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Herlyn-Werner-Wunderlich syndrome: Diagnosis and treatment of an atypical case and review of literature
INTRODUCTION: Herlyn-Werner-Wunderlich syndrome (HWWS), also known as OHVIRA syndrome, is a rare congenital abnormality of Müllerian duct development characterized by uterus didelphys, unilateral blind hemivagina, and ipsilateral renal agenesis. PRESENTATION OF CASE: In this report, the authors pres...
Autores principales: | , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Elsevier
2019
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6796699/ https://www.ncbi.nlm.nih.gov/pubmed/31586891 http://dx.doi.org/10.1016/j.ijscr.2019.08.035 |
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author | Girardi Fachin, Camila Aleixes Sampaio Rocha, João Lucas Atuati Maltoni, Amanda das Chagas Lima, Raquel Lins Arias Zendim, Vitória Agulham, Miguel Angelo Tsouristakis, Alina dos Santos Dias, André Ivan Bradley |
author_facet | Girardi Fachin, Camila Aleixes Sampaio Rocha, João Lucas Atuati Maltoni, Amanda das Chagas Lima, Raquel Lins Arias Zendim, Vitória Agulham, Miguel Angelo Tsouristakis, Alina dos Santos Dias, André Ivan Bradley |
author_sort | Girardi Fachin, Camila |
collection | PubMed |
description | INTRODUCTION: Herlyn-Werner-Wunderlich syndrome (HWWS), also known as OHVIRA syndrome, is a rare congenital abnormality of Müllerian duct development characterized by uterus didelphys, unilateral blind hemivagina, and ipsilateral renal agenesis. PRESENTATION OF CASE: In this report, the authors present the case of a 12-year-old patient presenting with progressive and disabling abdominal pain after the onset of menarche diagnosed with HWWS, describe the various diagnostic modalities and treatment options available, along with a current review of the literature. DISCUSSION: With normal external genitalia, HWWS is usually asymptomatic until menarche when patients present with worsening abdominal pain during menses and a palpable pelvic or abdominal mass. Untreated, HWWS may lead to a number of complications including endometriosis, infertility, and spontaneous abortion. CONCLUSION: Greater awareness of HWWS will lead to earlier detection and is the key to alleviating patient suffering and avoiding potentially severe complications. |
format | Online Article Text |
id | pubmed-6796699 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2019 |
publisher | Elsevier |
record_format | MEDLINE/PubMed |
spelling | pubmed-67966992019-10-22 Herlyn-Werner-Wunderlich syndrome: Diagnosis and treatment of an atypical case and review of literature Girardi Fachin, Camila Aleixes Sampaio Rocha, João Lucas Atuati Maltoni, Amanda das Chagas Lima, Raquel Lins Arias Zendim, Vitória Agulham, Miguel Angelo Tsouristakis, Alina dos Santos Dias, André Ivan Bradley Int J Surg Case Rep Article INTRODUCTION: Herlyn-Werner-Wunderlich syndrome (HWWS), also known as OHVIRA syndrome, is a rare congenital abnormality of Müllerian duct development characterized by uterus didelphys, unilateral blind hemivagina, and ipsilateral renal agenesis. PRESENTATION OF CASE: In this report, the authors present the case of a 12-year-old patient presenting with progressive and disabling abdominal pain after the onset of menarche diagnosed with HWWS, describe the various diagnostic modalities and treatment options available, along with a current review of the literature. DISCUSSION: With normal external genitalia, HWWS is usually asymptomatic until menarche when patients present with worsening abdominal pain during menses and a palpable pelvic or abdominal mass. Untreated, HWWS may lead to a number of complications including endometriosis, infertility, and spontaneous abortion. CONCLUSION: Greater awareness of HWWS will lead to earlier detection and is the key to alleviating patient suffering and avoiding potentially severe complications. Elsevier 2019-09-13 /pmc/articles/PMC6796699/ /pubmed/31586891 http://dx.doi.org/10.1016/j.ijscr.2019.08.035 Text en © 2019 The Authors http://creativecommons.org/licenses/by/4.0/ This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Article Girardi Fachin, Camila Aleixes Sampaio Rocha, João Lucas Atuati Maltoni, Amanda das Chagas Lima, Raquel Lins Arias Zendim, Vitória Agulham, Miguel Angelo Tsouristakis, Alina dos Santos Dias, André Ivan Bradley Herlyn-Werner-Wunderlich syndrome: Diagnosis and treatment of an atypical case and review of literature |
title | Herlyn-Werner-Wunderlich syndrome: Diagnosis and treatment of an atypical case and review of literature |
title_full | Herlyn-Werner-Wunderlich syndrome: Diagnosis and treatment of an atypical case and review of literature |
title_fullStr | Herlyn-Werner-Wunderlich syndrome: Diagnosis and treatment of an atypical case and review of literature |
title_full_unstemmed | Herlyn-Werner-Wunderlich syndrome: Diagnosis and treatment of an atypical case and review of literature |
title_short | Herlyn-Werner-Wunderlich syndrome: Diagnosis and treatment of an atypical case and review of literature |
title_sort | herlyn-werner-wunderlich syndrome: diagnosis and treatment of an atypical case and review of literature |
topic | Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6796699/ https://www.ncbi.nlm.nih.gov/pubmed/31586891 http://dx.doi.org/10.1016/j.ijscr.2019.08.035 |
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