Incidence and treatment of chylothorax in children undergoing corrective surgery for congenital heart diseases

Abstract Introduction: Chylothorax is a lymphatic extravasation into pleural cavity and its incidence is 0.25%-5.3% in children undergoing cardiac surgery. Objective: To evaluate the incidence of chylothorax in pediatrics patients operated, linking it in each surgical intervention. Evaluate treatment types and efficiency. Methods: Retrospective study using medical records of children undergoing cardiac surgery in the Hospital do Coração between 2004 and 2014. For statistical analysis, qualitative variables by absolute frequency and relative frequency; quantitative variables, by median of 25 and 75 percentiles, as they did not present normal distribution (Shapiro-Wilk, P<0.05). The Chi-square test was used for the association between type of treatment and result. The adopted confidence level was 95%. Results: Incidence of chylothorax was 2.1% (0.9% in intracardiac surgery, 1.7% correction of patent ductus arteriosus and aortic coarctation, 8.3% Glenn's surgery, 11.8% total cavopulmonary surgery and 3% in others). Among treatments, fasting associated with total parenteral nutrition (TPN) resolved 51% of the cases. Hypoglossal diet had failed treatment and surgical referral in 22% of the cases. Fasting with TPN associated with octreotide had success in the treatment of chylothorax in a period exceeding 15 days in 78% of cases, and 3.7% were referred for surgery. Conclusion: According to the results, incidence of chylothorax was 2.18%. Treatment with fasting and TPN leads to resolutions in 86.5%, and the association with octreotide was successful in 85.1% of cases, showing an efficient option, while the treatment with hypoglossal diet had therapeutic failure in 22% of the cases in which it was used.

Bilateral spontaneous chylothorax after severe vomiting in children / Quilotórax bilateral espontâneo após vômitos excessivos em criança

Abstract Objective: To report the case of a child with bilateral chylothorax due to infrequent etiology: thoracic duct injury after severe vomiting. Case description: Girl, 7 years old, with chronic facial swelling started after hyperemesis. During examination, she also presented with bilateral pleural effusion, with chylous fluid obtained during thoracentesis. After extensive clinical, laboratory, and radiological investigation of the chylothorax etiology, it was found to be secondary to thoracic duct injury by the increased intrathoracic pressure caused by the initial manifestation of vomiting, supported by lymphoscintigraphy findings. Comments: Except for the neonatal period, chylothorax is an infrequent finding of pleural effusion in children. There are various causes, including trauma, malignancy, infection, and inflammatory diseases; however, the etiology described in this study is poorly reported in the literature.

Resumo Objetivo: Relatar o caso de uma criança com quilotórax bilateral devido a etiologia pouco frequente: lesão do ducto torácico após quadro de vômitos excessivos. Descrição do caso: Menina, sete anos, apresentava edema facial crônico iniciado após quadro de hiperemese. À avaliação, também apresentava derrame pleural bilateral, com líquido quiloso obtido na toracocentese. Após extensa investigação clínica, laboratorial e radiológica da etiologia do quilotórax, foi definido ser secundário a lesão do ducto torácico por aumento da pressão intratorácica pela manifestação inicial de vômitos, corroborado por achados de linfocintilografia. Comentários: À exceção do período neonatal, o quilotórax é achado infrequente de efusão pleural em crianças. As causas são diversas, incluindo trauma, neoplasia, infecção e doenças inflamatórias; contudo, etiologia como a aqui descrita é pouco relatada na literatura.

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.

Videotoracoscopia como uma opção no tratamento cirúrgico do quilotórax após cirurgia cardíaca pediátrica / Video-assisted thoracoscopy as an option in the surgical treatment of chylothorax after cardiac surgery in children

OBJETIVO: Avaliar o uso de videotoracoscopia no tratamento cirúrgico do quilotórax após cirurgia para correção de cardiopatias congênitas em crianças. MÉTODOS: Revisamos os prontuários médicos de 3.092 crianças operadas para a correção de cardiopatias congênitas no Instituto do Coração/Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo (SP) entre fevereiro de 2002 e fevereiro de 2007. RESULTADOS: Das 3.092 crianças, 64 (2,2 por cento) apresentaram quilotórax como complicação pós-operatória. Em 50 (78,1 por cento) dessas, o tratamento clínico foi bem-sucedido, enquanto esse falhou em 14 (21,9 por cento), as quais foram submetidas à ligação do ducto torácico por videotoracoscopia. A ligação do ducto torácico obteve sucesso em 12 pacientes (86 por cento) e falhou em 2 casos, os quais foram resolvidos com medidas clínicas adicionais, como dieta pobre em gorduras e nutrição parenteral. Não houve morbidade ou mortalidade relacionada à operação. Dos 14 pacientes, 5 (35 por cento) faleceram em decorrência de complicações cardíacas ou infecciosas. CONCLUSÕES: A ligadura videoassistida do ducto torácico pode ser realizada com segurança em pacientes gravemente enfermos e com doença cardíaca grave, com resultados favoráveis.

