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Kim HJ, Lee EJ, Chang YW, Hong SS, Hwang J, Nam B, Bae SH. Anatomical Variations, Genitourinary Anomalies and Clinical Presentations in Obstructed Hemivagina and Ipsilateral Renal Anomaly Syndrome: Case Series. JOURNAL OF THE KOREAN SOCIETY OF RADIOLOGY 2025; 86:129-140. [PMID: 39958491 PMCID: PMC11822277 DOI: 10.3348/jksr.2024.0018] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 01/22/2024] [Revised: 05/23/2024] [Accepted: 07/13/2024] [Indexed: 02/18/2025]
Abstract
Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare Müllerian duct anomaly, commonly characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. While these are the three most common genitourinary anomalies in OHVIRA syndrome, a spectrum of urogenital anomalies can be present. Knowledge of this spectrum is crucial for proper patient management and treatment planning. In this case series, we report on five patients with OHVIRA syndrome, each presenting with a urogenital anomaly other than the typical renal agenesis or uterus didelphys. We highlight the gynecological complications encountered, which clinicians and radiologists should be aware of.
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Bhamidipaty-Pelosi S, Kyei-Barffour I, Volpert M, O'Neill N, Grimshaw A, Eriksson L, Vash-Margita A, Pelosi E. Müllerian anomalies and endometriosis: associations and phenotypic variations. Reprod Biol Endocrinol 2024; 22:157. [PMID: 39702195 DOI: 10.1186/s12958-024-01336-1] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/05/2024] [Accepted: 12/13/2024] [Indexed: 12/21/2024] Open
Abstract
Müllerian anomalies are congenital conditions characterized by the incomplete development of the female reproductive tract. Women affected by Müllerian anomalies often display additional malformations of the renal, skeletal, and cardiovascular system, and are at a higher risk for infertility and adverse pregnancy outcomes. Several Müllerian anomalies have been reported in association with endometriosis, but it is unclear if all classes or anatomical variations are associated with the disease. Most importantly, both Müllerian anomalies and endometriosis can manifest with a wide degree of variability, adding further complexity to their poorly defined relationship. Retrograde menstruation occurring in obstructive Müllerian anomalies is a well-accepted mechanism for the development of endometriosis. However, endometriosis can occur following surgical correction of the anomaly or in the absence of obstruction. This suggests that other mechanisms may be involved, although the specific pathogenesis remains elusive. This review provides a comprehensive summary of the current state of clinical research on endometriosis in Müllerian anomalies. This review also highlights research and knowledge gaps, informing the development of future experimental designs to address current limitations including heterogeneity of phenotypes, variable comorbidities, and lack of genetic information.
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Affiliation(s)
- Surya Bhamidipaty-Pelosi
- Department of Clinical Pharmacology, Indiana University School of Medicine, Indianapolis, IN, USA
- Royal Brisbane and Women's Hospital, The University of Queensland, Brisbane, QLD, Australia
| | - Isaac Kyei-Barffour
- Institute for Molecular Bioscience, The University of Queensland, Brisbane, QLD, Australia
| | - Marianna Volpert
- School of Biomedical Sciences, Queensland University of Technology, Brisbane, QLD, Australia
| | - Nora O'Neill
- Division of Pediatric and Adolescent Gynecology, Department of Obstetrics, Gynecology and Reproductive Sciences, Yale University School of Medicine, New Haven, CT, USA
| | - Alyssa Grimshaw
- Harvey Cushing/John Hay Whitney Medical Library, Yale University, New Haven, CT, USA
| | - Lars Eriksson
- Herston Health Sciences Library, The University of Queensland, Brisbane, QLD, Australia
| | - Alla Vash-Margita
- Division of Pediatric and Adolescent Gynecology, Department of Obstetrics, Gynecology and Reproductive Sciences, Yale University School of Medicine, New Haven, CT, USA
| | - Emanuele Pelosi
- Centre for Clinical Research, The University of Queensland, Brisbane, QLD, Australia.
- Department of Biochemistry and Molecular Biology, Indiana University School of Medicine, Indianapolis, IN, USA.
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Dayan D, Ebner F, Janni W, Hancke K, Adiyaman D, Huener B, Hensel M, Hartkopf AD, Schmid M, Lukac S. Endometriosis Coinciding with Uterus Didelphys and Renal Agenesis: A Literature Review. J Clin Med 2024; 13:7530. [PMID: 39768455 PMCID: PMC11727832 DOI: 10.3390/jcm13247530] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/02/2024] [Revised: 11/15/2024] [Accepted: 12/07/2024] [Indexed: 01/16/2025] Open
Abstract
Background/Objectives: Endometriosis and urogenital malformation with uterus didelphys and renal agenesis might occur concomitantly, and the question arises whether both entities are associated with each other. Methods: A literature search was conducted in PubMed and Web of Science, using the following search terms: "endometriosis and uterine malformation, endometriosis and Herlyn-Werner-Wunderlich syndrome", "endometriosis and OHVIRA (Obstructed Hemivagina and Ipsilateral Renal Anomaly) syndrome" and "uterus didelphys, renal agenesis and endometriosis". Results: We identified and examined 36 studies, comprising a total of 563 cases with coinciding endometriosis and OHVIRA. The most prevalent symptoms were dysmenorrhea and lower abdominal pain. Renal agenesis occurred more frequently on the right side. In the majority of cases, vaginal septum resection was performed to alleviate hematometrocolpos. Among the 97 cases necessitating abdominal exploration, endometriosis was identified in 61 patients (62.9%), although this figure is most likely an overestimation. However, a significantly heightened risk of endometriosis was evident. Conclusions: This literature review highlights the importance of considering the potential for urogenital malformation and endometriosis in cases of dysmenorrhea during adolescence. Ultrasound examination has proven to be a valuable diagnostic tool for identifying uterine abnormalities and guiding subsequent diagnostic and, if necessary, surgical interventions. Thorough assessment and appropriate management are imperative to mitigating the long-term consequences associated with deep infiltrating endometriosis.
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Affiliation(s)
- Davut Dayan
- Klinik für Frauenheilkunde und Geburtshilfe, Universitätsklinikum Ulm, 89075 Ulm, Germany; (F.E.); (W.J.); (K.H.); (D.A.); (B.H.); (M.H.); (A.D.H.); (M.S.)
| | - Florian Ebner
- Klinik für Frauenheilkunde und Geburtshilfe, Universitätsklinikum Ulm, 89075 Ulm, Germany; (F.E.); (W.J.); (K.H.); (D.A.); (B.H.); (M.H.); (A.D.H.); (M.S.)
- Abteilung für Frauenheilkunde und Geburtshilfe, Alb-Donau Klinikum Ehingen, 89584 Ehingen (Donau), Germany
| | - Wolfgang Janni
- Klinik für Frauenheilkunde und Geburtshilfe, Universitätsklinikum Ulm, 89075 Ulm, Germany; (F.E.); (W.J.); (K.H.); (D.A.); (B.H.); (M.H.); (A.D.H.); (M.S.)
| | - Katharina Hancke
- Klinik für Frauenheilkunde und Geburtshilfe, Universitätsklinikum Ulm, 89075 Ulm, Germany; (F.E.); (W.J.); (K.H.); (D.A.); (B.H.); (M.H.); (A.D.H.); (M.S.)
| | - Duygu Adiyaman
- Klinik für Frauenheilkunde und Geburtshilfe, Universitätsklinikum Ulm, 89075 Ulm, Germany; (F.E.); (W.J.); (K.H.); (D.A.); (B.H.); (M.H.); (A.D.H.); (M.S.)
| | - Beate Huener
- Klinik für Frauenheilkunde und Geburtshilfe, Universitätsklinikum Ulm, 89075 Ulm, Germany; (F.E.); (W.J.); (K.H.); (D.A.); (B.H.); (M.H.); (A.D.H.); (M.S.)
| | - Michelle Hensel
- Klinik für Frauenheilkunde und Geburtshilfe, Universitätsklinikum Ulm, 89075 Ulm, Germany; (F.E.); (W.J.); (K.H.); (D.A.); (B.H.); (M.H.); (A.D.H.); (M.S.)
| | - Andreas Daniel Hartkopf
- Klinik für Frauenheilkunde und Geburtshilfe, Universitätsklinikum Ulm, 89075 Ulm, Germany; (F.E.); (W.J.); (K.H.); (D.A.); (B.H.); (M.H.); (A.D.H.); (M.S.)
- Department für Frauengesundheit, Universitäts-Frauenklinik Tübingen, 72076 Tübingen, Germany
| | - Marinus Schmid
- Klinik für Frauenheilkunde und Geburtshilfe, Universitätsklinikum Ulm, 89075 Ulm, Germany; (F.E.); (W.J.); (K.H.); (D.A.); (B.H.); (M.H.); (A.D.H.); (M.S.)
| | - Stefan Lukac
- Klinik für Frauenheilkunde und Geburtshilfe, Universitätsklinikum Ulm, 89075 Ulm, Germany; (F.E.); (W.J.); (K.H.); (D.A.); (B.H.); (M.H.); (A.D.H.); (M.S.)
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Qi J, Zhou P, Peng H, Deng J, Shao Y, Ye L, Luo S. Clinical and Imaging Characteristics of Herlyn-Werner-Wunderlich Syndrome: a Comprehensive Analysis. Reprod Sci 2024; 31:3343-3350. [PMID: 38907127 PMCID: PMC11527966 DOI: 10.1007/s43032-024-01628-2] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/02/2024] [Accepted: 06/16/2024] [Indexed: 06/23/2024]
Abstract
PURPOSE To describe the clinical and imaging characteristics of Herlyn-Werner-Wunderlich syndrome (HWWS). METHODS This study presented an observational case series involving consecutive patients diagnosed with HWWS, whose medical records were retrospectively reviewed. From June 2012 to December 2022, there were a total of 85 patients with HWWS enrolled in our study. We obtained the medical history, including demographic characteristics, clinical presentation, treatment, complications, and radiologic examinations performed. Patients > 18 years of age (n = 58) were recontacted. RESULT In our analysis, 27 patients were categorised as having complete obstruction, and 58 were categorised as having incomplete obstruction. The mean age at the onset of symptoms and diagnosis of complete obstruction was significantly younger than incomplete obstruction (P < 0.05). For complete obstruction, the median time between menarche and the onset of symptoms was 2.1 years, while for incomplete obstruction, it was 5.3 years. There was a significantly lower incidence of intermittent mucopurulent discharge, irregular vaginal haemorrhage, and occasional examination findings of complete obstruction than incomplete obstruction (P < 0.05). Complete obstruction was significantly associated with dysmenorrhea and pelvic endometriosis compared with incomplete obstruction (P < 0.05). CONCLUSIONS There are distinct clinical differences between patients with complete obstruction of the hemivagina and those with incomplete obstruction. HWWS can manifest as various combinations of uterine anomalies, communications anomalies, and renal anomalies. Early recognition and treatment can avoid complications and preserve fertility. KEYSWORDS Herlyn-Werner-Wunderlich syndrome (HWWS); complete obstruction; incomplete obstruction; obstructed hemivagina; congenital malformation.
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Affiliation(s)
- Jiani Qi
- Department of Ultrasonography, The Third Xiangya Hospital of Central South University, Changsha, Hunan Province, China
| | - Ping Zhou
- Department of Ultrasonography, The Third Xiangya Hospital of Central South University, Changsha, Hunan Province, China
| | - Hong Peng
- Department of Ultrasonography, The Third Xiangya Hospital of Central South University, Changsha, Hunan Province, China
| | - Jin Deng
- Department of Ultrasonography, The Third Xiangya Hospital of Central South University, Changsha, Hunan Province, China
| | - Yang Shao
- Department of Ultrasonography, The Third Xiangya Hospital of Central South University, Changsha, Hunan Province, China
| | - Lingjuan Ye
- Department of Ultrasonography, The Third Xiangya Hospital of Central South University, Changsha, Hunan Province, China
| | - Shengjuan Luo
- Department of Ultrasonography, The Third Xiangya Hospital of Central South University, Changsha, Hunan Province, China.
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Lei XG, Zhang H. Vaginal clear cell adenocarcinoma in Herlyn-Werner-Wunderlich syndrome: A case report. World J Clin Oncol 2024; 15:1359-1365. [DOI: 10.5306/wjco.v15.i10.1359] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/26/2024] [Revised: 09/14/2024] [Accepted: 09/19/2024] [Indexed: 09/29/2024] Open
Abstract
BACKGROUND Herlyn-Werner-Wunderlich (HWW) syndrome is a rare Müllerian duct anomaly, characterized by a combination of urogenital abnormalities. The occurrence of primary cervico-vaginal carcinomas in patients with HWW syndrome is exceptionally rare, posing significant challenges for screening, early diagnosis, and effective management.
CASE SUMMARY We report a rare case of primary clear cell carcinoma of the vagina complicated in a 40-year-old woman with HWW syndrome. The patient presented with irregular vaginal bleeding for 4 years. On gynecological examination, an oblique vaginal septum was suspected. Surgical resection of the vaginal septum revealed a communicating fistula and a tumor on the left vagina and the left side of the septum, which was confirmed as clear cell carcinoma. One month later, she underwent a radical hysterectomy, vaginectomy, bilateral salpingo-oophorectomy, and pelvic lymph node dissection. Due to significant side effects, she completed only one course of chemotherapy. A year later, lung metastasis was detected and continued to grow. A thoracoscopic wedge resection of the right upper lobe was performed 4 years after the initial surgery. We also conducted a systemic review of the literature on primary cervical or vaginal carcinoma in HWW syndrome to explore this rare entity.
CONCLUSION Cervico-vaginal adenocarcinomas in patients with HWW syndrome are occult, and require early surgical intervention and regular imaging surveillance.
