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

Congenital chylous ascites: a case report

Due to the rarity of congenital chylous ascites and the lack of standards in diagnosis and therapy, this disease constitutes a medical challenge and individual therapy seems to be extremely important. A late preterm newborn with antenatally diagnosed ascites was born and chylous ascites was diagnosed after feeds were started. The baby was managed initially with nil per oral, parenteral nutrition and octreotide, followed by adding MCT formula feeds. Considering the rarity of neonatal chylous ascites and the non-uniformity in management plans and follow up, more case reports need to be published. Also, MCT formula, the main stay of management has to be discontinued as soon as possible with gradual introduction into breast feeds or normal newborn formula milk as long chain fatty acids are essential for optimal brain growth in newborns.

Very-long chain acyl coenzyme A dehydrogenase deficiency / 中国实用儿科杂志

Very long chain acyl-CoA dehydrogenase deficiency(VLCADD)is a disorder involving the initial step of fatty acid beta-oxidation in the mitochondrial matrix. VLCADD can present at various ages,from the neonatal period to adulthood,with symptoms including hypoglycemia,rhabdomyolysis,skeletal muscle weakness and cardiomyopathy,and poses the greatest risk of complications during intercurrent illness or after prolonged fasting. Early diagnosis,treatment,and surveillance can reduce mortality. The most common diagnostic evaluation methods are plasma acylcarnitine profiles and ACADVL gene molecular testing. Functional testing,including white blood cell or fibroblast enzyme assay,is a useful diagnostic adjunct if molecular sequencing alone is insufficient to deter-mine the diagnosis or uncharacterized mutations are identified. Treatment emphasizes the avoidance of fasting and often includes a specialized diet that is high carbohydrate/low longchain fat which is supplemented by medium chain triglycerides(MCT).very-long chain acyl coenzyme A dehydrogenase

How to select lipid emulsions for children with critical illness / 中国小儿急救医学

Intravenous lipid emulsions( LEs) are relevant for patients receiving parenteral nutrition because they prevent the depletion of essential fatty acids(FAs) and as a highly dense energy source. The prescription of LEs is complex,due mainly to their distinct FAs components,which may alter the immune re-sponse in different ways and distinctly influence inflammation according to their biochemical properties. The patient′s metabolic profile should guide the type of FAs and amount of lipids that are provided. For critically ill hypermetabolic patients,growing evidence indicates that standard LEs based solely on soybean oil should be avoided in favor of new LEs containing medium-chain triglycerides,olive oil,or fish oil to decrease the provision of potentially inflammatory/immunosuppressiveω-6 polyunsaturated fatty acids. As sources of eico-sapentaenoic and docosahexaenoic acids,LEs containing fish oil may be important for critically ill patients be-cause they allow better modulation of the immune response and likely reduce the length of intensive care unit stay. However,current evidence precludes the recommendation of a specific LE for clinical use in this patient population.

Current application and indications of dietary medium-chain triglycerides in hospitalized patients: an investigation and evaluation / 中华临床营养杂志

Objective To analyze the current dietary application of medium-chain triglycerides (MCT) in hospitalized patients,and clarify the appropriate indications and the role of MCT in clinical practice through evidence-based evaluation.Method A retrospective analysis was conducted on 46 cases of hospitalized patients who were administered dietary MCT between January,2012 and December,2013 in Peking Union Medical College Hospital.Results Dietary MCT were utilized in a small but increasing number of patients,who distributed in departments of gastroenterology,pediatrics,pneumology,general internal medicine,and general surgery.37.0％ of the patients had malnutrition,while 56.5％ had hypoalbuminemia.The leading indications of MCT therapy were gastrointestinal dysfunction (21 cases) and lymph circulation disorder (15 cases),while dyslipidemia (5 cases),exocrine pancreatic insufficiency (4 cases),and intractable epilepsy (1 case) served as other indications of MCT administration.Conclusions The indications of current dietary MCT application are approximately consistent with evidence from literature.However,given the scarcity of relevant researches and limited clinical application,further clinical researches with larger samples on dietary MCT are needed to provide more high-quality evidences for guidance of clinical practice.

