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Li J, Duan Q, Long N, Ji F, Hang Y, Yang Z, Xie Y, Zhanghuang C, Yan B. Misdiagnosis of Herlyn-Werner-Wunderlich syndrome combined with pelvic mesothelial cyst: a case report. Front Med (Lausanne) 2025; 12:1513725. [PMID: 40012981 PMCID: PMC11862998 DOI: 10.3389/fmed.2025.1513725] [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: 10/23/2024] [Accepted: 01/27/2025] [Indexed: 02/28/2025] Open
Abstract
Herlyn-Werner-Wunderlich syndrome (HWWS) combined with pelvic mesothelial cyst is a rare condition that can be easily misdiagnosed. Our center presents a case study of this disease to provide clinicians with valuable insights for accurate diagnosis and treatment. A 12-year-old girl initially received a misdiagnosis of left lower abdominal ectopic kidney hydronephrosis based on B-ultrasound and renography due to intermittent abdominal pain. However, surgical exploration revealed the correct diagnosis of HWWS with pelvic mesothelial cyst. Postoperative pathology confirmed the presence of a mesothelial cyst in the pelvis. Once the diagnosis was confirmed, the patient was transferred to an adult gynaecology department for further treatment. Two months later, during follow-up via telephone, it was reported that the patient had successfully recovered after undergoing transvaginal oblique septum resection. This case report provides detailed information on the diagnostic process, treatment procedures, and examination results, serving as a valuable reference for clinical practitioners dealing with HWWS combined with pelvic mesothelial cysts while reducing the risk of misdiagnosis and mistreatment.
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Affiliation(s)
- Jinrong Li
- Department of Urology, Kunming Children’s Hospital, Yunnan Province Clinical Research Center for Children’s Health and Disease, Kunming, China
| | - Qiong Duan
- Special Ward, Kunming Children’s Hospital, Kunming, China
| | - Na Long
- Special Ward, Kunming Children’s Hospital, Kunming, China
| | - Fengming Ji
- Department of Urology, Kunming Children’s Hospital, Yunnan Province Clinical Research Center for Children’s Health and Disease, Kunming, China
| | - Yu Hang
- Department of Urology, Kunming Children’s Hospital, Yunnan Province Clinical Research Center for Children’s Health and Disease, Kunming, China
| | - Zhen Yang
- Department of Oncology, Yunnan Children Solid Tumor Treatment Center, Kunming Children’s Hospital, Kunming, China
| | - Yucheng Xie
- Department of Pathology, Kunming Children’s Hospital, Kunming, China
| | - Chenghao Zhanghuang
- Department of Urology, Kunming Children’s Hospital, Yunnan Province Clinical Research Center for Children’s Health and Disease, Kunming, China
- Yunnan Key Laboratory of Children’s Major Disease Research, Yunnan Clinical Medical Center for Pediatric Diseases, Kunming Children’s Hospital, Kunming, China
| | - Bing Yan
- Department of Urology, Kunming Children’s Hospital, Yunnan Province Clinical Research Center for Children’s Health and Disease, Kunming, China
- Yunnan Key Laboratory of Children’s Major Disease Research, Yunnan Clinical Medical Center for Pediatric Diseases, Kunming Children’s Hospital, Kunming, China
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Shrestha P, Shrestha B, Mishra AK, Katuwal N. Acute urinary retention in an adolescent female with Herlyn-Werner-Wunderlich syndrome. Ann Med Surg (Lond) 2025; 87:331-334. [PMID: 40109601 PMCID: PMC11918556 DOI: 10.1097/ms9.0000000000002752] [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: 08/23/2024] [Accepted: 11/05/2024] [Indexed: 03/22/2025] Open
Abstract
Introduction and importance Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare congenital anomaly characterized by the triad of uterus didelphys, unilateral obstructed hemivagina, and ipsilateral renal agenesis. However, atypical presentations such as acute urinary retention are uncommon and pose diagnostic challenges. Early identification and treatment are crucial to prevent long-term complications like endometriosis and infertility. Case presentation A 12-year-old female presented with the unusual symptom of acute urinary retention, which was likely caused by compression of the bladder neck due to hematocolpos. Imaging via ultrasound and MRI confirmed the presence of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis, consistent with HWWS. Surgical excision of the vaginal septum was performed to relieve the hematocolpos, with the patient experiencing a full recovery. Clinical discussion Congenital abnormalities of Mullerian Duct are rare, and atypical presentations such as acute urinary retention, vaginal discharge, and even pyocolpos or hematosalpinx have been reported. MRI played a crucial role in confirming the diagnosis and guiding treatment. The importance of early detection and long-term monitoring is highlighted, though limitations exist due to the inability to assess future reproductive outcomes. Conclusion This case emphasizes the importance of recognizing atypical presentations of HWWS, such as acute urinary retention. Timely diagnosis and appropriate surgical intervention are critical for preventing complications and restoring normal function. Early recognition of this rare syndrome, especially in patients with unusual symptoms such as urine retention, is essential for ensuring optimal clinical outcomes.
