Van der woude syndrome - Case report

The Van der Woude Syndrome is a rare anomaly that affects orofacial structures and has been insufficiently reported in Latin America; its identification can be overlooked because it is closely related to cleft lip and palate. The objective of the article is to carry out, to our knowledge, the first...

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Autores: Reinoso Quezada, Santiago José, Moscoso Mesías, Micaela
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:2020
País:Ecuador
Institución:Universidad Central del Ecuador
Repositorio:Revista Odontología
Idioma:español
OAI Identifier:oai:revistadigital.uce.edu.ec:article/2285
Acceso en línea:https://revistadigital.uce.edu.ec/index.php/odontologia/article/view/2285
Access Level:acceso abierto
Palabra clave:Labio Leporino
Fisura del Paladar
Anomalías Congénitas
Anomalías Máxilofaciales
Síndrome
Cleft lip
cleft palate
congenital abnormalities
maxillofacial abnormalities
syndrome
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dc.title.none.fl_str_mv Van der woude syndrome - Case report
Síndrome de Van der woude - Informe de caso
title Van der woude syndrome - Case report
spellingShingle Van der woude syndrome - Case report
Reinoso Quezada, Santiago José
Labio Leporino
Fisura del Paladar
Anomalías Congénitas
Anomalías Máxilofaciales
Síndrome
Cleft lip
cleft palate
congenital abnormalities
maxillofacial abnormalities
syndrome
title_short Van der woude syndrome - Case report
title_full Van der woude syndrome - Case report
title_fullStr Van der woude syndrome - Case report
title_full_unstemmed Van der woude syndrome - Case report
title_sort Van der woude syndrome - Case report
dc.creator.none.fl_str_mv Reinoso Quezada, Santiago José
Moscoso Mesías, Micaela
author Reinoso Quezada, Santiago José
author_facet Reinoso Quezada, Santiago José
Moscoso Mesías, Micaela
author_role author
author2 Moscoso Mesías, Micaela
author2_role author
dc.subject.none.fl_str_mv Labio Leporino
Fisura del Paladar
Anomalías Congénitas
Anomalías Máxilofaciales
Síndrome
Cleft lip
cleft palate
congenital abnormalities
maxillofacial abnormalities
syndrome
topic Labio Leporino
Fisura del Paladar
Anomalías Congénitas
Anomalías Máxilofaciales
Síndrome
Cleft lip
cleft palate
congenital abnormalities
maxillofacial abnormalities
syndrome
description The Van der Woude Syndrome is a rare anomaly that affects orofacial structures and has been insufficiently reported in Latin America; its identification can be overlooked because it is closely related to cleft lip and palate. The objective of the article is to carry out, to our knowledge, the first report in Ecuador on this Syndrome characterized by the congenital association of sinuses in the lower lips with cleft lip and palate. In the Clinic of Lip and Cleft Palate of the Catholic University of Cuenca, a 6-year-old patient with the following characteristics presented in consultation: symmetrical lip fistulas on the lower lip; Asymptomatic and with sequelae of the lip and palate fissured with extreme salivation and dripping through the lip area upon chewing. The SVW may have other important clinical findings such as: skin folds in the limbs, hypodontia, bifid uvula, hookworm, and sensorineural hearing loss, of which the patient only presented bifid uvula and lip fistulas in addition to the sequela of LPF. The main diagnosis is purely clinical, therefore, complementary diagnoses are not needed, using routine studies such as preoperative laboratory exams, plus pediatric anesthesia and cardiology evaluation. The patient had sequelae from her first LPF surgery, so she underwent a new intervention and excision of the lip fistulas without any resulting sequela. Conclusion: In a case of cleft lip and palate with lip fistulas, an inter-consultation with pediatrics should always be carried out to rule out associated syndromes and proceed to their surgical excision, to avoid discomfort during the patient's chewing and for later cosmetic purposes. The surgical technique performed was the Spindle Technique (Fistulectomy), with which the ductal path is completely eliminated and a plane closure of the lip is performed, resulting in no sequelae.
