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Case Reports
. 2023 Nov 22;12(23):7227.
doi: 10.3390/jcm12237227.

Obstructed Hemivagina with Ipsilateral Renal Agenesis: A Challenging Case Report and a Management Flow Chart

Ewelina Malanowska-Jarema  1 , Andrzej Starczewski  1 , Yana Osnytska  1 , Mariola Krzyścin  2 , Elżbieta Sowińska-Przepiera  2 , Matteo Balzarro  3 , Emanuele Rubilotta  3
Affiliations
Case Reports

Obstructed Hemivagina with Ipsilateral Renal Agenesis: A Challenging Case Report and a Management Flow Chart

Ewelina Malanowska-Jarema et al. J Clin Med. .

Abstract

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.

Keywords: OHVIRA syndrome; congenital anomalies of kidney and urinary tract (CAKUT); laparoscopic surgery in female adolescents; obstructed vagina; recurrent urinary tract infections (rUTI); renal agenesis.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Ultrasound from extern hospital, when the diagnosis of hematocolpos was mistaken as an ovarian cyst (black arrow). Double uterus was not recognized (white arrow).
Figure 2
Figure 2
MRI presents hematocolpos (blue arrow) and hydrometrocolpos in right uterus (white arrow).
Figure 3
Figure 3
MRI presents hematocolpos (blue arrow) and a left healthy uterus (white arrow).
Figure 4
Figure 4
Intraoperative abdominal sonography and puncture of hematocolpos (white arrow). P is polish equivalent to R-right in english site of the ultrasound screen.
Figure 5
Figure 5
Intraoperative abdominal sonography and drainage of hematocolpos. P is polish equivalent to R-right in english site of the ultrasound screen.
Figure 6
Figure 6
Left healthy uterus in laparoscopic view.
Figure 7
Figure 7
Right uterus with hematometra in laparoscopic view.
Figure 8
Figure 8
Double uterus. Right uterus with hematometra.
Figure 9
Figure 9
Hematosalpinx of right fallopian tube.
Figure 10
Figure 10
Active endometriosis of peritoneum.
See this image and copyright information in PMC

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