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      Herlyn-Werner-Wunderlich syndrome: A rare cause of abdominal pain and dyspareunia

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          Abstract

          Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital disease characterized by Mullerian duct anomalies. The characteristic triad of this syndrome includes didelphys uterus, obstructed hemivagina, and ipsilateral renal agenesis (hence, also known as Obstructed Hemivagina and Ipsilateral Renal Agenesis or OHVIRA syndrome). The clinical presentation of HWW is nonspecific and patients may present with abdominal/pelvic pain, dyspareunia, or dysmenorrhea. However, the imaging findings of HWW are characteristic and discussed in this case report of a 16-year-old female who presented with abdominal pain and dyspareunia.

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

          P S Puligandla, Thomas Hui, Suad Gholoum … (2006)
          Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare müllerian anomaly consisting of uterine didelphy, hemivaginal septum, and ipsilateral renal agenesis. The purpose of this study was to evaluate the natural history and outcome of patients with HWWS. With ethics review board approval, all patients with uterine/vaginal anomalies were reviewed between 1982 and 2004. Patients with cloacal and/or anorectal anomalies were excluded. Presenting symptoms, preoperative investigations, operative management, and long-term follow-up were assessed. Of 80 patients identified with uterine/vaginal anomalies, 12 had HWWS. Median age at presentation was 13 years. Most patients (11/12) in this series presented with either abdominal pain and/or pelvic masses. Two patients had intra-abdominal abscesses. Seven patients were menstruating at presentation with 4 of these patients having dysmenorrhea. Symptom duration ranged from 0.5 to 12 months. Diagnosis was confirmed by ultrasound (n = 11), computed tomographic scan (n = 3), and/or magnetic resonance imaging (n = 2). Operative management included vaginal septectomy and drainage of the hematocolpos/hematometrocolpos. One patient required salpingectomy for pyosalpinx. Follow-up ultrasounds revealed no recurrent collections. Median follow-up was 3 years (2 months to 16 years). Eleven patients were asymptomatic after treatment. One patient complained of irregular menses. This is one of the largest reviews of HWWS in pediatric patients to date. Good long-term outcome occurs after vaginal septectomy. This diagnosis should be suspected in females with a pelvic mass and ipsilateral renal agenesis.
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            Herlyn-werner-wunderlich syndrome: MRI findings, radiological guide (two cases and literature review), and differential diagnosis

            Riccardo Del Vescovo, Sofia Battisti, Valerio Di Paola … (2012)
            Background Herlyn-Werner-Wunderlich (HWW) syndrome is a very rare congenital anomaly of the urogenital tract involving Müllerian ducts and Wolffian structures, and it is characterized by the triad of didelphys uterus, obstructed hemivagina and ipsilateral renal agenesis. It generally occurs at puberty and exhibits non-specific and variable symptoms with acute or pelvic pain shortly following menarche, causing a delay in the diagnosis. Moreover, the diagnosis is complicated by the infrequency of this syndrome, because Müllerian duct anomalies (MDA) are infrequently encountered in a routine clinical setting. Cases presentation two cases of HWW syndrome in adolescents and a differential diagnosis for one case of a different MDA, and the impact of magnetic resonance (MR) imaging technology to achieve the correct diagnosis. Conclusions MR imaging is a very suitable diagnostic tool in order to perform the correct diagnosis of HWW syndrome.
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              Imaging of müllerian duct anomalies.

              Spencer Behr, Jesse Courtier, Aliya Qayyum (2012)
              The müllerian ducts are paired embryologic structures that undergo fusion and resorption in utero to give rise to the uterus, fallopian tubes, cervix, and upper two-thirds of the vagina. Interruption of normal development of the müllerian ducts can result in formation of müllerian duct anomalies (MDAs). MDAs are a broad and complex spectrum of abnormalities that are often associated with primary amenorrhea, infertility, obstetric complications, and endometriosis. MDAs are commonly associated with renal and other anomalies; thus, identification of both kidneys is important. However, MDAs are not associated with ovarian anomalies. Hysterosalpingography (HSG) is routinely used in evaluation of infertility. Because a key component of MDA characterization is the external uterine fundal contour, HSG is limited for this purpose. Patients suspected of having an MDA are often initially referred for pelvic ultrasonography (US). Magnetic resonance (MR) imaging is typically reserved for complex or indeterminate cases. MR imaging is the imaging standard of reference because it is noninvasive, does not involve ionizing radiation, has multiplanar capability, allows excellent soft-tissue characterization, and permits a greater field of interrogation than does US. Use of MR imaging for evaluation of MDAs reduces the number of invasive procedures and related costs by guiding management decisions.
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                Author and article information

                Contributors
                Ninad Salastekar
                Journal
                Journal ID (nlm-ta): Radiol Case Rep
                Journal ID (iso-abbrev): Radiol Case Rep
                Title: Radiology Case Reports
                Publisher: Elsevier
                ISSN (Electronic): 1930-0433
                Publication date PMC-release: 20 August 2019
                Publication date Collection: October 2019
                Publication date (Electronic): 20 August 2019
                Volume: 14
                Issue: 10
                Pages: 1297-1300
                Affiliations
                [0001]Department of Radiology, SUNY Upstate Medical University, 750 East Adam Street, Syracuse, NY, 13210, USA
                Author notes
                [* ]Corresponding author. salasten@ 123456upstate.edu
                Article
                Publisher ID: S1930-0433(19)30266-3
                DOI: 10.1016/j.radcr.2019.08.003
                PMC ID: 6710296
                PubMed ID: 31462954
                SO-VID: 5e77c00b-d793-4e23-b3df-86ed0d0814a7
                Copyright © © 2019 The Authors
                License:

                This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

                History
                Date received : 15 May 2019
                Date revision received : 1 August 2019
                Date accepted : 2 August 2019
                Categories
                Subject: Pediatric

                Keywords: herlyn-werner-wunderlich syndrome,obstructed hemivagina and ipsilateral renal agenesis syndrome,dyspareunia,magnetic resonance imaging
                Data availability:
                Keywords: herlyn-werner-wunderlich syndrome, obstructed hemivagina and ipsilateral renal agenesis syndrome, dyspareunia, magnetic resonance imaging

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