Management and outcome of patients with combined vaginal septum, bifid uterus, and ipsilateral renal agenesis (Herlyn-Werner-Wunderlich syndrome)

Gholoum, Suad; Puligandla, Pramod S.; Hui, Thomas; Su, Wendy; Quiros, Elsa; Laberge, Jean-Martin

doi:10.1016/j.jpedsurg.2006.01.021

Cited by 149 publications

(128 citation statements)

References 13 publications

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“…However, MRI is currently considered the best imaging modality [11]. Surgical excision of the septum and drainage of the obstructed vagina is the definitive treatment [12]. Early recognition along with obstructing vaginal septum resection is necessary for prevention of complications related to chronic cryptomenorrhoea, such as endometriosis, pelvic adhesions, and infectious collections (pyocolpos, pyometros and pyosalpinx) along with preservation of fertility [12].…”

Section: Discussionmentioning

confidence: 99%

An Unusual Case of Recurrent Pyocolpos Following Midtrimester Miscarriage Revealed as Obstructed Hemivagina with Ipsilateral Renal Agenesis (OHVIRA) Syndrome

Khandaker¹

2014

Glob J Medical Clin Case Rep

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Obstructed hemivagina with ipsilateral renal agenesis (OHVIRA Syndrome) is a rare congenital anomaly. It mostly presents with severe dysmenorrhea and a palpable mass due to unilateral hematocolpos. Sometime it presents in unusual way with prolonged vaginal bleeding and profuse vaginal discharge. We report a case of an 18-year-old married female with OHVIRA syndrome presenting late with symptoms of recurrent pyocolpos following dilatation and evacuation for midtrimester miscarriage that was diagnosed on the basis of MRI and managed by vaginal septotomy.menarche was at 12 years of age with regular cycle and duration of 4-5 days but blood mixed discharge continued for another 5-6 days; for which she did not consulted at that time. She had also given history of cyclical dysmenorrhoea but not debilitating and treated with NSAIDS.

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Section: Discussionmentioning

confidence: 99%

An Unusual Case of Recurrent Pyocolpos Following Midtrimester Miscarriage Revealed as Obstructed Hemivagina with Ipsilateral Renal Agenesis (OHVIRA) Syndrome

Khandaker¹

2014

Glob J Medical Clin Case Rep

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“…Some time after menarche, the retention of menstrual blood in the obstructed hemivagina leads to the formation of a haematocolpos, which is usually clinically detected as a pelvic mass. A right sided prevalence of the obstructed system has been mentioned in the literature (7,12,13). Diagnosis may be further delayed if a communication between the two vaginas exists (3).…”

Section: Discussionmentioning

confidence: 99%

Herlyn-Werner-Wunderlich syndrome (uterus didelphys, blind hemivagina and ipsilateral renal agenesis) - a case report

Arıkan¹,

Harma²,

Harma³

et al. 2010

J Turkish German Gynecol Assoc

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Uterovaginal duplication with obstructed hemivagina and ipsilateral renal agenesis is referred to as the Herlyn-Werner-Wunderlich (HWW) syndrome. A 17 year old woman presented with right pelvic pain and dysmenorrhea, present since menarche at 13 and worsening over the past year. Ultrasound examination revealed a right pelvic mass (5x5 cm), double endometrial echoes, and hematocolpos. A right pelvic mass, agenesis of the right kidney, double uterus, and blind hemivagina with hematocolpos were detected by magnetic resonance imaging and intravenous pyelography. A right tubo-ovarian abscess with dense adhesions and a double uterus were observed on diagnostic laparoscopy. Adhesiolysis was carried out and purulent material irrigated. After a course of antibiotics, a vaginal septum resection was performed and the pyocolpos drained. She remained symptom free after four months of follow-up. Prompt and accurate diagnosis and treatment of this syndrome can significantly improve the lives of sufferers and prevent future complications. (J Turkish-German Gynecol Assoc 2010; 11: 107-9)

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“…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. A very few cases has been reported in the literature.…”

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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Management and outcome of patients with combined vaginal septum, bifid uterus, and ipsilateral renal agenesis (Herlyn-Werner-Wunderlich syndrome)

Cited by 149 publications

References 13 publications

An Unusual Case of Recurrent Pyocolpos Following Midtrimester Miscarriage Revealed as Obstructed Hemivagina with Ipsilateral Renal Agenesis (OHVIRA) Syndrome

An Unusual Case of Recurrent Pyocolpos Following Midtrimester Miscarriage Revealed as Obstructed Hemivagina with Ipsilateral Renal Agenesis (OHVIRA) Syndrome

Herlyn-Werner-Wunderlich syndrome (uterus didelphys, blind hemivagina and ipsilateral renal agenesis) - a case report

OHVIRA syndrome: a rare variant of mullerian duct anomaly

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