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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This ultimately affects the Mullerian and metanephric duct [ 9 ]. However, specific gene mutations have winderlich been identified as associated with most MDAs [ 2 ].

She was released 2 days after surgery, showing an uneventful recovery. Although a longitudinal vaginal septum was not directly visualised, its presence was admitted. The exact etiopathogenesis of HWW syndrome is still not known.

Piccinini PS, Doski J. Sonographic and MR findings in 11 cases. Chin Med J Engl. 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 region on the left side of midline. J Radiol Case Rep.

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

MRI with multiplanar image acquisition provides more detailed information. Urological complications associated to uterus didelphys with unilateral hematocolpos. Note the right uterine horn small arrow. Hematometra and hematosalpinx occurred in some more severely affected patients, as well as bleeding in the periadnexal and peritoneal space. J Minim Invasive Gynecol. Pelvic magnetic resonance imaging.

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

This new classification allows for earlier diagnosis and treatment. The patient subsequently underwent successful surgical 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. Case report and review of the literature. Delayed diagnosis can lead to complications that include endometriosis, adhesions, infertility and infectious complications arising from chronic cryptomenorrhoea [ 1 ].

In most cases, the blind hemi-vagina is opened, and the fluid drained. Double uterus, blind hemivagina, and ipsilateral renal agenesis: The syndrome is classified by qunderlich or incomplete obstruction of the vagina as classification 1 completely dyndrome hemivagina and classification 2 incompletely obstructed hemivagina.

Management and outcome of patients with combined vaginal septum, bifid uterus, and ipsilateral renal agenesis Herlyn-werner-wunderlich syndrome. Other associated anomalies include sndrome dysplasia, duplication of the kidneys and ureters, ectopic ureter, high-riding aortic bifurcation, IVC duplication, intestinal malrotation and ovarian malposition [ 2 ].

Drainage is often impeded due to small communication. Progression to secondary endometriosis, pelvic adhesions, pyosalpinx, pyocolpos. Hematocolpos may occur only a few months after menarche. Endometriosis in association with Herlyn-Werner-Wunderlich syndrome. CT and ultrasound syndrlme the most widely used diagnostic tools. Tubular hypodense pelvic mass, kidneys present. There is a spectrum of uterine fusion anomalies that can occur during early development.

New Classification of Herlyn-Werner-Wunderlich Syndrome

Author information Copyright and License information Disclaimer. The presenting symptoms may include the acute onset of abdominal pain, fever, and vomiting. She was on the fourth day of menstrual bleeding.

HWWS patients should be differentiated according to these two classifications. The exact cause of this developmental defect is not known. If neglected, the condition can progress to secondary endometriosis, pelvic adhesions, pyosalpinx and pyocolpos [ 1011 ]. We describe the case of a year-old girl with a known single kidney, recurrent abdominal wunderlichh during menstruation and presentation to the Emergency Department with acute abdomen.


Asymmetric lateral distribution of obstructed hemivagina and renal agenesis in women with uterus didelphys: As a result, two separate symmetrical uterine cavities develop with two cervices and no communication between these cavities; this is often associated with a vaginal septum, which can have a transverse wall that blocks one of the hemivaginas. Journal of Clinical and Diagnostic Research: Patients with this classification have an earlier age of onset, with a short time from menarche to attack.

These treatments inadvertently cause a delay in diagnosis as they diminish or eliminate menses. She denied any prior surgeries, use of werer or allergies.

Please review our privacy policy. Speculum exam revealed a fluctuant intravaginal mass.

New Classification of Herlyn-Werner-Wunderlich Syndrome

Transvaginal sonography has an advantage of low cost, better imaging of the uterus and adnexa with no radiation exposure. Treatment The patient underwent surgery with resection of the vaginal septum.

In this classification, the hemivagina is completely obstructed, and a small communication exists between the duplicated cervices. It can also affect the urethra, urethral sphincter, ureters, bladder and spleen.

Journal List Pol J Radiol v. European Journal of Radiology.