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Affiliation(s)
- Xian-Gao Lei
- Department of Radiology, Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education; West China Second University Hospital, Sichuan University, Chengdu 610041, Sichuan Province, China
| | - Heng Zhang
- Department of Radiology, Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education; West China Second University Hospital, Sichuan University, Chengdu 610041, Sichuan Province, China
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Jiang N, Xie Y, Qu W, Lei L, Gao S, Zhang H, Wang Q, Chen L, Sui L. Cervical Septum Incision Adversely Impacts Clinical Outcomes in Women With Complete Uterine Septum and Duplicated Cervix. J Minim Invasive Gynecol 2024; 31:756-760. [PMID: 38740131 DOI: 10.1016/j.jmig.2024.05.012] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/11/2023] [Revised: 05/02/2024] [Accepted: 05/05/2024] [Indexed: 05/16/2024]
Abstract
STUDY OBJECTIVE To investigate the reproductive outcomes of women with complete septate uterus and duplicated cervix who either did or did not receive cervical septum incision during hysteroscopic transcervical incision of the uterine septum. DESIGN Retrospective study approved by the hospital ethics committee. SETTING Obstetrics and Gynecology Hospital of Fudan University, Shanghai, China. PATIENTS Women with complete septate uterus and duplicated cervix who underwent hysteroscopic transcervical incision of the uterine septum in Obstetrics and Gynecology Hospital of Fudan University between January 2008 and December 2020 (n = 105). INTERVENTIONS Hysteroscopic incision of the septum. MEASUREMENTS AND MAIN RESULTS Included patients were grouped according to whether or not cervical septum incision was performed. Reproductive outcomes including gravidity, abortion rate, preterm birth rate, full-term birth rate, premature rupture of membranes, and cervical incompetence were assessed. In the no incision group, the abortion rate (7.4%) was significantly lower than that of the incision group (27.6%, p = .01); the preterm birth rate (4.6%) was significantly lower than that of the incision group (36.8%); and the full-term birth rate (95.5%) exceeded that of the incision group (63.2%, p <.01). Incidence of premature rupture of membranes and cervical incompetence during pregnancy was higher in the incision group (15.8% and 10.5%, p <.01 and p = .03). CONCLUSION Significantly improved reproductive outcomes were observed among patients with complete septate uterus and duplicated cervix whose cervical septum was preserved during the hysteroscopic transcervical incision of the uterine septum procedure.
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Affiliation(s)
- Ninghong Jiang
- Medical Center for Diagnosis and Treatment of Uterine Cavity and Tubal Diseases, Obstetrics and Gynecology Hospital, Fudan University, Shanghai, China (all authors)
| | - Yu Xie
- Medical Center for Diagnosis and Treatment of Uterine Cavity and Tubal Diseases, Obstetrics and Gynecology Hospital, Fudan University, Shanghai, China (all authors)
| | - Wenjie Qu
- Medical Center for Diagnosis and Treatment of Uterine Cavity and Tubal Diseases, Obstetrics and Gynecology Hospital, Fudan University, Shanghai, China (all authors)
| | - Lei Lei
- Medical Center for Diagnosis and Treatment of Uterine Cavity and Tubal Diseases, Obstetrics and Gynecology Hospital, Fudan University, Shanghai, China (all authors)
| | - Shujun Gao
- Medical Center for Diagnosis and Treatment of Uterine Cavity and Tubal Diseases, Obstetrics and Gynecology Hospital, Fudan University, Shanghai, China (all authors)
| | - Hongwei Zhang
- Medical Center for Diagnosis and Treatment of Uterine Cavity and Tubal Diseases, Obstetrics and Gynecology Hospital, Fudan University, Shanghai, China (all authors)
| | - Qing Wang
- Medical Center for Diagnosis and Treatment of Uterine Cavity and Tubal Diseases, Obstetrics and Gynecology Hospital, Fudan University, Shanghai, China (all authors)
| | - Limei Chen
- Medical Center for Diagnosis and Treatment of Uterine Cavity and Tubal Diseases, Obstetrics and Gynecology Hospital, Fudan University, Shanghai, China (all authors)
| | - Long Sui
- Medical Center for Diagnosis and Treatment of Uterine Cavity and Tubal Diseases, Obstetrics and Gynecology Hospital, Fudan University, Shanghai, China (all authors)..
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Xu X, Tian Y, Zhou J, Li Z, Meng L, Huang X, Zhang M. A rare case report of Herlyn-Werner-Wunderlich syndrome: Unraveling unusual urinary anomalies and literature review. Heliyon 2024; 10:e33558. [PMID: 39071571 PMCID: PMC11276916 DOI: 10.1016/j.heliyon.2024.e33558] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/15/2024] [Revised: 06/23/2024] [Accepted: 06/24/2024] [Indexed: 07/30/2024] Open
Abstract
Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare congenital genitourinary abnormality defined by uterine didelphys, obstructed hemivagina, and ipsilateral urological anomalies. Accurate diagnosis and prompt commencement of therapy can be difficult owing to heterogeneous genitourinary malformation among different patients. This is a case report of a patient with rare HWWS with uterine didelphys, obstructed hemivagina, vagina-ureteral remnant fistula (Gartner's duct cyst), and ipsilateral kidney dysgenesis who complained of intermittent abdominal pain during menstruation. The right ureteral remnant of the patient was distinctive, with three portions. The upper section was connected to the right dysplastic kidney, the lower section formed the fistulous tract with the vagina and bladder, while the middle section communicated with Gartner's Duct Cyst, which merged with the vagina and opened to the posterior cavity of hemivagina. The lower section of the right ureter was excised and ligated during laparoscopic surgery, while the upper section was excised. The patient recovered after surgery. We presented this rare case and conducted a literature review to provide a more comprehensive understanding of HWWS. This could help gynecologists effectively reduce misdiagnosis and missed diagnosis, especially when combined with complicated urinary malformation.
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Affiliation(s)
- Xiaotong Xu
- Department of Gynecology, The Second Hospital of Hebei Medical University, Shijiazhuang, Hebei, 050000, PR China
| | - Yanpeng Tian
- Department of Gynecology, The Second Hospital of Hebei Medical University, Shijiazhuang, Hebei, 050000, PR China
- Department of Gynecology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan, 450052, PR China
| | - Jingwen Zhou
- Department of Gynecology, The Second Hospital of Hebei Medical University, Shijiazhuang, Hebei, 050000, PR China
- Department of Gynecology, Loujiang New City Hospital of Taicang, Suzhou, Jiangsu, 215334, PR China
| | - Zhongkang Li
- Department of Gynecology, The Second Hospital of Hebei Medical University, Shijiazhuang, Hebei, 050000, PR China
| | - Li Meng
- Department of Gynecology, The Second Hospital of Hebei Medical University, Shijiazhuang, Hebei, 050000, PR China
| | - Xianghua Huang
- Department of Gynecology, The Second Hospital of Hebei Medical University, Shijiazhuang, Hebei, 050000, PR China
| | - Mingle Zhang
- Department of Gynecology, The Second Hospital of Hebei Medical University, Shijiazhuang, Hebei, 050000, PR China
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Mohd Adzlan F, Mohd K, Ahmad N, Ramli R. OHVIRA syndrome: clinical implications of a delayed diagnosis. BMJ Case Rep 2024; 17:e259861. [PMID: 38782440 DOI: 10.1136/bcr-2024-259861] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 05/25/2024] Open
Abstract
Obstructed Hemi Vagina with Ipsilateral Renal Agenesis (OHVIRA) syndrome is a rarely encountered müllerian duct anomaly. Delayed diagnosis is common due to normal onset of puberty and menstruation. We report a case of a woman in her early 20s with a background history of multiple emergency department visits, ward admissions and surgeries for chronic abdominal pain. She was reviewed at 1 month postlaparotomy for recurrent pelvic abscess and was finally diagnosed to have an OHVIRA syndrome, 11 years after her first clinical presentation. Excision of the vaginal septum completely resolved her symptoms. We are reporting this case to highlight the clinical implications resulting from the delayed diagnosis, to look into factors contributing to the delay and to highlight the importance of having a high index of suspicion to diagnose this unique condition.
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Affiliation(s)
- Fadzlin Mohd Adzlan
- Department of O&G, Ministry of Health, Hospital Sultanah Nur Zahirah/Hospital Kemaman, Kuala Terengganu/Kemaman, Malaysia
| | - Karimah Mohd
- Department of O&G, Ministry of Health, Hospital Sultanah Nur Zahirah/Hospital Kemaman, Kuala Terengganu/Kemaman, Malaysia
| | - Nasriah Ahmad
- Department of O&G, Ministry of Health, Hospital Sultanah Nur Zahirah/Hospital Kemaman, Kuala Terengganu/Kemaman, Malaysia
| | - Roziana Ramli
- Department of O&G, Ministry of Health, Hospital Sultanah Nur Zahirah/Hospital Kemaman, Kuala Terengganu/Kemaman, Malaysia
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Zhang L, Liu R, Ye S, Wu M, Liu R. Unilateral Genital Tract Obstruction with Ipsilateral Renal Anomaly Syndrome: Classification, Clinical Manifestations, and Precise Diagnose. J Multidiscip Healthc 2024; 17:2185-2202. [PMID: 38736541 PMCID: PMC11088864 DOI: 10.2147/jmdh.s459066] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/11/2024] [Accepted: 04/30/2024] [Indexed: 05/14/2024] Open
Abstract
Purpose To recommend the classification of unilateral genital tract obstruction with ipsilateral renal anomaly (UGTOIRA) syndrome into five types based on the site of obstruction, and to analyze the clinical manifestations and precise diagnosis of the syndrome. Methods The data, including demographic characteristics, symptoms, and precise diagnoses from 59 patients over the last decade, were retrospectively analyzed. Data analysis was conducted using the statistical software package SPSS 26.0. Results All 59 patients diagnosed with UGTOIRA syndrome were classified into five types based on the site of obstruction: Type I (vaginal obstruction) (45, 76.3%), Type II (cervicovaginal obstruction) (7, 11.9%), Type III (cervical obstruction) (3, 5.1%), Type IV (unilateral partial cervical aplasia) (3, 5.1%), and Type V (Unilateral isthmus atresia) (1, 1.7%). Of these cases, there were 34 cases (57.6%) with communication and 25 cases (42.4%) without communication between the left and right genital tracts. The chief complaints included dysmenorrhea alone in 28 cases (47.5%), dysmenorrhea accompanied by blood dripping after menstruation in 12 cases (20.3%), blood dripping after menstruation alone in 14 cases (23.7%), purulent vaginal secretions in one case (1.7%), vaginal pain in one case (1.7%), irregular menstruation in one case (l.7%), and infertility in two cases (3.4%). The precise diagnostic criteria include the affected side, abnormalities in the kidney and ureter, the site of obstruction, the location of blood accumulation, the size of the ipsilateral genital tract, whether there is communication and its site, the type based on the site of obstruction, and the presence and type of complications. Conclusion This classification of UGTOIRA syndrome encompasses the anatomical features of all cases reported in our study. Only by fully understanding the anatomical characteristics of this syndrome and identifying its clinical manifestations can clinicians make precise diagnoses as early as possible and provide individualized management.
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Affiliation(s)
- Ling Zhang
- Department of Obstetrics and Gynecology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, People’s Republic of China
- National Clinical Research Center for Obstetrics and Gynecology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, People’s Republic of China
| | - Ronghua Liu
- Department of Obstetrics and Gynecology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, People’s Republic of China
- National Clinical Research Center for Obstetrics and Gynecology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, People’s Republic of China
| | - Shuangmei Ye
- Department of Obstetrics and Gynecology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, People’s Republic of China
- National Clinical Research Center for Obstetrics and Gynecology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, People’s Republic of China
| | - Mingfu Wu
- Department of Obstetrics and Gynecology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, People’s Republic of China
- National Clinical Research Center for Obstetrics and Gynecology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, People’s Republic of China
| | - Rong Liu
- Department of Obstetrics and Gynecology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, People’s Republic of China
- National Clinical Research Center for Obstetrics and Gynecology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, People’s Republic of China
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Costa L, Garcia-Grau E, Toledo L, Burgaya N, Cos R, Rojas M, Giménez-Palop O, Caixas A. Herlyn-Werner-Wunderlinch: An unusual presentation in a patient with Prader-Willi syndrome. ENDOCRINOL DIAB NUTR 2024; 71:171-176. [PMID: 38735678 DOI: 10.1016/j.endien.2024.01.010] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/01/2023] [Revised: 01/15/2024] [Accepted: 01/16/2024] [Indexed: 05/14/2024]
Abstract
Herlyn-Werner-Wunderlich syndrome is an uncommon urogenital anomaly defined by uterus didelphys, obstructed hemi-vagina and unilateral renal anomalies. The most common clinical presentation is dysmenorrhoea following menarche, but it can also present as pain and an abdominal mass. Prader-Willi syndrome is a rare neuroendocrine genetic syndrome. Hypothalamic dysfunction is common and pituitary hormone deficiencies including hypogonadism are prevalent. We report the case of a 33-year-old female with Prader-Willi syndrome who was referred to the Gynaecology clinic due to vaginal bleeding and abdominal pain. Abdominal ultrasound revealed a haematometra and haematocolpos and computed tomography showed a uterus malformation and a right uterine cavity occupation (hematometra) as well as right kidney agenesis. Vaginoscopy and hysteroscopy were performed under general anaesthesia, finding a right bulging vaginal septum and a normal left cervix and hemiuterus. Septotomy was performed with complete haematometrocolpos drainage. The association of the two syndromes remains unclear.
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Affiliation(s)
- Laura Costa
- Obstetrics and Gynaecology Department, Gynaecological Unit, Spain.
| | - Emma Garcia-Grau
- Obstetrics and Gynaecology Department, Gynaecological Unit, Spain
| | - Laura Toledo
- Obstetrics and Gynaecology Department, Gynaecological Unit, Spain
| | - Nuria Burgaya
- Obstetrics and Gynaecology Department, Gynaecological Unit, Spain
| | - Ramon Cos
- Obstetrics and Gynaecology Department, Gynaecological Unit, Spain
| | - Mireia Rojas
- Obstetrics and Gynaecology Department, Gynaecological Unit, Spain
| | - Olga Giménez-Palop
- Endocrinology and Nutrition Department, Parc Taulí Hospital Universitari, Institut d'Investigació i Innovació Parc Taulí I3PT-CERCA-ISCIII, Department of Medicine, Universitat Autònoma de Barcelona, Sabadell, Barcelona, Spain
| | - Assumpta Caixas
- Endocrinology and Nutrition Department, Parc Taulí Hospital Universitari, Institut d'Investigació i Innovació Parc Taulí I3PT-CERCA-ISCIII, Department of Medicine, Universitat Autònoma de Barcelona, Sabadell, Barcelona, Spain
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Moon LM, Anderson Z, Cisneros-Camacho AL, Dietrich JE. Presentation and Management of Uterine Didelphys with Unilateral Cervicovaginal Agenesis/Dysgenesis (CVAD): A Multicenter Case Series. J Pediatr Adolesc Gynecol 2024; 37:72-77. [PMID: 37558159 DOI: 10.1016/j.jpag.2023.08.001] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/17/2023] [Revised: 07/04/2023] [Accepted: 08/04/2023] [Indexed: 08/11/2023]
Abstract
INTRODUCTION There are several well-described presentations of uterine didelphys (UD): UD without vaginal septum, UD with non-obstructed longitudinal vaginal septum, or UD with duplicated vaginas and an obstructed hemivagina on one side with ipsilateral renal anomaly. STUDY OBJECTIVE To describe another variant of UD and compare the presentation and management across different institutions METHODS: This was a retrospective case series approved by the NASPAG Fellows Research Consortium. Participating institutions obtained IRB approval. Inclusion criteria included a diagnosis of UD and unilateral cervicovaginal agenesis/dysgenesis (CVAD). Descriptive statistics were used. RESULTS Five patients met the inclusion criteria, with ages ranging from 13 to 27 years. Presenting symptoms included dysmenorrhea (80%), irregular bleeding (40%), acute onset left lower quadrant pain (20%), and abdominal mass (20%). Three patients had additional known abnormalities, including solitary kidney and solitary adrenal gland. All patients underwent pelvic magnetic resonance imaging. Two cases were only suspicious for unilateral CVAD on imaging and required pathology review postoperatively to confirm diagnosis. Two cases required a 2-staged approach with an initial diagnostic surgery followed by a second definitive procedure. Three patients were noted to have endometriosis intraoperatively. Postoperative follow-up ranged from 2 months to 2 years, with 1 patient reporting chronic pelvic pain. CONCLUSION Diagnosis on the basis of pelvic imaging can be difficult, as this unique variant may mimic classic obstructed hemivagina with ipsilateral renal anomaly. In patients with UD with unilateral CVAD, standard management is removal of the obstructed uterine horn. This multicenter series stresses awareness about the clinical presentation, distinguishes cases of cervical agenesis from dysgenesis, and reviews approaches to management.