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Affiliation(s)
- Pradeep Shrestha
- Maharajgunj Medical Campus, Tribhuvan University, Institute of Medicine, Maharajgunj, Nepal
| | - Bibek Shrestha
- Maharajgunj Medical Campus, Tribhuvan University, Institute of Medicine, Maharajgunj, Nepal
| | - Amit K Mishra
- Maharajgunj Medical Campus, Tribhuvan University, Institute of Medicine, Maharajgunj, Nepal
| | - Neeta Katuwal
- Department of Obstetrics and Gynaecology, Tribhuvan University Teaching Hospital, Maharajgunj, Nepal
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Telecan T, Capras RD, Filip GA, Ionutas EM, Stanca DV, Crivii CB. OHVIRA Syndrome and Ureteral Ectopy Draining in the Ipsilateral Hemiuterus, Diagnosed in the Prepubertal Age Group: Case-Report and Literature Review. MEDICINA (KAUNAS, LITHUANIA) 2024; 60:1922. [PMID: 39768804 PMCID: PMC11678595 DOI: 10.3390/medicina60121922] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Figures] [Subscribe] [Scholar Register] [Received: 10/30/2024] [Revised: 11/18/2024] [Accepted: 11/21/2024] [Indexed: 01/11/2025]
Abstract
Background: Müllerian (paramesonephric) duct anomalies (MDA) are a rare condition, occurring in 5.5% of female newborns. One of the most complex malformations is represented by Obstructed Hemivagina and Ipsilateral Renal Anomalies (OHVIRA) syndrome, also known as Herlyn -Werner-Wunderlich (HWW) syndrome. Case presentation: We present the case of a 7-year-old asymptomatic premenarchal female patient diagnosed with OHVIRA syndrome with ipsilateral renal hypoplasia and ectopic ureteral implantation at the level of the uterus. As the patient developed urinary incontinence after the incision of the vaginal septum, right-sided 3D laparoscopic total nephrectomy was performed. Literature review: OHVIRA syndrome associated with ureteral ectopy is a rare occurrence, being encountered in 0.0064% of cases. The premenarchal diagnosis represents a challenge, due to the underdeveloped status of the genital tract. However, it should be ruled out in female newborns with unilateral renal agenesia or multicystic dysplastic kidney. Most reported cases describe the obstructed hemivagina as the site of ureteral ectopy. To our knowledge, this is the first reported case of OHVIRA syndrome with ectopic ureter draining at the level of the ipsilateral hemiuterus, diagnosed before the pubertal age. Conclusions: OHVIRA syndrome is one of the rarest Müllerian duct abnormalities. The management of these patients should be conducted in multidisciplinary teams, with long-term urological and gynecological follow-ups.
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Affiliation(s)
- Teodora Telecan
- Department of Anatomy and Embryology, “Iuliu Hatieganu” University of Medicine and Pharmacy, 400012 Cluj-Napoca, Romania; (T.T.); (G.A.F.); (C.-B.C.)
- Department of Pathology, County Emergency Clinical Hospital, 400347 Cluj-Napoca, Romania
| | - Roxana Denisa Capras
- Department of Anatomy and Embryology, “Iuliu Hatieganu” University of Medicine and Pharmacy, 400012 Cluj-Napoca, Romania; (T.T.); (G.A.F.); (C.-B.C.)
| | - Gabriela Adriana Filip
- Department of Anatomy and Embryology, “Iuliu Hatieganu” University of Medicine and Pharmacy, 400012 Cluj-Napoca, Romania; (T.T.); (G.A.F.); (C.-B.C.)
| | - Emanuela Maria Ionutas
- Department of Urology, “Iuliu Hatieganu” University of Medicine and Pharmacy, 400012 Cluj-Napoca, Romania; (E.M.I.); (D.V.S.)
- Department of Urology, Clinical Municipal Hospital, 400139 Cluj-Napoca, Romania
| | - Dan Vasile Stanca
- Department of Urology, “Iuliu Hatieganu” University of Medicine and Pharmacy, 400012 Cluj-Napoca, Romania; (E.M.I.); (D.V.S.)
- Department of Urology, Clinical Municipal Hospital, 400139 Cluj-Napoca, Romania
| | - Carmen-Bianca Crivii
- Department of Anatomy and Embryology, “Iuliu Hatieganu” University of Medicine and Pharmacy, 400012 Cluj-Napoca, Romania; (T.T.); (G.A.F.); (C.-B.C.)
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Hayashi S, Ono M, Sagisaka M, Mimura T, Kojima J, Ono M, Sasaki T, Yamamoto A, Nishi H. A case of Herlyn-Werner-Wunderlich syndrome with exacerbation of hematometra after adnexectomy. Asian J Endosc Surg 2024; 17:e13361. [PMID: 39039742 DOI: 10.1111/ases.13361] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/12/2024] [Revised: 06/26/2024] [Accepted: 07/10/2024] [Indexed: 07/24/2024]
Abstract
A 27-year-old nulliparous woman presented with a feeling of fullness in the lower abdomen and abdominal pain. A left ovarian tumor, uterus didelphys, left renal agenesis, and left vaginal atresia were observed on imaging. The ovarian tumor was presumed to have caused the abdominal pain, and an abdominal left adnexectomy was performed. After 3 months, she reported severe lower abdominal pain during menstruation. Transvaginal ultrasonography revealed uterine enlargement. After 17 days, the patient presented with abdominal pain and fever. She was diagnosed with peritonitis due to infection and left uterine hematometra. Because she did not improve with antibiotic treatment, left laparoscopic hysterectomy was performed. Subsequently, she did not experience the lower abdominal pain. Appropriate diagnosis and treatment based on the morphology of the reproductive tract and symptoms must be considered in patients with Herlyn-Werner-Wunderlich syndrome. Treatment must permit the outflow of menstrual blood.
