Question:

What are the typical imaging findings of OHVIRA (obstructed hemivagina with ipsilateral renal agenesis)?
1. Septate uterus with hematometrocolpos and contralateral renal agenesis.
2. Didelphic uterus with obstructed hemivagina and ipsilateral renal agenesis.
3. Bicornuate uterus with hemivaginal atrophy and ipsilateral renal hypoplasia.
4. Hematometrocolpos with bilateral renal hypoplasia.
5. Vaginal hypoplasia with ipsilateral UPJ obstruction.





Answer:

The correct answer for the question "What are the typical imaging findings of OHVIRA (obstructed hemivagina with ipsilateral renal agenesis)?" is:

2. Didelphic uterus with obstructed hemivagina and ipsilateral renal agenesis.



Explanation
a. OHVIRA can have a septate uterus or didelphic uterus, but has hematometrocolpos/pyocolpos with ipsilateral renal agenesis, not contralateral renal agenesis.

b. Applies- Didelphic or septate uterus with obstructed hemivagina resulting in hematometrocolpos or pyocolpos with ipsilateral renal agenesis [Discussion paragraph 4].

c. OHVIRA is not associated with a bicornuate uterus, hemivaginal atrophy or renal hypoplasia. OHVIRA is a didelphic uterus with hemivaginal obstruction and ipsilateral renal agenesis.

d. There is no bilateral renal hypoplasia. The patient presents with renal agenesis on the side of hemivaginal obstruction.

e. OHVIRA does not present with vaginal hypoplasia or ipsilateral UPJ obstruction. Rather, there is a didelphic uterus with hemivaginal obstruction and ipsilateral renal agenesis.



From the manuscript:
Herlyn-Werner-Wunderlich syndrome: a rare presentation with pyocolpos
Radiology Case. 2012 Mar; 6(3):9-15


This article belongs to the OB/GYN section.




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From the manuscript

Herlyn-Werner-Wunderlich syndrome: a rare presentation with pyocolpos

Free full text article: Herlyn-Werner-Wunderlich syndrome: a rare presentation with pyocolpos

Abstract
Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), also known as Herlyn-Werner-Wunderlich syndrome, is a rare syndrome with only a few hundred reported cases described since 1922. Only a handful of these cases have been associated with pyocolpos. Mullerian duct anomalies have an incidence of 2-3%. While OHVIRA constitutes 0.16-10% of these Mullerian duct anomalies. Symptoms usually present shortly after menarche when hematocolpos develops during menstruation resulting in dysmenorrhea and a pelvic mass. The pelvic mass is the collection of blood products within the obstructed hemivagina. The first study in the diagnostic work-up is usually ultrasonography, which typically demonstrates a pelvic fluid collection which can simulate other disease processes thus confounding the diagnosis. MRI findings of the pelvis reveal a didelphic uterus. Imaging of the abdomen reveals agenesis of the ipsilateral kidney. MRI is beneficial in characterizing the didelphic uterus and vaginal septum for pre-operative planning. Understanding the imaging findings, in conjunction with the clinical presentation, is critical for early diagnosis in attempting to prevent complications such as endometriosis or adhesions from chronic infections with subsequent infertility.






References



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