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Affiliation(s)
- Lisa M Moon
- Department of Obstetrics & Gynecology, Baylor College of Medicine, Houston, Texas; Department of Obstetrics & Gynecology, University of Oklahoma College of Medicine, Oklahoma City, Oklahoma.
| | - Zachary Anderson
- University of Oklahoma College of Medicine, Oklahoma City, Oklahoma; Department of Obstetrics & Gynecology, University of Southern California, Los Angeles, California
| | | | - Jennifer E Dietrich
- Department of Obstetrics & Gynecology, Baylor College of Medicine, Houston, Texas
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Lim LM, Sivapatham L, Chong ASL, Wahab AVA. Obstructed Hemivagina Ipsilateral Renal Agenesis (OHVIRA) Syndrome-A Retrospective Cohort Analysis of the Sabah Population. J Pediatr Adolesc Gynecol 2023; 36:541-544. [PMID: 37451429 DOI: 10.1016/j.jpag.2023.07.002] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/20/2023] [Revised: 06/15/2023] [Accepted: 07/08/2023] [Indexed: 07/18/2023]
Abstract
OBJECTIVES The objectives of the study were to compare the demographic characteristics, presenting complaints, timeliness of diagnosis, and treatments given to all patients diagnosed with obstructive hemivagina ipsilateral renal agenesis (OHVIRA) between the adolescent group (age 10-19) and the adult group (age 20 and above) and to propose a feasible screening test consisting of routine bedside ultrasound to detect renal anomalies in addition to pelvic ultrasound during the assessment of females with gynecologic complications in resource-limited settings. METHOD AND ANALYSIS We conducted a retrospective cohort analysis of all patients with OHVIRA at our institution, Sabah Women and Children's Hospital, Malaysia, between the 2013 and 2022. Data were collected by reviewing patients' medical notes. RESULTS There were a total of 18 patients diagnosed with OHVIRA from 2013 to 2022, aged 10-41 years old. Nine patients (50%) presented during adolescence. Most (88.9%) came with symptoms such as abdominal pain, urinary symptoms, abnormal uterine bleeding, foul-smelling vaginal discharge, and primary infertility, whereas only 2 patients (11.1%) were asymptomatic and diagnosed incidentally. Acute abdomen was more common in the adolescent group (P = .015). No significant difference was found on the side of the renal anomaly. CONCLUSION Presenting symptoms vary and are often mimicked other gynecologic or surgical conditions, making the diagnosis difficult and delayed. Some patients were incidentally diagnosed while being managed for other problems, and it was not uncommon to have unnecessary surgery before the correct diagnosis was made. We suggest that all female patients with abdominal and pelvic complaints should be screened for renal anomaly during pelvic scan to improve diagnostic rates.
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Affiliation(s)
- Leek Mei Lim
- Department of Obstetrics & Gynaecology, Sabah Women and Children's Hospital, Kota Kinabalu, Sabah, Malaysia.
| | | | - Audrey Shuk Lan Chong
- Clinical Research Centre (CRC), Sabah Women and Children's Hospital, Kota Kinabalu, Sabah, Malaysia
| | - Ana Vetriana Abd Wahab
- Department of Obstetrics & Gynaecology, Sabah Women and Children's Hospital, Kota Kinabalu, Sabah, Malaysia
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Grammatis AL, Ajibade F, Warakaulle D, Dada T. Obstructed Hemi-Vagina with Ipsilateral Renal Agenesis Syndrome in Adulthood: A Diagnostic Challenge. Diagnostics (Basel) 2023; 13:3377. [PMID: 37958273 PMCID: PMC10649003 DOI: 10.3390/diagnostics13213377] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/02/2023] [Revised: 10/30/2023] [Accepted: 10/30/2023] [Indexed: 11/15/2023] Open
Abstract
A patient in her early 20s presented with constant and progressive lower abdominal and back pain, mainly on the right side of the abdomen, purulent vaginal discharge and pyrexia. A radiological assessment revealed a possible tubo-ovarian abscess and the incidental diagnosis of ipsilateral renal agenesis. The patient was treated for pelvic inflammatory disease (PID); however, after antibiotic administration and since the symptoms did not resolve, an abdominal MRI was requested, which revealed uterus didelphys with two cervices, an obstructed haemivagina and evidence of haematocolpos. The diagnosis of Obstructed Hemi-Vagina with Ipsilateral Renal Agenesis (OHVIRA) syndrome was confirmed, and the patient underwent the excision of the vaginal septum, the drainage of the haematopyocolpos and the laparoscopic drainage of the tubo-ovarian abscess. She achieved a good recovery.
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Affiliation(s)
| | - Femi Ajibade
- Department of Obstetrics and Gynaecology, Cumberland Infirmary Hospital, Carlisle CA2 7HY, UK;
| | - Dinuke Warakaulle
- Department of Radiology, Buckinghamshire Healthcare NHS Trust, Aylesbury HP21 8AL, UK;
| | - Tunde Dada
- Department of Obstetrics and Gynaecology, Buckinghamshire Healthcare NHS Trust, Aylesbury HP21 8AL, UK;
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Liu Y, Li Z, Dou Y, Wang J, Li Y. Anatomical variations, treatment and outcomes of Herlyn-Werner-Wunderlich syndrome: a literature review of 1673 cases. Arch Gynecol Obstet 2023; 308:1409-1417. [PMID: 36823415 DOI: 10.1007/s00404-022-06856-y] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/30/2022] [Accepted: 11/09/2022] [Indexed: 02/25/2023]
Abstract
PURPOSE This study aimed to perform a systematic review of patients with Herlyn-Werner-Wunderlich syndrome (HWWS) and present the prevalence of symptoms, anatomical variants, endometriosis, surgical interventions, and short- and long-term outcomes. METHODS A structured search was performed in PubMed, Scopus, Embase, and China National Knowledge Infrastructure, and studies published between 1 January, 2000 and 19 April, 2022 were included. The following data on HWWS were recorded: symptoms, anatomical variations, surgical interventions and short- and long-term outcomes. RESULTS A total of 1673 patients were included in our analysis. The main symptoms were dysmenorrhea (53.8%), abnormal uterine bleeding (28.9%), and vaginal discharge (26.6%). The principal anomalies were right-obstructed hemivagina (57.3%), haematocolpos (81.7%), uterus didelphys (88.8%), and ipsilateral renal agenesis (93.1%). A majority of patients belonged to classification 1.1 (46.7%), with a blind hemivagina, and classification 2.1 (39.2%), with a small communication between two vaginas. The mainstay of treatment was vaginal septum excision (91.8%). Minimally invasive surgery (48.5%) was performed only after vaginal surgery (61.9%), and only a few patients required a second surgery (2.2%). Endometriosis was found in 9.6% of the patients. Fifty-two percent of them had ipsilateral ovarian endometriosis cysts. Pregnancy rate of these patients was 72.1%. The rate of adverse pregnancy outcomes was 22.4%. The caesarean section rate was 61.2%. CONCLUSIONS Patients with HWWS presented with nonspecific symptoms and demonstrated various combinations of anomalies. The most common anatomical variants are classifications 1.1 and 2.1. Vaginal septum excision is effective in relieving symptoms and preventing complications, with hysteroscopic surgery as an option where there is concern about protecting the hymen from minor injury. The pregnancy rate for these patients after surgery was satisfactory, and the rate of adverse pregnancy outcomes after surgery was acceptable. We advise females with urological anomalies to be screened for Müllerian anomalies because of the close association between these two types of anomalies. Thus, HWWS contributes to the occurrence of endometriosis; however, more research is required to investigate the relationship between pelvic endometriosis and HWWS.
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Affiliation(s)
- Yu Liu
- Obstetrics and Gynecology of the Hainan Hospital of PLA General Hospital, Sanya, Hainan, China
| | - Zhen Li
- PLA, Obstetrics and Gynecology of the Southwest Hospital of Army Medical University, Shapingba District, Gaotanyan Main Street 29, Chongqing, 400000, China
| | - Yuya Dou
- PLA, Obstetrics and Gynecology of the Southwest Hospital of Army Medical University, Shapingba District, Gaotanyan Main Street 29, Chongqing, 400000, China
| | - Jingjing Wang
- PLA, Obstetrics and Gynecology of the Southwest Hospital of Army Medical University, Shapingba District, Gaotanyan Main Street 29, Chongqing, 400000, China
| | - Yudi Li
- PLA, Obstetrics and Gynecology of the Southwest Hospital of Army Medical University, Shapingba District, Gaotanyan Main Street 29, Chongqing, 400000, China.
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Borges AL, Sanha N, Pereira H, Martins A, Costa C. Herlyn-Werner-Wunderlich syndrome also known as obstructed hemivagina and ipsilateral renal anomaly: A case report and a comprehensive review of literature. Radiol Case Rep 2023; 18:2771-2784. [PMID: 37388267 PMCID: PMC10300495 DOI: 10.1016/j.radcr.2023.05.024] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/05/2023] [Accepted: 05/05/2023] [Indexed: 07/01/2023] Open
Abstract
Herlyn-Werner-Wunderlich syndrome, also known as obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), is a Müllerian duct anomaly. It is a rare clinical condition consisting of a duplicated uterus with an oblique vaginal septum that causes partial genital tract outflow obstruction. A urinary tract anomaly, most commonly renal agenesis, is usually present on the obstructed side. The diagnosis of genital tract outflow obstruction is often delayed due to the normal functioning of the unaffected side. The most frequent complications are dysmenorrhea, chronic pelvic pain, infection, infertility and endometriosis. This report describes a 17-year-old G0P0 patient with a history of severe dysmenorrhea and left-sided renal agenesis, who was admitted for complaints of foul vaginal discharge over the past 3 months that was unsuccessfully treated with antibiotics. Transrectal ultrasound revealed the presence of 2 separate hemicavities on transverse and longitudinal views. A cystic lesion with ground-glass opacities was detected between the bladder and a normal-appearing cervix, which was determined to be hematocolpos. The diagnosis of OHVIRA was made. This case highlights the importance of excluding a Müllerian anomaly in the presence of renal system abnormalities. Being aware of the type of anomalies, combinations and variants is crucial to determine the diagnosis and the best surgical approach. Ultrasound was an invaluable imaging exam to determine the type of anomaly and its complexity. Awareness of this syndrome and its variants will prevent misdiagnosis and will help to define the appropriate treatment for these patients.
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Affiliation(s)
- André Luís Borges
- Obstetrics and Gynecology Department, Hospital de São Francisco Xavier-Centro Hospitalar de Lisboa Ocidental, Estrada Forte do Alto Duque, Edificio 2 Piso 3, Lisboa, 1449-005, Portugal
- Faculdade de Ciências da Saúde, Universidade da Beira Interior, Av. Infante D. Henrique, Covilhã, Portugal
| | - Nhalim Sanha
- Obstetrics and Gynecology Department, Hospital de São Francisco Xavier-Centro Hospitalar de Lisboa Ocidental, Estrada Forte do Alto Duque, Edificio 2 Piso 3, Lisboa, 1449-005, Portugal
| | - Helena Pereira
- Obstetrics and Gynecology Department, Hospital de São Francisco Xavier-Centro Hospitalar de Lisboa Ocidental, Estrada Forte do Alto Duque, Edificio 2 Piso 3, Lisboa, 1449-005, Portugal
| | - Ana Martins
- Obstetrics and Gynecology Department, Hospital de São Francisco Xavier-Centro Hospitalar de Lisboa Ocidental, Estrada Forte do Alto Duque, Edificio 2 Piso 3, Lisboa, 1449-005, Portugal
| | - Cristina Costa
- Obstetrics and Gynecology Department, Hospital de São Francisco Xavier-Centro Hospitalar de Lisboa Ocidental, Estrada Forte do Alto Duque, Edificio 2 Piso 3, Lisboa, 1449-005, Portugal
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Zhang H, Zhang Y, Bao L, Ning G. MRI presentations of Müllerian duct anomalies in association with unilateral renal agenesis. Clin Radiol 2023; 78:168-174. [PMID: 36273955 DOI: 10.1016/j.crad.2022.09.119] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/09/2022] [Revised: 07/21/2022] [Accepted: 09/13/2022] [Indexed: 11/28/2022]
Abstract
AIM To evaluate the presentations of Müllerian duct anomalies (MDAs) associated with unilateral renal agenesis (URA) on magnetic resonance imaging (MRI). MATERIALS AND METHODS From 2014 to 2021, 92 patients with MDAs coexisting with URA who had undergone MRI were reviewed, and imaging patterns were analysed. RESULTS Based on the different imaging patterns, there were 74 cases of Herlyn-Werner-Wunderlich (HWW) syndrome (80.4%), nine cases of unicornuate uterus (10.9%), and nine cases of Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome (8.7%). In HWW syndrome, URA was ipsilateral to the vaginal or cervical obstruction. All the nine cases of unicornuate uterus were obstructive, and the most common subtype was unicornuate uterus with a non-communicating functioning rudimentary horn. The other two cases of unicornuate uterus with no rudimentary horn were obstructed due to cervical os obliteration, one of which was complicated with contralateral absent ovary. URA was contralateral to the unicornuate uterus in all cases. In MRKH syndrome, seven patients had bilateral rudiments and ovaries, all of which were unilaterally or bilaterally located above the pelvic brim, and URA was ipsilateral to the ectopic ovary in all cases. The other two patients had unilateral absent rudiment, ovary, and ipsilateral URA. CONCLUSIONS MRI presentations of URA-associated MDAs are diverse, with HWW syndrome being the most common form, followed by different patterns of unicornuate uterus and MRKH syndrome. An ectopic or absent ovary might be associated with URA in MRKH syndrome and unicornuate uterus.