OBJECTIVE: To evaluate the use of video-assisted thoracoscopy in the surgical treatment of chylothorax developed after the surgical correction of congenital heart disease in children. METHODS: We reviewed the medical charts of 3,092 children who underwent surgery for congenital heart disease between February of 2002 and February of 2007 at the Heart Institute of the University of São Paulo School of Medicine Hospital das Clínicas, in São Paulo, Brazil. RESULTS: Of the 3,092 children, 64 (2.2 percent) presented with chylothorax as a postoperative complication. In 50 (78.1 percent) of those patients, the clinical management was successful, whereas it failed in 14 (21.9 percent), all of whom were then submitted to thoracic duct ligation by video-assisted thoracoscopy. The thoracic duct ligation was successful in 12 patients (86 percent) but failed in 2. In the postoperative period, additional clinical measures, such as a low-fat diet and parenteral nutrition, were required in order to resolve those 2 cases. There was no surgical morbidity or mortality. Of the 14 patients who underwent thoracic duct ligation, 5 (35 percent) died due to cardiac or infectious complications. CONCLUSIONS: Video-assisted thoracic duct ligation can be safely performed in patients with severe heart disease, and the outcomes are favorable.

Quilotórax secundario a herida por proyectil de arma de fuego / Chylothorax secondary to gunshot wound

Introducción: El quilotórax es la presencia de quilo en el espacio pleural secundaria a lesión u obstrucción del conducto torácico. Su etiología puede ser traumática o no traumática. El aspecto lechoso del líquido pleural y su alto contenido de triglicéridos orientan el diagnóstico. El objetivo de este trabajo es presentar un caso de quilotórax secundario a herida por proyectil de arma de fuego y revisar los conceptos actuales y opciones terapéuticas. Caso clínico: Paciente de 33 años que presentó quilotórax secundario a herida por proyectil de arma de fuego. Se manejó a base de ayuno, alimentación parenteral y drenaje pleural. Su evolución fue satisfactoria. Conclusiones: El quilotórax es una entidad asociada a elevada morbilidad y mortalidad. Su diagnóstico temprano y oportuno, como en el paciente descrito, es fundamental para asegurar la evolución satisfactoria.

BACKGROUND: Chylothorax is the occurrence of chyle in the pleural space due to damage or obstruction of the thoracic duct. Its etiology can be traumatic or nontraumatic. Traumatic lesion of the thoracic duct occurs after accidents or cardiothoracic surgery. Non-Hodgkin's lymphoma is the most frequent nontraumatic etiology. Milky aspect and high content of triglycerides in pleural fluid confirm the diagnosis. The objective of this paper is to present a case of chylothorax secondary to shotgun lesion as well as to review current concepts about chylothorax and its treatment. CLINICAL CASE: We present the case of a 33-year-old male patient with chylothorax secondary to gunshot lesion. Management was based on fasting, parenteral nutrition and pleural drainage. Patient's evolution was satisfactory. CONCLUSIONS: Chylothorax is an entity associated with high morbidity and mortality. Early diagnosis and appropriate treatment are essential.

Quilotórax congénito: presentación de un caso / Congenital quilothorax: presentation of a case

Se presenta una paciente que presentó en el estudio ultrasonográfico prenatal efusión pleural bilateral. Posterior a su nacimiento se estableció el diagnóstico de quilotórax bilateral de etiología no determinada, limitándose el mismo con la terapéutica empleada en los primeros 7 días de vida. Se comentan sus principales manifestaciones clínicas, estudios para establecer el diagnóstico y tratamiento utilizado.

We present a patient who showed a bilateral pleural effusion at the prenatal ultrasonographic study. After she was born, the diagnostic was established: bilateral quilothorax of unknown etiology, limited with the therapy applied during the first 7 days of life. We state the main clinical manifestations, trials to arrive to a diagnostic and used treatment.

Fístula linfática após tratamento cirúrgico de síndrome do desfiladeiro torácico à direita / Chylothorax after surgical treatment of right-sided thoracic outlet syndrome

A fístula linfática como complicação de correção de síndrome do desfiladeiro torácico é um evento muito raro. Relatamos um caso de fístula linfática à direita e apresentamos uma breve revisão do tratamento de quilotórax pós-cirúrgico.

Chylothorax as a complication of the surgical treatment of thoracic outlet syndrome is a quite rare event. We report a case of right-sided chylothorax and present a brief review on the treatment of postoperative chylothorax.

Gorhams Disease: Vanishing Bone Syndrome.