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Affiliation(s)
- Shigehiro Hayashi
- Department of Obstetrics and Gynaecology, Tokyo Medical University, Tokyo, Japan
| | - Masanori Ono
- Department of Obstetrics and Gynaecology, Tokyo Medical University, Tokyo, Japan
| | - Masahiro Sagisaka
- Department of Obstetrics and Gynaecology, Tokyo Medical University, Tokyo, Japan
| | - Takayuki Mimura
- Department of Obstetrics and Gynaecology, Tokyo Medical University, Tokyo, Japan
| | - Junya Kojima
- Department of Obstetrics and Gynaecology, Tokyo Medical University, Tokyo, Japan
| | - Masataka Ono
- Department of Obstetrics and Gynaecology, Tokyo Medical University, Tokyo, Japan
| | - Toru Sasaki
- Department of Obstetrics and Gynaecology, Tokyo Medical University, Tokyo, Japan
| | - Akiko Yamamoto
- Department of Obstetrics and Gynaecology, Tokyo Medical University, Tokyo, Japan
| | - Hirotaka Nishi
- Department of Obstetrics and Gynaecology, Tokyo Medical University, Tokyo, Japan
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Peixoto Silva A, Souza Neves S, Tannure Saraiva PH, Bicalho Bretas TA. Hemihysterectomy in a patient with uterus didelphys, vaginal septum and ipsilateral renal agenesis: A case report and literature review. Int J Gynaecol Obstet 2024; 165:969-974. [PMID: 37968793 DOI: 10.1002/ijgo.15247] [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: 09/21/2023] [Accepted: 10/30/2023] [Indexed: 11/17/2023]
Abstract
Uterus didelphys is a rare Müllerian anomaly, often diagnosed during menarche or in women with a personal history of infertility and/or recurrent pregnancy loss. Its association with other genitourinary anomalies is frequent and may determine the existence of established syndromes. This case report refers to a 13-year-old female patient diagnosed with OHVIRA syndrome (Obstructed Hemivagina with Ipsilateral Renal Agenesis), a condition wherein the presence of a didelphic uterus is associated with hemivagina obstruction and ipsilateral renal agenesis. The patient presented with cyclic pelvic pain, related to the presence of hematocolpos and hematometra, which persisted despite several surgical approaches, including vaginal septum excision and correction of cervical stenosis. The recurrence of the condition indicated exploratory laparotomy, revealing two hemi-uteri and two uterine cervixes, with hematometra on the right. A subtotal hemihysterectomy was performed on the right. Post-procedure, the patient developed with regular menstrual cycles and improvement of pelvic pain complaints. Given the limited prevalence and low index of suspicion, the potential requirement for surgical intervention and its potential impact on reproductive future, diagnosing and treating OHVIRA syndrome and other Müllerian anomalies poses notable challenges in clinical practice. Hence, sharing different therapeutic approaches of a rare diagnosis with the scientific community is of paramount importance to aid in early diagnosis and effective management of similar clinical cases.
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Affiliation(s)
- Ananda Peixoto Silva
- Department of Gynecology and Obstetrics, Hospital Metropolitano Odilon Behrens, Belo Horizonte, Brazil
| | - Sasha Souza Neves
- Department of Gynecology and Obstetrics, Hospital Metropolitano Odilon Behrens, Belo Horizonte, Brazil
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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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Navarro V, Acién M, Acién P. Applicability and Suitability of the Embryological-Clinical Classification of Female Genital Malformations: A Systematic Review. J Clin Med 2024; 13:2988. [PMID: 38792529 PMCID: PMC11121905 DOI: 10.3390/jcm13102988] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/17/2024] [Revised: 05/13/2024] [Accepted: 05/16/2024] [Indexed: 05/26/2024] Open
Abstract
Complex urogenital malformations are clinically highly relevant; thus, they must be appropriately diagnosed and classified before initiating treatment. Background/Objectives: This study aimed to evaluate the applicability and suitability of the embryological-clinical classification of female genital malformations. Methods: A systematic review of cases of genital malformations reported in the literature from 2000 to 2020 was conducted. Case reports and series with the following combinations: "female genital tract" AND (malformation OR anomaly OR müllerian anomaly OR uterine anomaly OR cervical anomaly OR vaginal anomaly OR cloacal anomaly OR urogenital sinus); and "female genital tract" AND (renal agenesis OR ectopic ureter) were searched. A total of 3124 articles were identified, of which 824 cases of genital malformation were extracted. The characteristics of each malformation were included in a database for further analyses. Results: Using the embryological-clinical classification, 89.9% of the published cases and 86.5% of the 52 cases defined as unclassifiable by their authors have been classified in this review. In 73 cases (72.2%), the classification of the malformation using the AFS system was incomplete because although the type of uterine anomaly of the AFS classification matched that of the embryological-clinical classification, characteristics of the urinary system or the vagina were overlooked when using the AFS system. Following a dispersion matrix, we have been able to show that the embryological-clinical classification system is able to classify and subclassify the genitourinary malformations more accurately. Conclusions: The applicability of the embryological-clinical classification has been confirmed after classifying most of the cases of genital malformation previously published. This system also provides a more complete and accurate classification than other classifying systems exclusively based on Müllerian duct development or uterovaginal parameters, demonstrating its suitability.