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Affiliation(s)
- H Zhang
- Department of Radiology, Key Laboratory of Obstetric & Gynecologic and Pediatric Diseases and Birth Defects of Ministry of Education, National Key Laboratory of Biotherapy, West China Second University Hospital, Sichuan University, China
| | - Y Zhang
- Department of Radiology, Key Laboratory of Obstetric & Gynecologic and Pediatric Diseases and Birth Defects of Ministry of Education, National Key Laboratory of Biotherapy, West China Second University Hospital, Sichuan University, China
| | - L Bao
- Department of Radiology, Key Laboratory of Obstetric & Gynecologic and Pediatric Diseases and Birth Defects of Ministry of Education, National Key Laboratory of Biotherapy, West China Second University Hospital, Sichuan University, China
| | - G Ning
- Department of Radiology, Key Laboratory of Obstetric & Gynecologic and Pediatric Diseases and Birth Defects of Ministry of Education, National Key Laboratory of Biotherapy, West China Second University Hospital, Sichuan University, China.
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Malhotra V, Nanda S, Chauhan M, Bhardwaj R, Chauhan A. Successful management of cervical dysgenesis: Case report and review. Trop Doct 2023; 53:271-275. [PMID: 36705083 DOI: 10.1177/00494755221149228] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/28/2023]
Abstract
Cervical agenesis or dysgenesis is a rare Mullerian anomaly that is usually associated with vaginal aplasia. A literature review revealed reports of 83 cases including ours, of which 57 (68.6%) presented with obstruction of the external OS, 11 (13.2%) had the cervix replaced by a fibrous cord and 5 (6.02%) had a fragmented cervix. A total of 24 (28.9%) were managed by core and drilling technique (CDT), 16(19.2%) patients underwent uterovaginal anastomosis (UVA), 7(8.4%) underwent total abdominal hysterectomy preserving the ovaries and 5 (6.02%) were managed by cervical reconstruction. Unfortunately, 31 failed to return after their clinical and radiological diagnosis was confirmed. Early diagnosis and treatment are necessary to avoid long-term complications.
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Affiliation(s)
- Vani Malhotra
- Professor, Department of Obstetrics & Gynaecology, 29061PGIMS, Rohtak, Haryana, India
| | - Smiti Nanda
- Professor, Department of Obstetrics & Gynaecology, 29061PGIMS, Rohtak, Haryana, India
| | - Meenakshi Chauhan
- Professor, Department of Obstetrics & Gynaecology, 29061PGIMS, Rohtak, Haryana, India
| | - Ritu Bhardwaj
- Professor, Department of Obstetrics & Gynaecology, 29061PGIMS, Rohtak, Haryana, India
| | - Aastha Chauhan
- Professor, Department of Obstetrics & Gynaecology, 29061PGIMS, Rohtak, Haryana, India
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Zhang H, Zheng Y, Ning G, Fu C, Bao L. Preoperative MRI presentations of Herlyn-Werner-Wunderlich syndrome. Congenit Anom (Kyoto) 2022; 62:228-235. [PMID: 35941518 DOI: 10.1111/cga.12489] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/24/2021] [Revised: 01/22/2022] [Accepted: 06/18/2022] [Indexed: 11/28/2022]
Abstract
Herlyn-Werner-Wunderlich (HWW) syndrome is a rare complex female urogenital anomaly, with diverse anatomical presentations. Due to obstruction, most patients with HWW syndrome need to be addressed surgically. The treatment strategy should be tailored to the different anatomical variants of each patient. Therefore, a detailed and comprehensive preoperative evaluation is needed. In this review, we describe the embryology and clinical manifestations of HWW syndrome and discuss and illustrate its diverse preoperative magnetic resonance imaging presentations to guide clinical treatment.
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Affiliation(s)
- Heng Zhang
- Department of Radiology, Key Laboratory of Obstetric & Gynecologic and Pediatric Diseases and Birth Defects of Ministry of Education, National Key Laboratory of Biotherapy, West China Second University Hospital, Sichuan University, Chengdu, Sichuan, China
| | - Ying Zheng
- Department of Gynecology, Key Laboratory of Obstetric & Gynecologic and Pediatric Diseases and Birth Defects of Ministry of Education, National Key Laboratory of Biotherapy, West China Second University Hospital, Sichuan University, Chengdu, Sichuan, China
| | - Gang Ning
- Department of Radiology, Key Laboratory of Obstetric & Gynecologic and Pediatric Diseases and Birth Defects of Ministry of Education, National Key Laboratory of Biotherapy, West China Second University Hospital, Sichuan University, Chengdu, Sichuan, China
| | - Chuan Fu
- Department of Radiology, Key Laboratory of Obstetric & Gynecologic and Pediatric Diseases and Birth Defects of Ministry of Education, National Key Laboratory of Biotherapy, West China Second University Hospital, Sichuan University, Chengdu, Sichuan, China
| | - Li Bao
- Department of Radiology, Key Laboratory of Obstetric & Gynecologic and Pediatric Diseases and Birth Defects of Ministry of Education, National Key Laboratory of Biotherapy, West China Second University Hospital, Sichuan University, Chengdu, Sichuan, China
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Zarfati A, Lucchetti MC. OHVIRA (Obstructed Hemivagina and Ipsilateral Renal Anomaly or Herlyn-Werner-Wunderlich syndrome): Is it time for age-specific management? J Pediatr Surg 2022; 57:696-701. [PMID: 35487798 DOI: 10.1016/j.jpedsurg.2022.04.002] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/17/2021] [Revised: 03/18/2022] [Accepted: 04/04/2022] [Indexed: 11/19/2022]
Abstract
BACKGROUND OHVIRA (Obstructed Hemivagina and Ipsilateral Renal Anomaly or Herlyn-Werner-Wunderlich syndrome) is a rare Müllerian malformation. Usually, symptoms begin with worsening dysmenorrhea in post-menarche adolescents. The management in pre-menarche period is controversial and has only recently been subject of study. AIMS To review the experience of a pediatric tertiary center and to propose an age-specific management protocol for patients diagnosed before menarche. METHODS A retrospective cohort study (review of medical records - period 2009-2021). RESULTS Twenty-eight patients were diagnosed (mean age 11.9 years), seven (25%) before menarche, one (3%) perinatally. One patient had Floating-Harbor syndrome. Twenty-three patients had ipsilateral renal agenesis, while five had a multicystic-dysplastic kidney. The contralateral kidney showed hypertrophy in 25 patients, pelvicalyceal ectasia in 8 and dysplasia in 1. Twenty-four patients were symptomatic. Three of the seven diagnosed prior to menarche had symptoms. All post-menarche diagnosed patients were symptomatic. Twenty-six patients underwent surgery (one-stage drainage, vaginal septal resection, and vaginoplasty). Asymptomatic pre-menarche patients were followed-up until surgery after menarche onset. No patient underwent surgery prior to menarche solely for OHVIRA diagnosis. At follow-up (median 3.5 years, 3 lost to follow-up), eighteen patients were asymptomatic, one developed endometriosis, one had impaired renal function, two needed reoperations. CONCLUSIONS Pre-menarche OHVIRA patients, without symptoms, should undergo regular follow-up until the onset of menarche. Surgery must be considered in post-menarche or symptomatic patients. Post-operative, long-term follow-up is required, evaluating both renal and gynecological issues. LEVEL-OF-EVIDENCE IV.
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Affiliation(s)
- Angelo Zarfati
- Department of Pediatric Surgery, Bambino Gesù Pediatric Hospital, Piazza di Sant'Onofrio, 4, Roma 00165, Italy; University of Rome Tor Vergata, Via Cracovia, 50, Roma 00133, Italy.
| | - Maria Chiara Lucchetti
- Department of Pediatric Surgery, Bambino Gesù Pediatric Hospital, Piazza di Sant'Onofrio, 4, Roma 00165, Italy
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20
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Mendoza KIR, Nicer DPC. Obstructed hemivagina with ipsilateral renal and urinary tract anomaly presenting as an unusual cause of acute abdomen: a radiologic perspective. BMJ Case Rep 2022; 15:e249959. [PMID: 35760501 PMCID: PMC9237859 DOI: 10.1136/bcr-2022-249959] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 06/08/2022] [Indexed: 11/03/2022] Open
Abstract
Obstructed hemivagina with ipsilateral renal anomaly (OHVIRA) is a rare congenital genitourinary defect with a triad of unilateral vaginal obstruction, uterine anomaly and ipsilateral renal agenesis. This paper reports an unusual presentation of OHVIRA, with our patient experiencing severe abdominal pain from a left tubo-ovarian abscess that is contralateral to the side of the vaginal outflow obstruction. Another reportable finding is our patient's rare association of a trifid ureter that fuses distally before inserting ectopically in her vaginal canal. Lastly, this case report also emphasises the importance of radiologists' expertise in suspecting the diagnosis early on and in contributing to the preoperative evaluation of patients with OHVIRA, thereby providing adequate management for these patients.
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Affiliation(s)
- Kim Ivan R Mendoza
- Department of Radiology, Philippine General Hospital, Manila, Philippines
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Image-Guided Transvaginal and Transabdominal Drain Placement for Surgical Navigation in a Patient with Tubo-Ovarian Abscess and Obstructed Hemivagina and Ipsilateral Renal Anomaly Syndrome. J Pediatr Adolesc Gynecol 2022; 35:94-97. [PMID: 34289429 DOI: 10.1016/j.jpag.2021.07.006] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/22/2021] [Revised: 06/10/2021] [Accepted: 07/09/2021] [Indexed: 11/21/2022]
Abstract
BACKGROUND We describe a minimally invasive, image-guided technique for obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome complicated by tubo-ovarian abscess (TOA). CASE A 15-year-old female adolescent with OHVIRA syndrome presented with TOA. Magnetic resonance imaging of the abdomen/pelvis showed a loculated pelvic mass and fluid collection near the obstructed left hemivagina. Tissue quality and ill-defined surgical planes prevented surgical excision. A transabdominal drain was placed via laparoscopic port by Interventional Radiology. She later underwent transvaginal and transabdominal drain placement into the hemivaginal collection using computed tomography and ultrasound guidance, allowing for surgical excision of the vaginal septum, drainage and excision of the TOA, and neosalpingostomy. SUMMARY AND CONCLUSION Because of the anatomic complexity in OHVIRA syndrome, preoperative minimally invasive techniques with Interventional Radiology collaboration can assist intraoperative anatomic navigation for successful surgical treatment.
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Feng T, Rao X, Yang X, Yu X, Xia F, Du X. A rare variant of obstructed hemivagina and ipsilateral renal agenesis and its improvement of classification. J Obstet Gynaecol Res 2022; 48:869-874. [PMID: 35014127 DOI: 10.1111/jog.15148] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/26/2021] [Revised: 12/24/2021] [Accepted: 12/30/2021] [Indexed: 11/30/2022]
Abstract
Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) is a rare congenital malformation of the female reproductive organs, including uterine dysplasia, blind hemivagina, and ipsilateral renal dysplasia. It has a serious impact on patients' quality of life and fertility. We report a rare case of OHVIRA with a complete septate uterus, ectopic dysplasia of the left kidney, left ectopic ureter, and three oblique vaginal septa. The dysplastic left kidney and the ectopic ureter were removed during laparoscopy. In addition, the two layers of vaginal septa, which were ignored in the past 10 years, were removed by hysteroscopy. As the detection rate of OHVIRA has gradually increased, manifestations that previous classification systems could not recapitulate have emerged. By retrieving the relevant cases, we have tried to propose a new classification system, which would allow clinicians to have a complete understanding of the manifestations of OHVIRA and would be helpful in the treatment of patients.
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Affiliation(s)
- Tongfu Feng
- Maternal and Child Health Hospital of Hubei Province, Wuhan, China
| | - Xiaoyan Rao
- Department of School of Medicine, Wuhan University of science and technology, Wuhan, China
| | - Xiaohong Yang
- Maternal and Child Health Hospital of Hubei Province, Wuhan, China
| | - Xudong Yu
- Maternal and Child Health Hospital of Hubei Province, Wuhan, China
| | - Feng Xia
- Maternal and Child Health Hospital of Hubei Province, Wuhan, China
| | - Xin Du
- Maternal and Child Health Hospital of Hubei Province, Wuhan, China
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Gutiérrez-Montufar OO, Zambrano-Moncayo CP, Otálora-Gallego MC, Meneses-Parra AL, Díaz-Yamal I. Síndrome de Herlyn-Werner-Wunderlich: reporte de caso y revisión de la literatura. REVISTA COLOMBIANA DE OBSTETRICIA Y GINECOLOGÍA 2021; 72:407-422. [PMID: 35134287 PMCID: PMC8833243 DOI: 10.18597/rcog.3699] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 04/22/2021] [Accepted: 11/08/2021] [Indexed: 11/16/2022]
Abstract
Objetivo: Presentar un reporte de caso de síndrome de Herlyn Werner Wunderlich (SHWW) y hacer una revisión de la literatura para determinar los hallazgos clínicos e imagenológicos en estas pacientes. Materiales y métodos: Se presenta el caso de una mujer de 16 años que consultó, en un hospital de las fuerzas militares en Bogotá, por dolor pélvico recurrente, su diagnostico final fue SHWW. Se realizó una búsqueda sistemática de la literatura en las diferentes bases de datos, revisiones sistemáticas, cohortes, series de casos y reportes de casos en pacientes con diagnóstico de SHWW en cualquier edad, se obtuvo información sobre las características de presentación clínica, y las tecnologías diagnósticas más frecuentemente utilizadas. Se hace resumen narrativo de los hallazgos. Resultados: Se incluyeron 77 publicaciones, un total de 676 pacientes. Los síntomas más frecuentes fueron dismenorrea (63,9 %), seguido de dolor pélvico (35,2%). Las tecnologías diagnósticas más utilizadas fueron el ultrasonido pélvico en un 92,1% y la resonancia magnética nuclear en un 74,6% de los casos. La histeroscopia y laparoscopia son poco utilizados en el diagnóstico. Conclusión: El SHWW es una entidad poco frecuente, debe hacer parte del estudio complementario de la agenesia renal del paciente pediátrico y del diagnóstico diferencial de la dismenorrea primaria en pacientes en la adolescencia. Se requiere evaluar con estudios de cohorte más grandes la utilidad de la histeroscopia en estas pacientes.
