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Herlyn-Werner-Wunderlich Syndrome: Case Report and Review of the Literature

Herlyn-Werner-Wunderlich (HWW) syndrome is a very rare congenital malformation of the urogenital tract involving both the müllerian and the wolffian ducts, and it consists of the association of didelphys uterus, ipsilateral obstructed hemivagina, and ipsilateral kidney agenesis. Its etiology is rela...

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Autores principales: Panaitescu, Anca Maria, Peltecu, Gheorghe, Gică, Nicolae
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9599958/
https://www.ncbi.nlm.nih.gov/pubmed/36292155
http://dx.doi.org/10.3390/diagnostics12102466
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author Panaitescu, Anca Maria
Peltecu, Gheorghe
Gică, Nicolae
author_facet Panaitescu, Anca Maria
Peltecu, Gheorghe
Gică, Nicolae
author_sort Panaitescu, Anca Maria
collection PubMed
description Herlyn-Werner-Wunderlich (HWW) syndrome is a very rare congenital malformation of the urogenital tract involving both the müllerian and the wolffian ducts, and it consists of the association of didelphys uterus, ipsilateral obstructed hemivagina, and ipsilateral kidney agenesis. Its etiology is related to the abnormal development of two embryonic structures—mesonephros and paramesonephros—although its precise mechanism is not known, but theories involving signaling molecules and gene expression are studied for potential explanations. Because of its rarity, there is limited literature on this subject. We present a case diagnosed in our department and elaborate on management. In HWW syndrome, symptoms appear after menarche and include pelvic pain, with progressive intensity due to the developing of hematocolpos. Menstruation may be present or absent depending on the type of anomaly. The use of magnetic resonance imaging (MRI) is the most recommended method of investigation since, in most cases, at this age, sexual life has not started yet and transvaginal ultrasound cannot be used. Surgical treatment in our case consisted of an exploratory laparoscopy followed by a vaginal surgical approach to resect the vaginal septum of the obstructed hemivagina.
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spelling pubmed-95999582022-10-27 Herlyn-Werner-Wunderlich Syndrome: Case Report and Review of the Literature Panaitescu, Anca Maria Peltecu, Gheorghe Gică, Nicolae Diagnostics (Basel) Case Report Herlyn-Werner-Wunderlich (HWW) syndrome is a very rare congenital malformation of the urogenital tract involving both the müllerian and the wolffian ducts, and it consists of the association of didelphys uterus, ipsilateral obstructed hemivagina, and ipsilateral kidney agenesis. Its etiology is related to the abnormal development of two embryonic structures—mesonephros and paramesonephros—although its precise mechanism is not known, but theories involving signaling molecules and gene expression are studied for potential explanations. Because of its rarity, there is limited literature on this subject. We present a case diagnosed in our department and elaborate on management. In HWW syndrome, symptoms appear after menarche and include pelvic pain, with progressive intensity due to the developing of hematocolpos. Menstruation may be present or absent depending on the type of anomaly. The use of magnetic resonance imaging (MRI) is the most recommended method of investigation since, in most cases, at this age, sexual life has not started yet and transvaginal ultrasound cannot be used. Surgical treatment in our case consisted of an exploratory laparoscopy followed by a vaginal surgical approach to resect the vaginal septum of the obstructed hemivagina. MDPI 2022-10-12 /pmc/articles/PMC9599958/ /pubmed/36292155 http://dx.doi.org/10.3390/diagnostics12102466 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Case Report
Panaitescu, Anca Maria
Peltecu, Gheorghe
Gică, Nicolae
Herlyn-Werner-Wunderlich Syndrome: Case Report and Review of the Literature
title Herlyn-Werner-Wunderlich Syndrome: Case Report and Review of the Literature
title_full Herlyn-Werner-Wunderlich Syndrome: Case Report and Review of the Literature
title_fullStr Herlyn-Werner-Wunderlich Syndrome: Case Report and Review of the Literature
title_full_unstemmed Herlyn-Werner-Wunderlich Syndrome: Case Report and Review of the Literature
title_short Herlyn-Werner-Wunderlich Syndrome: Case Report and Review of the Literature
title_sort herlyn-werner-wunderlich syndrome: case report and review of the literature
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9599958/
https://www.ncbi.nlm.nih.gov/pubmed/36292155
http://dx.doi.org/10.3390/diagnostics12102466
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