Efeitos da suplementação dietética com óleo de coco no perfil lipídico e cardiovascular de indivíduos dislipidêmicos / Effects of dietary supplementation with coconut oil on lipid and cardiovascular profile of dyslipidemic subjects

Objetivo. Avaliar o efeito da suplementação dietética com óleo de coco extravirgem no perfil lipídico e cardiovascular de indivíduos hipercolesterolêmicos. Método. Trata-se de uma pesquisa de intervenção realizada no ambulatório de cardiologia de Valparaíso de Goiás. A amostra foi composta de 32 pacientes, hipercolesterolêmicos, 50% do sexo feminino, idade média de 48 anos. Todos os pacientes foram suplementados com 30 mL/dia de óleo de coco extravirgem durante três meses. Analisou-se o peso corporal, o índice de massa corporal, o perímetro abdominal, a relação abdômen-quadris, o consumo alimentar (recordatório de 24 horas), assim como lipidograma completo, glicemia de jejum, apolipoproteínas (apo) A-I e B, proteína C reativa ultrassensível (PCR-us), lipoproteína (a) [Lp(a)] e fibrinogênio antes e depois da suplementação. Usou-se, para análise dos dados, o teste estatístico t de Student, e considerou-se significância de 5%. Resultados. Após a suplementação com óleo de coco, observou-se redução significativa do peso, índice de massa corporal, relação abdômen-quadris, perímetro abdominal, triglicérides, lipoproteína de muito baixa densidade (VLDL-c) e PCR-us, bem como aumento significativo nas concentrações de apo A-I. Observou-se, também, tendência à redução do colesterol total, lipoproteína de baixa densidade (LDL-c) e Lp(a), assim como um ligeiro aumento de lipoproteína de alta densidade (HDL-c) e fibrinogênio, porém esses resultados não foram significativos. Conclusão. Os resultados sugerem que a suplementação dietética com óleo de coco extravirgem é capaz de exercer benefícios no perfil lipídico e cardiovascular de indivíduos dislipidêmicos.

Objective. To evaluate the effect of dietary supplementation with extra virgin coconut oil on lipid profile and cardiovascular dyslipidemic subjects.Method. This is an intervention research conducted in a cardiology clinic of Valparaiso de Goiás. The sample consisted of 32 patients with hypercholesterolemia, 50% female, mean age 48 years. All patients were supplemented with 30 mL/day coconut oil for three months. The authors analyzed body weight, body mass index, abdominal perimeter and abdomen-hip ratio, dietary intake (24 hour recall), as well as full lipid profile, fasting glucose, apolipoprotinein AI and B, high-sensitivity C-reactive protein, lipoprotein (a) [Lp (a)] and fibrinogen before and after supplementation. The t-Student test with 5% significance was used.Results. After supplementation with coconut oil, there was significant reduction in weight, body mass index, abdomen hip ratio, abdominal perimeter, triglycerides, very low density lipoprotein (VLDL-c), and high-sensitivity C-reactive protein, as well as significant increase in the levels of apo A-I . There was also a trend towards a reduction in total cholesterol, low-density lipoprotein (LDL-C) and Lp (a), as well as a slight increase in high density lipoprotein (HDL-C) and fibrinogen, but these results were not significant. Conclusion. The results suggest that dietary supplementation with extra virgin coconut oil is able to exert ?benefits on lipid profile and cardiovascular hypercholesterolemic subjects. However, randomized controlled trials are needed.

Post- Intercostal Intubation Chylothorax.

Chylothorax is a rare condition in childhood . It usually occurs as a post operative complication following cardiac surgery for heart diseases . we report a 16 months old girl with atrial septal defect and severe pulmonary stenosis, hospitalised for empyema and who developed chylothorax following intercostal drainage, an entity which has never been reported.

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.