Gorhams disease, also known as massive osteolysis or vanishing bone disease is an extremely rare bone disease. It is characterized by angiomatosis with adjacent bone resorption. We report an 8-years old boy with the disease who was managed successfully with alpha 2b interferon therapy.

Hernia diafragmática izquierda: a propósito de un caso en el Centro de Emergencia Infantil Coromoto enero 2008 / Left diaphragmatic hernia: a purpose of a case in Centro Emergencia Infaltil Coromoto january 2008

Es una Patología Neonatal por persistencia del Canal Pleuroperitoneal ó la Agenesia Total o Parcial del Diafragma con introducción de las vísceras abdominales: (estómago, intestino delgado y grueso, incluso bazo, riñón y páncreas) en el tórax, asociado a una hipoplasia pulmonar uni o bilateral. Los síntomas se presentan inmediatamente después del nacimiento con frecuencia requieren maniobras, la dificultad respiratoria es severa y conlleva a la insuficiencia respiratoria con hipoxia, hipercapnea y acidosis severa. Se presenta caso diagnosticado en el Centro de Emergencia Infantil Coromoto. Lactante menor de 5 meses de edad, antecedentes de tos alérgica persistente desde el nacimiento con mejoría parcial por tratamiento vía oral e inhalatoria hasta la edad de 5 meses cuyo síntoma empeoro, se trató como síndrome viral agudo, hasta que se le realiza su primera RX de tórax evidenciándose Hernia Diafragmática izquierda o de Bochdalek, la cual fue corregida Quirúrgicamente con evolución satisfactoria.

Spontaneous recovery of chylothorax caused by lymphoma

Chylothorax is a rare complication with both traumatic and non-traumatic causes. The most common non-traumatic cause is malignancy, especially lymphoma. Lymph discharges into the pleural space, causing nutritional, metabolic, immunologic and respiratory complications. We evaluated the case of a patient suffering from a low-grade lymphoma who developed chylothorax that did not respond to chemotherapy and conservative treatment. This patient recovered spontaneously

A respeito de um caso de quilotórax bilateral e quiloascite espontâneos: aspectos clínicos e cirúrgicos / About a case of espontaneous bilateral chylothorax and chyloascitis: clinical and surgical aspects

Efusão quilosa pleural ou quilotórax significa acúmulo quiloso no espaço pleural, geralmente como ruptura secundária dos ductos linfáticos torácicos, que pode ser promovida por vários estímulos, desde trauma, doenças malignas até causas idiopáticas. É definido como uma efusão de linfa na cavidade pleural, podendo ter origem no tórax ou na cavidade abdominal, ou em ambos. É de aspecto leitoso, inodoro, branco, de pH alcalino com gravidade específica acima de 1012, bacteriostático, não irritativo à pleura. É opalescente, formado quando triglicérides de cadeia longa da dieta são transformados em quilomícrons de baixa densidade lipoprotéica secretados no intestino. O quilo é transportado através do ducto torácico e drenado na veia subclávia esquerda. O diagnóstico é baseado em análise clínica da efusão pleural contendo quilomícrons e níveis de triglicérides maior que 110 mg/dL como indicativo praticamente certo de efusão pleural quilosa. A conduta depende da causa e das circunstâncias individuais. Um relato de quilotórax bilateral e quiloascite espontâneos é apresentado com ótima evolução através de tratamento conservador com a utilização de dieta enteral oligomérica, rica em aminoácidos, com glutamina e mínima oferta de triglicérides de cadeia média e octeotride

Chylous pleural effusion or chylothorax means chyle accumulation in the pleural space generally as a secondary disruption of thoracic lymphatics, that can be promoted by various stimulants, since trauma, malignancies, to idiopathic causes. Is defined as an effusion of limphin pleural cavity. Chyle may have its origin in the thorax or in the abdomen, or both. Is a milky, white, alkaline pH with a specific gravity above 1012, bacteriostatic and nonirritating to thepleural space, opalescent fluid formed when long-chain triglycerides in the diet are transformed into chylomicrons and very-low-density lipoproteins and secreted into intestinal lacteals. The chyle is transported through the thoracic duct and drained into the left subclavian vein. Diagnosis is based on a chemical analysis of the pleural effusion presenting chylomicrons, pleural triglyceride with levels greater than 110 mg/dL being nearly always indicative of a chylous pleural effusion. Management depends on the underlying cause and the individual circumstances. A case report of spontaneous chylothorax and chyloascitis is presented successfullytreated by conservative means, using oligomeric enteral feeding, rich in amino acids with minimum quantity of medium-chain-triglycerides, glutamine, and octeotride

Hernia diafragmática traumática: reporte de un caso / Traumatic diaphragmatic hernia: case-report

La hernia diafragmática traumática es poco frecuente en niños y continúa siendo una patología de difícil diagnóstico. Es considerada un indicador de gravedad del trauma y presenta una elevada tasa de morbilidad y mortalidad. El objetivo de esta publicación es presentar un caso de hernia diafragmática secundario a un trauma toracoabdominal cerrado. Reportamos el caso de una niña de 8 años atropellada que presentó una hernia diafragmática izquierda y una fractura inestable de pelvis. Evolucionó con un quilotórax y un infarto renal izquierdo que fueron manejados en forma conservadora.

