o pericardiectomía parcial constituyen las opciones de manejo más acertado que hasta el día de hoy se conocen con buenos resultados a corto y mediano plazo

Introduction: Chylopericardium is the presence of chylous fluid in the pericardial space. The most frequent causes of this rare entity are secondary to surgery and other invasive techniques on the thorax, followed by idiopathic ones, although they have also been associated with congenital anomalies of the lymphatic system, lymphangiomas and other mediastinal tumors, tuberculosis, etc. Its appearance is more common in adults, but lately cases have been described equally between the pediatric population and adults. Cases Reports: The presence of two patients of 8 and 11 months of age who present with significant amount of fluid at the level of the pericardium with involvement of right cardiac chambers in whom the pericardiocentesis performed reveals chyle without a frank pathological association is reported. Different managements with a single purpose, to achieve the ideal treatments for each case. Results: Two patients were identified from our surgical registry in the period described. The first corresponded to a male, of 11 months of age, without significant antecedents, with evidence of chylous pericardial effusion of spontaneous appearance that did not yield to the established conservative measures and later went to surgery for ligation of the thoracic duct and partial pericardiectomy. The second is a patient recently evaluated in the area of pediatrics, male, of 8 months, with Down Syndrome who comes to emergency of our hospital due to the presence of dyspnea, general malaise and food intolerance. The chest X-ray revealed the presence of cardiomegaly and a complementary transthoracic echocardiogram revealed severe pericardial effusion with signs of cardiac tamponade. Diagnostic and evacuation pericardiocentesis was performed with a pediatric central catheter, obtaining serous fluid and draining 35 cc, but three days after the puncture, the exit of chylous fluid through the catheter maintained in the pericardial space was observed. Conservative management is conditioned for a period of 7 days. The rest of the literature review identifies 18 pediatric patients with the presence of a spontaneously occurring pericardial effusion with different treatments offered. Discussion and Conclusions: Chylopericardium is a pathology of rare appearance, the main etiologies define patients who have been taken to cardiac or thoracic surgery. The most categorical description is given for adulthood but in the pediatric age (under 18 years) has not been described a consensus management yet for the variability of its diagnosis, however management remains uncertain after its appearance in those who debuted with primary chylopericardium or spontaneous origin, but despite this the implementation of conservative measures such as enteral nutrition with fatty acids of each media and use of parenteral nutrition between 7 to 15 days; and after the failure of these measures, surgery with thoracic duct ligation by right thoracotomy plus pleuropericardial window confection or partial pericardiectomy are the most successful management options that are known to date with good results in the short and medium term

Quilopericardio secundario a cirugía cardíaca / Chylopericardium after cardiac surgery

Introducción: El quilopericardio post cirugía cardíaca en el adulto es extremadamente infrecuente. Su aspecto lechoso característico y análisis bioquímico permiten el diagnóstico. El objetivo es presentar una complicación excepcional de la cirugía cardíaca, su fisiopatología y tratamiento médico. Presentación del caso: Mujer de 61 años, con historia de disnea progresiva, ortopnea y disnea paroxística nocturna de 12 años de evolución. Se pesquisó en ecocardiograma comunicación interauricular (CIA) tipo ostium secundum con shunt izquierda-derecha, disfunción ventricular, dilatación de cavidades derechas, insuficiencia tricuspídea masiva e hipertensión pulmonar grave. Se discutió caso y se decidió resolución quirúrgica. Se abordó por esternotomía media, y en circulación extracorpórea, se realizó, cierre de CIA con parche de poliéster y plastía tricuspídea. Al tercer día postoperatorio presentó aumento de débito pericárdico con líquido de aspecto lechoso. Análisis bioquímico permitió realizar el diagnóstico de quilopericardio. Se indicó tratamiento conservador con nutrición parenteral total y posterior uso de octreotide a lo largo de aproximadamente un mes. La paciente respondió a la terapia y fue dada de alta, no requiriendo de reintervención. Ecocardiograma de control mostró ausencia de derrame pericárdico. Al mes de seguimiento la paciente se encontró asintomática en capacidad funcional I. Discusión: La cirugía cardíaca es la causa del 9% de los casos de quilopericardio en adultos. Se han descrito diversas etiologías que van desde la lesión directa del conducto torácico hasta la asociación con la hipertensión pulmonar. En el caso presentado, el tratamiento con medidas nutricionales y octreotide fue efectivo evitando una nueva exploración quirúrgica.

