Herlyn—Werner—Wunderlich Syndrome: Premenarche

David, Anita; Gudi, Shobha; Shankar, R

doi:10.5005/jp-journals-10006-1496

Cited by 7 publications

(8 citation statements)

References 16 publications

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“…Diagnosis is delayed due to the occurrence of normal menstrual flow from the patent hemivagina. Sometimes, muco or hydrocolpos has been reported in infants 20 and rarely vaginal clear cell carcinoma and cervical adenocarcinoma of the obstructed side have been reported. 21 Diagnosis of OHVIRA syndrome requires a multimodal approach, which includes a detailed history, meticulous examination, and appropriate imaging studies.…”

Section: Casementioning

confidence: 99%

“…26 Also, it is advisable that whenever fetal renal anomalies are diagnosed on the antenatal scan look for obstructed müllerian anomalies in order to detect and treat them at the earliest. 20 Hysterosalpingogram was used in the past quite often but now its role is limited to class 2.2 only to confirm communication between the two cervices. Laparoscopy can help in confirming the diagnosis, detection, and treatment of Flowchart 1: OHVIRA syndrome classification, clinical features and management 10 (USG, ultrasonography; MRI, magnetic resonance imaging; EUA, examination under anesthesia; HSG, hysterosalpingograph) consequences on the reproductive and mental health.…”

Section: Casementioning

confidence: 99%

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OHVIRA Syndrome—Diagnostic Dilemmas and Review of Literature

Sharma¹,

Mishra²,

Seth³

et al. 2020

Journal of South Asian Federation of Obstetrics and Gynaecology

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Obstructed hemivagina, ipsilateral renal agenesis (OHVIRA) syndrome, as well as didelphic uterus, is a rare, obstructed müllerian anomaly, affecting the physical, reproductive, sexual, and mental health of the patient. The diagnosis is often delayed because of unawareness of the fact that even common gynecological complaints like vaginal discharge can be associated with this rare entity. The most common symptoms at presentation, however, include pain in the abdomen and menstrual complaints. Therefore, while evaluating them, the possibility of this rare entity should be kept in mind; hence, emphasizing the importance of local examination. A 3D ultrasound and a magnetic resonance imaging are helpful in confirming the diagnosis. In this article, we report case series of OHVIRA syndrome with extreme clinical symptoms at presentation. We reviewed the literature and discussed various classifications used for this syndrome, including the newer classification based on the type of obstruction. The minimally invasive "vaginoscopic no-touch" technique of surgery is an alternative option available for adolescents where preservation of hymen seems to be important.

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Section: Casementioning

confidence: 99%

Section: Casementioning

confidence: 99%

OHVIRA Syndrome—Diagnostic Dilemmas and Review of Literature

Sharma¹,

Mishra²,

Seth³

et al. 2020

Journal of South Asian Federation of Obstetrics and Gynaecology

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“…Herlyn-Werner-Wunderlich syndrome (HWWS), a Mullerian anomaly has an incidence of 0.1-3.8% 1 The diagnosis is aided by ultrasound, CT or MRI scans. The treatment depends on the variants of the syndrome whether it is class 1 or class 2 2,3 .…”

Section: Introductionmentioning

confidence: 99%

Incidental diagnosis of Herlyn-Werner-Wunderlich Syndrome in a nulliparous woman: a case report in Bhutan

Sonam¹,

Pradhan²,

Tshomo³

2021

Bhutan Health Journal

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Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare congenital syndrome with features of uterus didelphys, ipsilateral absent kidney and obstructed hemivagina. Ultrasound findings of absent kidney or abnormalities in the kidney should alert the clinicians about the syndrome. Complications like endometriosis, infertility and pelvic inflammation occurs with late diagnosis.A 42-yearold nulliparious married woman who presented with right sided abdominal pain to the National Referral Hospital was diagnosed to have HWWS. The diagnosis was supported by ultrasound, CT and MRI findings. Patient was treated for endometriosis and had symptomatic improvement. The diagnosis of the syndrome is challenging as it is rare but clinicians should suspect the syndrome in women who present with infertility. Ultrasound scan is the basic investigation to screen the syndrome which is available in most hospitals.