Caso clínico-radiológico: quilotorax / Radiological diagnosis of chylothorax

Se presenta el caso clínico de una paciente de 10 años que debutó con derrame pleural masivo con características de quilotórax cuya causa fue secundaria a un linfoma linfoblástico de células T. El quilotórax secundario se trató con manejo conservador consistente en dieta baja en grasas y drenaje pleural en un primer momento y posteriormente -ya teniéndose el diagnóstico establecido- sólo con dieta baja en grasa, presentando una evolución clínica satisfactoria y sin recaídas posteriores. Se discute la etiología del quilotórax, dentro de las cuales se encuentra el linfoma no Hodgkin postulándose como causa la obstrucción del conducto torácico por las masas tumorales. Se revisa el diagnóstico y manejo actual del quilotórax mostrando alguna evidencia del buen resultado del manejo conservador con éxito cercano al 80 por ciento durante las primeras 4 semanas. En los últimos años el uso del análogo de somatostatina, octeotride, con resultados promisorios, está sustentado por series clínicas pequeñas que requieren mayor evaluación antes de una recomendación.

Quilotórax / Chylothorax

O quilotórax, normalmente secundário a doenças malignas, trauma, doenças congênitas, infecções e trombose da veia cava superior, é uma causa pouco freqüente de derrame pleural. O diagnóstico e tratamento precoces são importantes no sentido de prevenir a mais temida conseqüência do quilotórax, a má nutrição e conseqüente comprometimento do estado imunológico.

Chylothorax, an uncommon cause of pleural effusion, is usually secondary to malignancy, trauma, congenital diseases, infections and superior vena cava thrombosis. The early diagnosis and treatment are important to prevent the most fearful consequence of chylothorax, the malnutrition with a compromised immunological status.

Uso de octreotide (análogo de somatostatina) en un caso de quilotórax bilateral secundario a linfoma: Report of one case / Octreotide for bilateral chylothorax secondary to lymphoma. Report of one case

Since chylothorax is uncommon and has multiple causes, its best treatment choice is not clear. Recent reports show that octreotide is useful in the treatment of chlylothorax secondary to cancer or caused by surgical procedures. We report a 21 years old male with a chylothorax secondary to a non Hodgkin lymphoma. Treatment with octreotide changed the color and triglyceride content of pleural effusion. Serum albumin and proteins increased and the effusion subsided after the second cycle of chemotherapy with CHOP. Thus octreotide was discontinued on the 27th day of therapy and pleural drainages were whithdrawn. He was discharged in good conditions, 38 days after admission.

Chylothorax and chyloascites complicating cirrhosis of liver

Chylothorax and chyloascites is the accumulation of chyle in pleural and peritoneal spaces and are rarely seen. This case report describes the occurrence and management in an old lady. However, she expired due to frank haematemesis and encephalopathy. Chylothorax/chyloascites are difficult to manage and associated with poor prognosis

Experiencia clínica: quilotorax en cirugía cardiovascular pediátrica / Chylothorax in paediatric cardiovascular surgery

El quilotórax es una complicación infrecuente de la cirugía cardiovascular (CCV). Los estudios pediátricos son escasos y las cifras de incidencia y mortalidad son variables. En una revisión retrospectiva de 314 pacientes postoperados cardíacos identificamos 15 casos de quilotórax. La complicación se asoció tanto a procedimientos extrapericárdiacos como intrapericárdiacos y se manifestó antes de una semana luego de iniciada la alimentación enteral. En 11/15 pacientes fue suficiente el tratamiento con dieta pobre en grasa; en 3/15 fue necesario emplear nutrición parenteral total. El tratamiento médico fue efectivo en las primeras 2 semanas. Sólo en paciente requirió tratamiento quirúrgico; este caso presentaba trombosis de vena cava superiror. La presencia de quilotórax se asoció a períodos más prolongados de ventilación mecánica y de hospitalización en la unidad de cuidados intensivos, sin observarse mortalidad. El quilotórax es una complicacion poco frecuente de la CCV y de buen pronóstico; la gran mayoría de los casos mejora con tratamiento médico exclusivo. Esta complicación puede aumentar los costos de la CCV al prolongar los períodos de hospitalización en unidades de alta complejidad