Introduction: Adult post cardiac surgery chylopericardium is extremely rare. Its characteris- tic milky look and biochemical analysis allow diagnosis. The objective is to present a rare complication of cardiac surgery, its pathophysiology and treatment. Case report: A 61 years old woman, with a history of progressive dyspnea, orthopnea and paroxysmal nocturnal dyspnea of 12 years of evolution. Echocardiography found an ostium secundum atrial septal defect (ASD) with left-right shunt, ventricular dysfunction, dilated right chambers, massive tricuspid regurgitation and severe pulmonary hypertension. The case was discussed and surgical resolution was decided. A median sternotomy, ASD closure polyester patch and tricuspid plasty was performed. On the third postoperative day presented increased pericardial debit with milky liquid. Biochemical analysis allowed the diagnosis of chylopericardium. Conservative treatment was decided with total parenteral nutrition and subsequent use of octreotide over about one month. The patient responded well to treatment and was discharged, not requiring reoperation. Control echocardiogram showed no pericardial effusion. One-month follow-up the patient was asymptomatic and in functional capacity I. Discussion: Cardiac surgery is the cause of 9% of cases chylopericardium in adults. Various etiologies are described ranging from direct injury to the thoracic duct to the association with pulmonary hypertension. In the case presented, nutritional therapy and octreotide were effective, avoiding a new surgical exploration.

Chylopericardium after Cardiac Surgery

Chylopericardium after intra-thoracic surgery is rare. Its incidence is reported to be between 0.22% and 0.5%[1, 2] following paediatric cardiac surgery, but is not quantified following cardiac surgery in the adult population. A delay in diagnosis can lead to serious consequences with tamponade and death[3]. Chronic lymph leak can also lead to immunosuppresion, hypoproteinemia and malnutrition[3]. The majority of published literatures on this condition after cardiac surgery are in children. There are few reports of chylopericardium in adults following coronary artery bypass grafting and valvular surgery[4–6], and these advocate treatment with either total parental nutrition or surgical intervention. We report on the case of chylopericardium after a trial. Septal defect surgical closure in an adult patient treated successfully by oral dietary manipulation.

Quilopericardio y quilotórax bilateral en una mujer con cáncer de mama / Chylopericardium and bilateral chylothorax in a breast cancer patient

El quilopericardio no traumático suele deberse a enfermedades infecciosas, congénitas o neoplásicas que infiltran los ganglios mediastinales, alteran el flujo linfático normal y acumulan quilo en la cavidad pericárdica. Se asocia a quilotórax en, aproximadamente, un 3% de los casos. Se presenta el caso de una paciente de 52 años con cáncer de mama avanzado que ingresa con derrame pleural bilateral y signos ecocardiográficos de taponamiento cardíaco. La TC mostró múltiples adenopatías en mediastino. El dosaje de triglicéridos en líquido pleural fue 372 mg/ dl. Por pericardiocentesis se obtuvo un líquido lechoso con triglicéridos de 984 mg/dl y colesterol 90 mg/dl. Se modificó el esquema terapéutico. Se revisan la fisiopatología, los criterios diagnósticos y el tratamiento de esta rara entidad.

Non traumatic chylopericar dium is mostly secondary to infection, congenital or neoplastic disease that invade mediastinal lymph nodes and modify the normal lymphatic flow. It is associated to chylothorax in approximately 3% of cases. We report the case of a 52 years old woman with diagnosis of advanced breast cancer. She was admitted with bilateral pleural effusion and echocardiographic signs of cardiac tamponade. A CT scan disclosed multiple mediastinal lymphadenopathy. The level of tryglicerides in pleural effusion was 372 mg/dl. A percutaneous pericardiocentesis was performed, obtaining chyle, with 984 mg/dl of tryglicerides and cholesterol 90 mg/dl levels. Treatment strategy was modified. We reviewed pathophysiology, diagnostic criteria and treatment of this rare entity.

