Diprosopus: Systematic review and report of two cases

Autores
Bidondo, Maria Paz; Groisman, Boris; Tardivo, Agostina; Tomasoni, Fabián; Tejeiro, Verónica; Camacho, Inés; Vilas, Mariana; Liascovich, Rosa; Barbero, Pablo Miguel
Año de publicación
2016
Idioma
inglés
Tipo de recurso
artículo
Estado
versión publicada
Descripción
Background: Diprosopus is a subtype of symmetric conjoined twins with one head, facial duplication and a single trunk. Diprosopus is a very rare congenital anomaly. Methods: This is a systematic review of published cases and the presentation of two new cases born in Argentina. We estimated the prevalence of conjoined twins and diprosopus using data from the National Network of Congenital Anomalies of Argentina (RENAC). Results: The prevalence of conjoined twins in RENAC was 19 per 1,000,000 births (95% confidence interval, 12–29). Diprosopus prevalence was 2 per 1,000,000 births (95% confidence interval, 0.2–6.8). In the systematic review, we identified 31 diprosopus cases. The facial structures more frequently duplicated were nose and eyes. Most frequent associated anomalies were: anencephaly, duplication of cerebral hemispheres, craniorachischisis, oral clefts, spinal abnormalities, congenital heart defects, diaphragmatic hernia, thoracic and/or abdominal visceral laterality anomalies. One of the RENAC cases and three cases from the literature had another discordant nonmalformed twin. Conclusion: The conjoined twins prevalence was similar to other studies. The prevalence of diprosopus was higher. The etiology is still unknown. The presence of visceral laterality anomalies may indicate the link between diprosopus and the alteration or duplication of the primitive node in the perigastrulation period (12–15 days postfertilization). Pregnancies of more than two embryos may be a risk factor for diprosopus. Given the low prevalence of this defect, it would be useful to perform studies involving several surveillance systems and international consortiums. Birth Defects Research (Part A), 2016. © 2016 Wiley Periodicals, Inc. Birth Defects Research (Part A) 106:993–1007, 2016. © 2016 Wiley Periodicals, Inc.
Fil: Bidondo, Maria Paz. Dirección Nacional de Instituto de Investigación. Administración Nacional de Laboratorio e Instituto de Salud “Dr. C. G. Malbrán”; Argentina. Universidad de Buenos Aires. Facultad de Medicina. Departamento de Biología Celular e Histología; Argentina
Fil: Groisman, Boris. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina. Dirección Nacional de Instituto de Investigación. Administración Nacional de Laboratorio e Instituto de Salud “Dr. C. G. Malbrán”; Argentina
Fil: Tardivo, Agostina. Dirección Nacional de Instituto de Investigación. Administración Nacional de Laboratorio e Instituto de Salud “Dr. C. G. Malbrán”; Argentina
Fil: Tomasoni, Fabián. Dirección Nacional de Instituto de Investigación. Administración Nacional de Laboratorio e Instituto de Salud “Dr. C. G. Malbrán”; Argentina. Hospital "Evita Pueblo"; Argentina
Fil: Tejeiro, Verónica. Dirección Nacional de Instituto de Investigación. Administración Nacional de Laboratorio e Instituto de Salud “Dr. C. G. Malbrán”; Argentina. Hospital "Evita Pueblo"; Argentina
Fil: Camacho, Inés. Dirección Nacional de Instituto de Investigación. Administración Nacional de Laboratorio e Instituto de Salud “Dr. C. G. Malbrán”; Argentina. Hospital "25 de Mayo"; Argentina
Fil: Vilas, Mariana. Dirección Nacional de Instituto de Investigación. Administración Nacional de Laboratorio e Instituto de Salud “Dr. C. G. Malbrán”; Argentina
Fil: Liascovich, Rosa. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina. Dirección Nacional de Instituto de Investigación. Administración Nacional de Laboratorio e Instituto de Salud “Dr. C. G. Malbrán”; Argentina