publishDate 2020
dc.date.none.fl_str_mv 2020-07-01
dc.type.none.fl_str_mv info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
format article
status_str publishedVersion
dc.identifier.none.fl_str_mv https://revistadigital.uce.edu.ec/index.php/odontologia/article/view/2285
10.29166/odontologia.vol22.n2.2020-119-129
url https://revistadigital.uce.edu.ec/index.php/odontologia/article/view/2285
identifier_str_mv 10.29166/odontologia.vol22.n2.2020-119-129
dc.language.none.fl_str_mv spa
language spa
dc.relation.none.fl_str_mv https://revistadigital.uce.edu.ec/index.php/odontologia/article/view/2285/2369
https://revistadigital.uce.edu.ec/index.php/odontologia/article/view/2285/2448
https://revistadigital.uce.edu.ec/index.php/odontologia/article/view/2285/2449
dc.rights.none.fl_str_mv Derechos de autor 2020 Santiago José Reinoso Quezada, Micaela Moscoso Mesías
info:eu-repo/semantics/openAccess
rights_invalid_str_mv Derechos de autor 2020 Santiago José Reinoso Quezada, Micaela Moscoso Mesías
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
text/html
text/html
dc.publisher.none.fl_str_mv Universidad Central del Ecuador
publisher.none.fl_str_mv Universidad Central del Ecuador
dc.source.none.fl_str_mv Odontología; Vol. 22 Núm. 2 (2020): Julio - Diciembre; 119-129
1390-9967
10.29166/odontologia.vol22.n2.2020
reponame:Revista Odontología
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instname_str Universidad Central del Ecuador
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repository.name.fl_str_mv Revista Odontología - Universidad Central del Ecuador
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spelling Van der woude syndrome - Case reportSíndrome de Van der woude - Informe de casoReinoso Quezada, Santiago JoséMoscoso Mesías, MicaelaLabio LeporinoFisura del PaladarAnomalías CongénitasAnomalías MáxilofacialesSíndromeCleft lipcleft palatecongenital abnormalitiesmaxillofacial abnormalitiessyndromeThe Van der Woude Syndrome is a rare anomaly that affects orofacial structures and has been insufficiently reported in Latin America; its identification can be overlooked because it is closely related to cleft lip and palate. The objective of the article is to carry out, to our knowledge, the first report in Ecuador on this Syndrome characterized by the congenital association of sinuses in the lower lips with cleft lip and palate. In the Clinic of Lip and Cleft Palate of the Catholic University of Cuenca, a 6-year-old patient with the following characteristics presented in consultation: symmetrical lip fistulas on the lower lip; Asymptomatic and with sequelae of the lip and palate fissured with extreme salivation and dripping through the lip area upon chewing. The SVW may have other important clinical findings such as: skin folds in the limbs, hypodontia, bifid uvula, hookworm, and sensorineural hearing loss, of which the patient only presented bifid uvula and lip fistulas in addition to the sequela of LPF. The main diagnosis is purely clinical, therefore, complementary diagnoses are not needed, using routine studies such as preoperative laboratory exams, plus pediatric anesthesia and cardiology evaluation. The patient had sequelae from her first LPF surgery, so she underwent a new intervention and excision of the lip fistulas without any resulting sequela. Conclusion: In a case of cleft lip and palate with lip fistulas, an inter-consultation with pediatrics should always be carried out to rule out associated syndromes and proceed to their surgical excision, to avoid discomfort during the patient's chewing and for later cosmetic purposes. The surgical technique performed was the Spindle Technique (Fistulectomy), with which the ductal path is completely eliminated and a plane closure of the lip is performed, resulting in no sequelae.El Síndrome de Van der Woude (SVW) es una anomalía infrecuente que afecta a estructuras orofaciales y ha sido insuficientemente reportada en América Latina, su identificación puede ser pasada por alto debido a que está íntimamente relacionada con labio y paladar fisurado. El objetivo del artículo es realizar según nuestro conocimiento, el primer reporte en Ecuador sobre este Síndrome que se caracteriza por la asociación congénita de senos en labios inferiores con labio leporino y paladar hendido. En la Clínica de Labio y Paladar Fisurado de la Universidad Católica de Cuenca se presentó en consulta una paciente de 6 años con las siguientes características: fístulas labiales simétricas en el labio inferior; asintomático y con secuelas de labio y paladar fisurado (LPF) con salivación extrema y goteo por la zona labial a la masticación. El SVW puede contar con otros hallazgos clínicos importantes como: pliegues cutáneos en los miembros, hipodoncia, úvula bífida, anquiloglosia y pérdida de la audición neurosensorial, de los cuales la paciente únicamente presentó úvula bífida y las fístulas labiales además de la secuela de LPF. El principal diagnóstico es meramente clínico, por lo tanto, no se necesitan diagnósticos complementarios, utilizándose los estudios de rutina como exámenes de laboratorio pre operatorios, más la valoración anestésica y cardiológica pediátrica. La paciente presentó secuelas de su primera cirugía de LPF por lo que se procedió a una nueva intervención y a la escisión de las fístulas labiales sin ningún tipo de secuela resultante. Conclusión: En un caso de Labio y Paladar Fisurado con fístulas labiales siempre se deberá realizar interconsulta con Pediatría para descartar síndromes asociados y proceder a su escisión quirúrgica, para evitar molestias durante la masticación del paciente y con fines posteriormente estéticos. La técnica quirúrgica realizada fue la Técnica en Huso (Fistulectomía), con la cual elimina de manera completa el trayecto ductal y se realiza un cierre por planos del labio, resultando sin secuelasUniversidad Central del Ecuador2020-07-01info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionapplication/pdftext/htmltext/htmlhttps://revistadigital.uce.edu.ec/index.php/odontologia/article/view/228510.29166/odontologia.vol22.n2.2020-119-129Odontología; Vol. 22 Núm. 2 (2020): Julio - Diciembre; 119-1291390-996710.29166/odontologia.vol22.n2.2020reponame:Revista Odontologíainstname:Universidad Central del Ecuadorinstacron:UCEspahttps://revistadigital.uce.edu.ec/index.php/odontologia/article/view/2285/2369https://revistadigital.uce.edu.ec/index.php/odontologia/article/view/2285/2448https://revistadigital.uce.edu.ec/index.php/odontologia/article/view/2285/2449Derechos de autor 2020 Santiago José Reinoso Quezada, Micaela Moscoso Mesíasinfo:eu-repo/semantics/openAccess2021-08-20T00:58:12Zoai:revistadigital.uce.edu.ec:article/2285Portal de revistashttps://revistadigital.uce.edu.ec/Universidad públicahttps://uce.edu.ec/**Ecuador*1390-99671390-7468opendoar:02021-08-20T00:58:12Revista Odontología - Universidad Central del Ecuadorfalse
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