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Affiliation(s)
| | | | | | | | - Ivonne Díaz-Yamal
- Docente de la Universidad Militar Nueva Granada, Bogotá (Colombia)..
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Yu JH, Lee SR, Choi H, Kim KS, Kang BM. A New Case of Herlyn–Werner–Wunderlich Syndrome: Uterine Didelphys with Unilateral Cervical Dysgenesis, Vaginal Agenesis, Cervical Distal Ureteral Remnant Fistula, Ureterocele, and Renal Agenesis in a Patient with Contralateral Multicystic Dysplastic Kidney. Diagnostics (Basel) 2021; 12:diagnostics12010083. [PMID: 35054250 PMCID: PMC8774431 DOI: 10.3390/diagnostics12010083] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/03/2021] [Revised: 12/25/2021] [Accepted: 12/28/2021] [Indexed: 11/16/2022] Open
Abstract
The aim of this study was to present a new case of congenital Herlyn–Werner–Wunderlich syndrome, a rare anomaly of the female reproductive tract, and review the related literature. A 12-year-old girl presented with severe dysmenorrhea since menarche and magnetic resonance imaging showing a bicornuate uterus, double cervix, right hematometra, and hematosalpinx with ipsilateral renal agenesis, accompanied by a remnant distal ureter with hydroureter. A diagnostic cystoscopy and a reduced-port robot-assisted laparoscopy with chromopertubation were performed in order to identify the anomaly. Uterine didelphys and right cervical dysgenesis with ipsilateral vaginal agenesis, cervical distal ureteral remnant fistula, ureterocele, and renal agenesis were diagnosed on the basis of histopathologic findings, and she subsequently underwent a robotic unilateral right total hysterectomy with salpingectomy. This case report reinforces the importance of the intraoperative biopsy for an accurate diagnosis, despite magnetic resonance imaging being considered the gold-standard diagnostic tool.
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Affiliation(s)
- Jin-Hee Yu
- Department of Obstetrics and Gynecology, Seoul Asan Medical Center, University of Ulsan College of Medicine, 88, Olympic-ro 43-gil, Songpa-gu, Seoul 05505, Korea; (J.-H.Y.); (H.C.); (B.-M.K.)
| | - Sa-Ra Lee
- Department of Obstetrics and Gynecology, Seoul Asan Medical Center, University of Ulsan College of Medicine, 88, Olympic-ro 43-gil, Songpa-gu, Seoul 05505, Korea; (J.-H.Y.); (H.C.); (B.-M.K.)
- Correspondence: ; Tel.: +82-2-3010-3648; Fax: +82-2-3010-3630
| | - Heayeon Choi
- Department of Obstetrics and Gynecology, Seoul Asan Medical Center, University of Ulsan College of Medicine, 88, Olympic-ro 43-gil, Songpa-gu, Seoul 05505, Korea; (J.-H.Y.); (H.C.); (B.-M.K.)
| | - Kun-Suk Kim
- Department of Urology, Seoul Asan Medical Center, University of Ulsan College of Medicine, 88, Olympic-ro 43-gil, Songpa-gu, Seoul 05505, Korea;
| | - Byung-Moon Kang
- Department of Obstetrics and Gynecology, Seoul Asan Medical Center, University of Ulsan College of Medicine, 88, Olympic-ro 43-gil, Songpa-gu, Seoul 05505, Korea; (J.-H.Y.); (H.C.); (B.-M.K.)
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Pfeifer SM, Attaran M, Goldstein J, Lindheim SR, Petrozza JC, Rackow BW, Siegelman E, Troiano R, Winter T, Zuckerman A, Ramaiah SD. ASRM müllerian anomalies classification 2021. Fertil Steril 2021; 116:1238-1252. [PMID: 34756327 DOI: 10.1016/j.fertnstert.2021.09.025] [Citation(s) in RCA: 162] [Impact Index Per Article: 40.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/22/2021] [Accepted: 09/22/2021] [Indexed: 11/21/2022]
Abstract
There are many proposed classification systems for müllerian anomalies. The American Fertility Society (AFS) Classification from 1988 has been the most recognized and utilized. The advantages of this iconic classification include its simplicity, recognizability, and correlation with clinical pregnancy outcomes. However, the AFS classification has been criticized for its focus primarily on uterine anomalies, with exclusion of those of the vagina and cervix, its lack of clear diagnostic criteria, and its inability to classify complex aberrations. Despite this classification and others, the wide range of müllerian anomalies is still largely unknown and confusing to many providers. Consequently, müllerian anomalies may go undiagnosed for extended periods, receive inappropriate or inadequate surgical interventions, and result in persistent issues such as pain or loss of reproductive function. The American Society for Reproductive Medicine Task Force on Müllerian Anomalies Classification was formed and charged with designing a new classification. The Task Force set goals for a new classification and chose to base it on the iconic AFS classification from 1988 because of its simplicity and recognizability, while expanding and updating it to include all categories of anomalies. In addition, this was recognized as an opportunity to raise awareness of this area of medicine, educate providers and learners, and promote patient advocacy. Presented here is the new American Society for Reproductive Medicine Müllerian Anomalies Classification 2021.
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Romanski PA, Bortoletto P, Pfeifer SM. Unilateral Obstructed Müllerian Anomalies: A Series of Unusual Variants of Known Anomalies. J Pediatr Adolesc Gynecol 2021; 34:749-757. [PMID: 33910086 DOI: 10.1016/j.jpag.2021.04.005] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/05/2021] [Revised: 03/27/2021] [Accepted: 04/09/2021] [Indexed: 10/21/2022]
Abstract
BACKGROUND Obstructed Müllerian anomalies in adolescents can be grouped into complete outflow obstruction or unilateral outflow obstruction. The challenge with unilateral obstructions is that diagnosis can be delayed for weeks to years, as menstruation occurs normally through the patent side and thus obstruction is often not initially considered in the differential diagnosis. CASES In this case series, we present 3 unusual and challenging cases of unilateral Müllerian obstructions in adolescent female patients, along with strategies for diagnosis and management. Each case involves a unique variation of a recognized Müllerian anomaly that was initially misdiagnosed, leading to a significant delay in definitive diagnosis and treatment. SUMMARY AND CONCLUSION These cases highlight that even among the well-described Müllerian anomalies, there can be unusual variations. Patients who do not respond to initial management or who develop new symptoms should be further evaluated to confirm the correct diagnosis Tools that may be helpful in making the correct diagnosis include imaging studies that use contrast dye to better delineate cavities and their connections, magnetic resonance imaging with a radiologist experienced in Müllerian anomalies, and an examination under anesthesia. A definitive diagnosis is critical to the successful management of these conditions, and individualized management plans are required for each patient depending on their specific anomaly and their preferences for treatment.
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Affiliation(s)
- Phillip A Romanski
- The Ronald O. Perelman and Claudia Cohen Center for Reproductive Medicine, Weill Cornell Medical College, New York, New York.
| | - Pietro Bortoletto
- The Ronald O. Perelman and Claudia Cohen Center for Reproductive Medicine, Weill Cornell Medical College, New York, New York
| | - Samantha M Pfeifer
- The Ronald O. Perelman and Claudia Cohen Center for Reproductive Medicine, Weill Cornell Medical College, New York, New York
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Kudela G, Wiernik A, Drosdzol-Cop A, Machnikowska-Sokołowska M, Gawlik A, Hyla-Klekot L, Gruszczyńska K, Koszutski T. Multiple variants of obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome - one clinical center case series and the systematic review of 734 cases. J Pediatr Urol 2021; 17:653.e1-653.e9. [PMID: 34274235 DOI: 10.1016/j.jpurol.2021.06.023] [Citation(s) in RCA: 25] [Impact Index Per Article: 6.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/10/2020] [Revised: 05/10/2021] [Accepted: 06/24/2021] [Indexed: 11/16/2022]
Abstract
INTRODUCTION Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare female urogenital tract malformation. STUDY OBJECTIVE To present 10 patients with OHVIRA treated at the clinical center. To perform a systematic review of OHVIRA case series related to the prevalence of anatomical variants, surgical interventions and endometriosis, and to compare them with our case series. MATERIALS AND METHODS Medical records from 10 OHVIRA patients treated between 2016 and 2020 were retrospectively reviewed. For the systematic review, PubMed and Web of Science were used to search for relevant studies. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were strictly followed. RESULTS The most common anatomical variant includes left obstructed hemivagina (50.7%) with isolated hematocolpos or hydrocolpos (55.9%), uterus didelphys (82.9%), and ipsilateral renal agenesis (92.2%). Vaginal septectomy was the most common surgical approach (86.5%). Hemivaginectomy (2.2%), hemihysterectomy (4.2%), or total hysterectomy (0.7%) were also performed in several patients. Some subjects required salpingectomy (3.3%) or oophorectomy (1.8%). 7.5% of patients, mainly infants, did not require surgery due to the spontaneous resolution of hydrocolpos. Endometriosis was fortuitously found in 13.6% of the selected cases who underwent laparoscopy or laparotomy. DISCUSSION The most common variant of OHVIRA includes isolated hematocolpos and a thick vaginal septum between adjacent hemivaginas. Endometriosis was present in approximately 14% of OHVIRA patients, but this number is probably underestimated. Routine laparoscopy is not required. However, all patients need further monitoring due to a higher risk of endometriosis. Based on the analyzed studies and our case series, vaginal septectomy is a sufficient surgical technique to relieve symptoms and prevent possible complications in most OHVIRA patients.
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Affiliation(s)
- Grzegorz Kudela
- Department of Pediatric Surgery and Urology, Medical University of Silesia, Katowice, Poland.
| | - Agnieszka Wiernik
- Department of Pediatric Surgery and Urology, Medical University of Silesia, Katowice, Poland
| | - Agnieszka Drosdzol-Cop
- Department of Woman's Health, School of Health Sciences in Katowice, Medical University of Silesia, Katowice, Poland
| | | | - Aneta Gawlik
- Department of Pediatrics and Pediatric Endocrinology, Medical University of Silesia, Katowice, Poland
| | - Lidia Hyla-Klekot
- Department of Pediatric Surgery and Urology, Medical University of Silesia, Katowice, Poland
| | | | - Tomasz Koszutski
- Department of Pediatric Surgery and Urology, Medical University of Silesia, Katowice, Poland
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Kim YN, Han JH, Lee YS, Lee I, Han SW, Seo SK, Yun BH. Comparison between prepubertal and postpubertal patients with obstructed hemivagina and ipsilateral renal anomaly syndrome. J Pediatr Urol 2021; 17:652.e1-652.e7. [PMID: 34187747 DOI: 10.1016/j.jpurol.2021.06.008] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/16/2021] [Revised: 04/10/2021] [Accepted: 06/06/2021] [Indexed: 11/30/2022]
Abstract
BACKGROUND Obstructive hemivagina with ipsilateral renal anomaly (OHVIRA) syndrome is a rare, complex congenital anomaly with an unknown prevalence. However, case reports and small studies on OHVIRA syndrome have increased rapidly in the last 20 years, which may be related to increased use of imaging, surgical techniques, and prenatal sonography. OBJECTIVE This study aimed to analyze and compare patients with OHVIRA syndrome diagnosed in the prepubertal and postpubertal periods to understand the disease characteristics and improve clinical management. STUDY DESIGN A retrospective cohort study was conducted including 65 patients with OHVIRA syndrome who were diagnosed between January 2004 and September 2018 at a tertiary university hospital. RESULTS Among the 65 patients, 44 patients were diagnosed with OHVIRA syndrome during the prepubertal period and 21 patients were diagnosed postpubertally. Compared with postpubertally diagnosed patients with OHVIRA syndrome, those diagnosed prepubertally were mostly asymptomatic at initial presentation (82% versus [vs.] 0%, P < 0.001), had a higher incidence of ectopic ureter (68% vs. 24%, P = 0.001), and presented with a higher incidence of multicystic dysplastic kidney (61% vs. 19%, P = 0.01). Approximately half of the patients with prepubertal OHVIRA syndrome (53%) showed spontaneous resolution of hemivaginal fluid within 5 years. Among the patients with postpubertally diagnosed OHVIRA syndrome, those in the pain-dominant group had a larger amount of hemivaginal fluid than those in the painless discharge-dominant group (54% vs. 10%, P = 0.036). Superimposed infection of hemivaginal fluid was markedly present in the discharge-dominant group (9% vs. 75%, P = 0.006). CONCLUSIONS Clinical characteristics of patients with OHVIRA syndrome are altered based on the time of initial diagnosis. Follow-up and timely intervention should be proceeded accordingly.
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Affiliation(s)
- Yoo-Na Kim
- Department of Obstetrics and Gynecology, Severance Hospital, Yonsei University College of Medicine, Seoul, South Korea; Institute of Women's Life Science, Yonsei University College of Medicine, Seoul, South Korea
| | - Jang Hee Han
- Department of Urology and Urological Science Institute, Severance Hospital, Yonsei University College of Medicine, Seoul, Republic of Korea
| | - Yong Seung Lee
- Department of Urology and Urological Science Institute, Severance Hospital, Yonsei University College of Medicine, Seoul, Republic of Korea
| | - Inha Lee
- Department of Obstetrics and Gynecology, Severance Hospital, Yonsei University College of Medicine, Seoul, South Korea; Institute of Women's Life Science, Yonsei University College of Medicine, Seoul, South Korea
| | - Sang Won Han
- Department of Urology and Urological Science Institute, Severance Hospital, Yonsei University College of Medicine, Seoul, Republic of Korea
| | - Seok Kyo Seo
- Department of Obstetrics and Gynecology, Severance Hospital, Yonsei University College of Medicine, Seoul, South Korea; Institute of Women's Life Science, Yonsei University College of Medicine, Seoul, South Korea
| | - Bo Hyon Yun
- Department of Obstetrics and Gynecology, Severance Hospital, Yonsei University College of Medicine, Seoul, South Korea; Institute of Women's Life Science, Yonsei University College of Medicine, Seoul, South Korea.