Fil: Barbero, Pablo Miguel. Dirección Nacional de Instituto de Investigación. Administración Nacional de Laboratorio e Instituto de Salud “Dr. C. G. Malbrán”; Argentina
Materia
Conjoined Twins
Craniofacial Duplication
Diprosopus
Heterotaxy
Neural Tube Defects
Very Rare Congenital Anomalies
Nivel de accesibilidad
acceso abierto
Condiciones de uso
https://creativecommons.org/licenses/by-nc-sa/2.5/ar/
Repositorio
CONICET Digital (CONICET)
Institución
Consejo Nacional de Investigaciones Científicas y Técnicas
OAI Identificador
oai:ri.conicet.gov.ar:11336/66344
Acceder
id CONICETDig_88ad9841a6f1829c72459c00357e3b91
oai_identifier_str oai:ri.conicet.gov.ar:11336/66344
network_acronym_str CONICETDig
repository_id_str 3498
network_name_str CONICET Digital (CONICET)
spelling Diprosopus: Systematic review and report of two casesBidondo, Maria PazGroisman, BorisTardivo, AgostinaTomasoni, FabiánTejeiro, VerónicaCamacho, InésVilas, MarianaLiascovich, RosaBarbero, Pablo MiguelConjoined TwinsCraniofacial DuplicationDiprosopusHeterotaxyNeural Tube DefectsVery Rare Congenital Anomalieshttps://purl.org/becyt/ford/3.3https://purl.org/becyt/ford/3Background: Diprosopus is a subtype of symmetric conjoined twins with one head, facial duplication and a single trunk. Diprosopus is a very rare congenital anomaly. Methods: This is a systematic review of published cases and the presentation of two new cases born in Argentina. We estimated the prevalence of conjoined twins and diprosopus using data from the National Network of Congenital Anomalies of Argentina (RENAC). Results: The prevalence of conjoined twins in RENAC was 19 per 1,000,000 births (95% confidence interval, 12–29). Diprosopus prevalence was 2 per 1,000,000 births (95% confidence interval, 0.2–6.8). In the systematic review, we identified 31 diprosopus cases. The facial structures more frequently duplicated were nose and eyes. Most frequent associated anomalies were: anencephaly, duplication of cerebral hemispheres, craniorachischisis, oral clefts, spinal abnormalities, congenital heart defects, diaphragmatic hernia, thoracic and/or abdominal visceral laterality anomalies. One of the RENAC cases and three cases from the literature had another discordant nonmalformed twin. Conclusion: The conjoined twins prevalence was similar to other studies. The prevalence of diprosopus was higher. The etiology is still unknown. The presence of visceral laterality anomalies may indicate the link between diprosopus and the alteration or duplication of the primitive node in the perigastrulation period (12–15 days postfertilization). Pregnancies of more than two embryos may be a risk factor for diprosopus. Given the low prevalence of this defect, it would be useful to perform studies involving several surveillance systems and international consortiums. Birth Defects Research (Part A), 2016. © 2016 Wiley Periodicals, Inc. Birth Defects Research (Part A) 106:993–1007, 2016. © 2016 Wiley Periodicals, Inc.Fil: Bidondo, Maria Paz. Dirección Nacional de Instituto de Investigación. Administración Nacional de Laboratorio e Instituto de Salud “Dr. C. G. Malbrán”; Argentina. Universidad de Buenos Aires. Facultad de Medicina. Departamento de Biología Celular e Histología; ArgentinaFil: Groisman, Boris. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina. Dirección Nacional de Instituto de Investigación. Administración Nacional de Laboratorio e Instituto de Salud “Dr. C. G. Malbrán”; ArgentinaFil: Tardivo, Agostina. Dirección Nacional de Instituto de Investigación. Administración Nacional de Laboratorio e Instituto de Salud “Dr. C. G. Malbrán”; ArgentinaFil: Tomasoni, Fabián. Dirección Nacional de Instituto de Investigación. Administración Nacional de Laboratorio e Instituto de Salud “Dr. C. G. Malbrán”; Argentina. Hospital "Evita Pueblo"; ArgentinaFil: Tejeiro, Verónica. Dirección Nacional de Instituto de Investigación. Administración Nacional de Laboratorio e Instituto de Salud “Dr. C. G. Malbrán”; Argentina. Hospital "Evita Pueblo"; ArgentinaFil: Camacho, Inés. Dirección Nacional de Instituto de Investigación. Administración Nacional de Laboratorio e Instituto de Salud “Dr. C. G. Malbrán”; Argentina. Hospital "25 de Mayo"; ArgentinaFil: Vilas, Mariana. Dirección Nacional de Instituto de Investigación. Administración Nacional de Laboratorio e Instituto de Salud “Dr. C. G. Malbrán”; ArgentinaFil: Liascovich, Rosa. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina. Dirección Nacional de Instituto de Investigación. Administración Nacional de Laboratorio e Instituto de Salud “Dr. C. G. Malbrán”; ArgentinaFil: Barbero, Pablo Miguel. Dirección Nacional de Instituto de Investigación. Administración Nacional de Laboratorio e Instituto de Salud “Dr. C. G. Malbrán”; ArgentinaWiley-liss, Div John Wiley & Sons Inc2016-12info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionhttp://purl.org/coar/resource_type/c_6501info:ar-repo/semantics/articuloapplication/pdfapplication/pdfhttp://hdl.handle.net/11336/66344Bidondo, Maria Paz; Groisman, Boris; Tardivo, Agostina; Tomasoni, Fabián; Tejeiro, Verónica; et al.; Diprosopus: Systematic review and report of two cases; Wiley-liss, Div John Wiley & Sons Inc; Birth Defects Research Part A: Clinical and Molecular Teratology; 106; 12; 12-2016; 993-10071542-0752CONICET DigitalCONICETenginfo:eu-repo/semantics/altIdentifier/doi/10.1002/bdra.23549info:eu-repo/semantics/altIdentifier/url/https://onlinelibrary.wiley.com/doi/abs/10.1002/bdra.23549info:eu-repo/semantics/openAccesshttps://creativecommons.org/licenses/by-nc-sa/2.5/ar/reponame:CONICET Digital (CONICET)instname:Consejo Nacional de Investigaciones Científicas y Técnicas2025-10-22T11:04:04Zoai:ri.conicet.gov.ar:11336/66344instacron:CONICETInstitucionalhttp://ri.conicet.gov.ar/Organismo científico-tecnológicoNo correspondehttp://ri.conicet.gov.ar/oai/requestdasensio@conicet.gov.ar; lcarlino@conicet.gov.arArgentinaNo correspondeNo correspondeNo correspondeopendoar:34982025-10-22 11:04:04.539CONICET Digital (CONICET) - Consejo Nacional de Investigaciones Científicas y Técnicasfalse
dc.title.none.fl_str_mv Diprosopus: Systematic review and report of two cases
title Diprosopus: Systematic review and report of two cases
spellingShingle Diprosopus: Systematic review and report of two cases
Bidondo, Maria Paz
Conjoined Twins
Craniofacial Duplication
Diprosopus
Heterotaxy
Neural Tube Defects
Very Rare Congenital Anomalies
title_short Diprosopus: Systematic review and report of two cases
title_full Diprosopus: Systematic review and report of two cases
title_fullStr Diprosopus: Systematic review and report of two cases
title_full_unstemmed Diprosopus: Systematic review and report of two cases
title_sort Diprosopus: Systematic review and report of two cases
dc.creator.none.fl_str_mv Bidondo, Maria Paz
Groisman, Boris
Tardivo, Agostina
Tomasoni, Fabián
Tejeiro, Verónica
Camacho, Inés
Vilas, Mariana
Liascovich, Rosa
Barbero, Pablo Miguel
author Bidondo, Maria Paz
author_facet Bidondo, Maria Paz
Groisman, Boris
Tardivo, Agostina
Tomasoni, Fabián
Tejeiro, Verónica
Camacho, Inés
Vilas, Mariana
Liascovich, Rosa
Barbero, Pablo Miguel
author_role author
author2 Groisman, Boris
Tardivo, Agostina
Tomasoni, Fabián
Tejeiro, Verónica
Camacho, Inés
Vilas, Mariana
Liascovich, Rosa
Barbero, Pablo Miguel
author2_role author
author
author
author
author
author
author
author
dc.subject.none.fl_str_mv Conjoined Twins
Craniofacial Duplication
Diprosopus
Heterotaxy
Neural Tube Defects
Very Rare Congenital Anomalies
topic Conjoined Twins
Craniofacial Duplication
Diprosopus
Heterotaxy
Neural Tube Defects
Very Rare Congenital Anomalies
purl_subject.fl_str_mv https://purl.org/becyt/ford/3.3
https://purl.org/becyt/ford/3
