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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| Format: | Article |
| Language: | English |
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MDPI AG
2022-10-01
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| Series: | Diagnostics |
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| Online Access: | https://www.mdpi.com/2075-4418/12/10/2466 |
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| author | Anca Maria Panaitescu Gheorghe Peltecu Nicolae Gică |
| author_facet | Anca Maria Panaitescu Gheorghe Peltecu Nicolae Gică |
| author_sort | Anca Maria Panaitescu |
| collection | DOAJ |
| 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. |
| first_indexed | 2024-03-09T20:22:08Z |
| format | Article |
| id | doaj.art-2cd882dc99274aeaa511d8c8ff6b2cf7 |
| institution | Directory Open Access Journal |
| issn | 2075-4418 |
| language | English |
| last_indexed | 2024-03-09T20:22:08Z |
| publishDate | 2022-10-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Diagnostics |
| spelling | doaj.art-2cd882dc99274aeaa511d8c8ff6b2cf72023-11-23T23:45:46ZengMDPI AGDiagnostics2075-44182022-10-011210246610.3390/diagnostics12102466Herlyn-Werner-Wunderlich Syndrome: Case Report and Review of the LiteratureAnca Maria Panaitescu0Gheorghe Peltecu1Nicolae Gică2Department of Obstetrics and Gynecology, Carol Davila University of Medicine and Pharmacy, 020021 Bucharest, RomaniaDepartment of Obstetrics and Gynecology, Carol Davila University of Medicine and Pharmacy, 020021 Bucharest, RomaniaDepartment of Obstetrics and Gynecology, Carol Davila University of Medicine and Pharmacy, 020021 Bucharest, RomaniaHerlyn-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.https://www.mdpi.com/2075-4418/12/10/2466Herlyn-Werner-Wunderlich syndromediagnosistreatmentprognosis |
| spellingShingle | Anca Maria Panaitescu Gheorghe Peltecu Nicolae Gică Herlyn-Werner-Wunderlich Syndrome: Case Report and Review of the Literature Diagnostics Herlyn-Werner-Wunderlich syndrome diagnosis treatment prognosis |
| 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 | Herlyn-Werner-Wunderlich syndrome diagnosis treatment prognosis |
| url | https://www.mdpi.com/2075-4418/12/10/2466 |
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