Herlyn Werner Wunderlich Syndrome with Hematocolpos: An
Unusual Case Report of Full Diagnostic Approach and Treatment

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Abstract

Herlyn-Werner-Wunderlich (HWW) syndrome is an uncommon combined müllerian duct anomalies (MDAs) and mesonephric duct malformation of female urogenital tract characterized by uterus didelphys and obstructed hemi-vagina and ipsilateral renal agenesis (OHVIRA) syndrome. We present a rare and unusual case of this syndrome in a 19 year-old female who suffered from hypomenorrhoea and abdominal pain. She had an obstructed hemi-vagina on right side which led to marked distention of ipsilateral cervix, while proximal hemi-vagina compressed the contralateral side causing its partial obstruction resulting in hypomenorrhoea. Understanding the imaging findings of this rare condition is important for early diagnosis in order to prevent complications which may lead to infertility.

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Most cited references 12

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Müllerian Anomalies: A Proposed Classification (An Analysis of 144 Cases)

Veasy C. Buttram, William E. Gibbons (1979)

Fertility and Sterility, 32(1), 40-46

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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: uterus didelphys, blind hemivagina and ipsilateral renal agenesis. Sonographic and MR findings in 11 cases.

Paolo M S Schingo, Cinzia Orazi, P Marchetti … (2007)

Uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis is a rare entity, sometimes referred to as Herlyn-Werner-Wunderlich syndrome (HWW). It usually presents after menarche with progressive pelvic pain, sometimes with regular menses, and a palpable mass due to hemihaematocolpos. The diagnosis is generally made only if the suspicion of this genitourinary syndrome is raised. To highlight the imaging diagnostic clues in this rare condition. We report on 11 adolescents with this condition. Sonography mostly allowed the correct diagnosis by showing uterovaginal duplication, haematocolpos or haematometrocolpos, and the absence of the ipsilateral kidney. MRI provided more detailed information regarding uterine morphology, the continuity with each vaginal channel (obstructed and nonobstructed), and the bloody nature of the contents. Early and accurate diagnosis of this syndrome is important so that adequate and prompt surgical therapy (excision of the vaginal septum) can provide relief of pain and prevent further complications. It is also advisable to look for an obstructed Müllerian system whenever a multicystic dysplastic kidney or the absence of a kidney is discovered in a fetus, or girl postnatally.

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Author and article information

Journal

Journal ID (nlm-ta): Int J Fertil Steril

Journal ID (iso-abbrev): Int J Fertil Steril

Journal ID (publisher-id): Royan Institute

Title: International Journal of Fertility & Sterility

Publisher: Royan Institute

ISSN (Print): 2008-076X

ISSN (Electronic): 2008-0778

Publication date (Print): Apr-Jun 2016

Publication date (Electronic): 5 April 2016

Volume: 10

Issue: 1

Pages: 136-140

Affiliations

Department of Radiodiagnosis, Dr. Rajendra Prasad Government Medical College, Kangra, HP, India

Author notes

[*Corresponding Address: ]Department of RadiodiagnosisDr. Rajendra Prasad Government Medical CollegeKangraHPIndia Email: rohitbhoil@ 123456gmail.com

Article

Other ID: Int-J-Fertil-Steril-10-136

DOI: 10.22074/ijfs.2016.4779

PMC ID: 4845524

PubMed ID: 27123211

SO-VID: 206e6c29-e618-43c3-94d7-a662d93b1583

License:

This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

History

Date received : 22 December 2014

Date accepted : 14 March 2015

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Citation Bhoil R, Ahluwalia A, Chauhan N. Herlyn werner wunderlich syndrome with hematocolpos: an unusual case report of full diagnostic approach and treatment. Int J Fertil Steril. 2016; 10(1): 136-140.

Herlyn Werner Wunderlich Syndrome with Hematocolpos: An Unusual Case Report of Full Diagnostic Approach and Treatment

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Most cited references 12

Müllerian Anomalies: A Proposed Classification (An Analysis of 144 Cases)

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

Herlyn-Werner-Wunderlich syndrome: uterus didelphys, blind hemivagina and ipsilateral renal agenesis. Sonographic and MR findings in 11 cases.

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