Isolated Chylopericardium after Mitral Valve Replacement: the First Description of Adult Heart Disease in Korea / 대한구급학회지

Isolated chylopericardium as a complication of cardiac surgery is very rare. Two cases of chylopericardium have been previously reported in Korea; both patients suffered from chylopericardium after a corrective cardiac surgery for a congenital heart disease such as atrial or ventricular septal defect. We report a case of chylopericardium in a 55-year-old mitral valve replacement patient. The reason for chylopericardium was unclear, but it might have been related with the damaged lymph nodes and blunt dissection of the thymus. While most chylopericardium cases require surgical intervention, we managed this chylopericardium case with a low-fat diet for 3 days.

Chylopericardial Tamponade in a Patient with Chylothorax after Pulmonary Lobectomy / 대한구급학회지

Chylopericardium is a very rare, yet potentially fatal, complication following intrathoracic surgery, and can further lead to other life-threatening complications such as cardiac tamponade. A 54-year-old female underwent right upper lobectomy for lung cancer. Chylothorax developed on the 2nd postoperative day, and was managed conservatively with dietary modification. On the 9th postoperative day, the patient suddenly developed hypotension and severe cardiac dysfunction requiring cardiopulmonary resuscitation followed by VA ECMO. Transthoracic echocardiography revealed a large amount of pericardial effusion. Prompt pericardiocentesis was performed and the aspirated fluid showed features of chyle. Thoracic duct ligation with pericardial window operation was performed because the daily amount of chyle drained did not decrease after 3 weeks. Here, we review etiologies and therapeutic options of chylopericardial tamponade following intrathoracic surgery, which should not be underestimated even when the patient seems to demonstrate a good recovery.

Chylopericardium Secondary to Lymphangiomyoma - A case report -

Chylopericardium is a rare disease entity characterized by the accumulation of chylous fluid in the pericardial sac. It usually arises from mediastinal neoplasms, thrombosis of the subclavian vein, tuberculosis, nonsurgical trauma, thoracic or cardiac surgery. The spectrum of symptoms for chylopericardium varies from an incidental finding of cardiomegaly to dyspnea, upper abdominal discomfort, cough, chest pain, palpitation, fatigue. However, most of the patients are asymptomatic. The main purpose of treatment of chylopericardium is the prevention of cardiac tamponade and prevention of metabolic, nutritional, and immunological compromise due to chyle leak. Here, we report a case of chylopercardium secondary to lymphangiomyoma with review of the literature.

Quilopericardio secundario a cirugía cardiovascular / Chylopericardium secondary to cardiovascular surgery

El quilopericardio constituye una complicación rara de la cirugía de defectos cardíacos congénitos. Es causada por la obstrucción mecánica del drenaje del conducto torácico o sus tributarios linfáticos en la vena subclavia o por daño quirúrgico. Se reporta el caso de un paciente de dos meses de vida con diagnóstico de ventrículo izquierdo hipoplásico, en quien se encontró quilopericardio posterior a dos intervenciones quirúrgicas, y recibió tratamiento exitoso con drenaje pericárdico y sustitución de las grasas de la dieta por triglicéridos de cadena media.

Chylopericardium is a rare complication after congenital heart disease surgery. It is caused by mechanical obstruction of the thoracic duct drainage or its lymphatic tributaries to the subclavian vein or by direct surgical injury. We report the case of a two month-old boy with hypoplastic left ventricle diagnosis who developed chylopericardium secondary to two cardiac surgeries and who underwent a successful pericardial drainage procedure. Dietary fats were substituted by medium chain triglycerides.

Quilopericárdio idiopático primário: relato de caso / Quilopericardio idiopático primario: informe de caso clínico / Primary idiopathic chylopericardium: case report

O acúmulo de quilo no espaço pericárdico ou quilopericárdio é uma condição que, com maior frequência, ocorre após trauma, cirurgia cardíaca e torácica ou associado a tumores, tuberculose ou linfoangiomatose. Quando não é possível a identificação precisa da etiologia, o quilopericárdio é denominado primário ou idiopático. Essa é uma situação clínica rara. Descrevemos um caso em paciente do sexo feminino, com 20 anos de idade, tratada cirurgicamente. A propósito do caso, apresentamos breve revisão da literatura e comentários sobre quadro clínico, etiopatogenia, exames diagnósticos complementares e opções de tratamento.

