ESTUDIO DE UN CASO DE OTOCEFALIA: CAUSA, CONTROL Y CONSECUENCIA

Otocephaly is a very rare and always mortal congenital malformation, that shows the displacement ventromedial of the ears (sinotia), mandibular aplasia (agnasia), absence of the tongue (aglosia) and microstomia. In this article, after a review of the studies achievements about the theme in the scien...

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Autores principales: Yohanna Romero, Jose Loaiza, Catherine Marquez
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Publicado: 2020
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author Yohanna Romero
Jose Loaiza
Catherine Marquez
author_browse Catherine Marquez
Jose Loaiza
Yohanna Romero
author_facet Yohanna Romero
Jose Loaiza
Catherine Marquez
author_sort Yohanna Romero
collection Producción científica
description Otocephaly is a very rare and always mortal congenital malformation, that shows the displacement ventromedial of the ears (sinotia), mandibular aplasia (agnasia), absence of the tongue (aglosia) and microstomia. In this article, after a review of the studies achievements about the theme in the scientific medical, perinatal and obstetric literature, we show a clinical case in the Perinatology unit of the Hospital “Prince Lara” from the Puerto Cabello city, in Venezuela, of a fetus of a teen mother, sixteen years old, pregnant for 32 weeks, primigest, without pathological antecedents but no perinatal control. The pacient was refers with polidramnios to Perinatal Unit. A study ecographic has been made and this shows that there are few aspects that determines to the doctors to make a therapeutic amniodrainage to protect the mother´s life. The necrosic study and autopsy confirms the prenatal finds. The attention to the case shows the utility of the bidimensional ecography for the valoration of the cervico fetal facial pathology and the necessity of a good prenatal control as condition of a good and opportune diagnostic that allows a genetic advice adequate to the situation that always goes to mortal conclusion. Keywords: otocephaly, sinotia, agnatia, microstomia. References [1]C. Diez del Arco, A. Pascual Martín, A. Oliva, E. Aguilar Navarro and A. Pelayo Alarcón, «Síndrome adnatiamicrostomia (otocefalia): a propósito de un caso,» 7 Enero 2018. [En línea]. Available: https_//www.seap.es/posteres2018/-/asset publisher/SOiwqcZWkULE/content/diaz-del-arco-s-pascual-martin-a-oliva-a-aguilar-navarro-e-pelayo-alarcon-a?inheritRedirect-false. [2]D. Escrivano Abad, J. Arbuéz Gabarre, E. Gómez Montes, J. Puente Agueda, I. Herráiez García and A. GalindoIzquierdo, «Diagnóstico prenatal de otocefalia aislada. Utilidad de la ecografía tridimensional.,» Ginecologíaobstétrica de México, vol. 79, nº 8 agosto, pp. 493-496, 2011. [3]L. Gómez, J. Pérez, C. Bermúdez, C. Sosa and F. Guevara, «Residencia de perinatología en el Hospital Universitario de Caracas (Venezuela),» Revista Colombiana de Obstetricia y ginecología, vol. 58, nº 1, pp. 53-59, 2007. [4]S. Santisteban, S. Águila, A. Breto, E. Cabezas and J. Delgado, Obstetricia y perinatología. Diagnóstico ytratamiento, La Habana: Editorial de Ciencias Médicas, 2012. [5]M. Roséndiz Moran, A. Valencia Gutiérrez, V. Soto Abraham and A. Durán, «Otocefalia. Informe de un casode autopsia.,» Revista Mexicana de Pediatría, vol. 70, nº 4 julio-agosto, pp. 181-184, 2003. [6]M. Hernández Rodríguez, M. Romero de Fasolino, C. Silva García, A. Morales Machín, I. Sabatini Sáezand C. Fasolino Romero, «Complejo agnatia tocefalia: extendiendo el espectro de anomalías,» Revista Vitae.Academia Biomédica Digital, vol. 1, nº 53 Enero- marzo, pp. 6-12, 2013. [7]J. Ramírez, H. Pachajoa, C. Isaza and W. Saldarriaga, «Síndrome de aglosia adactilia y exposición prenatal amisoprostol ¿Relación causal o casual? Reporte de un caso,» Revista Colombiana de Obstetricia y Ginecología,vol. 59, nº 3, pp. 248-252, 2008.