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Affiliation(s)
- Victoria Navarro
- Obstetrics and Gynecology Service, Elda University Hospital, 03600 Elda, Alicante, Spain;
- Reproductive Biopathologies Mixed Research Unit FISABIO-UA-UMH, 03550 San Juan, Alicante, Spain
| | - Maribel Acién
- Reproductive Biopathologies Mixed Research Unit FISABIO-UA-UMH, 03550 San Juan, Alicante, Spain
- Obstetrics and Gynecology Service, San Juan University Hospital, 03550 San Juan, Alicante, Spain
- Division of Gynecology, Miguel Hernández University, Campus of San Juan, 03550 San Juan, Alicante, Spain;
- Grant Next Generation EU-EGA Institute for Women’s Health, University College London, London WC1E 6DE, UK
| | - Pedro Acién
- Division of Gynecology, Miguel Hernández University, Campus of San Juan, 03550 San Juan, Alicante, Spain;
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Yu BR, Chae HS, Rheu CH. Early-onset severe ovarian endometriosis in adolescents with completely obstructed Müllerian anomalies accompanied by ipsilateral renal agenesis: two case reports. J Surg Case Rep 2024; 2024:rjae272. [PMID: 38706491 PMCID: PMC11066802 DOI: 10.1093/jscr/rjae272] [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: 03/01/2024] [Accepted: 04/06/2024] [Indexed: 05/07/2024] Open
Abstract
Endometriosis is known to occur frequently in adolescents with obstructed Müllerian anomalies. Our cases emphasize that endometriosis can rapidly progress to a severe stage in obstructed hemivagina and ipsilateral renal anomaly syndrome, one of the completely obstructed Müllerian anomalies. The first patient was a 14-year-old girl who complained of cyclic abdominal pain. Imaging revealed a uterine didelphys with unilateral hematocolpos and a left adnexal endometrioma. The second, an 11-year-old girl, visited the hospital complaining of cyclic abdominal pain, had a unicornuate uterus with a functioning horn and left adnexal endometrioma. Also, both patients had unilateral renal agenesis. The surgery in both cases revealed Stage IV endometriosis. Adjuvant hormone therapy was administered for 1 year, and there was no recurrence until 3 years after surgery. We emphasize that patients diagnosed with renal agenesis should be screened to check for gynecological anomalies when menstrual cramps occur after menarche.
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Affiliation(s)
- Bo Ram Yu
- Department of Obstetrics and Gynecology, Jeonbuk National University Hospital, Jeonju 54907, South Korea
| | - Hee-Suk Chae
- Department of Obstetrics and Gynecology, Jeonbuk National University Hospital, Jeonju 54907, South Korea
- Department of Obstetrics and Gynecology, Jeonbuk National University, Jeonju 54907, South Korea
- Research Institute of Clinical Medicine of Jeonbuk National University-Biomedical Research Institute, Jeonbuk National University Hospital, Jeonju 54907, South Korea
| | - Chul-Hee Rheu
- Department of Obstetrics and Gynecology, Jeonbuk National University Hospital, Jeonju 54907, South Korea
- Department of Obstetrics and Gynecology, Jeonbuk National University, Jeonju 54907, South Korea
- Research Institute of Clinical Medicine of Jeonbuk National University-Biomedical Research Institute, Jeonbuk National University Hospital, Jeonju 54907, South Korea
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Mori A, Fujino S, Honda R, Kamijo K, Sano M, Imai T, Muramoto T, Kobayashi Y. Significance of expeditious diagnosis and treatment following menarche in obstructed hemivagina and ipsilateral renal agenesis syndrome: A case report. Radiol Case Rep 2024; 19:141-145. [PMID: 37941983 PMCID: PMC10628782 DOI: 10.1016/j.radcr.2023.09.070] [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: 09/12/2023] [Accepted: 09/22/2023] [Indexed: 11/10/2023] Open
Abstract
Obstructed hemivagina and ipsilateral renal agenesis syndrome is a rare complex of structural abnormalities of female urogenital tract. Moreover, delayed treatment of this condition can reportedly lead to pelvic infection, endometriosis, and infertility. We present a rare case of obstructed hemivagina and ipsilateral renal agenesis syndrome wherein appropriate treatment was initiated early due to the identification of left renal agenesis. A 14-year-old girl visited the gynecology department due to worsening left lower abdominal pain after menarche. She was diagnosed with renal dysplasia in the pediatric department at the ages of 3 and 9 years, and it was recommended that she visits the gynecology department right after menarche. She experienced her first menarche at the age of 14 years. Imaging studies revealed uterine didelphys with occlusion of the left vagina and formation of hematocolpos. Additionally, hematomas were observed in the left uterine cavity and the left fallopian tube; left renal agenesis was confirmed. Based on these findings, we diagnosed this patient with obstructed hemivagina and ipsilateral renal agenesis syndrome and performed a surgical incision and excision of the closed left vaginal wall, resulting in the disappearance of the hematocolpos. Approximately 2 years postoperatively, the patient has remained recurrence-free. Early diagnosis and treatment are crucial for preserving fertility in patients with obstructed hemivagina and ipsilateral renal agenesis syndrome, and early referral to the obstetrics and gynecology department is recommended if renal dysplasia is detected during childhood.
