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      Congenital anomalies causing hemato/hydrocolpos: imaging findings, treatments, and outcomes

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          Abstract

          Hemato/hydrocolpos due to congenital urogenital anomalies are rare conditions discovered in neonatal, infant, and adolescent girls. Diagnosis is often missed or delayed owing to its rare incidence and nonspecific symptoms. If early correct diagnosis and treatment cannot be performed, late complications such as tubal adhesion, pelvic endometriosis, and infertility may develop. Congenital urogenital anomalies causing hemato/hydrocolpos are mainly of four types: imperforate hymen, distal vaginal agenesis, transverse vaginal septum, and obstructed hemivagina and ipsilateral renal anomaly, and clinicians should have adequate knowledge about these anomalies. This article aimed to review the diagnosis and treatment of these urogenital anomalies by describing embryology, clinical presentation, imaging findings, surgical management, and postoperative outcomes.

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          Most cited references58

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          The American Fertility Society classifications of adnexal adhesions, distal tubal occlusion, tubal occlusion secondary to tubal ligation, tubal pregnancies, müllerian anomalies and intrauterine adhesions.

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            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.
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              New Classification of Herlyn-Werner-Wunderlich Syndrome

              Background: Uterus didelphys and blind hemivagina associated with ipsilateral renal agenesis are collectively known as Herlyn-Werner-Wunderlich syndrome (HWWS). In the literature, the syndrome often appears as a single case report or as a small series. In our study, we reviewed the characteristics of all HWWS patients at Peking Union Medical College Hospital (PUMCH) and suggested a new classification for this syndrome because the clinical characteristics differed significantly between the completely and incompletely obstructed vaginal septum. This new classification allows for earlier diagnosis and treatment. Methods: From January 1986 to March 2013, all diagnosed cases of HWWS at PUMCH were reviewed. A retrospective long-term follow-up study of the clinical presentation, surgical prognosis, and pregnancy outcomes was performed. Statistical analyses were performed using SPSS, version 15.0 (IBM, Armonk, NY, USA). Between-group comparisons were performed using the χ 2 test, Fisher's exact test, and the t-test. The significance level for all analyses was set at P < 0.05. Results: The clinical data from 79 patients with HWWS were analyzed until March 31, 2013. According to our newly identified characteristics, we recommend that the syndrome be classified by the complete or incomplete obstruction of the hemivagina as follows: Classification 1, a completely obstructed hemivagina and Classification 2, an incompletely obstructed hemivagina. The clinical details associated with these two types are distinctly different. Conclusions: HWWS patients should be differentiated according to these two classifications. The two classifications could be generalized by gynecologists world-wide.
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                Author and article information

                Contributors
                tntrad@gmail.com
                Journal
                Jpn J Radiol
                Jpn J Radiol
                Japanese Journal of Radiology
                Springer Singapore (Singapore )
                1867-1071
                1867-108X
                11 April 2021
                11 April 2021
                2021
                : 39
                : 8
                : 733-740
                Affiliations
                [1 ]GRID grid.414173.4, ISNI 0000 0000 9368 0105, Department of Diagnostic Radiology, , Hiroshima Prefectural Hospital, ; Minami-ku, Ujinakanda, Hiroshima, 734-8530 Japan
                [2 ]Department of Radiology, Hiroshima City Hiroshima Citizens Hospital, Hiroshima, Japan
                [3 ]GRID grid.414173.4, ISNI 0000 0000 9368 0105, Department of Obstetrics and Gynecology, , Hiroshima Prefectural Hospital, ; Hiroshima, Japan
                [4 ]GRID grid.414173.4, ISNI 0000 0000 9368 0105, Department of Maternal and Child Health Research Center, , Hiroshima Prefectural Hospital, ; Hiroshima, Japan
                Author information
                http://orcid.org/0000-0002-5825-376X
                Article
                1115
                10.1007/s11604-021-01115-7
                8338850
                33840015
                3f01606a-a0a9-4583-8de0-e236eb643344
                © The Author(s) 2021

                Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/.

                History
                : 8 February 2021
                : 30 March 2021
                Categories
                Invited Review
                Custom metadata
                © Japan Radiological Society 2021

                hematocolpos,imperforate hymen,distal vaginal agenesis,transverse vaginal septum,ohvira

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