Obstructed Hemivagina with Ipsilateral Renal Agenesis: A Challenging Case Report and a Management Flow Chart
We present here a case of complex uterine anomaly—obstructed hemivagina with ipsilateral renal agenesis (OHVIRA), also known as Herlyn-Werner-Wunderlich syndrome in a 13-year-old girl with a history of recurrent urinary tract infections (rUTI). In the emergency room, a trans-abdominal sonography rev...
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| Format: | Article |
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MDPI AG
2023-11-01
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| Series: | Journal of Clinical Medicine |
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| Online Access: | https://www.mdpi.com/2077-0383/12/23/7227 |
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| author | Ewelina Malanowska-Jarema Andrzej Starczewski Yana Osnytska Mariola Krzyścin Elżbieta Sowińska-Przepiera Matteo Balzarro Emanuele Rubilotta |
| author_facet | Ewelina Malanowska-Jarema Andrzej Starczewski Yana Osnytska Mariola Krzyścin Elżbieta Sowińska-Przepiera Matteo Balzarro Emanuele Rubilotta |
| author_sort | Ewelina Malanowska-Jarema |
| collection | DOAJ |
| description | We present here a case of complex uterine anomaly—obstructed hemivagina with ipsilateral renal agenesis (OHVIRA), also known as Herlyn-Werner-Wunderlich syndrome in a 13-year-old girl with a history of recurrent urinary tract infections (rUTI). In the emergency room, a trans-abdominal sonography revealed an ovarian cyst and renal agenesis, without any suspicion of vaginal obstruction. This led to a delay in the diagnosis of this uncommon anomaly. Finally, MRI findings confirmed the presence of OHVIRA syndrome. As the congenital anomalies of the kidney and urinary tract (CAKUT) are present in almost one third of cases associated with genital malformations, urologists should carefully screen patients with rUTI. The patient underwent simultaneous laparoscopy and vaginoscopy, which was in our opinion the most appropriate therapeutic decision. In this article, we are also going to discuss the role of laparoscopy in the management of OHVIRA syndrome, as well as other surgical techniques described in the literature. |
| first_indexed | 2024-03-09T01:48:57Z |
| format | Article |
| id | doaj.art-794c1cdb039b49b3a6727ccdb0d45be1 |
| institution | Directory Open Access Journal |
| issn | 2077-0383 |
| language | English |
| last_indexed | 2024-03-09T01:48:57Z |
| publishDate | 2023-11-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Journal of Clinical Medicine |
| spelling | doaj.art-794c1cdb039b49b3a6727ccdb0d45be12023-12-08T15:18:42ZengMDPI AGJournal of Clinical Medicine2077-03832023-11-011223722710.3390/jcm12237227Obstructed Hemivagina with Ipsilateral Renal Agenesis: A Challenging Case Report and a Management Flow ChartEwelina Malanowska-Jarema0Andrzej Starczewski1Yana Osnytska2Mariola Krzyścin3Elżbieta Sowińska-Przepiera4Matteo Balzarro5Emanuele Rubilotta6Department of Gynecology, Endocrinology and Gynecologic Oncology, Pomeranian Medical University, 70-204 Szczecin, PolandDepartment of Gynecology, Endocrinology and Gynecologic Oncology, Pomeranian Medical University, 70-204 Szczecin, PolandDepartment of Gynecology, Endocrinology and Gynecologic Oncology, Pomeranian Medical University, 70-204 Szczecin, PolandDepartment of Endocrinology, Metabolic and Internal Diseases, Pomeranian Medical University, 70-204 Szczecin, PolandDepartment of Endocrinology, Metabolic and Internal Diseases, Pomeranian Medical University, 70-204 Szczecin, PolandDepartment of Urology, Azienda Ospedaliera Universitaria Integrata Verona, Piazzale Stefani 1, 37100 Verona, ItalyDepartment of Urology, Azienda Ospedaliera Universitaria Integrata Verona, Piazzale Stefani 1, 37100 Verona, ItalyWe present here a case of complex uterine anomaly—obstructed hemivagina with ipsilateral renal agenesis (OHVIRA), also known as Herlyn-Werner-Wunderlich syndrome in a 13-year-old girl with a history of recurrent urinary tract infections (rUTI). In the emergency room, a trans-abdominal sonography revealed an ovarian cyst and renal agenesis, without any suspicion of vaginal obstruction. This led to a delay in the diagnosis of this uncommon anomaly. Finally, MRI findings confirmed the presence of OHVIRA syndrome. As the congenital anomalies of the kidney and urinary tract (CAKUT) are present in almost one third of cases associated with genital malformations, urologists should carefully screen patients with rUTI. The patient underwent simultaneous laparoscopy and vaginoscopy, which was in our opinion the most appropriate therapeutic decision. In this article, we are also going to discuss the role of laparoscopy in the management of OHVIRA syndrome, as well as other surgical techniques described in the literature.https://www.mdpi.com/2077-0383/12/23/7227obstructed vaginaOHVIRA syndromerecurrent urinary tract infections (rUTI)congenital anomalies of kidney and urinary tract (CAKUT)renal agenesislaparoscopic surgery in female adolescents |
| spellingShingle | Ewelina Malanowska-Jarema Andrzej Starczewski Yana Osnytska Mariola Krzyścin Elżbieta Sowińska-Przepiera Matteo Balzarro Emanuele Rubilotta Obstructed Hemivagina with Ipsilateral Renal Agenesis: A Challenging Case Report and a Management Flow Chart Journal of Clinical Medicine obstructed vagina OHVIRA syndrome recurrent urinary tract infections (rUTI) congenital anomalies of kidney and urinary tract (CAKUT) renal agenesis laparoscopic surgery in female adolescents |
| title | Obstructed Hemivagina with Ipsilateral Renal Agenesis: A Challenging Case Report and a Management Flow Chart |
| title_full | Obstructed Hemivagina with Ipsilateral Renal Agenesis: A Challenging Case Report and a Management Flow Chart |
| title_fullStr | Obstructed Hemivagina with Ipsilateral Renal Agenesis: A Challenging Case Report and a Management Flow Chart |
| title_full_unstemmed | Obstructed Hemivagina with Ipsilateral Renal Agenesis: A Challenging Case Report and a Management Flow Chart |
| title_short | Obstructed Hemivagina with Ipsilateral Renal Agenesis: A Challenging Case Report and a Management Flow Chart |
| title_sort | obstructed hemivagina with ipsilateral renal agenesis a challenging case report and a management flow chart |
| topic | obstructed vagina OHVIRA syndrome recurrent urinary tract infections (rUTI) congenital anomalies of kidney and urinary tract (CAKUT) renal agenesis laparoscopic surgery in female adolescents |
| url | https://www.mdpi.com/2077-0383/12/23/7227 |
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