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Affiliation(s)
- Atsushi Mori
- Gynecology Service, Nagano Municipal Hospital, 1333-1 Tomitake, Nagano City, Nagano Prefecture, Japan
| | - Shotaro Fujino
- Gynecology Service, Nagano Municipal Hospital, 1333-1 Tomitake, Nagano City, Nagano Prefecture, Japan
| | - Riku Honda
- Gynecology Service, Nagano Municipal Hospital, 1333-1 Tomitake, Nagano City, Nagano Prefecture, Japan
| | - Kyosuke Kamijo
- Gynecology Service, Nagano Municipal Hospital, 1333-1 Tomitake, Nagano City, Nagano Prefecture, Japan
| | - Megumi Sano
- Gynecology Service, Nagano Municipal Hospital, 1333-1 Tomitake, Nagano City, Nagano Prefecture, Japan
| | - Takashi Imai
- Gynecology Service, Nagano Municipal Hospital, 1333-1 Tomitake, Nagano City, Nagano Prefecture, Japan
| | - Tsutomu Muramoto
- Gynecology Service, Nagano Municipal Hospital, 1333-1 Tomitake, Nagano City, Nagano Prefecture, Japan
| | - Yaeko Kobayashi
- Gynecology Service, Nagano Municipal Hospital, 1333-1 Tomitake, Nagano City, Nagano Prefecture, Japan
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Gudapati S, Chaudhari K, Dave A, Shinde D. An Incidental Diagnosis of Herlyn-Werner-Wunderlich Syndrome in a Young Female: A Case Report of a Rare Entity. Cureus 2024; 16:e53227. [PMID: 38425614 PMCID: PMC10903378 DOI: 10.7759/cureus.53227] [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: 12/19/2023] [Accepted: 01/29/2024] [Indexed: 03/02/2024] Open
Abstract
Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome, a rare genetic condition of the urogenital system, is characterized by distinctive features such as ipsilateral renal agenesis, obstructed hemivagina, and uterus didelphys. It is also referred to as Herlyn-Werner-Wunderlich (HWW) syndrome. Its clinical manifestations include dysmenorrhea, consistent abdominal pain, and infrequent periods. It is typically diagnosed after menarche. We report a case of a 20-year-old female who was admitted to the casualty ward following a road accident. She was Incidentally found to have uterine didelphys with hemorrhagic cystic lesion and left renal agenesis on ultrasonography (USG). She also had gallbladder stones, along with the findings mentioned above. Clinicians should exclude HWW syndrome in cases where uterine didelphys and unilateral renal agenesis coexist. Prompt identification and treatment of the condition can help avoid potential untoward pregnancy-related issues in the future.
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Affiliation(s)
- Sravya Gudapati
- Department of Obstetrics and Gynaecology, Jawaharlal Nehru Medical College, Datta Meghe Institute of Higher Education and Research (DMIHER), Wardha, IND
| | - Kamlesh Chaudhari
- Department of Obstetrics and Gynaecology, Jawaharlal Nehru Medical College, Datta Meghe Institute of Higher Education and Research (DMIHER), Wardha, IND
| | - Apoorva Dave
- Department of Obstetrics and Gynaecology, Jawaharlal Nehru Medical College, Datta Meghe Institute of Higher Education and Research (DMIHER), Wardha, IND
| | - Dhanajay Shinde
- Department of Radiodiagnosis, Datta Meghe Institute of Higher Education and Research (DMIHER), Wardha, IND
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Arab W, Abdelkhalek Y, Zoghbi A, Atallah D. Late diagnosis of Herlyn-Werner-Wunderlich syndrome: Is there a need for an early screening? Congenit Anom (Kyoto) 2023; 63:219-220. [PMID: 37736683 DOI: 10.1111/cga.12542] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/20/2023] [Accepted: 09/02/2023] [Indexed: 09/23/2023]
Affiliation(s)
- Wissam Arab
- Obstetrics and Gynecology Department, Hotel-Dieu de France Hospital, Saint Joseph University, Beirut, Lebanon
| | - Yara Abdelkhalek
- Obstetrics and Gynecology Department, Hotel-Dieu de France Hospital, Saint Joseph University, Beirut, Lebanon
| | - Antoine Zoghbi
- Emergency Department, Hotel-Dieu de France Hospital, Saint Joseph University, Beirut, Lebanon
| | - David Atallah
- Obstetrics and Gynecology Department, Hotel-Dieu de France Hospital, Saint Joseph University, Beirut, Lebanon
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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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Kim SJ, Shim SY, Cho HH, Park MH, Lee KA. Prenatal Diagnosis of Fetal Obstructed Hemivagina and Ipsilateral Renal Agenesis (OHVIRA) Syndrome. Medicina (B Aires) 2023; 59:medicina59040703. [PMID: 37109661 PMCID: PMC10146732 DOI: 10.3390/medicina59040703] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/14/2023] [Revised: 03/26/2023] [Accepted: 04/02/2023] [Indexed: 04/07/2023] Open
Abstract
Background: Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome, also known as Herlyn-Werner-Wunderlich syndrome, is a rare syndrome characterized by the triad of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. Most cases of OHVIRA have been reported in adolescents or adults. Gartner duct cysts, including those manifesting as vaginal wall cysts, are also rare. Fetal OHVIRA syndrome and Gartner duct cysts are difficult to diagnose. Case Presentation: Here, the authors report a case of combined OHVIRA and Gartner duct cyst diagnosed prenatally by ultrasonography, along with a brief review of the relevant published reports. A 30-year-old nulliparous female was referred to our institution at 32 weeks’ gestation for fetal right kidney agenesis. Detailed ultrasonographic examinations using 2D, 3D, and Doppler ultrasounds revealed hydrocolpometra, and uterus didelphys, with a normal anus and right kidney agenesis. Conclusions: When encountering female fetuses with ipsilateral renal agenesis or vaginal cysts, clinicians should be aware of OHVIRA syndrome and Gartner duct cysts and perform systematic ultrasonographic examinations for other genitourinary anomalies.
