Congenital chylothorax in siblings

Fox, Grenville; Challis, D; O'Brien, Kirsty K; Kelly, E. N.; Ryan, Greg

doi:10.1080/080352598750031734

Cited by 6 publications

(7 citation statements)

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“…En la etapa posnatal, el manejo puede ser clasificado en tres categorías: Manejo de la enfermedad subyacente, tratamiento conservador y tratamiento quirúrgico 24 . No es objeto de esta revisión, sin embargo se debe enfocar el proceso diagnóstico diferencial para así, direccionar la identificación y el manejo de la enfermedad subyacente.…”

Section: Descripción De Casounclassified

“…Otra de las medidas usadas como tratamiento conservador, es el uso de somatostatina, la cual se indica si no hay respuesta a las medidas dietéticas, su tasa de éxito puede ser alrededor del 50% y se basa en su efecto atenuante sobre el flujo de la linfa del conducto torácico 25 . Finalmente, el manejo quirúrgico se recomienda en casos donde a pesar de tratamiento conservador, el paciente drena más de 100 mL/Kg de peso corporal por día y en quienes se ha producido un rápido declive en el estado nutricional a pesar del tratamiento conservador 24 . Se incluye manejo con pleurodesis o pleurectomía, ligadura del conducto torácico y finalmente toracoscopia o toracotomía 25 .…”

Section: Descripción De Casounclassified

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Recién nacido pretérmino con quilotórax congénito bilateral, una entidad infrecuente que amenaza la vida

Aguilar-López

Rodríguez-Moncada

Díaz-Díaz

et al. 2017

revmed

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médicas uis revista de los estudiantes de medicina de la universidad industrial de santander RESUMENEl quilotórax corresponde a la acumulación de linfa en el espacio pleural. El diagnóstico se confirma por la presencia de quilomicrones en el líquido pleural, sin embargo en la práctica clínica se utilizan los criterios de Büttiker, para establecer el diagnóstico. El objetivo de esta publicación es presentar el caso de un recién nacido con quilotórax congénito bilateral, realizando una revisión actualizada del tema, que incentive la recolección de datos y la generación de guías nacionales para su abordaje estandarizado. Se trató de un recién nacido pretérmino, quien tras parto por cesárea presentó síndrome de distrés respiratorio, ameritó terapia temprana de surfactante logrando retiro de ventilación mecánica al día de vida, con posterior presentación de signos de dificultad respiratoria y hallazgo radiológico y ecográfico de derrame pleural bilateral. Se realizó toracentesis con citoquímico compatible con quilotórax. Nuevamente ameritó soporte ventilatorio invasivo, toracostomía cerrada bilateral y nutrición parenteral, con posterior evolución clínica satisfactoria. Preterm newborn with bilateral congenital chylothorax, a rare life-threatening entity ABSTRACTChylothorax refers to the accumulation of lymphatic fluid in the pleural space. Diagnosis is confirmed by the presence of chylomicrons in the pleural fluid, however in clinical practice Büttiker criteria are widely used. The aim of this publication is to present the case of a newborn with bilateral congenital chylothorax with an updated review of the literature that encourages data collection and generation of national guidelines for standardized approach. This was a newborn preterm, who after a cesarean delivery presented respiratory distress syndrome who required early surfactant therapy achieving withdrawal of mechanical ventilation on his first day, with subsequent presentation of signs of respiratory distress, and a radiological and ultrasound finding of bilateral pleural effusion. Thoracentesis was performed obtaining cytochemical compatible with chylothorax. Again required invasive ventilatory support, closed bilateral thoracostomy and parenteral nutrition, with a satisfactory clinical course. MÉD.UIS. 2017;30(1):87-92.

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Recién nacido pretérmino con quilotórax congénito bilateral, una entidad infrecuente que amenaza la vida

Aguilar-López

Rodríguez-Moncada

Díaz-Díaz

et al. 2017

revmed

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“…Sometimes massive pleural effusion and progressive pulmonary infiltration cause respiratory failure [15]. Pleural effusions are often chylous, due to spontaneous rupture of diseased lymph vessels within the lymphangiomas [26], and patients may have associated chyloptysis [29], haemoptysis [6,30,31] or chylopericardium [32]. In some case reports, chylous ascites, protein wasting enteropathy, peripheral lymphoedema, hemihyperplasia and lymphopenia have been also described [1] (figure 3).…”

Section: Clinical Manifestationsmentioning

confidence: 99%

“…The "lymphatic dysplasia syndrome" comprises lymphoedema, and effusions of the pericardium, pleura and peritoneum when there is no identifiable cause, and in the absence of lymphangiomas, lymphangiectasis or GLA [30,67]. The syndrome includes primary lymphoedema, congenital chylothorax, idiopathic chylous effusions and the yellow nail syndrome (i.e.…”

Section: Differential Diagnosismentioning

confidence: 99%

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Thoracic involvement in generalised lymphatic anomaly (or lymphangiomatosis)

2016

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Generalised lymphatic anomaly (GLA), also known as lymphangiomatosis, is a rare disease caused by congenital abnormalities of lymphatic development. It usually presents in childhood but can also be diagnosed in adults. GLA encompasses a wide spectrum of clinical manifestations ranging from singleorgan involvement to generalised disease. Given the rarity of the disease, most of the information regarding it comes from case reports. To date, no clinical trials concerning treatment are available. This review focuses on thoracic GLA and summarises possible diagnostic and therapeutic approaches. @ERSpublications Possible diagnostic and therapeutic approaches to generalised lymphatic anomaly (lymphangiomatosis)

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Fetal Hydrops and Familial Pulmonary Lymphatic Hypoplasia

Thibeault¹,

Black²,

Taboada³

2002

Am J Perinatol

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Two siblings were born with pleural effusions and hydrops. The first infant was a 26-week-old gestation male and died at 8 hours of life with radiographically small lungs and pulmonary insufficiency. No lung tissue was obtainable. This pregnancy was followed by two normal term infants, a male and female. The fourth pregnancy resulted in a female born at 35 weeks' gestation with pleural effusion and hydrops who died at 32 months of age. This infant was discharged from hospital at 32 days of age with small pleural effusions, but needed supplemental oxygen and daily diuretics to control edema. There were 14 additional admissions until death-all for respiratory distress or infections. An open lung biopsy at 2 months, showed dilated pleural lymphatics, with hypoplasia of the acinar and terminal bronchiolar lymphatics. At postmortem examination there was a markedly thickened pleura, and slit-like hypoplastic lymphatics of the acinar and terminal bronchioles and interlobular septal regions. This is the second family reported with these distinctive pulmonary intra-acinar and peri-acinar hypoplastic lymphatics. This disease is compatible with an autosomal recessive mode of inheritance.

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Congenital chylothorax in siblings

Cited by 6 publications

References 6 publications

Recién nacido pretérmino con quilotórax congénito bilateral, una entidad infrecuente que amenaza la vida

Recién nacido pretérmino con quilotórax congénito bilateral, una entidad infrecuente que amenaza la vida

Thoracic involvement in generalised lymphatic anomaly (or lymphangiomatosis)

Fetal Hydrops and Familial Pulmonary Lymphatic Hypoplasia

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