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Liu M, Zhang L, Xia Y, Huang X, Ye T, Zhang Y, Qi Z, Wang L, Lai X, Dai Q, Jiang Y. New Consideration of Herlyn-Werner-Wunderlich Syndrome Diagnosed by Ultrasound. JOURNAL OF ULTRASOUND IN MEDICINE : OFFICIAL JOURNAL OF THE AMERICAN INSTITUTE OF ULTRASOUND IN MEDICINE 2021; 40:1893-1900. [PMID: 33236794 DOI: 10.1002/jum.15572] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 06/19/2020] [Revised: 10/17/2020] [Accepted: 10/20/2020] [Indexed: 06/11/2023]
Abstract
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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Affiliation(s)
- Meijuan Liu
- Department of Ultrasound, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
- Department of Ultrasound, Yantai Yuhuangding Hospital, Affiliated Hospital of Qingdao Medical University, Yantai, China
| | - Li Zhang
- Department of Ultrasound, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Yu Xia
- Department of Ultrasound, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Xuepei Huang
- Department of Ultrasound, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Tiantian Ye
- Department of Ultrasound, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Yixiu Zhang
- Department of Ultrasound, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Zhenhong Qi
- Department of Ultrasound, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Liang Wang
- Department of Ultrasound, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Xingjian Lai
- Department of Ultrasound, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Qing Dai
- Department of Ultrasound, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Yuxin Jiang
- Department of Ultrasound, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
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López-Alza LC, Mesa-Espinel MS. Síndrome de Herlyn-Werner-Wunderlich. Reporte de caso. REVISTA DE LA FACULTAD DE MEDICINA 2021. [DOI: 10.15446/revfacmed.v69n4.83840] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/09/2022] Open
Abstract
Introducción. El síndrome de Herlyn-Werner-Wunderlich (SHWW) es una malformación congénita rara del tracto urogenital que se caracteriza por la triada útero didelfo, hemivagina obstruida y anomalía renal ipsilateral, y que suele diagnosticarse después de la menarquia. Su tratamiento consiste en resección del tabique vaginal y drenaje de la hemivagina obstruida, con un buen pronóstico postquirúrgico.
Presentación del caso. Mujer de 22 años con un cuadro clínico de 9 años de evolución que inició cuando tuvo su primera menstruación, consistente en dismenorrea, dolor pélvico y ciclos menstruales irregulares, quien asistió al servicio de ginecología de un hospital de segundo nivel en Sogamoso, Boyacá (Colombia). La paciente reportó haber visitado múltiples especialistas y recibido tratamiento con vitamina E, metformina y anticonceptivos, sin mejoría de los síntomas y signos. Luego de ser valorada, y teniendo en cuenta los hallazgos en ecografía y resonancia magnética, fue diagnosticada con SHWW, por lo que se le realizó colpotomía más resección de masa paracervical derecha de aproximadamente 60x60 mm y de tabique vaginal, lográndose la resolución completa de los síntomas. Finalmente, 10 meses después de la cirugía, la joven refirió encontrarse en estado de embarazo, sin presentar complicaciones.
Conclusión. El SHWW es una malformación poco común que debe considerarse como diagnóstico diferencial en mujeres de cualquier edad con anomalías de los conductos paramesonéfricos, dolor pélvico, dismenorrea y masa en el tracto genital, pues su diagnóstico temprano y un tratamiento oportuno mejoran considerablemente la calidad de vida de estas pacientes al reducir la severidad de los síntomas, disminuir la incidencia de complicaciones y mejorar el pronóstico obstétrico.
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Sunyecz A, Snider M, Minniear E, Duenas-Garcia O, Payton A, Shapiro R. Herlyn-Werner-Wunderlich Syndrome: A Case Report on a Congenital Uterine Anomaly With Literature Review. JOURNAL OF DIAGNOSTIC MEDICAL SONOGRAPHY 2021. [DOI: 10.1177/87564793211012633] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022]
Abstract
During fetal development, abnormalities during Mullerian duct formation can lead to varied types of uterine, cervical, or vaginal anomalies. Herlyn-Werner-Wunderlich Syndrome (HWWS) is a rare, congenital Mullerian duct anomaly characterized by the triad of uterine didelphys, obstructed hemi-vagina, and ipsilateral renal agenesis. If the diagnosis is delayed or missed, permanent sequalae such as chronic pain, infection, and infertility can result. This is a case of a 16-year-old woman with heavy vaginal bleeding and menstrual cramping in which sonography was used in the diagnosis of HWWS. Sonography plays a vital role in this diagnosis, as it is the most common initial imaging examination. For this reason, it is important that the characteristics of uterine anomalies, like HWWS, be recognized with sonography. It is important that this diagnosis is made early and treatment can be initiated to prevent irreversible complications.
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Affiliation(s)
- Alec Sunyecz
- Department of Obstetrics and Gynecology, School of Medicine, West Virginia University, Morgantown, WV, USA
| | - Megan Snider
- Department of Obstetrics and Gynecology, School of Medicine, West Virginia University, Morgantown, WV, USA
| | - Elizabeth Minniear
- Department of Obstetrics and Gynecology, School of Medicine, West Virginia University, Morgantown, WV, USA
| | - Omar Duenas-Garcia
- Department of Obstetrics and Gynecology, School of Medicine, West Virginia University, Morgantown, WV, USA
| | - Amber Payton
- Department of Obstetrics and Gynecology, School of Medicine, West Virginia University, Morgantown, WV, USA
| | - Robert Shapiro
- Department of Obstetrics and Gynecology, School of Medicine, West Virginia University, Morgantown, WV, USA
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Yang M, Wen S, Liu X, He D, Wei G, Wu S, Huang Y, Ni Y, Shi Y, Hua Y. Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA): Early diagnosis, treatment and outcomes. Eur J Obstet Gynecol Reprod Biol 2021; 261:12-16. [PMID: 33873082 DOI: 10.1016/j.ejogrb.2021.03.018] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/12/2020] [Revised: 01/25/2021] [Accepted: 03/11/2021] [Indexed: 10/21/2022]
Abstract
OBJECTIVE To describe and summarize the early diagnosis, treatment and outcomes of obstructed hemivagina and ipsilateral renal anomaly (OHVIRA). METHODS Data from patients with OHVIRA treated at the study centre over the last decade were analysed retrospectively, including demographic characteristics, symptoms, diagnosis, treatment and outcomes. Relevant published literature was also reviewed in this study. RESULTS In total, 11 cases were diagnosed with OHVIRA over the study period, ranging in age from 3 to 14 years (median 11 years). Four cases were asymptomatic, four had abdominal pain and three had urinary symptoms. All 11 cases underwent vaginoplasty. Ureteral bladder re-implantation (n = 1), dysplastic nephrectomy (n = 1), separation of labia minora adhesions (n = 1), perineoplasty (n = 1) and urethroplasty (n = 1) were performed. Cases were followed up for 2 months to 8 years (median 5 years) with good follow-up outcomes. CONCLUSIONS Based on the cases in this study and the relevant literature, ultrasound appears to be an effective method for the diagnosis of OHVIRA. Experienced ultrasonographers are able to achieve a high corrected diagnosis rate. The differential diagnosis of OHVIRA should be considered by paediatric surgeons, urologists, emergency physicians and gynaecologists when they identify patients with didelphys uterus or renal agenesis. Early surgery can reduce the risk of potential genital tract infection and gynaecological complications.
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Affiliation(s)
- Meng Yang
- Department of Urology, Children's Medicine Affiliated to Chongqing Medical University, Chongqing, PR China; Paediatrics Research Institute, Children's Hospital of Chongqing Medical University, Ministry of Education Key Laboratory of Child Development and Disorders, National Clinical Research Centre for Child Health and Disorders, China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Children's Hospital of Chongqing Medical University, Chongqing, PR China; Chongqing Key Laboratory of Paediatrics, Chongqing Key Laboratory of Children Urogenital Development and Tissue Engineering, PR China
| | - Sheng Wen
- Department of Urology, Children's Medicine Affiliated to Chongqing Medical University, Chongqing, PR China; Paediatrics Research Institute, Children's Hospital of Chongqing Medical University, Ministry of Education Key Laboratory of Child Development and Disorders, National Clinical Research Centre for Child Health and Disorders, China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Children's Hospital of Chongqing Medical University, Chongqing, PR China; Chongqing Key Laboratory of Paediatrics, Chongqing Key Laboratory of Children Urogenital Development and Tissue Engineering, PR China
| | - Xing Liu
- Department of Urology, Children's Medicine Affiliated to Chongqing Medical University, Chongqing, PR China; Paediatrics Research Institute, Children's Hospital of Chongqing Medical University, Ministry of Education Key Laboratory of Child Development and Disorders, National Clinical Research Centre for Child Health and Disorders, China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Children's Hospital of Chongqing Medical University, Chongqing, PR China; Chongqing Key Laboratory of Paediatrics, Chongqing Key Laboratory of Children Urogenital Development and Tissue Engineering, PR China
| | - Dawei He
- Department of Urology, Children's Medicine Affiliated to Chongqing Medical University, Chongqing, PR China; Paediatrics Research Institute, Children's Hospital of Chongqing Medical University, Ministry of Education Key Laboratory of Child Development and Disorders, National Clinical Research Centre for Child Health and Disorders, China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Children's Hospital of Chongqing Medical University, Chongqing, PR China; Chongqing Key Laboratory of Paediatrics, Chongqing Key Laboratory of Children Urogenital Development and Tissue Engineering, PR China
| | - Guanghui Wei
- Department of Urology, Children's Medicine Affiliated to Chongqing Medical University, Chongqing, PR China; Paediatrics Research Institute, Children's Hospital of Chongqing Medical University, Ministry of Education Key Laboratory of Child Development and Disorders, National Clinical Research Centre for Child Health and Disorders, China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Children's Hospital of Chongqing Medical University, Chongqing, PR China; Chongqing Key Laboratory of Paediatrics, Chongqing Key Laboratory of Children Urogenital Development and Tissue Engineering, PR China
| | - Shengde Wu
- Department of Urology, Children's Medicine Affiliated to Chongqing Medical University, Chongqing, PR China; Paediatrics Research Institute, Children's Hospital of Chongqing Medical University, Ministry of Education Key Laboratory of Child Development and Disorders, National Clinical Research Centre for Child Health and Disorders, China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Children's Hospital of Chongqing Medical University, Chongqing, PR China; Chongqing Key Laboratory of Paediatrics, Chongqing Key Laboratory of Children Urogenital Development and Tissue Engineering, PR China
| | - Yitian Huang
- Department of Urology, Children's Medicine Affiliated to Chongqing Medical University, Chongqing, PR China
| | - Yuansong Ni
- Department of Urology, Children's Medicine Affiliated to Chongqing Medical University, Chongqing, PR China
| | - Yan Shi
- Department of Urology, Children's Medicine Affiliated to Chongqing Medical University, Chongqing, PR China
| | - Yi Hua
- Department of Urology, Children's Medicine Affiliated to Chongqing Medical University, Chongqing, PR China; Paediatrics Research Institute, Children's Hospital of Chongqing Medical University, Ministry of Education Key Laboratory of Child Development and Disorders, National Clinical Research Centre for Child Health and Disorders, China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Children's Hospital of Chongqing Medical University, Chongqing, PR China; Chongqing Key Laboratory of Paediatrics, Chongqing Key Laboratory of Children Urogenital Development and Tissue Engineering, PR China.
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Crowley CM, Botros K, Hegazy IF, O'Donnell E. Uterine didelphys: diagnosis, management and pregnancy outcome. BMJ Case Rep 2021; 14:14/3/e242233. [PMID: 33782076 PMCID: PMC8009243 DOI: 10.1136/bcr-2021-242233] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
Abstract
This report addresses and discusses two cases of uterine didelphys in pregnancy. The first case describes the diagnosis, management and subsequent pregnancies in a 28-year-old woman, para 2, with known didelphys uterus, left-obstructed hemi-vagina and ipsilateral renal agenesis. This uterine anomaly was diagnosed at 13 years of age, after pelvic imaging identified a haematocolpos and two uteri. To drain this haematocolpos, a hymenectomy was performed. In the second case, an incidental finding of uterine didelphys and vaginal septum in a 28-year-old primigravida is described. Both patients delivered healthy male infants at term via emergency and planned lower segment caesarean sections, indicating women with major uterine anomalies can have successful obstetric outcomes.
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Affiliation(s)
| | - Karim Botros
- Obstetrics and Gynaecology, University Hospital Waterford, Waterford, Ireland
| | | | - Edward O'Donnell
- Obstetrics and Gynaecology, University Hospital Waterford, Waterford, Ireland
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34
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Valera PGV, Domingo MVC. Herlyn Werner Wunderlich Syndrome with Vagino-umbillical Fistula: A Most Unique Presentation. FERTILITY & REPRODUCTION 2021. [DOI: 10.1142/s2661318221300014] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/18/2022] Open
Abstract
The Herlyn–Werner–Wunderlich (HWW) syndrome is an uncommon Mullerian duct anomaly composed of a triad of obstructed hemivagina, renal agenesis, uterine didelphys with a prevalence of <1/1,000,000 females. Aside from its usual presentation of cyclic hypogastric pain and abdominal mass, there has been no other reported case in literature of the syndrome presenting with a vagino-umbilical fistula. Presented here is a unique case of a 35-year-old woman presenting with purulent, foul-smelling discharge extruding from a fistula on her umbilicus and her vagina associated with a tender hypogastric mass. Three-dimensional transvaginal ultrasonography aided the diagnosis of HWW syndrome with a concomitant vagino-umbilical fistula. Excision of the vaginal septum together with antibiotic coverage resolved her symptoms. The syndrome’s embryopathogenesis, classification, diagnostics, definitive surgical management and other issues during follow up are discussed.
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Affiliation(s)
- Pamela Grace Vidal Valera
- Division of Reproductive Endocrinology and Infertility, University of the Philippines Manila, Philippine General Hospital, Ermita, Manila, Metro Manila, Philippines
| | - Madonna Victoria Calderon Domingo
- Division of Reproductive Endocrinology and Infertility, University of the Philippines Manila, Philippine General Hospital, Ermita, Manila, Metro Manila, Philippines
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35
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Yi S, Jiang J. Clinical characteristics and management of patients with complete septate uterus, double cervix, obstructed hemivagina, and ipsilateral renal agenesis. J Obstet Gynaecol Res 2021; 47:1497-1501. [PMID: 33410178 DOI: 10.1111/jog.14662] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/15/2020] [Revised: 12/18/2020] [Accepted: 12/26/2020] [Indexed: 11/28/2022]
Abstract
AIM To analyze the clinical characteristics and management of patients with complete septate uterus, double cervix, obstructed hemivagina, and ipsilateral renal agenesis. METHODS This retrospective study reviewed the medical records of 17 patients with complete septate uterus, double cervix, obstructed hemivagina, and ipsilateral renal agenesis admitted to the Third Xiangya Hospital of Central South University between June 2007 and December 2019. RESULTS The median age at surgery was 23 years. The most common presenting complaint was infertility. Seven (41.2%) patients were misdiagnosed previously. The obstruction was complete in five patients. All 17 patients underwent vaginoplasty, in which seven adolescent girls underwent vaginoscopic vaginal septum resections. Eight patients underwent hysteroscopic resections of the uterine septum for infertility or spontaneous miscarriage. Laparoscopy was performed in seven patients for specific indications, and only one patient was found to have pelvic endometriosis during the laparoscopy. During the follow-up, seven patients wished to conceive, and there were five living infants (four cesarean deliveries at term and one preterm vaginal delivery). CONCLUSION Complete septate uterus with double cervix, obstructed hemivagina, and ipsilateral renal agenesis is an variant of obstructed hemivagina and ipsilateral renal agenesis syndrome. Consequently, healthcare providers should be aware of this potential variant.
