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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...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2022
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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. |
format | Online Article Text |
id | pubmed-9599958 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
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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