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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J Minim Invasive Gynecol.

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

Rev Chil Obstet Ginecol ; Diagnosis Ultrasonography and MRI are useful in its detection. New classification of Herlyn-Werner-Wunderlich syndrome. Tubular hypodense pelvic mass, kidneys present. Wuunderlich Referenced in the case presentation. On examination, there was a tender lump in the hypogastric region on the left side of midline.

OHVIRA – Wikipedia

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Hwrlyn paramesonephric ducts of the genetically female embryo fuse together in the midline and form the uterus, cervix and the upper four-fifths of the vagina.

Herlyn-Werner-Wunderlich syndrome | Radiology Reference Article |

In patients with uterine and vaginal abnormalities, work up for associated renal anomalies should be performed. J Radiol Case Rep. It presents soon after menarche or shows delayed presentation depending on the type. A Uterus didelphys bicollis and B a distended and blood-filled right hemivagina arrows.

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

CT Heryln density mass in the pelvis with absent ipsilateral kidney.


A diagnosis of uterus didelphys with left haematometra, hameatotrachelos and haematosalpinx with left renal agenesis — suggestive of the Herlyn-Werner-Wunderlich syndrome classification 1. Case Report Presentation of a clinical case of a year-old patient with HWW syndrome presenting with regular menses, dysmenorrhea and painful lump in hypogastric wernet on the left side of midline.

Herlyn-Werner-Wunderlich syndrome uterus didelphys, blind hemivagina and ipsilateral renal agenesis — a case report. These patients have a later age of onset. To alleviate symptoms and retain fertility in these patients, the most effective treatment is surgery.

Sgndrome presentation Pathology Radiographic features Treatment and wundedlich Differential diagnosis References Images: If one of the Wolffian ducts is absent, the ipsilateral kidney and ureter will fail to fuse in the midline. We present an unusual case of a didelphic uterus with pyocolpos in an obstructed hemivagina and ipsilateral renal anomaly figure 1. J Pediatr Adolesc Gynecol ; Retrieved from ” https: We described the role of imaging modalities in diagnosis of the Herlyn-Werner-Wunderlich syndrome with a review of literature.

She had not been sexually active yet. Case presentation A year-old girl, presented to the Emergency Department with a 4-day history of increasing right lower quadrant RLQ and hypogastric pain, nausea and sporadic vomiting without fever, diarrhoea or urinary symptoms. A didelphic uterus results due to embryologic arrest during the 8 th week of gestation. The pelvic mass is the collection of blood products within the obstructed hemivagina.

An analysis of the medical records from these patients revealed several important clinical characteristics of HWWS that were not previously reported in the literature [ Table 1 ]. Of these cases, 79 patients 3. J Womens Med ; 2: Selected MRI images of the pelvis and abdomen.

Speculum exam revealed a fluctuant intravaginal mass.


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

Management and outcome of patients with combined vaginal septum, bifid uterus, and ipsilateral renal agenesis Herlyn-Werner-Wunderlich syndrome J Pediatr Surg. All patients were preoperatively diagnosed by ultrasonography and pelvic examination. Institutional case series and a systematic literature review. Syndrom occurrence of an open Wunderlihc cyst, a homolateral aplasia of the kidney and a double uterus as a typical syndrome of abnormalities.

A didelphic uterus with an obstructed hemivagina and ipsilateral renal agenesis OHVIRA is a rare congenital anomaly constituting 0. Herlyn U, Werner H.

According to our newly identified characteristics, we recommend that the syndrome be classified by the complete or syndeome obstruction of the hemivagina as follows: It is sydnrome to be confused with the Wunderlich syndrome. From Wikipedia, the free encyclopedia.

MRI is considered as a gold standard for diagnosis and pre-operative planning of the Herlyn-Werner-Wunderlich syndrome. Copyright Journal of Radiology Case Reports. MRI of the abdomen and pelvis revealed: J Magn Reson Imaging. An interesting case report on Herlyn-Werner-Wunderlich syndrome with hematoclopos and communicating blind duplex ureters. Although a longitudinal vaginal septum was not directly visualised, its presence was admitted.

The Herlyn-Werner-Wunderlich syndrome is a syndrime, congenital disorder characterised by uterus didelphys, unilateral obstructed hemivagina and ipsilateral renal agenesis, all being secondary to mesonephric duct-induced Mullerian anomalies.

The patient was sent to radiology for further characterization. By using this site, you agree to the Terms of Use and Privacy Policy.