OHVIRA Syndrome (obstructed hemivagina and ipsilateral renal anomaly) with Uterus Didelphys, an Unusual Presentation

Mandava, Anitha; Prabhakar, Rajmohan; S, Smitha K.

doi:10.1016/j.jpag.2011.11.004

Cited by 67 publications

(57 citation statements)

References 8 publications

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“…The pathogenesis of OHVIRA syndrome is related to anomalous development of both the paramesonephric (Mullerian) and mesonephric (Wolffian) ducts 7 . The Wolffian ducts induce normal Mullerian duct fusion and give origin to the kidneys.…”

Section: Discussionmentioning

confidence: 99%

“…It may remain unrecognised initially as regular menstrual flow from the patent unobstructed hemivagina gives the appearance of normal menses, and dysmenorrhoea, if present, is a common complaint in this age group 7 . Symptom relief of dysmenorrhoea with non‐steroidal anti‐inflammatory and oral contraceptive medication may also lead to a delay in diagnosis 9 .…”

Section: Discussionmentioning

confidence: 99%

“…Symptom relief of dysmenorrhoea with non‐steroidal anti‐inflammatory and oral contraceptive medication may also lead to a delay in diagnosis 9 . The clinical presentation may be variable and non‐specific features such as acute abdominal, 4 , 10 pelvic 9 , 11 or vaginal 12 pain, dysuria, 13 urinary retention, 7 vaginal discharge 14 , 15 and infertility 16 have been reported. The two cases presented were both known to have an absent kidney and this should prompt investigation for Mullerian duct anomalies and vice versa.…”

Section: Discussionmentioning

confidence: 99%

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Sonographic diagnosis of Obstructed Hemivagina and Ipsilateral Renal Anomaly Syndrome: a report of two cases

Mishra

2014

Australas J Ultrason Med

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Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare urogenital anomaly. Patients typically present at puberty, shortly after menarche with increasing pelvic pain, dysmenorrhea and pelvic mass. There may be a known history of unilateral renal agenesis. Diagnosis can usually be established by ultrasound or magnetic resonance imaging. Resection of the vaginal septum is the treatment of choice. Early diagnosis and treatment can relieve symptoms, prevent complications and preserve fertility. We present two cases of OHVIRA syndrome diagnosed by ultrasound to promote recognition of this rare but important condition.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Sonographic diagnosis of Obstructed Hemivagina and Ipsilateral Renal Anomaly Syndrome: a report of two cases

Mishra

2014

Australas J Ultrason Med

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“…Strong suspicion and knowledge of this anomaly are essential for a precise diagnosis. 2 The triad of uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis, known as OHVIRA syndrome or HWW syndrome, is a congenital anomaly of Müllerian duct with associated mesonephric duct anomaly. 3,4 This is a very rare but well documented variety of mullerian anomaly.…”

Section: Introductionmentioning

confidence: 99%

OHVIRA syndrome: a rare variant of mullerian duct anomaly

Rudra¹,

Dahiya²

2016

Int J Reprod Contracept Obstet Gynecol

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OHVIRA is a rare variety of congenital anomaly of mullerian duct. The syndrome includes uterus didelphys, obstructed hemivagina and ipsilateral renal anomaly, also known as Herlyn-Werner-Wunderlich (HWW) syndrome. A 15yrs old girl was referred to our department with regular menstrual period and cyclic pain in lower abdomen of 5-6 months duration. USG and MRI established the final diagnosis of OHVIRA syndrome. Drainage of hematocolpos of obstructed hemivagina and excision of septum was done and patient was discharged under satisfactory condition to come for follow-up. Patient had her normal menses on 28/8/2015. A repeat USG was done which showed didelphus uterus with no collection in endometrial cavity.

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“…In 1976, Wunderlich [5] reported a rare form of bicornuate uterus with simple vagina and isolated hematocervix, in association with aplasia of the right kidney and ureter. The HWWS is also referred in the literature as the obstructed hemivagina and ipsilateral renal anomaly syndrome (OHVIRA) [6,7].…”

mentioning

confidence: 99%

Herlyn–Werner–Wunderlich syndrome: pre- and post-surgical MRI and US findings

Dias

Jogo

2015

Abdom Imaging

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Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare congenital anomaly of the female urogenital tract that associates Mu¨llerian duct anomalies with mesonephric duct anomalies. The triad of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis characterizes this syndrome. Patients generally present with non-specific symptoms after menarche. Pelvic pain, dysmenorrhea, and palpable mass due to hematocolpos or hematometra are the most common findings. Pyohematocolpos and pyosalpinx may appear as acute complications, while endometriosis and pelvic adhesions constitute potential long-term complications. When a prenatal diagnosis of unilateral renal agenesis in newborn girls is known, a gynecological imaging study should be performed to exclude uterine and vaginal abnormalities. These patients should be followed up to ensure that a timely surgical correction is performed. The diagnosis of HWWS is difficult due to the lack of specific symptoms or findings upon physical examination. An accurate imaging description of these congenital anomalies is crucial to guide patients toward surgical treatment, relieving acute complications, and preserving the normal fertility. The authors provide a pictorial review of the magnetic resonance imaging and ultrasonography findings of the HWWS with correlation to embryological, clinical, and surgical features.

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OHVIRA Syndrome (obstructed hemivagina and ipsilateral renal anomaly) with Uterus Didelphys, an Unusual Presentation

Cited by 67 publications

References 8 publications

Sonographic diagnosis of Obstructed Hemivagina and Ipsilateral Renal Anomaly Syndrome: a report of two cases

Sonographic diagnosis of Obstructed Hemivagina and Ipsilateral Renal Anomaly Syndrome: a report of two cases

OHVIRA syndrome: a rare variant of mullerian duct anomaly

Herlyn–Werner–Wunderlich syndrome: pre- and post-surgical MRI and US findings

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