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ACTUALIZACION Y PERSPECTIVAS DE LA PENTALOGIA DE CANTRELL

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La pentalogía de Cantrell es una enfermedad compleja que requiere la intervención multidisciplinaria del equipo de salud para llegar al diagnóstico preciso y decidir el mejor tratamiento.
hernandezgonzalez9.jpg Autor:
Martha alicia Hernández gonzález
Columnista Experto de SIIC

Institución:
Instituto Mexicano del Seguro Social

Artículos publicados por Martha alicia Hernández gonzález
Coautores
Sergio Solorio* Sonia Lazcano Bautista** Blanca Olivia Murillo Ortiz*** 
Doctor en Ciências Médicas, Cardióloga Pediatra, Instituto Mexicano del Seguro Social, León, México*
Cardióloga Pediatra, Instituto Mexicano del Seguro Social, León, México**
Doctor en Ciências Médicas, Instituto Mexicano del Seguro Social, León, México***
Recepción del artículo
10 de Junio, 2008 		Aprobación
16 de Julio, 2008
Primera edición
5 de Diciembre, 2008 		Segunda edición, ampliada y corregida
7 de Junio, 2021

ACTUALIZACION Y PERSPECTIVAS DE LA PENTALOGIA DE CANTRELL

Resumen
La pentalogía de Cantrell es una enfermedad rara, con características clínicas, anatómicas y embriológicas peculiares. Esta entidad es una variedad toracoabdominal de la ectopia cordis en la que se asocian cinco anomalías: defecto epigástrico de la línea media abdominal supraumbilical, defecto del tercio inferior del esternón, deficiencia del segmento anterior del diafragma, defectos pericárdicos y malformaciones cardíacas congénitas. La comunicación interventricular e interauricular se encuentra en la mitad de los casos y puede asociarse a otras anomalías extracardíacas como espina bífida, diversas malformaciones del sistema nervioso central, divertículo de Meckel, poliesplenia, entre otros. El diagnóstico se hace mediante ultrasonografía fetal en el primer trimestre de la gestación; en casos incompletos la tomografía helicoidal y la resonancia magnética complementan el diagnóstico. El tratamiento es quirúrgico y en varias etapas, lo primordial es proteger las vísceras expuestas y corregir la cardiopatía congénita. Los resultados aún son controversiales. El pronóstico de la enfermedad depende de la extensión del defecto, el diagnóstico temprano y el manejo perioperatorio. Nosotros consideramos que la pentalogía de Cantrell es una enfermedad compleja en la que es necesaria la intervención multidisciplinaria del equipo de salud para llegar al diagnóstico preciso y decidir el mejor tratamiento.

Palabras clave
pentalogía de Cantrell, diagnóstico, tratamiento, pronóstico


Artículo completo
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Pentalogy of Cantrell: Update and New Perspectives

Abstract
The pentalogy of Cantrell is a rare disease, with unique clinical, anatomical and embryological characteristics. This disease is a thoraco-abdominal variety of ectopia cordis. Five anomalies are associated: epigastric defect of the over umbilical abdominal mean line, defect of inferior breastbone third, defect of the anterior segment of the diaphragm, pericardial defects and congenital cardiac malformations. Ventricular septal defect and atrial septal defects are in half of the cases and it could be associated to other extra cardiac anomalies as bifid spine, several malformations of central nervous system, meckel diverticulum and polispleenia, among others. The diagnosis is made by fetal ultrasonography in the first trimester of pregnancy. In incomplete cases, helical tomography and magnetic resonance imaging complete the diagnosis. The treatment is surgical and in several stages. It is fundamental to protect the exposed viscera and to correct the congenital heart disease. The results still are controversial. The prognosis of the disease depends on the defect extension, time of diagnosis and peri-operative handling. We considered that the pentalogy of Cantrell is a complex pathology. A multidisciplinary health team intervention is necessary to reach a precise diagnosis and to decide the best treatment.

Key words
pentalogy of Cantrell, diagnosis, treatment, prognosis

Clasificación en siicsalud
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Especialidades
Principal: Pediatría
Relacionadas: Cardiología, Diagnóstico por Imágenes, Epidemiología, Genética Humana, Obstetricia y Ginecología



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Enviar correspondencia a:
Sergio Solorio, Instituto Mexicano del Seguro Social Unidad Médica de Alta Especialidad No.1. Bajío Unidad de Investigación en Epidemiología Clínica, 37320, Blvd.. Adolfo López Mateos esq. Av. Insurgentes sin número. Col. Los Paraísos, León, México
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