dc.description.none.fl_txt_mv Background: Diprosopus is a subtype of symmetric conjoined twins with one head, facial duplication and a single trunk. Diprosopus is a very rare congenital anomaly. Methods: This is a systematic review of published cases and the presentation of two new cases born in Argentina. We estimated the prevalence of conjoined twins and diprosopus using data from the National Network of Congenital Anomalies of Argentina (RENAC). Results: The prevalence of conjoined twins in RENAC was 19 per 1,000,000 births (95% confidence interval, 12–29). Diprosopus prevalence was 2 per 1,000,000 births (95% confidence interval, 0.2–6.8). In the systematic review, we identified 31 diprosopus cases. The facial structures more frequently duplicated were nose and eyes. Most frequent associated anomalies were: anencephaly, duplication of cerebral hemispheres, craniorachischisis, oral clefts, spinal abnormalities, congenital heart defects, diaphragmatic hernia, thoracic and/or abdominal visceral laterality anomalies. One of the RENAC cases and three cases from the literature had another discordant nonmalformed twin. Conclusion: The conjoined twins prevalence was similar to other studies. The prevalence of diprosopus was higher. The etiology is still unknown. The presence of visceral laterality anomalies may indicate the link between diprosopus and the alteration or duplication of the primitive node in the perigastrulation period (12–15 days postfertilization). Pregnancies of more than two embryos may be a risk factor for diprosopus. Given the low prevalence of this defect, it would be useful to perform studies involving several surveillance systems and international consortiums. Birth Defects Research (Part A), 2016. © 2016 Wiley Periodicals, Inc. Birth Defects Research (Part A) 106:993–1007, 2016. © 2016 Wiley Periodicals, Inc.
Fil: Bidondo, Maria Paz. Dirección Nacional de Instituto de Investigación. Administración Nacional de Laboratorio e Instituto de Salud “Dr. C. G. Malbrán”; Argentina. Universidad de Buenos Aires. Facultad de Medicina. Departamento de Biología Celular e Histología; Argentina
Fil: Groisman, Boris. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina. Dirección Nacional de Instituto de Investigación. Administración Nacional de Laboratorio e Instituto de Salud “Dr. C. G. Malbrán”; Argentina
Fil: Tardivo, Agostina. Dirección Nacional de Instituto de Investigación. Administración Nacional de Laboratorio e Instituto de Salud “Dr. C. G. Malbrán”; Argentina
Fil: Tomasoni, Fabián. Dirección Nacional de Instituto de Investigación. Administración Nacional de Laboratorio e Instituto de Salud “Dr. C. G. Malbrán”; Argentina. Hospital "Evita Pueblo"; Argentina
Fil: Tejeiro, Verónica. Dirección Nacional de Instituto de Investigación. Administración Nacional de Laboratorio e Instituto de Salud “Dr. C. G. Malbrán”; Argentina. Hospital "Evita Pueblo"; Argentina
Fil: Camacho, Inés. Dirección Nacional de Instituto de Investigación. Administración Nacional de Laboratorio e Instituto de Salud “Dr. C. G. Malbrán”; Argentina. Hospital "25 de Mayo"; Argentina
Fil: Vilas, Mariana. Dirección Nacional de Instituto de Investigación. Administración Nacional de Laboratorio e Instituto de Salud “Dr. C. G. Malbrán”; Argentina
Fil: Liascovich, Rosa. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina. Dirección Nacional de Instituto de Investigación. Administración Nacional de Laboratorio e Instituto de Salud “Dr. C. G. Malbrán”; Argentina
Fil: Barbero, Pablo Miguel. Dirección Nacional de Instituto de Investigación. Administración Nacional de Laboratorio e Instituto de Salud “Dr. C. G. Malbrán”; Argentina