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Affiliation(s)
- Soo Jung Kim
- Department of Obstetrics and Gynecology, Ewha Womans University College of Medicine, Ewha Womans University Seoul Hospital, Seoul 07804, Republic of Korea
| | - So-Yeon Shim
- Department of Pediatrics, Ewha Womans University College of Medicine, Ewha Womans University Seoul Hospital, Seoul 07804, Republic of Korea
| | - Hyun-Hae Cho
- Department of Radiology, Ewha Womans University College of Medicine, Ewha Womans University Seoul Hospital, Seoul 07804, Republic of Korea
| | - Mi-Hye Park
- Department of Obstetrics and Gynecology, Ewha Womans University College of Medicine, Ewha Womans University Seoul Hospital, Seoul 07804, Republic of Korea
| | - Kyung A. Lee
- Department of Obstetrics and Gynecology, Ewha Womans University College of Medicine, Ewha Womans University Seoul Hospital, Seoul 07804, Republic of Korea
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14
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Martini N, Alabdallah E, Al-Ghotani B, ALAWAD IEMAN, Tawashi N. Successful management of Herlyn-Werner-Wunderlich syndrome in a 17-year-old virgin girl in the challenging socio-cultural-logistic setting of Syria: a case report. Ann Med Surg (Lond) 2023; 85:1223-1226. [PMID: 37113823 PMCID: PMC10129165 DOI: 10.1097/ms9.0000000000000510] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/05/2023] [Accepted: 03/18/2023] [Indexed: 04/29/2023] Open
Abstract
Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare developmental anomaly of the female reproductive system caused by a failure of fusion during Mullerian duct development. The triad of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis characterizes HWWS. The most common presenting symptoms are dysmenorrhoea, pelvic pain, primary infertility in later years, and an abdominal mass due to hematometrocolpos. Case Presentation A 17-year-old girl presented to the authors' department with recurrent low back pain, which was neither responsive to analgesics nor associated with urinary complaints, vomiting, or fever. Imaging techniques confirmed she had the triad of uterus didelphys, obstructed hemivagina, and right renal agenesis. Clinical Discussion The genital system is the same for males and females before 6 weeks of pregnancy. HWWS is a rare congenital disorder as a result of the failure of fusion during Mullerian duct development. It consists of a didelphic uterus, hemivaginal septum, and unilateral renal agenesis. Conclusion Shame and social stigma associated with virginity continue to endanger the lives of many girls in Syria. To complicate matters further, the low resources in Syria produced by war pose a difficult challenge in managing many gynecological conditions, including HWWS, like this case, in which endoscopic technologies were not available, necessitating open surgery while keeping in mind preserving hymen intactness. So, the authors indicate that preserving virginity could be conducted even though the approach is open surgery by very careful intervention and experienced surgeons.
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Affiliation(s)
- Nafiza Martini
- Faculty of Medicine, Damascus University
- Stemosis for Scientific Research, Damascus
| | - Ebaa Alabdallah
- Faculty of Medicine, Damascus University
- Stemosis for Scientific Research, Damascus
| | - Basel Al-Ghotani
- Faculty of Medicine, Damascus University
- Stemosis for Scientific Research, Damascus
- Corresponding author. Address: Faculty of Medicine, Damascus University, Damascus, Syrian Arab Republic. E-mail address: (B. Al-Ghotani)
| | - IEMAN ALAWAD
- Stemosis for Scientific Research, Damascus
- Alassad Medical Complex, Hama, Syrian Arab Republic
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15
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Eleje GU, Ihekwoaba EC, Onu OA, Igbodike EP, Ilokanuno CN, Chukwuanukwu TO, Okoro CC, Obioha KC, Okpala BC. Conception following successful repair of two previous failed attempts at repair of partial transverse vaginal septum diagnosed after marital union: A case report. SAGE Open Med Case Rep 2022; 10:2050313X221141532. [PMID: 36507061 PMCID: PMC9726837 DOI: 10.1177/2050313x221141532] [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: 04/06/2022] [Accepted: 11/09/2022] [Indexed: 12/07/2022] Open
Abstract
Transverse vaginal septum is a congenital anomaly in which a membrane obstructs the vagina. This can be partial or complete in type. Although rare, it presents peculiar challenges in symptomatology, diagnosis, and ultimate management. To our knowledge, we are the first to report a shortest vaginoplasty-conception interval following successful repair of previously failed repair of partial transverse vaginal septum. A 28-year-old Nigerian married nulliparous lady who presented to us with history of inability of penile-vaginal penetration with the presence of normal menstrual flow after two previous failed attempts at repair. She had a vaginoplasty with placement of a vaginal mold to prevent stenosis. She was subsequently able to have successful sexual intercourse and achieved pregnancy after 2 months, without recurrence of vaginal stenosis. When transverse vaginal septum is encountered in a married nullipara, a thorough clinical evaluation of the viability and feasibility of a vaginoplasty should be made during the first surgery. If repeated failed repair occurs, as in our case, we recommend meticulous and experienced surgical attention from the outset. The originality in this report lies in the very short period between repair and successful conception. Thus, we obtained satisfactory short-term clinical outcome of successful conception at the 2 months follow-up.