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Affiliation(s)
- Shuijing Yi
- Department of Obstetrics and Gynecology, The Third Xiangya Hospital of Central South University, Changsha, China
| | - Jianfa Jiang
- Department of Obstetrics and Gynecology, The Third Xiangya Hospital of Central South University, Changsha, China
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36
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Affiliation(s)
- Swati Jha
- Sheffield Teaching Hospitals, Sheffield, UK
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37
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Jeffery H, Jha S. Acute urinary retention during menstruation: Answers. Pediatr Nephrol 2021; 36:321-322. [PMID: 32556542 PMCID: PMC7815573 DOI: 10.1007/s00467-020-04646-9] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/25/2020] [Accepted: 06/02/2020] [Indexed: 11/28/2022]
Affiliation(s)
- Hannah Jeffery
- Department of Paediatric Surgery, Sheffield Children's Hospitals, Sheffield, UK
| | - Swati Jha
- Department of Urogynaecology, Sheffield Teaching Hospitals, Level 4 Jessop Wing, Tree Root Walk, Sheffield, S10 2SF, UK.
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38
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Basnet T, Pradhan T, Yadav P, Sah MK, Yadav J, Rai Y, Thapa R. Obstructed Hemivagina and Ipsilateral Renal Anomaly Syndrome Rare Obstructive Uterovaginal Anomaly: A Case Report. JNMA J Nepal Med Assoc 2020; 58:805-808. [PMID: 34504376 PMCID: PMC7654496 DOI: 10.31729/jnma.5043] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/24/2020] [Indexed: 11/23/2022] Open
Abstract
Obstructed Hemivagina and Ipsilateral Renal Anomaly syndrome is a rare obstructive uterovaginal anomaly involving both mesonephric and paramesonephric ducts. It usually presents after menarche with non-specific symptoms like pelvic pain, dysmenorrhea, or paravaginal mass and examination findings of paravaginal or pelvic mass. Because of non-specific symptoms and signs, the diagnosis is usually overlooked, which leads to complications like endometriosis, tubo-ovarian abscess compromising patient's fertility, and quality of life. Therefore, in presence of these nonspecific clinical features along with imaging findings of uterine didelphys and unilateral renal agenesis, this syndrome should be considered in the diagnosis. We present a case of a 17-year-old lady with Obstructed Hemivagina and Ipsilateral Renal Anomaly syndrome, diagnosed by finding of paravaginal mass on examination and uterine didelphys with ipsilateral renal agenesis in USG and managed successfully by resection of vaginal septum.
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Affiliation(s)
- Tulasa Basnet
- Department of Obstetrics and Gynaecology, B. P. Koirala Institute of Health Sciences, Dharan, Nepal
| | - Tarun Pradhan
- Department of Obstetrics and Gynaecology, B. P. Koirala Institute of Health Sciences, Dharan, Nepal
| | - Punita Yadav
- Department of Obstetrics and Gynaecology, B. P. Koirala Institute of Health Sciences, Dharan, Nepal
| | - Manoj Kumar Sah
- Department of Obstetrics and Gynaecology, B. P. Koirala Institute of Health Sciences, Dharan, Nepal
| | - Jyotsna Yadav
- Department of Obstetrics and Gynaecology, B. P. Koirala Institute of Health Sciences, Dharan, Nepal
| | - Yashaswi Rai
- Department of Obstetrics and Gynaecology, B. P. Koirala Institute of Health Sciences, Dharan, Nepal
| | - Rashmi Thapa
- Department of Obstetrics and Gynaecology, B. P. Koirala Institute of Health Sciences, Dharan, Nepal
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39
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Oka E, Chiyoda T, Iwata T, Yamagami W, Aoki D. Uterine cervical cancer associated with obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome: A case report and review of the literature. Gynecol Oncol Rep 2020; 34:100645. [PMID: 32995456 PMCID: PMC7502329 DOI: 10.1016/j.gore.2020.100645] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/18/2020] [Accepted: 09/08/2020] [Indexed: 10/27/2022] Open
Abstract
•Cervical cancer occurring in patients with OHVIRA syndrome is very rare.•This case reports on HPV-negative adenocarcinoma located in the nonvisible cervix.•Colposcopy and imaging are essential for cervical cancer with uterine malformation.•In OHVIRA syndrome, cervical malignancies may be overlooked.
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Affiliation(s)
- Emiko Oka
- Department of Obstetrics and Gynecology, Keio University School of Medicine, Tokyo, Japan
| | - Tatsuyuki Chiyoda
- Department of Obstetrics and Gynecology, Keio University School of Medicine, Tokyo, Japan
| | - Takashi Iwata
- Department of Obstetrics and Gynecology, Keio University School of Medicine, Tokyo, Japan
| | - Wataru Yamagami
- Department of Obstetrics and Gynecology, Keio University School of Medicine, Tokyo, Japan
| | - Daisuke Aoki
- Department of Obstetrics and Gynecology, Keio University School of Medicine, Tokyo, Japan
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40
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Zhang H, Ning G, Fu C, Bao L, Guo Y. Herlyn–Werner–Wunderlich syndrome: diverse presentations and diagnosis on MRI. Clin Radiol 2020; 75:480.e17-480.e25. [DOI: 10.1016/j.crad.2020.01.016] [Citation(s) in RCA: 18] [Impact Index Per Article: 3.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/22/2019] [Accepted: 01/29/2020] [Indexed: 11/24/2022]
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41
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Zhang J, Zhang M, Zhang Y, Liu H, Yuan P, Peng X, Cao Z, Wang L. Proposal of the 3O (Obstruction, Ureteric Orifice, and Outcome) Subclassification System Associated with Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA). J Pediatr Adolesc Gynecol 2020; 33:307-313. [PMID: 31931122 DOI: 10.1016/j.jpag.2020.01.001] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/16/2019] [Revised: 12/31/2019] [Accepted: 01/02/2020] [Indexed: 11/27/2022]
Abstract
STUDY OBJECTIVE To propose a "3O" (obstruction, ureteric orifice, and outcome) subclassification system associated with obstructed hemivagina and ipsilateral renal anomaly (OHVIRA). DESIGN Retrospective case series. SETTING Xiangya Hospital, Central South University, Changsha, Hunan, China. PARTICIPANTS A total of 26 women with obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) over a 9-year period. INTERVENTIONS Not applicable. MAIN OUTCOME MEASURES In all cases, the obstruction, ureteric orifice, outcome and surgical strategy were reviewed. RESULTS In our study, the "obstruction" category included 14 cases of blind hemivagina, 8 cases of buttonhole septum, 3 cases of cervical fistula, and 1 case of cervical atresia. A total of 25 patients with vaginal obstruction underwent resection of the vaginal septum. The patient with cervical atresia underwent a failed cervicoplasty, followed by hemi-hysterectomy. The "ureteric orifice" category included 24 cases of absent ureter with no orifice, as well as 2 cases of ureteric orifice emptying into the obstructed hemivagina. The 2 patients were treated with laparoscopic extirpation of the ectopic ureter and renal moiety. Regarding the "outcome" category, 5 patients with severe recurrent hematometra, hematosalpinx, and ovarian endometrioma underwent hemi-hysterectomy, salpingectomy, and cystectomy of the ovarian endometrioma. Both patients (1 with a septate uterus and 1 with a bicornuate uterus) who experienced recurrent abortion accepted uterine correction. CONCLUSION We provide new insights into the anatomical variants of this rare syndrome with the relevant surgical implications. Magnetic resonance imaging is the most useful tool in 3O diagnosis.
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Affiliation(s)
- Junjie Zhang
- Department of Urology, Xiangya Hospital, Central South University, Changsha, Hunan, China
| | - Mengda Zhang
- Department of Urology, The Third Xiangya Hospital, Central South University, Changsha, Hunan, China
| | - Yu Zhang
- Department of Obstetrics and Gynecology, Xiangya Hospital, Central South University, Changsha, Hunan, China
| | - Huining Liu
- Department of Obstetrics and Gynecology, Xiangya Hospital, Central South University, Changsha, Hunan, China
| | - Peng Yuan
- Department of Urology, Xiangya Hospital, Central South University, Changsha, Hunan, China
| | - Xiaoyan Peng
- Department of Respiratory Medicine, Xiangya Hospital, Central South University, Changsha, Hunan, China
| | - Zhenzhen Cao
- Department of Gynecologic Oncology, Hunan Cancer Hospital and The Affiliated Cancer Hospital of Xiangya School of Medical, Central South University, Changsha, Hunan, China
| | - Long Wang
- Department of Urology, The Third Xiangya Hospital, Central South University, Changsha, Hunan, China.
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42
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Liu Y, Mapow B. Coexistence of urogenital malformations in a female fetus with de novo 15q24 microdeletion and a literature review. Mol Genet Genomic Med 2020; 8:e1265. [PMID: 32400031 PMCID: PMC7336734 DOI: 10.1002/mgg3.1265] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/24/2019] [Revised: 03/24/2020] [Accepted: 03/25/2020] [Indexed: 12/13/2022] Open
Abstract
Background 15q24 microdeletion is a relatively new syndrome caused by nonallelic homologous recombination (NAHR) between low‐copy repeats (LCRs) in the 15q24 chromosome region. This syndrome is characterized by a spectrum of clinical symptoms including global developmental delay, intellectual disability, facial dysmorphisms, and congenital malformations of the extremities, eye, gastrointestinal tract, genitourinary system, and genitalia. Method Molecular cytogenetic analysis was performed using whole genome single‐nucleotide polymorphism (SNP) microarray analysis. Autopsy examination including gross and microscopic examination were performed. In addition, a thorough review of the literature on 15q24 microdeletion was completed and summarized in table format. Result Molecular cytogenetic analysis revealed a 3.88 MB interstitial deletion within 15q24.1 to 15q24.3 (74,353,735–78,228,485 bp) in our case. Autopsy examination showed congenital malformations within the genitourinary system and genitalia, including left kidney agenesis and uterus didelphys. After thorough literature review, we found a series of midline defects associated with 15q24 microdeletion syndrome. Conclusion We report the first case of coexistence of urogenital abnormalities, including left kidney agenesis and uterus didelphys, with 15q24 microdeletion syndrome, which is also associated with midline defects secondary to abnormal development. Since 15q24 microdeletion syndrome is a relatively new entity, fully characterizing its variation and severity requires additional examination of the genetics, molecular profile and structural and functional abnormalities in affected patients. Due to the limited data in the literature, statistical analysis of abnormalities in each organ system is not possible. However, we can predict that novel genetic pathways involving cell migration, adhesion, apoptosis, and embryo development might be discovered with the advanced study of 15q24 microdeletion syndrome.
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Affiliation(s)
- Yaobin Liu
- Department of Pathology, Pennsylvania Hospital, Philadelphia, Pennsylvania, USA
| | - Beth Mapow
- Department of Pathology and Laboratory Medicine, Jefferson Health New jersey, Cherry Hill, New Jersey, USA
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43
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Wu CQ, Childress KJ, Traore EJ, Smith EA. A Review of Mullerian Anomalies and Their Urologic Associations. Urology 2020; 151:98-106. [PMID: 32387292 DOI: 10.1016/j.urology.2020.04.088] [Citation(s) in RCA: 10] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/06/2020] [Revised: 04/17/2020] [Accepted: 04/22/2020] [Indexed: 10/24/2022]
Abstract
Structural anomalies of the female reproductive tract, known as Mullerian anomalies, can occur in isolation or in association with anomalies of other organ systems. Due to shared embryology, the most common association in up to 40% of patients is with renal, ureteral, and bladder anomalies. Affected girls can have a wide range of genitourinary symptoms with urologists playing an integral role in their diagnosis and treatment. To facilitate the recognition and management of these conditions, we provide a review of Mullerian anomalies including the embryology, classifications, syndromes, evaluation, and treatments with attention to their urologic applicability.
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Affiliation(s)
- Charlotte Q Wu
- Division of Pediatric Urology, Children's Healthcare of Atlanta; Emory University School of Medicine, Atlanta, GA.
| | - Krista J Childress
- Division of Gynecologic Specialties, Department of Gynecology and Obstetrics, Emory University School of Medicine; Divisions of Pediatric Surgery and Pediatrics, Children's Healthcare of Atlanta, Atlanta, GA
| | - Elizabeth J Traore
- Division of Pediatric Urology, Children's Healthcare of Atlanta; Emory University School of Medicine, Atlanta, GA
| | - Edwin A Smith
- Division of Pediatric Urology, Children's Healthcare of Atlanta; Emory University School of Medicine, Atlanta, GA
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44
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Wiener JS, Huck N, Blais AS, Rickard M, Lorenzo A, Di Carlo HNM, Mueller MG, Stein R. Challenges in pediatric urologic practice: a lifelong view. World J Urol 2020; 39:981-991. [PMID: 32328778 DOI: 10.1007/s00345-020-03203-1] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/23/2019] [Accepted: 04/08/2020] [Indexed: 12/11/2022] Open
Abstract
The role of the pediatric urologic surgeon does not end with initial reconstructive surgery. Many of the congenital anomalies encountered require multiple staged operations while others may not involve further surgery but require a life-long follow-up and often revisions. Management of most of these disorders must extend into and through adolescence before transitioning these patients to adult colleagues. The primary goal of management of all congenital uropathies is protection and/or reversal of renal insult. For posterior urethral valves, in particular, avoidance of end-stage renal failure may not be possible in severe cases due to the congenital nephropathy but usually can be prolonged. Likewise, prevention or minimization of urinary tract infections is important for overall health and eventual renal function. Attainment of urinary continence is an important goal for most with a proven positive impact on quality of life; however, measures to achieve that goal can require significant efforts for those with neuropathic bladder dysfunction, obstructive uropathies, and bladder exstrophy. A particular challenge is maximizing future self-esteem, sexual function, and reproductive potential for those with genital anomalies such as hypospadias, the bladder exstrophy epispadias complex, prune belly syndrome, and Mullerian anomalies. Few endeavors are rewarding as working with children and their families throughout childhood and adolescence to help them attain these goals, and modern advances have enhanced our ability to get them to adulthood in better physical and mental health than ever before.
