Clinical characteristics and treatment of Herlyn–Werner–Wunderlich syndrome

Wang, Jinhui; Zhu, Lan; Lang, Jinghe; Liu, Zhufeng; Sun, Dawei; Leng, Jinhua; Fan, Qingbo

doi:10.1007/s00404-014-3286-5

Cited by 53 publications

(58 citation statements)

References 10 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…About 75% of patients with didelphys uterus have a partial or complete vaginal septum which is commonly longitudinal in the Herlyn-Werner-Wunderlich syndrome, which reflects a disorder of lateral fusion between the inferior portions of two Mullerian ducts [7]. Studies of uterine organogenesis have implicated the Hox and Wnt genes as regulators of uterine morphology.…”

Section: Discussionmentioning

confidence: 99%

The Herlyn-Werner-Wunderlich Syndrome – A Case Report with Radiological Review

Khaladkar

Kamal

et al. 2016

Pol J Radiol

View full text Add to dashboard Cite

SummaryBackgroundHWW syndrome is a very rare congenital anomaly of urogenital tract involving Mullerian ducts and mesonephric ducts. It is characterised by a triad of symptoms - uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis. It can be classified based on a completely or incompletely obstructed hemivagina. It presents soon after menarche or shows delayed presentation depending on the type. It can exhibit acute pelvic pain shortly after menarche and may show non-specific and variable symptoms with resultant delay in diagnosis. The most common presentation is pain and dysmenorrhea, and pain and abdominal mass in the lower abdomen secondary to haematocolpos and/or haematometra.Case ReportPresentation of a clinical case of a 13-year-old patient with HWW syndrome presenting with regular menses, dysmenorrhea and painful lump in hypogastric region on the left side of midline. We described the role of imaging modalities in diagnosis of the Herlyn-Werner-Wunderlich syndrome with a review of literature. USG and MRI showed left renal agenesis with compensatory hypertrophy of the right kidney, uterus didelphys with haematometra and haematocervix in the left uterus with evidence of blood in a dilated retort-shaped left fallopian tube and a normal right uterus. The unique feature of our case is haematometra and haematocervix with cervical and vaginal atresia found on the left side (classification 1.2) with associated left renal agenesis.ConclusionsHWW syndrome can present early or late, depending on the type. In patients with uterine and vaginal abnormalities, a work-up for associated renal anomalies should be performed. Early intervention is needed to reduce the risk of endometriosis and infertility.

show abstract

Section: Discussionmentioning

confidence: 99%

The Herlyn-Werner-Wunderlich Syndrome – A Case Report with Radiological Review

Khaladkar

Kamal

et al. 2016

Pol J Radiol

View full text Add to dashboard Cite

show abstract

“…Vaginal septum resection is frequently used in the treatment. Hysteroscopic septum resection may be considered in cases in which the patient and the family wish to preserve the hymen (13). Hemihisterectomy should be considered among the treatment options since vaginal septum resection is inadequate to prevent hematometra development or only sep-tum resection cannot prevent obstruction in cases of cervical atresia (14).…”

Section: Discussionmentioning

confidence: 99%

Primer amenore ve servikal agenezi ile görülen Herlyn-Werner-Wunderlich sendromu(HWWS),nadir bir olgu sunumu

Topdaği¹,

Yılmaz

Topdagi

et al. 2019

Zeynep Kamil Tıp Bülteni

View full text Add to dashboard Cite

The classic triad of Herlyn-Werner-Wunderlich syndrome includes blind hemivagina, uterine didelphys and ipsilateral renal agenesis. Herlyn-Werner-Wunderlich syndrome is a variant of Mullerian duct anomalies. Clinical presentation of the Herlyn-Werner-Wunderlich syndrome is usually accompanied by severe dysmenorrhea secondary to hematometrocolpos beginning with menarche and palpable pelvic mass. Early diagnosis and treatment are important in protecting the patient's fertility and preventing complications in the long term. In this study, when a 13-year old patient from the pediatric age group was being investigated for the pelvic mass, HWWS confirmed by MRI was identified. After the patient and his family were informed, and their consent was taken, the diagnostic laparoscopy was performed. Subsequently, after hematometra was removed by paravaginal repair, her complaints were resolved. The patient was followed up regarding fertility and endocrine functions, and a second operation could be performed in accordance with her age. This syndrome should be kept in mind in pediatric and adolescent patient groups who are investigated for severe dysmenorrhea, primary amenorrhea or pelvic mass.

show abstract

“…Various case reports have described congenital abnormalities of the unilateral hematocolpos and ipsilateral renal agenesis (1)(2)(3). In 1985, Bian named this condition congenital vaginal oblique septum syndrome (CVOS) (4) and it is also known as Herlyn-Werner-Wunderlich syndrome (3,5,6). CVOS is usually caused by congenital malformation in the vagina, which results in the development of a double uterus and cervix (6).…”

Section: Introductionmentioning

confidence: 99%

Ultrasonic evaluation of congenital vaginal oblique septum syndrome: A study of 21 cases

et al. 2018

View full text Add to dashboard Cite

The aim of the present study was to investigate the ultrasound features and classify the lesion types of congenital vaginal oblique septum syndrome (CVOS) in 21 patients prior to surgery. Grey-scale pelvic ultrasound was performed to evaluate the uterus, vagina and kidneys in 21 patients with suspected CVOS. Ultrasound features, including the presence of a double uterus, hematocolpos masses and renal absence, in CVOS types I, II and III were studied and compared with intra-operative results and the results of surgery. Ultrasound identified the presence of double uteruses and cervices with ipsilateral renal agenesis on the oblique septum side in all 21 patients. There were 14 hematocolpos lesions on the right and 7 on the left of the vagina. Type I CVOS was diagnosed in 15 patients with a large hematocolpos mass (volume, 64-268 ml) and these diagnoses were confirmed by surgery. Furthermore, there were 4 patients with type II and 2 patients with type III CVOS exhibiting small hematocolpos lesions (volume, 5-36 ml) identified by ultrasound, which were all confirmed by surgery. Therefore, ultrasound imaging is useful tool to evaluate the abnormal features of CVOS and determine the type of CVOS in patients prior to surgical intervention.

show abstract

Clinical characteristics and treatment of Herlyn–Werner–Wunderlich syndrome

Abstract: Clinical presentation in untreated HWWS suggests the anatomic anomaly. Early recognition and treatment can reduce symptoms. Pregnancies occur in both the affected and unaffected uterus.

Cited by 53 publications

References 10 publications

The Herlyn-Werner-Wunderlich Syndrome – A Case Report with Radiological Review

The Herlyn-Werner-Wunderlich Syndrome – A Case Report with Radiological Review

Primer amenore ve servikal agenezi ile görülen Herlyn-Werner-Wunderlich sendromu(HWWS),nadir bir olgu sunumu

Ultrasonic evaluation of congenital vaginal oblique septum syndrome: A study of 21 cases

Contact Info

Product

Resources

About