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OBJECTIVE: This study aimed to analyze prenatal diagnosis, perinatal outcomes, and postnatal follow-up in fetuses with ectopia cordis (EC). METHODS: This retrospective analysis accessed 31 patients with EC who were either diagnosed or referred to a tertiary Fetal Medicine centers for EC diagnosis in Brazil, Germany, Italy, and Poland. We analyzed prenatal diagnosis, perinatal outcomes, and follow-up in these patients. RESULTS: Our study included a cohort of 31 fetuses with EC, 4 and 27 of whom had partial and complete protrusion of the heart through a ventral defect in the thoracoabdominal wall, respectively. EC was diagnosed by fetal echocardiography at a mean gestational age of 20.3 ± 8.6 weeks (range, 8-35 weeks). Of the four cases, in which the karyotype was performed, all of them had a normal result (1 - 46,XX and 3 - 46,XY). Five patients showed conotruncal abnormalities and six ventricular septal defects. Termination of pregnancy (TOP) was performed in 15 cases (48%) and seven pregnant women had spontaneous fetal demise (22.5%). Of the seven fetuses that were born alive, four of them died, and three infants underwent surgery. Among these three infants, all of them survived, one was 5 months, 13 years old and 29 years old at the time of study completion. CONCLUSIONS: Ectopia cordis is associated with high mortality rates and intracardiac/extra-cardiac defects. Ventricular septal defects and conotruncal anomalies were the more common intracardiac defects associated with EC. However, in this cohort of fetuses with EC the incidence of PC was lower than reported in the literature.

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Resumen Objetivo Comunicar la experiencia en el tratamiento de pectus excavatum en el Instituto Nacional del Tórax. Material y Método: Estudio descriptivo con seguimiento. Se recolectaron datos de las variables de interés, mediante la revisión de fichas clínicas. Se incluyeron pacientes operados entre marzo de 2007 y abril de 2018. Resultados Se incluyeron 86 pacientes operados con técnica mínimamente invasiva en el período descrito. De ellos, 74 pacientes de sexo masculino. El promedio de edad al momento de la cirugía fue de 17,8 años. El principal motivo de consulta fue por desmedro estético que correspondió al 41,8% de los pacientes. Salvo en 1 paciente, en todo el resto se ocupó una sola barra. En nuestra serie hubo 12 pacientes que presentaron alguna complicación operatoria y 1 paciente fallecido. Hasta el cierre del seguimiento se retiraron 61 barras, 2 de ellas previo al período estipulado de tratamiento, por morbilidad. Hay 20 barras in situ y 4 pacientes de los cuales no se tiene registro por abandono de controles. Discusión El pectus excavatum es la más frecuente de las deformidades de la pared torácica, es 4 a 6 veces más frecuente en hombres que en mujeres. En general los pacientes son asintomáticos, aunque algunos pueden presentar síntomas cardiopulmonares. La reparación con cirugía mínimamente invasiva con técnica de Nuss aparece hoy en día como el gold standard de manejo. Conclusión El manejo de los pacientes con pectus excavatum en nuestra Institución se asemeja a lo reportado en la literatura internacional. Nuestros esfuerzos deben apuntar a disminuir la morbimortalidad asociada.

Aim To communicate the experience in the treatment of pectus excavatum in the National Institute of Thorax. Materials and Method: Descriptive study with follow-up. Data of variables of interest were collected through the review of clinical records. Patients operated between March 2007 and April 2018 were included. Results 86 patients operated with the Nuss technique were included in the period described. Of them, 74 male patients. The average age at the time of surgery was 17.8 years. The main reason for consultation was due to cosmetic detriment that corresponded to 41.8% of the patients. Except in 1 patient, in all the rest a single bar was occupied. In our series, there were 12 patients who presented some operative complication and 1 patient died. Up to the end of the follow-up, 61 bars were removed, 2 of them in non-scheduled surgery. There are 20 bars in situ and 4 patients of which there is no registration due to abandonment of controls. Discussion Pectus excavatum is the most frequent of the deformities of the chest wall, it is 4 to 6 times more frequent in men than in women. In general, patients are asymptomatic, although some may have cardiopulmonary symptoms. The repair with minimally invasive surgery with Nuss technique appears today as the goldstandard of management. Conclusion The management of patients with pectus excavatum in our Institution is similar to that reported in the international literature. Our efforts should aim to reduce the associated morbidity and mortality.