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Affiliation(s)
- John S Wiener
- Division of Urologic Surgery, Duke University Medical Center, Durham, NC, USA.
| | - Nina Huck
- Department of Pediatric, Adolescent and Reconstructive Urology, Medical Faculty Mannheim, University Medical Center Mannheim, Heidelberg University, Mannheim, Germany
| | - Anne-Sophie Blais
- Division of Pediatric Urology, Hospital for Sick Children, Toronto, ON, Canada
| | - Mandy Rickard
- Division of Pediatric Urology, Hospital for Sick Children, Toronto, ON, Canada
| | - Armando Lorenzo
- Division of Pediatric Urology, Hospital for Sick Children, Toronto, ON, Canada.,Department of Surgery, University of Toronto, Toronto, ON, Canada
| | - Heather N McCaffrey Di Carlo
- The James Buchanan Brady Urologic Institute, The Johns Hopkins University School of Medicine, Baltimore, MD, USA
| | - Margaret G Mueller
- Division of Female Pelvic Medicine and Reconstructive Surgery, Departments of Obstetrics & Gynecology and Urology, Feinberg School of Medicine, Northwestern University, Chicago, IL, USA
| | - Raimund Stein
- Department of Pediatric, Adolescent and Reconstructive Urology, Medical Faculty Mannheim, University Medical Center Mannheim, Heidelberg University, Mannheim, Germany
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45
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Miyazaki Y, Orisaka M, Nishino C, Onuma T, Kurokawa T, Yoshida Y. Herlyn-Werner-Wunderlich syndrome with cervical atresia complicated by ovarian endometrioma: A case report. J Obstet Gynaecol Res 2019; 46:347-351. [PMID: 31814219 DOI: 10.1111/jog.14175] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/20/2019] [Accepted: 11/19/2019] [Indexed: 11/30/2022]
Abstract
Herlyn-Werner-Wunderlich syndrome (HWWs) is a rare congenital malformation that involves uterus dideslphys, obstructed hemivagina and ipsilateral renal agenesis. The obstructed vagina affects menstrual flow and causes related clinical features after menarche. Pelvic endometriosis is one of the common complications of HWWs. Resection of the vaginal septum can release the obstruction and result in good outcomes. However, in the case of cervical atresia, a rare variant of HWWs, ipsilateral hysterectomy is recommended because it is difficult to canalize cervical atresia surgically. Here we present a case of HWWs with cervical atresia complicated with pelvic endometriosis. She was treated with laparoscopic ovarian cystectomy followed by hormonal therapy. Six months after surgery, there is no evidence of recurrence of endometrioma.
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Affiliation(s)
- Yumiko Miyazaki
- Department of Obstetrics and Gynecology, University of Fukui Faculty of Medical Sciences, Fukui, Japan
| | - Makoto Orisaka
- Department of Obstetrics and Gynecology, University of Fukui Faculty of Medical Sciences, Fukui, Japan
| | - Chihiro Nishino
- Department of Obstetrics and Gynecology, University of Fukui Faculty of Medical Sciences, Fukui, Japan
| | - Toshimichi Onuma
- Department of Obstetrics and Gynecology, University of Fukui Faculty of Medical Sciences, Fukui, Japan
| | - Tetsuji Kurokawa
- Department of Obstetrics and Gynecology, University of Fukui Faculty of Medical Sciences, Fukui, Japan
| | - Yoshio Yoshida
- Department of Obstetrics and Gynecology, University of Fukui Faculty of Medical Sciences, Fukui, Japan
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Gungor Ugurlucan F, Dural O, Yasa C, Kirpinar G, Akhan SE. Diagnosis, management, and outcome of obstructed hemivagina and ipsilateral renal agenesis (OHVIRA syndrome): Is there a correlation between MRI findings and outcome? Clin Imaging 2019; 59:172-178. [PMID: 31821975 DOI: 10.1016/j.clinimag.2019.11.013] [Citation(s) in RCA: 21] [Impact Index Per Article: 3.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/02/2019] [Revised: 10/23/2019] [Accepted: 11/04/2019] [Indexed: 10/25/2022]
Abstract
AIM To review the experience of a single tertiary center with obstructed hemivagina and ipsilateral renal agenesis (OHVIRA syndrome) and evaluate if MRI findings correlate with outcome. METHODS Patients (n = 32) diagnosed with OHVIRA syndrome between 2001 and 2019 were analyzed. Presenting symptoms, age of menarche, age at operation, MRI findings, management, and outcome were reviewed. In sagittal MRI planes, distance from hematocolpos to perineum was measured. Measurements were compared among patients who underwent single-stage vaginoplasty and hemihysterectomy. RESULTS Mean age at diagnosis and mean age of menarche was 16.8 ± 6.4 (10-33) and 12.8 ± 1.0 (10-15). Main presenting complaints were abdominal pain/dysmenorrhea. Eighteen anomalies (56.3%) were on right side. Twenty-eight had ipsilateral renal agenesis, 3 patients had normal renal anatomy and one had unilateral multicystic dysplastic kidney. In MRI, 21 patients had hematocolpos, 11 patients had both hematocolpos and hematometra. 28 patients underwent single-stage vaginoplasty and vaginal septum resection. One had hemihysterectomy due to sepsis at presentation. Three patients had hemihysterectomy due to proximal vaginal septum and impossibility of vaginoplasty. During follow-up, 7 cases (21.9%) had married and 5 of these (71.4%) were pregnant or had delivered. Five patients had reoperation during follow-up. MRI images of 19 patients indicated mean distances from hematocolpos to perineum whom underwent vaginoplasty or hemihysterectomy were 33.9 ± 18.1 mm (10-79 mm) and 87.3 ± 11.0 mm (80-100), respectively (p = .009). CONCLUSIONS Gold standard treatment of OHVIRA syndrome is single-stage vaginoplasty. Distance from hematocolpos to perineum in MRI may correlate with surgical outcome. Hemihysterectomy may be an alternative for extreme proximal vaginal septum or infectious complications.
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Affiliation(s)
- Funda Gungor Ugurlucan
- Istanbul University Istanbul Faculty of Medicine, Department of Obstetrics and Gynecology, Istanbul, Turkey.
| | - Ozlem Dural
- Istanbul University Istanbul Faculty of Medicine, Department of Obstetrics and Gynecology, Istanbul, Turkey
| | - Cenk Yasa
- Istanbul University Istanbul Faculty of Medicine, Department of Obstetrics and Gynecology, Istanbul, Turkey
| | - Gamze Kirpinar
- Istanbul University Istanbul Faculty of Medicine, Department of Obstetrics and Gynecology, Istanbul, Turkey
| | - Suleyman Engin Akhan
- Istanbul University Istanbul Faculty of Medicine, Department of Obstetrics and Gynecology, Istanbul, Turkey
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Dabi Y, Dray G, Allanche M, Skalli D, Paniel BJ, Haddad B, Touboul C. Fertility and pregnancy outcomes in patients with bicorporeal uterus and blind hemivagina: 20 years of experience in a tertiary referral gynaecological department. J Gynecol Obstet Hum Reprod 2019; 49:101651. [PMID: 31760181 DOI: 10.1016/j.jogoh.2019.101651] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/19/2019] [Revised: 09/28/2019] [Accepted: 10/18/2019] [Indexed: 11/15/2022]
Abstract
BACKGROUND To evaluate fertility and pregnancy outcomes in patients with uterus bicorporeal and blind hemivagina. Our second objective was to investigate factors predicting fertility and pregnancy outcomes in those patients. MATERIAL AND METHODS We conducted a retrospective analysis in a tertiary referring gynaecological department, in France. We included all patients with uterus bicorporeal and blind hemivagina who underwent at least one surgery in our centre. Initial characteristics of the patients included were extracted from their medical charts and patients were contacted to assess their fertility and pregnancies outcomes upon their consent to participate to the study. RESULTS Between 1989 and 2010, 79 patients fulfilled inclusion criteria and were selected for analysis. Mean follow up of those patients was of 16.15 (QI 10-21) years. Forty-six patients (58.2%) returned the survey and among them, 21 (45.7%) were fertile, 8 (17.4%) were infertile and 17 (37%) never attempted to get pregnant following initial management. Forty-nine pregnancies were included to assess pregnancies outcomes: 11 (22.5%) early miscarriages, 1 (2.0%) extra-uterine pregnancy, 2 (4.1%) second semester miscarriages and 35 (71.4%) leaded to living birth. Nineteen (54.3%) deliveries occurred by caesarean section and 14 (40.0%) by vaginal delivery. Fifteen pregnancies (42.9%) were complicated. In univariate analysis, adhesiolysis performed at the time of initial surgery was the only factor associated with infertility (p=0.004). CONCLUSIONS Fertility seems to be perfectly conserved in those patients and they do not have increased rate of adverse pregnancies outcomes.
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Affiliation(s)
- Y Dabi
- Faculté de médecine de Créteil UPEC - Paris XII, Service de Gynécologie-Obstétrique et Médecine de la Reproduction, Centre Hospitalier Intercommunal de Créteil, 40 Avenue de Verdun, 94000 Créteil, France
| | - G Dray
- Faculté de médecine de Créteil UPEC - Paris XII, Service de Gynécologie-Obstétrique et Médecine de la Reproduction, Centre Hospitalier Intercommunal de Créteil, 40 Avenue de Verdun, 94000 Créteil, France
| | - M Allanche
- Faculté de médecine de Créteil UPEC - Paris XII, Service de Gynécologie-Obstétrique et Médecine de la Reproduction, Centre Hospitalier Intercommunal de Créteil, 40 Avenue de Verdun, 94000 Créteil, France
| | - D Skalli
- Faculté de médecine de Créteil UPEC - Paris XII, Service de Gynécologie-Obstétrique et Médecine de la Reproduction, Centre Hospitalier Intercommunal de Créteil, 40 Avenue de Verdun, 94000 Créteil, France
| | - B J Paniel
- Faculté de médecine de Créteil UPEC - Paris XII, Service de Gynécologie-Obstétrique et Médecine de la Reproduction, Centre Hospitalier Intercommunal de Créteil, 40 Avenue de Verdun, 94000 Créteil, France
| | - B Haddad
- Faculté de médecine de Créteil UPEC - Paris XII, Service de Gynécologie-Obstétrique et Médecine de la Reproduction, Centre Hospitalier Intercommunal de Créteil, 40 Avenue de Verdun, 94000 Créteil, France
| | - C Touboul
- Faculté de médecine de Créteil UPEC - Paris XII, Service de Gynécologie-Obstétrique et Médecine de la Reproduction, Centre Hospitalier Intercommunal de Créteil, 40 Avenue de Verdun, 94000 Créteil, France; UMR INSERM U965: Angiogenèse et Recherche translationnelle, Hôpital Lariboisière, 49 bd de la chapelle 75010 Paris, France.
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Girardi Fachin C, Aleixes Sampaio Rocha JL, Atuati Maltoni A, das Chagas Lima RL, Arias Zendim V, Agulham MA, Tsouristakis A, Dos Santos Dias AIB. Herlyn-Werner-Wunderlich syndrome: Diagnosis and treatment of an atypical case and review of literature. Int J Surg Case Rep 2019; 63:129-134. [PMID: 31586891 PMCID: PMC6796699 DOI: 10.1016/j.ijscr.2019.08.035] [Citation(s) in RCA: 21] [Impact Index Per Article: 3.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/17/2019] [Revised: 08/15/2019] [Accepted: 08/26/2019] [Indexed: 11/29/2022] Open
Abstract
In this report we present a rare case of HWWS diagnosed in a 12-year-old female presenting with disabling abdominal pain after the onset of menarche. We reviewed patient’s clinical history, utility of diagnostic modalities, usual treatments, and recent literature. The purpose of this case report is to offer a better understanding of the pathophysiology of HWWS. When a young female presents with common, nonspecific symptoms, HWWS should be on differential diagnoses list. Greater awareness of HWWS will lead to earlier detection and, consequently, reduced complications caused by delayed diagnosis. Introduction Herlyn-Werner-Wunderlich syndrome (HWWS), also known as OHVIRA syndrome, is a rare congenital abnormality of Müllerian duct development characterized by uterus didelphys, unilateral blind hemivagina, and ipsilateral renal agenesis. Presentation of case In this report, the authors present the case of a 12-year-old patient presenting with progressive and disabling abdominal pain after the onset of menarche diagnosed with HWWS, describe the various diagnostic modalities and treatment options available, along with a current review of the literature. Discussion With normal external genitalia, HWWS is usually asymptomatic until menarche when patients present with worsening abdominal pain during menses and a palpable pelvic or abdominal mass. Untreated, HWWS may lead to a number of complications including endometriosis, infertility, and spontaneous abortion. Conclusion Greater awareness of HWWS will lead to earlier detection and is the key to alleviating patient suffering and avoiding potentially severe complications.
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Affiliation(s)
- Camila Girardi Fachin
- Pediatric Surgery, Hospital de Clínicas, Federal University of Paraná, Curitiba, PR, Brazil.
| | | | | | | | | | - Miguel Angelo Agulham
- Pediatric Surgery, Hospital de Clínicas, Federal University of Paraná, Curitiba, PR, Brazil
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Reproductive surgery for müllerian anomalies: a review of progress in the last decade. Fertil Steril 2019; 112:408-416. [DOI: 10.1016/j.fertnstert.2019.07.005] [Citation(s) in RCA: 17] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/02/2019] [Accepted: 07/03/2019] [Indexed: 11/21/2022]
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Egbe TO, Kobenge FM, Wankie EM. Virginity-sparing management of hematocolpos with imperforate hymen: case report and literature review. SAGE Open Med Case Rep 2019; 7:2050313X19846765. [PMID: 31105948 PMCID: PMC6501474 DOI: 10.1177/2050313x19846765] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/27/2018] [Accepted: 04/03/2019] [Indexed: 11/21/2022] Open
Abstract
Imperforate hymen results from failure of the endoderm of the urogenital sinus to completely canalize and has an incidence of 0.01% to 0.05%. This sometimes presents as a pelvic mass that compresses the bladder causing acute urinary retention. A 13-year-old girl was referred to our department with a history of primary amenorrhea, cyclic lower abdominal pain, abdominal–pelvic mass, constipation and acute urinary retention. She had an ultrasonography misdiagnosis of a huge ovarian mass before referral to our unit. On examination, the vagina was bulging and compressing the rectum. Repeat abdominal ultrasonography confirmed the diagnosis of hematometrocolpos. She underwent X-shaped hymenotomy with a favorable outcome. Diagnosis of imperforate hymen requires high suspicion index. Virginity-sparing surgery constitutes a good treatment option for cultural and religious reasons.
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