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“…The manifestations of abnormalities are didelphys uterus, hemivagina with obstruction, and ipsilateral renal agenesis. 4 Ipsilateral Mullerian duct anomalies that have obstruction greater than 50 % are actually predictive factors for renal agenesis. This anomaly is dominant, twice as often on the right side.…”

Section: Introductionmentioning

confidence: 99%

“…Early diagnosis will provide benefits because late diagnosis can cause several complications, like retrograde tubal reflux, leading to endometriosis and infertility. 4 Lan Zhu et al looked at patients with HWW syndrome at Peking Medical College Hospital (PUMCH), and suggested a new classification for HWW syndrome, based on the presence or absence of complete or incomplete vaginal obstruction. This syndrome is classified by classification 1 (complete obstruction hemivagina) and classification 2 (incomplete hemivaginal obstruction).…”

Section: Introductionmentioning

confidence: 99%

Surgical therapy for herlyn-werner-wunderlich (HWW) syndrome cases

Pangastuti¹,

Rahman²,

Setyawan³

2021

IJFCM

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Introduction: One of the rare forms of Mullerian congenital ducts is Herlyn-Werner-Wunderlich (HWW) syndrome. HWW syndrome is usually found at puberty with nonspecific symptoms. Pelvic pain can be found both acute and chronic, dysmenorrhoea, as well as hematometra and hematocolpos. Early detection and treatment results in a good prognosis with fertility preservation. Case presentation: Two cases of Herlyn-Werner-Wunderlich (HWW) syndrome surgically performed were reported at Dr. Sardjito Yogyakarta Indonesia Central General Hospital in 2017-2018. Both cases are still children and at the age of puberty. The chief complaint in both cases it was reported to have the same main complaint, abdominal pain, with the condition having never experienced menstruation yet. On further examination it is known that pain is caused due to hematometra and hematosalping that occur in both cases. The first case with hematometra in the right hemiuterus and right hematosalpinx, while second case with hematometra in the uterus didelphys and bilateral hematosalpinx. One case with a normal vagina, and the other case of vaginal agenesis. There was previous history of surgery in both of cases. There are abnormalities in the urinary tract that occur together with these genital abnormalities. Surgical therapy is carried out with the main goal being to drain menstrual blood from hematometra and hematosalpinx. In first case which has a vagina within the normal range (9 cm long) and the inferior area of the right hemiuterus which is bulging toward the vagina, an area can be identified for the drainage incision to drain the entire hematometra and hematosalpinx. In the second case with vaginal agenesis, neovagina which is made by sigmoid colon vaginoplasty surgery can be a way of menstrual blood flow for a lifetime. Conclusion: Clinical manifestations of Herlyn-Werner-Wunderlich (HWW) syndrome cases can be classified into 2 groups, complete and incomplete. Surgical treatment is based on the condition of the uterus and vagina. In the condition of cervical agenesis but with a normal vagina, surgery can be done by making an incision in the drainage of the uterine cervical tissue. If vaginal agenesis is obtained, vaginoplasty surgery can be performed, and one of the options is sigmoid colon vaginoplasty.

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Herlyn—Werner—Wunderlich Syndrome: Premenarche

Cited by 7 publications

References 16 publications

OHVIRA Syndrome—Diagnostic Dilemmas and Review of Literature

OHVIRA Syndrome—Diagnostic Dilemmas and Review of Literature

Incidental diagnosis of Herlyn-Werner-Wunderlich Syndrome in a nulliparous woman: a case report in Bhutan

Surgical therapy for herlyn-werner-wunderlich (HWW) syndrome cases

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Herlyn—Werner—Wunderlich Syndrome: Premenarche

Cited by 7 publications

References 16 publications

OHVIRA Syndrome&mdash;Diagnostic Dilemmas and Review of Literature

OHVIRA Syndrome&mdash;Diagnostic Dilemmas and Review of Literature

Incidental diagnosis of Herlyn-Werner-Wunderlich Syndrome in a nulliparous woman: a case report in Bhutan

Surgical therapy for herlyn-werner-wunderlich (HWW) syndrome cases

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OHVIRA Syndrome—Diagnostic Dilemmas and Review of Literature

OHVIRA Syndrome—Diagnostic Dilemmas and Review of Literature