OHVIRA: Uterus didelphys, blind hemivagina and ipsilateral renal agenesis: Advantage MRI

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Abstract

We present here a case of an uncommon complex uterine anomaly – Obstructed HemiVagina with Ipsilateral Renal Agenesis (OHVIRA), also known as Herlyn-Werner-Wunderlich syndrome in a 14-year-old girl along with sonographic (trans-abdominal and trans labial), and MRI findings. The patient underwent surgery wherein imaging findings were confirmed. An MRI has proved to be of great help in correct diagnosis avoiding surgical interventions/ laparoscopy, which were needed in past to diagnose this rare anomaly. We also discuss the development of this anomaly with the help of a relatively new theory of uro-genital development by Acien and review the literature.

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Most cited references 7

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Incidence of Müllerian defects in fertile and infertile women.

P Acién (1997)

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Embryological observations on the female genital tract.

P Acién (1992)

The embryology of the genital tract and urinary system is described, and the hypothesis is advanced that the vagina is an organ embryologically derived from the mesonephric or Wolffian ducts in addition to the Müllerian tubercle. This is based on experimental studies and case reports in the literature and our own cases of genital malformations, especially in patients with unilateral renal agenesis and an ipsilateral blind vagina. The importance of the mesonephric ducts as guides or 'inductor' elements for adequate Müllerian development is emphasized. A new embryological classification of female genital malformations is proposed, based on these embryological concepts.

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Association of renal agenesis and mullerian duct anomalies.

S. Li, Mohsin A Qayyum, F Coakley … (2015)

The purpose of this work was to determine the association of renal agenesis with the different types of mullerian duct anomalies (MDAs). A 5 year retrospective review of MR records identified 57 patients with MDAs. Associated renal anomalies were correlated with the various types of MDAs. Renal agenesis was found in 17 (29.8%) of 57 patients. No other renal anomalies were identified. Renal agenesis was more frequent in patients with uterus didelphys (13/16 cases). Renal agenesis was also seen in patients with uterine agenesis (2/5 cases) and unicornuate uterus (2/7 cases). All 11 cases of obstructed uterus didelphys were associated with renal agenesis ipsilateral to the side of the obstructing transverse hemivaginal septum. Renal agenesis is more commonly seen in uterus didelphys than in other types of MDAs. Renal agenesis in patients with uterus didelphys is often ipsilateral to an obstructing, transverse, hemivaginal septum.

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Author and article information

Journal

Journal ID (nlm-ta): J Hum Reprod Sci

Journal ID (iso-abbrev): J Hum Reprod Sci

Journal ID (publisher-id): JHRS

Title: Journal of Human Reproductive Sciences

Publisher: Medknow Publications & Media Pvt Ltd (India )

ISSN (Print): 0974-1208

ISSN (Electronic): 1998-4766

Publication date (Print): Jan-Apr 2012

Volume: 5

Issue: 1

Pages: 67-70

Affiliations

[1]Department of Radiodiagnosis, Safdarjung Hospital and VM Medical College, New Delhi, India

Author notes

Address for correspondence: Dr. Sunil K Bajaj, Department of Radiodiagnosis, Safdarjung Hospital and VM Medical College, New Delhi - 110029, India. E-mail: drskbajaj@ 123456rediffmail.com

Article

Publisher ID: JHRS-5-67

DOI: 10.4103/0974-1208.97811

PMC ID: 3409925

PubMed ID: 22870020

SO-VID: 552c333f-6c4d-4c9a-91c1-703a64dc5d56

License:

This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

History

Date received : 17 October 2011

Date revision received : 09 December 2011

Date accepted : 07 March 2012

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