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INTRODUCCIÓN: El blastema pleuropulmonar (BPP) es la neoplasia primaria maligna más común de los pulmones en la infancia. Se presenta con mayor frecuencia en niños entre 1 y 4 años, con sínto mas respiratorios. Han sido definidos tres tipos por histopatología (tipo I, II y III), relacionados con sobrevida y pronóstico. OBJETIVO: Reportar el primer caso de un paciente con un BPP que se presentó como una deformación de la pared torácica. CASO CLÍNICO: Paciente de un año de vida que se presentó con una deformidad de la pared torácica a los 10 meses de edad. El estudio imagenológico reveló una gran masa quística en el hemitórax derecho. No desarrolló sintomatología respiratoria hasta el ingreso hospitalario. Se realizó una lobectomía superior derecha y la biopsia confirmó un BPP tipo I. Se trató con resección quirúrgica y realización periódica de imágenes torácicas como seguimiento. CONCLUSIONES: Se reporta el caso de un BPP que se manifestó con deformidad torácica, forma de presentación no descrita previamente en la literatura. El BPP es un cáncer poco frecuente que debe ser considerado en el diagnóstico diferencial de lesiones quísticas pulmonares, sobre todo en la edad pediátrica. Reconocerlo como un tumor maligno en vez de una anomalía del desarrollo, es determinante para que el paciente sea sometido a resección quirúrgica, terapia adyuvante y seguimiento apropiado.

INTRODUCTION: Pleuropulmonary blastema (PPB) is the most common primary malignancy of the lungs in childhood. It occurs more frequently in children between one and four years of age, and respiratory symptoms are a common manifestation. Three types have been defined (type I, II and III), which are related to survival and prognosis. OBJECTIVE: To report the first case of a patient with a PPB who presented with a chest wall deformity. CASE REPORT: One year old male patient who had a chest wall deformity at ten months of age. Imaging revealed a giant cyst in the right hemithorax. He did not develop respiratory symptoms until hospital admission. A right upper lobectomy was perfor med and the biopsy confirmed a type I pleuropulmonary blastoma. He was considered successfully treated with complete surgical excision and routine follow-up with thoracic imaging is conducted. CONCLUSIONS: PPB is a very rare cancer that needs to be considered in the differential diagnosis of cystic lung diseases in children. The recognition of this lesion as a malignant tumour rather than a developmental cystic malformation is vital so the child can receive complete excision and appropriate follow-up care.

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El pectus excavatum representa del 90 al 92% de las deformidades congénitas de la pared anterior del tórax; el resto comprende pectus carinatum, tórax hendido, síndrome de Cantrelly síndrome de Poland. En México, se presenta en 1 de cada 1,000 nacidos vivos. La deformidad es poco evidente al nacimiento; sin embargo, en la infancia se hace más evidente y puede llegar a constituir un problema relevante funcional, psicosocial y/o estético para el adolescente. La patogenia se atribuye al crecimiento anormal de los cartílagos costales. Siguiendo el principio fundamental de que toda deformación congénita que produce síntomas reclama la atención del cirujano, se recomienda la cirugía correctiva en la edad temprana de la vida, porque la manipulación operatoria se lleva a cabo en un palmo de extensión. Presentamos el caso de un varón de 18 años de edad, de 1.90 metros de estatura, jugador de baloncesto, a quien se le colocó una prótesis modelada de silicon por debajo del plano fasciomuscular, para corregir el defecto físico.

Pectus excavatum is the commonest congenital defect of the anterior chest wall, accounting for 90-92% of all cases, the rest is distributed between pectus carinatum, cleft sternum, Cantrell's syndrome and Poland's syndrome. In Mexico, pectus excavatum occurs in 1 of 1,000 live births. This deformity is barely noticed at birth but becomes more evident during childhood and may constitute a functional, psychosocial and/or aesthetic problem for the adolescent. The pathophysiology involved is an abnormal growth of costal cartilages. Following the fundamental principle that any symptomatic congenital deformity claims the surgeon's attention, corrective surgery is recommended early in life, when the surgical manipulation is made in a small field. We present the case of an 18 year old, 1.9 m tall, basketball player, who had a modelled silicon implant inserted beneath the fasciomuscular layer, as a cosmetic repair of his deformity.