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Affiliation(s)
- George Uchenna Eleje
- Effective Care Research Unit, Department of Obstetrics and Gynecology, Nnamdi Azikiwe University, Nnewi, Nigeria,Department of Obstetrics and Gynecology, Nnamdi Azikiwe University Teaching Hospital, Nnewi, Nigeria,George Uchenna Eleje, Effective Care Research Unit, Department of Obstetrics and Gynaecology, Nnamdi Azikiwe University, P.M.B. 5001, Nnewi, 435001, Nigeria. Emails: ;
| | - Eric Chukwudi Ihekwoaba
- Department of Surgery, Faculty of Medicine, Nnamdi Azikiwe University, Awka, Nigeria,Department of Surgery, Nnamdi Azikiwe University Teaching Hospital, Nnewi, Nigeria
| | - Onyekachi Amos Onu
- Department of Surgery, Nnamdi Azikiwe University Teaching Hospital, Nnewi, Nigeria
| | - Emeka Philip Igbodike
- Department of Obstetrics and Gynecology, Nnamdi Azikiwe University Teaching Hospital, Nnewi, Nigeria
| | - Chinedu Nnaemeka Ilokanuno
- Plastic and Reconstructive Surgery Unit, Department of Surgery, Nnamdi Azikiwe University, Awka, Nigeria,Plastic and Reconstructive Surgery Unit, Department of Surgery, Nnamdi Azikiwe University Teaching Hospital, Nnewi, Nigeria
| | - Titus Osita Chukwuanukwu
- Plastic and Reconstructive Surgery Unit, Department of Surgery, Nnamdi Azikiwe University, Awka, Nigeria,Plastic and Reconstructive Surgery Unit, Department of Surgery, Nnamdi Azikiwe University Teaching Hospital, Nnewi, Nigeria
| | | | - Kingsley Chukwu Obioha
- Department of Obstetrics and Gynecology, University of Nigeria Teaching Hospital, Ituku Ozalla, Nigeria
| | - Boniface Chukwuneme Okpala
- Effective Care Research Unit, Department of Obstetrics and Gynecology, Nnamdi Azikiwe University, Nnewi, Nigeria,Department of Obstetrics and Gynecology, Nnamdi Azikiwe University Teaching Hospital, Nnewi, Nigeria
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16
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Aljahdali EA, Kurdi MO. Hymen-saving hymenotomy of imperforate hymen in neonates and adolescents: tertiary medical center experience. ANNALS OF PEDIATRIC SURGERY 2022. [DOI: 10.1186/s43159-022-00192-1] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/10/2022] Open
Abstract
Abstract
Background
Imperforate hymen (IH) is the most common obstructive anomaly of female genital tract. Hymen saving has a great importance for both cultural and religious reasons. Conventional surgical treatment for this condition is cruciate incision hymenotomy or hymen-saving central annular hymenotomy (HSCAH). This study aimed to review presentations and management of neonate and adolescents with IH at one tertiary hospital over 12 year’s period. The aim also was to stress upon importance of hymen saving to our Muslim community and improve knowledge, enable targeted education, and reduce unnecessary tests and diagnostic delay for this condition.
Methods
Twenty-five patients with IH were diagnosed and treated in this retrospective cohort study over 12 years, from January 2010 till December 2021. Demographic characteristics of the patients, their symptoms and signs, investigations, type of operations, recurrence, and infections were recorded and analyzed.
Results
Saudi accounted for the majority of IH cases (88%). There were fifteen adolescents, and ten were neonates. Bulging imperforate hymen, pelvic/abdominopelvic mass, constipation, acute urine retention, abdominal discomfort, and lower abdominal distension were among the signs and symptoms. All 25 patients had HSCAH, 21 of whom had primary HSCAH and four of whom had secondary HSCAH due to recurrence. To avoid recurrence, HSCAH was performed with suturing of the inner vaginal mucosa to the exterior vestibular mucosa.
Conclusion
Early diagnosis of imperforate hymen for neonate and adolescent girls and prompt definitive HSCAH could have positive impact on treatment outcome and decrease complications and fulfill integrity of hymen as virginity of high importance in our Muslim community and other similar culture, where hymen-sparing procedure is preferred by most of these patients and families.
