Uterus didelphys with an obstructed unilateral vagina and ipsilateral renal agenesis: A rare cause of dysmenorrhoea

Attar, Rukset; Yıldırım, Gazi; Inan, Yücel; Küzılkale, Ozge; Karateke, Ateş

doi:10.5152/jtgga.2013.44341

Cited by 11 publications

(16 citation statements)

References 15 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Resection of the vaginal septum is the most effective treatment of this abnormality leading to immediate relief. It can also prevent long‐term complications, preserve future fertility, and achieve a better pregnancy outcome 1, 3 . The recent years, because many patients with HWW syndrome have an imperforate hymen, minimally invasive approaches such as hysteroscopic resection of the septum have been reported in the literature with excellent results 12 .…”

Section: Discussionmentioning

confidence: 99%

“…This condition's exact cause and pathogenesis are unclear, but it seems like a lesion occurs during the eighth week of gestation and affects the adjacent Mullerian and metanephric ducts. The clinical presentation is nonspecific, with patients usually complaining of abdominal/pelvic pain, dyspareunia, dysmenorrhea, or palpable mass in the vagina 3 . If an ascending infection occurs in the obstructed vagina, the patient may present with pyohematocolpos, pyosalpinx, and peritonitis.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Herlyn–Werner–Wunderlich syndrome: Report of a delayed diagnosed case with video presentation of the operative technique of vaginal septum resection

Pittokopitou

Kathopoulis

Πρωτόπαπας

et al. 2021

J of Obstet and Gynaecol

View full text Add to dashboard Cite

Herlyn–Werner–Wunderlich (HWW) syndrome is a rare congenital anomaly of Mullerian duct development characterized by uterus didelphys with blind hemivagina and ipsilateral renal agenesis. We present a case of a 29‐year‐old nulliparous woman, who was referred to our hospital complaining of chronic pelvic pain, dyspareunia, and a palpable mass in her vagina. At the age of 12, she underwent surgery because of a didelphys uterus diagnosis. Subsequently, she was operated on twice for endometriomas. At our institution, clinical and imaging findings revealed an obstructed hemivagina setting the diagnosis of HWW syndrome. Some of the various syndrome types may go unnoticed for months or even years after the onset of menstruation. Early diagnosis, followed by proper surgical treatment, is the key to avoid potentially severe complications.

show abstract

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Herlyn–Werner–Wunderlich syndrome: Report of a delayed diagnosed case with video presentation of the operative technique of vaginal septum resection

Pittokopitou

Kathopoulis

Πρωτόπαπας

et al. 2021

J of Obstet and Gynaecol

View full text Add to dashboard Cite

show abstract

“…[ 7 ] Both the syndromes are rare and are discussed as same entities in many case reports and their incidence is estimated to be between 0.1% and 3.5% of all mullerian anomalies. [ 8 ] The cause is attributed to embryologic arrest affecting both the mullerian and metanephric ducts concurrently at about 8 weeks gestation. [ 9 ] The most common presenting symptoms are dysmenorrhea within first few years of menarche and increasing pelvic pain.…”

Section: Introductionmentioning

confidence: 99%

An eventful journey from menarche to successful motherhood

et al. 2016

View full text Add to dashboard Cite

Herlyn–Werner–Wunderlich syndrome is an uncommon variant of mullerian duct anomaly and the approach to its diagnosis requires a high index of suspicion and vigilant work up. Presented here is a case of a 26-year-old woman who had the aforementioned anomaly and was pursued for nearly 10 years to provide her with a fruitful obstetric outcome after having undergone Strassman's metroplasty at a young age of 15 years.

show abstract

“…These malformations result from anomalous development of the paramesonephric ducts, also known as Müller's ducts, during the embryologic period. 1,2 The prevalence of Müllerian anomalies remains uncertain among studies, ranging from less than 1%-5% within the general population. [2][3][4] In certain specific cohorts, such as women with unfavorable…”

mentioning

confidence: 99%

“…obstetric outcomes, this prevalence might even exhibit a higher magnitude. 1,4 Moreover, it is believed that there is underreporting of cases, particularly in those with milder symptoms. This exacerbates the challenge of precisely ascertaining the total count of affected women.…”

mentioning

confidence: 99%

Hemihysterectomy in a patient with uterus didelphys, vaginal septum and ipsilateral renal agenesis: A case report and literature review

Peixoto Silva,

Souza Neves,

Tannure Saraiva

et al. 2023

Intl J Gynecology & Obste

View full text Add to dashboard Cite

Uterus didelphys is a rare Müllerian anomaly, often diagnosed during menarche or in women with a personal history of infertility and/or recurrent pregnancy loss. Its association with other genitourinary anomalies is frequent and may determine the existence of established syndromes. This case report refers to a 13‐year‐old female patient diagnosed with OHVIRA syndrome (Obstructed Hemivagina with Ipsilateral Renal Agenesis), a condition wherein the presence of a didelphic uterus is associated with hemivagina obstruction and ipsilateral renal agenesis. The patient presented with cyclic pelvic pain, related to the presence of hematocolpos and hematometra, which persisted despite several surgical approaches, including vaginal septum excision and correction of cervical stenosis. The recurrence of the condition indicated exploratory laparotomy, revealing two hemi‐uteri and two uterine cervixes, with hematometra on the right. A subtotal hemihysterectomy was performed on the right. Post‐procedure, the patient developed with regular menstrual cycles and improvement of pelvic pain complaints. Given the limited prevalence and low index of suspicion, the potential requirement for surgical intervention and its potential impact on reproductive future, diagnosing and treating OHVIRA syndrome and other Müllerian anomalies poses notable challenges in clinical practice. Hence, sharing different therapeutic approaches of a rare diagnosis with the scientific community is of paramount importance to aid in early diagnosis and effective management of similar clinical cases.

show abstract

Uterus didelphys with an obstructed unilateral vagina and ipsilateral renal agenesis: A rare cause of dysmenorrhoea

Cited by 11 publications

References 15 publications

Herlyn–Werner–Wunderlich syndrome: Report of a delayed diagnosed case with video presentation of the operative technique of vaginal septum resection

Herlyn–Werner–Wunderlich syndrome: Report of a delayed diagnosed case with video presentation of the operative technique of vaginal septum resection

An eventful journey from menarche to successful motherhood

Hemihysterectomy in a patient with uterus didelphys, vaginal septum and ipsilateral renal agenesis: A case report and literature review

Contact Info

Product

Resources

About