Cargando…

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...

Descripción completa

Detalles Bibliográficos
Autores principales: 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
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2019
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
_version_ 1783459666885345280
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
work_keys_str_mv AT girardifachincamila herlynwernerwunderlichsyndromediagnosisandtreatmentofanatypicalcaseandreviewofliterature
AT aleixessampaiorochajoaolucas herlynwernerwunderlichsyndromediagnosisandtreatmentofanatypicalcaseandreviewofliterature
AT atuatimaltoniamanda herlynwernerwunderlichsyndromediagnosisandtreatmentofanatypicalcaseandreviewofliterature
AT daschagaslimaraquellins herlynwernerwunderlichsyndromediagnosisandtreatmentofanatypicalcaseandreviewofliterature
AT ariaszendimvitoria herlynwernerwunderlichsyndromediagnosisandtreatmentofanatypicalcaseandreviewofliterature
AT agulhammiguelangelo herlynwernerwunderlichsyndromediagnosisandtreatmentofanatypicalcaseandreviewofliterature
AT tsouristakisalina herlynwernerwunderlichsyndromediagnosisandtreatmentofanatypicalcaseandreviewofliterature
AT dossantosdiasandreivanbradley herlynwernerwunderlichsyndromediagnosisandtreatmentofanatypicalcaseandreviewofliterature