25
views
0
recommends
+1 Recommend
0 collections
    0
    shares
      • Record: found
      • Abstract: found
      • Article: found
      Is Open Access

      Case Report: A Rare Cause of Complicated Urinary Tract Infection in a Woman with Herlyn-Werner-Wunderlich Syndrome

      case-report
      1 , 2 , 3 , 4 , *
      Iranian Red Crescent Medical Journal
      Kowsar
      Urinary Tract Infection, HWWS, OHVIRA Syndrome

      Read this article at

      Bookmark
          There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

          Abstract

          Introduction

          Urinary tract infection is a common disease in the general population. However, in patients with frequent urinary tract infection, it is important to determine any treatable cause to avoid recurrence.

          Case Presentation

          Herlyn-Werner-Wunderlich syndrome or OHVIRA syndrome is a very rare congenital anomaly with uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. The earliest presentation of this syndrome is hematocolpos that develops during menstruation and results in dysmenorrhea and a pelvic mass shortly after menarche. Herein, we report a patient with Herlyn-Werner-Wunderlich syndrome manifested with unusual symptoms, delayed onset and without surgery. The unique point of this patient is the partial obstruction of cervico-vaginal junction.

          Conclusions

          Early diagnosis and timely treatment of OHVIRA syndrome can prevent long-term complications, such as recurrent urinary tract infection and infertility. A high index of suspicion is required, even though OHVIRA syndrome is extremely rare and may have an atypical presentation.

          Related collections

          Most cited references12

          • Record: found
          • Abstract: found
          • Article: not found

          Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome: management and follow-up.

          To review the experience at two large referral centers with the syndrome of obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), and to review the risks, benefits, and complications of surgical management options. Retrospective case series. Children's Hospital Boston and Brigham and Women's Hospital, Boston, Massachusetts. Twenty-seven cases of obstructed hemivagina over a 12-year period. For each patient who presented symptoms, diagnostic studies, anatomic findings, surgical management, outcomes, and complications were reviewed. The mean age of diagnosis was 14 years. Twenty-three patients had ipsilateral renal anomalies, including 20 patients who had renal agenesis. Two had dysplastic ipsilateral kidneys requiring nephrectomy in infancy. Twenty-six patients underwent vaginal reconstruction, and eight of those additionally underwent laparoscopy for clarification of diagnosis. Six required a two-stage vaginoplasty because of incomplete previous resection (n = 1), infection or anatomic distortion (n = 4), or restenosis (n = 2). Vaginal septum adenosis was seen in eight patients. Most patients with OHVIRA syndrome can be treated solely with single-stage vaginoplasty. Routine laparoscopy is not essential to management. Vaginal stenosis is a postoperative possibility, and may be associated with vaginal adenosis.
            Bookmark
            • Record: found
            • Abstract: found
            • Article: not found

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

            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.
              Bookmark
              • Record: found
              • Abstract: found
              • Article: not found

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

              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.
                Bookmark

                Author and article information

                Journal
                Iran Red Crescent Med J
                Iran Red Crescent Med J
                10.5812/ircmj
                Kowsar
                Iranian Red Crescent Medical Journal
                Kowsar
                2074-1804
                2074-1812
                30 October 2016
                November 2016
                : 18
                : 11
                : e40267
                Affiliations
                [1 ]MD, Department of Family Medicine, Cheng Ching General Hospital, Taichung, Taiwan
                [2 ]MD, Division of Nephrology, Department of Internal Medicine, Taichung Veterans General Hospital, Taichung, Taiwan
                [3 ]School of Medicine, China Medical University, Taichung, Taiwan
                [4 ]Department of Life Science, Tunghai University, Taichung, Taiwan
                Author notes
                [* ]Corresponding Author: Shang-Feng Tsai, MD, Division of Nephrology, Department of Medicine, Taichung Veterans General Hospital, Taichung, Taiwan. Tel: +886-423592525, Fax: +886-423594980, E-mail: s881056@ 123456gmail.com
                Article
                10.5812/ircmj.40267
                5294448
                28203451
                b112ea2f-e78d-4b9d-bfcb-dd15236178be
                Copyright © 2016, Iranian Red Crescent Medical Journal

                This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 International License ( http://creativecommons.org/licenses/by-nc/4.0/) which permits copy and redistribute the material just in noncommercial usages, provided the original work is properly cited.

                History
                : 21 June 2016
                : 22 August 2016
                : 16 October 2016
                Categories
                Case Report

                Medicine
                urinary tract infection,hwws,ohvira syndrome
                Medicine
                urinary tract infection, hwws, ohvira syndrome

                Comments

                Comment on this article