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spelling openalex:W3088855613ESTUDIO DE UN CASO DE OTOCEFALIA: CAUSA, CONTROL Y CONSECUENCIAYohanna RomeroJose LoaizaCatherine Marquezotocephalyagnathiaaglossiamicrostomiacongenital malformationsprenatal diagnosisfetal echocardiographyadolescent pregnancyperinatal caregenetic counselingfetal autopsyfetal facial anomaliesCongenital Ear and Nasal AnomaliesCraniofacial Disorders and TreatmentsFetal and Pediatric Neurological DisordersOtocephaly is a very rare and always mortal congenital malformation, that shows the displacement ventromedial of the ears (sinotia), mandibular aplasia (agnasia), absence of the tongue (aglosia) and microstomia. In this article, after a review of the studies achievements about the theme in the scientific medical, perinatal and obstetric literature, we show a clinical case in the Perinatology unit of the Hospital “Prince Lara” from the Puerto Cabello city, in Venezuela, of a fetus of a teen mother, sixteen years old, pregnant for 32 weeks, primigest, without pathological antecedents but no perinatal control. The pacient was refers with polidramnios to Perinatal Unit. A study ecographic has been made and this shows that there are few aspects that determines to the doctors to make a therapeutic amniodrainage to protect the mother´s life. The necrosic study and autopsy confirms the prenatal finds. The attention to the case shows the utility of the bidimensional ecography for the valoration of the cervico fetal facial pathology and the necessity of a good prenatal control as condition of a good and opportune diagnostic that allows a genetic advice adequate to the situation that always goes to mortal conclusion. Keywords: otocephaly, sinotia, agnatia, microstomia. References [1]C. Diez del Arco, A. Pascual Martín, A. Oliva, E. Aguilar Navarro and A. Pelayo Alarcón, «Síndrome adnatiamicrostomia (otocefalia): a propósito de un caso,» 7 Enero 2018. [En línea]. Available: https_//www.seap.es/posteres2018/-/asset publisher/SOiwqcZWkULE/content/diaz-del-arco-s-pascual-martin-a-oliva-a-aguilar-navarro-e-pelayo-alarcon-a?inheritRedirect-false. [2]D. Escrivano Abad, J. Arbuéz Gabarre, E. Gómez Montes, J. Puente Agueda, I. Herráiez García and A. GalindoIzquierdo, «Diagnóstico prenatal de otocefalia aislada. Utilidad de la ecografía tridimensional.,» Ginecologíaobstétrica de México, vol. 79, nº 8 agosto, pp. 493-496, 2011. [3]L. Gómez, J. Pérez, C. Bermúdez, C. Sosa and F. Guevara, «Residencia de perinatología en el Hospital Universitario de Caracas (Venezuela),» Revista Colombiana de Obstetricia y ginecología, vol. 58, nº 1, pp. 53-59, 2007. [4]S. Santisteban, S. Águila, A. Breto, E. Cabezas and J. Delgado, Obstetricia y perinatología. Diagnóstico ytratamiento, La Habana: Editorial de Ciencias Médicas, 2012. [5]M. Roséndiz Moran, A. Valencia Gutiérrez, V. Soto Abraham and A. Durán, «Otocefalia. Informe de un casode autopsia.,» Revista Mexicana de Pediatría, vol. 70, nº 4 julio-agosto, pp. 181-184, 2003. [6]M. Hernández Rodríguez, M. Romero de Fasolino, C. Silva García, A. Morales Machín, I. Sabatini Sáezand C. Fasolino Romero, «Complejo agnatia tocefalia: extendiendo el espectro de anomalías,» Revista Vitae.Academia Biomédica Digital, vol. 1, nº 53 Enero- marzo, pp. 6-12, 2013. [7]J. Ramírez, H. Pachajoa, C. Isaza and W. Saldarriaga, «Síndrome de aglosia adactilia y exposición prenatal amisoprostol ¿Relación causal o casual? Reporte de un caso,» Revista Colombiana de Obstetricia y Ginecología,vol. 59, nº 3, pp. 248-252, 2008.Articlehttps://uctunexpo.autanabooks.com/index.php/uct/article/download/309/550https://uctunexpo.autanabooks.com/index.php/uct/article/view/309W3088855613esUniversidad Ciencia y Tecnología1316-48212542-34012020-06-03
spellingShingle otocephaly
agnathia
aglossia
microstomia
congenital malformations
prenatal diagnosis
fetal echocardiography
adolescent pregnancy
perinatal care
genetic counseling
fetal autopsy
fetal facial anomalies
Congenital Ear and Nasal Anomalies
Craniofacial Disorders and Treatments
Fetal and Pediatric Neurological Disorders
Yohanna Romero
Jose Loaiza
Catherine Marquez
ESTUDIO DE UN CASO DE OTOCEFALIA: CAUSA, CONTROL Y CONSECUENCIA
title ESTUDIO DE UN CASO DE OTOCEFALIA: CAUSA, CONTROL Y CONSECUENCIA
title_full ESTUDIO DE UN CASO DE OTOCEFALIA: CAUSA, CONTROL Y CONSECUENCIA
title_fullStr ESTUDIO DE UN CASO DE OTOCEFALIA: CAUSA, CONTROL Y CONSECUENCIA
title_full_unstemmed ESTUDIO DE UN CASO DE OTOCEFALIA: CAUSA, CONTROL Y CONSECUENCIA
title_short ESTUDIO DE UN CASO DE OTOCEFALIA: CAUSA, CONTROL Y CONSECUENCIA
title_sort estudio de un caso de otocefalia causa control y consecuencia
topic otocephaly
agnathia
aglossia
microstomia
congenital malformations
prenatal diagnosis
fetal echocardiography
adolescent pregnancy
perinatal care
genetic counseling
fetal autopsy
fetal facial anomalies
Congenital Ear and Nasal Anomalies
Craniofacial Disorders and Treatments
Fetal and Pediatric Neurological Disorders
topic_facet otocephaly
agnathia
aglossia
microstomia
congenital malformations
prenatal diagnosis
fetal echocardiography
adolescent pregnancy
perinatal care
genetic counseling
fetal autopsy
fetal facial anomalies
Congenital Ear and Nasal Anomalies
Craniofacial Disorders and Treatments
Fetal and Pediatric Neurological Disorders
url https://uctunexpo.autanabooks.com/index.php/uct/article/download/309/550
https://uctunexpo.autanabooks.com/index.php/uct/article/view/309
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AT joseloaiza estudiodeuncasodeotocefaliacausacontrolyconsecuencia
AT catherinemarquez estudiodeuncasodeotocefaliacausacontrolyconsecuencia