Influência da fisioterapia complexa descongestiva associada à ingestão de triglicerídeos de cadeia média no tratamento do linfedema de membro superior / Influence of complex descongestive physical therapy associated with intake of medium-chain triglycerides for treating upper-limb lymphedema

OBJETIVO: Verificar a influência da utilização da fisioterapia complexa descongestiva associada à dietoterapia com triglicerídeos de cadeia média (TCM) como forma de intervenção no linfedema de membro superior (MS). MÉTODOS: Para a avaliação do linfedema, foram utilizadas cirtometria, volumetria, pregas cutâneas e quantidade de água corporal total. A Escala Visual Análoga (EVA) foi utilizada para avaliar as sensações de desconforto, peso e dor no MS. Participaram deste estudo dez mulheres mastectomizadas com linfedema de MS homolateral à cirurgia, com idade média de 65,9 ± 10,4 anos e índice de massa corpórea (IMC) de 26,8 ± 3,0kg/m² que, após avaliação nutricional, foram divididas aleatoriamente em dois grupos: Grupo Controle (n= 5), submetido ao tratamento fisioterapêutico constando da terapia complexa descongestiva (massagem clássica, drenagem linfática manual, bandagem compressiva e cuidados com a pele) três vezes na semana, durante quatro semanas; Grupo TCM (n= 5), submetido ao mesmo protocolo fisioterapêutico somado ao tratamento dietético diário com ingestão de TCM, por quatro semanas. RESULTADOS: Ao final da intervenção, a análise da cirtometria e da volumetria mostraram diferenças significativas entre os grupos (< 0,05), com maior redução do linfedema no Grupo TCM. Não houve diferença significativa nos valores das pregas cutâneas e da quantidade de água corporal total. A sensação de peso no membro superior, antes e após a intervenção, foi significativamente menor (< 0,05) no Grupo TCM. CONCLUSÕES: O tratamento fisioterapêutico somado à dietoterapia com ingestão de TCM em mulheres portadoras de linfedema de MS pós-cirurgia e tratamento de câncer de mama foi efetivo na involução desta condição.

OBJECTIVE: To investigate the influence of complex decongestive physical therapy (CDP) in association with diet therapy using medium-chain triglycerides (MCT), as an intervention in cases of upper-limb lymphedema. METHODS: The lymphedema was evaluated by measuring circumferences, volumes, skin folds and whole-body water content. Feelings of discomfort, pain and heaviness in the arms were evaluated using a visual analog scale. Ten women who had undergone mastectomy and presented upper-limb lymphedema homolateral to the surgery participated in this study. Their mean age was 65.9 ± 10.4 years and their mean body mass index (BMI) was 26.8 ± 3.0kg/m². After nutritional evaluation, they were randomly divided into two groups: the Control Group (n= 5), which underwent physical therapy treatment consisting of CDP (classical massage, manual lymphatic drainage, compression taping and skincare) three times a week, for four weeks; and the MCT Group (n= 5), which underwent the same physical therapy protocol with the addition of daily diet therapy consisting of intake of MCT, for four weeks. RESULTS: At the end of the intervention, analysis of the circumference and volume measurements showed significant differences between the groups (< 0.05), with a greater reduction in lymphedema in the MCT Group. There were no significant differences in the skin fold measurements or whole-body water content. The feeling of heaviness in the arms after the intervention was significantly less in the MCT Group (< 0.05), compared with before the intervention. CONCLUSION: Physical therapy treatment together with diet therapy with intake of MCT in women with upper-limb lymphedema following surgery and breast cancer treatment was effective in reverting this condition.

Enteral nutrition with dietary fiber and medium chain triglycerides in postoperative patients with gastric cancer / 肠外与肠内营养

Objective: To evaluate the role of enteral nutrition with dietary fiber and medium chain triglycerides in postoperative patients with gastric cancer. Methods: Forty patientswith gastric cancer receivedenteral diet with dietary fiber and medium chain triglycerides for 7 days. Enteral feeding was initiated 48hours after surgery. Host biochemistryindicators, nutritional parameters and immunity were monitored. Results: With surgical trauma, patients'nutritional status and immunity were impaired, and after nutritional support, the nutritional and immune parameters increased significantly. Conclusions: In postoperative patientswith gastric cancer, enteral nutrition with dietary fiber and medium chain triglycerides can improve the nutritional status and immunity.