description Background: Diprosopus is a subtype of symmetric conjoined twins with one head, facial duplication and a single trunk. Diprosopus is a very rare congenital anomaly. Methods: This is a systematic review of published cases and the presentation of two new cases born in Argentina. We estimated the prevalence of conjoined twins and diprosopus using data from the National Network of Congenital Anomalies of Argentina (RENAC). Results: The prevalence of conjoined twins in RENAC was 19 per 1,000,000 births (95% confidence interval, 12–29). Diprosopus prevalence was 2 per 1,000,000 births (95% confidence interval, 0.2–6.8). In the systematic review, we identified 31 diprosopus cases. The facial structures more frequently duplicated were nose and eyes. Most frequent associated anomalies were: anencephaly, duplication of cerebral hemispheres, craniorachischisis, oral clefts, spinal abnormalities, congenital heart defects, diaphragmatic hernia, thoracic and/or abdominal visceral laterality anomalies. One of the RENAC cases and three cases from the literature had another discordant nonmalformed twin. Conclusion: The conjoined twins prevalence was similar to other studies. The prevalence of diprosopus was higher. The etiology is still unknown. The presence of visceral laterality anomalies may indicate the link between diprosopus and the alteration or duplication of the primitive node in the perigastrulation period (12–15 days postfertilization). Pregnancies of more than two embryos may be a risk factor for diprosopus. Given the low prevalence of this defect, it would be useful to perform studies involving several surveillance systems and international consortiums. Birth Defects Research (Part A), 2016. © 2016 Wiley Periodicals, Inc. Birth Defects Research (Part A) 106:993–1007, 2016. © 2016 Wiley Periodicals, Inc.
publishDate 2016
dc.date.none.fl_str_mv 2016-12
dc.type.none.fl_str_mv info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
http://purl.org/coar/resource_type/c_6501
info:ar-repo/semantics/articulo
format article
status_str publishedVersion
dc.identifier.none.fl_str_mv http://hdl.handle.net/11336/66344
Bidondo, Maria Paz; Groisman, Boris; Tardivo, Agostina; Tomasoni, Fabián; Tejeiro, Verónica; et al.; Diprosopus: Systematic review and report of two cases; Wiley-liss, Div John Wiley & Sons Inc; Birth Defects Research Part A: Clinical and Molecular Teratology; 106; 12; 12-2016; 993-1007
1542-0752
CONICET Digital
CONICET
url http://hdl.handle.net/11336/66344
identifier_str_mv Bidondo, Maria Paz; Groisman, Boris; Tardivo, Agostina; Tomasoni, Fabián; Tejeiro, Verónica; et al.; Diprosopus: Systematic review and report of two cases; Wiley-liss, Div John Wiley & Sons Inc; Birth Defects Research Part A: Clinical and Molecular Teratology; 106; 12; 12-2016; 993-1007
1542-0752
CONICET Digital
CONICET
dc.language.none.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv info:eu-repo/semantics/altIdentifier/doi/10.1002/bdra.23549
info:eu-repo/semantics/altIdentifier/url/https://onlinelibrary.wiley.com/doi/abs/10.1002/bdra.23549
dc.rights.none.fl_str_mv info:eu-repo/semantics/openAccess
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eu_rights_str_mv openAccess
rights_invalid_str_mv https://creativecommons.org/licenses/by-nc-sa/2.5/ar/
dc.format.none.fl_str_mv application/pdf
application/pdf
dc.publisher.none.fl_str_mv Wiley-liss, Div John Wiley & Sons Inc
publisher.none.fl_str_mv Wiley-liss, Div John Wiley & Sons Inc
dc.source.none.fl_str_mv reponame:CONICET Digital (CONICET)
instname:Consejo Nacional de Investigaciones Científicas y Técnicas
reponame_str CONICET Digital (CONICET)
collection CONICET Digital (CONICET)
instname_str Consejo Nacional de Investigaciones Científicas y Técnicas
repository.name.fl_str_mv CONICET Digital (CONICET) - Consejo Nacional de Investigaciones Científicas y Técnicas
repository.mail.fl_str_mv dasensio@conicet.gov.ar; lcarlino@conicet.gov.ar
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