Uterine didelphys associated with obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome

Han, Boram; Herndon, Christopher N.; Rosen, Mitchell P.; Wang, Z. Jane

doi:10.2484/rcr.v5i1.327

Cited by 28 publications

(16 citation statements)

References 13 publications

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“…Uterine anomalies can result from failure of the Müllerian ducts to fuse in the midline, to connect with the urogenital sinus, or to create the appropriate lumen in the upper vagina and uterus by resorption of the central vaginal cells and the septum between the fused Müllerian ducts [ 2 4 ]. Lack of fusion of the two Müllerian ducts results in duplication of corpus and cervix [ 5 ]. Occasionally, uterus didelphys is associated with an obstructed hemivagina and often with ipsilateral renal agenesis [ 6 ].…”

Section: Discussionmentioning

confidence: 99%

Herlyn-Werner-Wunderlich syndrome: An unusual presentation with pyocolpos

et al. 2017

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Herlyn-Werner-Wunderlich syndrome is a rare congenital anomaly of the urogenital tract, which is characterized by the triad of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. It usually presents at puberty with pelvic pain, dysmenorrhea, and a vaginal or pelvic mass. Although rare, it may present with purulent vaginal discharge due to secondary infection of the obstructed hemivagina, making diagnosis difficult. A careful pelvic examination to identify the cervix and vagina is the key to the diagnosis of Müllerian duct anomalies and magnetic resonance imaging can provide additional useful information. The optimal treatment is full excision and marsupialization of the obstructing vaginal septum so that both uteri can drain through the patent vagina. The authors report a case of a 22-year-old female with an unusual presentation of Herlyn-Werner-Wunderlich syndrome complicated by pyocolpos, which was successfully managed by vaginal septum resection and drainage of pus.

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Section: Discussionmentioning

confidence: 99%

Herlyn-Werner-Wunderlich syndrome: An unusual presentation with pyocolpos

et al. 2017

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“…In case of septate uterus, septa is demonstrated unequivocally by MRI. [7][8][9][10] The standard management of these patients is excision of vaginal septum and drainage of hematometrocolpos. 9,10 …”

Section: Discussionmentioning

confidence: 99%

Uterine didelphys with obstructed hemivagina and ipsilateral renal anomaly - OHVIRA syndrome: a rare congenital anomaly

Patil

Nagaraju

2015

Int J Reprod Contracept Obstet Gynecol

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OHVIRA or Herlyn-Werner-Wunderlich syndrome is a rare congenital anomaly characterized by uterine anomaly with obstructed hemivagina and ipsilateral renal anomaly. We present a case of 16 year female who presented with cyclical lower abdominal pain. Ultrasonography and computed tomography in this patient revealed classic triad of findings. Our case is significant as the diagnosis was made at an earlier stage of the disease before development of gross obstruction and hematometrocolpos.

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“…Ultrasonography is frequently used as initial investigation and preferred as it is non-invasive, free of radiation, and widely available but cannot identify types of MDA. [28][29][30] Magnetic resonance imaging is ideal imaging modality for noninvasive evaluation of female pelvic anatomy because it provides more detailed information regarding uterine morphology, continuity with each vaginal lumen, character of vaginal septum, and nature of fluid contents. It is more useful in detection of associated conditions, such as, endometriosis, pelvic inflammation and adhesions, and coexisting urologic anomalies.…”

Section: Ijrsmsmentioning

confidence: 99%

Radiological Manifestations of Obstructed Hemivagina and Ipsilateral Renal Anomaly Syndrome: A Rare Complex Müllerian and Wolffian Duct Anomaly

Resident

Consultant

2016

IJRSMS

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Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare complex Müllerian and Wolffian duct anomaly. It is also known as Herlyn–Werner–Wunderlich syndrome (HWWS). It includes unilateral renal anomalies and uterine didelphys. It generally occurs at puberty and exhibits non-specific and variable symptoms with acute or pelvic pain shortly following menarche, causing a delay in the diagnosis. We report here a 16-year-old female presenting with progressive cyclical pelvic pain, where magnetic resonance imaging (MRI) suggested the diagnosis of the OHVIRA syndrome. She was managed by surgical resection of the septum and draining of the obstructed vagina.

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Uterine didelphys associated with obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome

Cited by 28 publications

References 13 publications

Herlyn-Werner-Wunderlich syndrome: An unusual presentation with pyocolpos

Herlyn-Werner-Wunderlich syndrome: An unusual presentation with pyocolpos

Uterine didelphys with obstructed hemivagina and ipsilateral renal anomaly - OHVIRA syndrome: a rare congenital anomaly

Radiological Manifestations of Obstructed Hemivagina and Ipsilateral Renal Anomaly Syndrome: A Rare Complex Müllerian and Wolffian Duct Anomaly

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