Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), also known as Herlyn-Werner-Wunderlich syndrome, is a rare syndrome with. Herlyn-Werner-Wunderlich (HWW) syndrome, is a rare anomaly characterised by Müllerian duct anomalies (MDA) associated with mesonephric duct anomalies. Herlyn-Werner-Wunderlich (HWW) syndrome is a very rare congenital anomaly of the urogenital tract involving Müllerian ducts and Wolffian.

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The diagnosis is often difficult due to the infrequency of the syndrome, and a high index of suspicion is required.

The Herlyn-Werner-Wunderlich Syndrome – A Case Report with Radiological Review

She was on the fourth day of menstrual bleeding. Treatment The patient underwent surgery with resection of the vaginal septum. It can exhibit acute pelvic pain shortly after menarche and may show non-specific and variable symptoms with resultant delay in diagnosis. Uterus didelphys, blind hemivagina and ipsilateral renal agenesis. A small communication exists between two vaginas with resultant incomplete obstruction. J Minim Invasive Gynecol. A case report and review of the literature. However, if one of the Wolffian ducts is absent, the kidney and ureter on the ipsilateral side will fail to fuse at midline.

Sitemap What’s New Feedback Disclaimer. Views Read Edit View history. We described the role of imaging modalities in diagnosis of the Herlyn-Werner-Wunderlich syndrome with a review of literature. Case 3 Case 3. The surgical approach depends on the imaging findings. As cervical agenesis is difficult to correct surgically, laparoscopic or transabdominal resection of the affected ipsilateral uterus is suggested [ 3 ].

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The syndrome is classified by complete or incomplete obstruction of the vagina as classification 1 completely obstructed hemivagina and classification 2 incompletely obstructed hemivagina. Case 2 Case 2.

The Herlyn-Werner-Wunderlich Syndrome – A Case Report with Radiological Review

In our study, we reviewed the characteristics of all HWWS patients at Peking Union Medical College Hospital PUMCH and suggested a new classification for this syndrome because the clinical characteristics differed significantly between the completely and incompletely obstructed vaginal septum.

This enable us to diagnose some patients before menarche and carry out a surgical correction of the obstruction before any damage has occurred because of haematocolpos, haematometra and retrograde menstruation.

A Uterus didelphys bicollis and B a distended and blood-filled right hemivagina arrows. National Center for Biotechnology InformationU. Family history was relevant for a paternal aunt with ovarian and cervical cancer and a mother with questionable cervical cancer.

The attack often comes years syndromf menarche. Obstructed hemivagina and ipsilateral renal anomaly OHVIRAalso known as Herlyn-Werner-Wunderlich syndrome, is a rare syndrome with only a few hundred reported cases described since There was no history of menorrhagia. There is no communication between the duplicated uterus wunderlkch vagina.

It is not to be confused with the Wunderlich syndrome. The left endometrial cavity was moderately distended and contiguous with the obstructed hemivagina. Each ureteric bud develops from the Wolffian duct which is ultimately responsible for the development of the kidney. Awareness is necessary in order to diagnose and treat this disorder properly before complications occur.


She denied fevers, chills, nausea, vomiting or wnderlich.

Herlyn–Werner–Wunderlich syndrome: a rare cause of pelvic pain in adolescent girls

It can be classified based on a completely or incompletely obstructed hemivagina. Studies of uterine organogenesis have implicated the Hox and Wnt genes as regulators of uterine morphology.

Clinical suspicion and awareness of the syndrome are therefore imperative to making a timely diagnosis and preventing these complications. Haemoperitoneum due to bleeding from the fallopian tube can be found at surgery [ 10 ].

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Rare disease: Herlyn–Werner–Wunderlich syndrome: a rare cause of pelvic pain in adolescent girls

Heterogeneous echogenic pelvic mass with peripheral flow. She had menarche at 12 years of age and her menses were regular. Copyright Journal of Radiology Case Reports. Cervical agenesis is difficult to correct surgically.

How to cite this article: A didelphic uterus with an obstructed hemivagina and ipsilateral renal agenesis OHVIRA is a rare congenital anomaly constituting 0. Support Center Support Center. Complete septate wujderlich, obstructed hemivagina, and ipsilateral renal anomaly: