Herlyn-Werner-Wunderlich Syndrome—Timely Diagnosis is Important to Preserve Fertility

Güdücü, Nilgün; Gönenç, Gökçenur; İşçi, Herman; Yiğiter, Alin Başgül; Dünder, İlkkan

doi:10.1016/j.jpag.2012.05.013

Cited by 28 publications

(26 citation statements)

References 6 publications

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“…Primary amenorrhea, dyspareunia, urinary retention, spontaneous rupture of the hematocolpos, infertility, and obstetric complications are less common manifestations [13,21,22]. Patients may also complain of mucopurulent discharge in the presence of vaginal communications, as well as intermenstrual bleeding when uterine cavities communicate [23]. In rare cases, a large hematocolpos results in a huge paravaginal mass that may mimic ischiorectal swelling on initial examination, as reported by Asha and Manila [24].…”

Section: Clinical Presentationmentioning

confidence: 92%

“…Several reasons may justify the delayed symptoms: first, due to its distention proprieties, the vagina can accommodate a large volume of blood; second, some of the blood is absorbed between menses [23]; third, anti-inflammatory drugs and oral contraceptives are usually prescribed to relieve dysmenorrhea [2,26]; and finally, some patients presenting with isolated vaginal discharge are erroneously treated with long-term antibiotics. At the emergency department, the diagnosis is also challenging and frequently simulates inflammatory and infectious disorders like tubo-ovarian abscess [25].…”

Section: Clinical Presentationmentioning

confidence: 99%

“…Nonetheless, some studies like that of Tong et al [1] showed that the incidence of primary infertility is not increased among women with HWWS. Actually, infertility is a potential complication of HWWS when the diagnosis is delayed and treatment is inappropriate, and appears to be more related to endometriosis and infectious complications than to uterus didelphys [20,23,29].…”

Section: Complicationsmentioning

confidence: 99%

“…It better depicts pelvic anatomy and documents uterine anomalies, thus being useful in the management of surgical treatment options [9,23]. MRI is more accurate than hysterosalpingography (HSG) or US not only in the evaluation of the uterine contours, the shape of the endometrial cavity and the course of the vaginal septum, but also in the detection of acute and chronic complications [9].…”

Section: Diagnostic Proceduresmentioning

confidence: 99%

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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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Section: Clinical Presentationmentioning

confidence: 92%

Section: Clinical Presentationmentioning

confidence: 99%

Section: Complicationsmentioning

confidence: 99%

Section: Diagnostic Proceduresmentioning

confidence: 99%

See 2 more Smart Citations

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

Dias

Jogo

2015

Abdom Imaging

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“…Reproductive performance of these patients is affected by the delayed diagnosis: retrograde blood flow destroys tubal function and leads to endometriosis [4]. Another important factor affecting fertility is the occurrence of infectious complications.…”

mentioning

confidence: 99%

Herlyn Werner Wunderlich syndrome (HWWS): an unusual presentation of acute abdominal pain

2014

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New Consideration of Herlyn‐Werner‐Wunderlich Syndrome Diagnosed by Ultrasound

Liu

Zhang

Xia

et al. 2020

J of Ultrasound Medicine

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Objectives-As a rare malformation of the female reproductive system, Herlyn-Werner-Wunderlich syndrome (HWWS) was categorized into 3 classifications. It was recommended recently that, on the basis of the past classification, cervicovaginal atresia without communicating uteri should be newly added as classification 4. The surgical intervention will differ by type. To better optimize patient counseling as well as the preoperative evaluation and planning, our objective was to describe the ultrasound characteristics of each type of HWWS, including the new type.Methods-From January 1995 to November 2015, 37 cases of HWWS in with complete ultrasound information confirmed by surgery in the Peking Union Medical College Hospital were reviewed. We analyzed their ultrasound features, including hematometra, hematocervix, hematocolpos, and an ovarian chocolate cyst.Results-All of the ultrasound images of the 37 patients showed uterus didelphys with ipsilateral renal agenesis. Compared with the other 3 types, classification 4 showed distinctive ultrasound characteristics. Most cases of classification 4 showed hematometra (5 of 7 [71.4%]) and an ipsilateral ovarian chocolate cyst (6 of 7 [85.7%]), which was significantly higher than in the other 3. A rudimentary uterine horn was also a distinctive characteristic in this type. Meanwhile none of the classification 4 cases showed hematocervix or hydrocolpos, which were common signs of the other 3.Conclusions-According to this new classification criteria for HWWS, ultrasound characteristics of the new classification 4 differ from the others. As classification 4 was suggested to have a different surgical option, we should pay attention to its ultrasound characteristics, which might help in providing more information about the treatment and prognosis to the gynecologist.

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Herlyn-Werner-Wunderlich Syndrome—Timely Diagnosis is Important to Preserve Fertility

Cited by 28 publications

References 6 publications

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

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

Herlyn Werner Wunderlich syndrome (HWWS): an unusual presentation of acute abdominal pain

New Consideration of Herlyn‐Werner‐Wunderlich Syndrome Diagnosed by Ultrasound

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