The accumulation of chyle in the pericardial space, or chylopericardium, is a condition occurring most frequently after trauma, cardiac and thoracic surgery, or in association with tumors, tuberculosis or lymphangiomatosis. When its precise cause cannot be identified, it is called primary or idiopathic chylopericardium. This is a rare clinical entity. We report the case of a surgically treated 20-year-old female patient. A brief review of the literature and comments on the clinical presentation, etiopathogenesis, ancillary diagnostic tests and treatment options are also presented.

La acumulación de quilo en el espacio pericárdico o quilopericardio es una condición que con mayor frecuencia ocurre después de trauma, cirugía cardíaca y torácica o asociado a tumores, tuberculosis o linfoangiomatosis. Cuando no es posible la identificación precisa de la etiología, el quilopericardio se denomina primario o idiopático. Esta es una situación clínica rara. Describimos un caso en paciente del sexo femenino, con 20 años de edad, tratada quirúrgicamente. A propósito del caso, presentamos una breve revisión bibliográfica y comentarios sobre el cuadro clínico, la etiopatogenia, exámenes diagnósticos complementarios y opciones de tratamiento.

A Case of Chylopericardium Secondary to the Superior Vena Cava Syndrome in Behcet's Disease / 대한류마티스학회지

Behcet's disease (BD) is a chronic inflammatory disorder associated with oral aphthous ulcer, genital ulcer and uveitis. Vascular lesions in BD can affect all types and sizes of vessels. The venous thrombosis, which is uncommon feature of other vasculitis, is relatively common clinical manifestation of BD. Sometimes the cardiovascular involvement in BD results in serious complications as the leading cause of morbidity and mortality. We report a 41-year-old male patient who suffered BD and presented massive chylopericardium due to the superior vena cava (SVC) syndrome. After thrombectomy of SVC, the highly productive left-sided chylothorax and restenosis of anastomosis site at SVC had occurred, which were successfully treated by the ligation of the thoracic duct and balloon-stent angioplasty. We discussed the mechanism and treatment of chylopericardium in SVC syndrome, and the possible complications after the surgical management.

Primary Idiopathic Chylopericardium Associated with Cervicomediastinal Cystic Hygroma

Chylopericardium is a rare clinical entity in which chylous fluid accumulates in the pericardial cavity. We report a case of primary idiopathic chylopericardium associated with multiple, small cervicomediastinal cystic hygromas occurring in an asymptomatic 43-year-old woman with no history of trauma, thoracic surgery, malignancy, infection or tuberculosis. Echocardiography showed a large amount of pericardial effusions and pericardial fluid analysis revealed inappropriately elevated triglyceride. We did not demonstrate communication between the thoracic duct and the pericardial sac by lymphangiography and chest computed tomography. She successfully responded to 30 days of continuous pericardial drainage and 15 days of a medium-chain triglyceride diet after 30 days of total parenteral nutrition. Follow-up echocardiography 6 months after treatment commencement showed a minimal reaccumulation of pericardial fluid without symptom. We conclude that if a patient is asymptomatic and can well tolerate daily life, surgery including pericardiectomy or ligation of the thoracic duct is not necessarily required.

Isolated spontaneous chylopericardium during the treatment of non-Hodgkin's lymphoma / 대한내과학회지

Although malignant pericardial effusion is a common complication of malignancy, chylopericardium is a rare entity characterized by the accumulation of chylous fluid in the pericardial sac. The lymphatic vessels of the pericardium drain into the thoracic duct. The mechanical obstruction of venous drainage plays an important role in the pathophysiology of this disease. In most cases, pericardiocenthesis is needed to prevent cardiac tamponade. We present a case of a 15-year-old boy diagnosed as non-Hodgkin's lymphoma, diffuse large B cell, with superior vena cava syndrome, who developed an isolated chylopericardium after the first cycle of chemotherapy. We confirmed the diagnosis with an echocardiography, lymphangiography, and fluid analysis. Chylopericardium was managed successfully with pericardiocenthesis and drainage. If pericardial effusion were developed during the treatment of non-Hodgkin's lymphoma, chylopericardium should be considered as one of the differential diagnoses and associated venous thrombosis around the thoracic duct outlet should be ruled out.