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17
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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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18
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Fontana E, Parma M, Fedele F, Girardelli S, Parazzini F, Candiani M. Forty-two normomenstruating adolescents with Müllerian obstructive anomalies: Presentation, pitfalls in the dagnosis and surgical management. Acta Obstet Gynecol Scand 2022; 102:92-98. [PMID: 36285343 PMCID: PMC9780719 DOI: 10.1111/aogs.14454] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/23/2022] [Revised: 07/03/2022] [Accepted: 08/18/2022] [Indexed: 12/30/2022]
Abstract
INTRODUCTION We analyzed the frequency, presentation and pitfalls in the diagnosis and surgical management of a large group of normomenstruating adolescents with obstructive reproductive tract anomalies. MATERIAL AND METHODS Retrospective analysis of prospectively collected data. Of the 143 outpatients referred for severe dysmenorrhea and persistent pelvic pain, 42 (29.3%) young women with obstructive Müllerian anomalies and regular menstrual flow were identified. These patients were divided into four groups: (1) patients with duplicate uterine cavities, obstructed hemivagina and ipsilateral renal agenesis (n = 34); (2) patients with unicornuate uterus and noncommunicating cavitated rudimentary horn (n = 5); (3) patients with accessory cavitated uterine mass (n = 2); (4) patients with partially obstructed transverse vaginal septum (n = 1). All 42 patients were conservatively treated via laparoscopy and 35/42 patients had also vaginal surgery. RESULTS Of the four groups, patients in groups 2 and 3 (n = 7) were conservatively managed by laparoscopy alone; for patients in groups 1 and 4 (n = 35), laparoscopy and the vaginal approach were used. Patients of group 1 were treated by resecting the obstructed vaginal septum with drainage of retained collections. In patients in group 2, surgery consisted of the removal of the rudimentary horn. Patients of group 3 were treated by the removal of myometrial neoformations. In the patient in group 4, treatment consisted of removal of the septum. All surgical procedures were successful and no major complications were recorded. Follow-up reports highlighted the disappearance of obstruction and clear improvement in pain symptoms. CONCLUSIONS Unilateral obstructive anomalies of the female genital tract are difficult to identify. Early diagnosis allows the preservation of reproductive activity and avoids potential complications.
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Affiliation(s)
- Eleonora Fontana
- Obstetrics and Gynecology DepartmentIRCCS San Raffaele Scientific InstituteMilanItaly,Fondazione IRCCS Ca' Granda Ospedale Maggiore PoliclinicoUniversity of MilanMilanItaly
| | - Marta Parma
- Obstetrics and Gynecology DepartmentIRCCS San Raffaele Scientific InstituteMilanItaly
| | - Francesco Fedele
- Obstetrics and Gynecology DepartmentIRCCS San Raffaele Scientific InstituteMilanItaly,Fondazione IRCCS Ca' Granda Ospedale Maggiore PoliclinicoUniversity of MilanMilanItaly
| | - Serena Girardelli
- Obstetrics and Gynecology DepartmentIRCCS San Raffaele Scientific InstituteMilanItaly
| | - Fabio Parazzini
- Fondazione IRCCS Ca' Granda Ospedale Maggiore PoliclinicoUniversity of MilanMilanItaly
| | - Massimo Candiani
- Obstetrics and Gynecology DepartmentIRCCS San Raffaele Scientific InstituteMilanItaly
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19
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Matsubara S, Watanabe T. An adolescent girl with acute abdominal pain and abdominal mass. BMJ 2022; 379:e068444. [PMID: 36265874 DOI: 10.1136/bmj-2021-068444] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/04/2022]
Affiliation(s)
- Shigeki Matsubara
- Jichi Medical University, Department of Obstetrics and Gynecology, Tochigi, Japan
| | - Takashi Watanabe
- Haga Red Cross Hospital, Department of Obstetrics and Gynecology, Tochigi, Japan
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20
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Mabuchi S, Hayashida H, Kubo C, Takemura M, Kamiura S. Herlyn-Werner-Wunderlich syndrome (HWWS)-associated gynecological malignancies: A case report and literature review. Gynecol Oncol Rep 2022; 43:101051. [PMID: 35923167 PMCID: PMC9340499 DOI: 10.1016/j.gore.2022.101051] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/30/2022] [Revised: 07/18/2022] [Accepted: 07/22/2022] [Indexed: 10/26/2022] Open
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21
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Hayat AM, Yousaf KR, Chaudhary S, Amjad S. The Herlyn-Werner-Wunderlich (HWW) syndrome – A case report with radiological review. Radiol Case Rep 2022; 17:1435-1439. [PMID: 35265236 PMCID: PMC8899131 DOI: 10.1016/j.radcr.2022.02.017] [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: 12/29/2021] [Accepted: 02/02/2022] [Indexed: 11/05/2022] Open
Abstract
Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital anomaly of female urogenital tract involving combined mullerian duct anomalies and mesonephric duct malformation characterized by uterus didelphys, obstructed hemi-vagina and ipsilateral renal agenesis which is also known as OHVIRA syndrome. It can be classified based on a completely or incompletely obstructed hemivagina. It presents soon after menarche or shows delayed presentation depending on the type. The most common presentation is lower abdominal pain, dysmenorrhea, and abdominal mass in the lower abdomen secondary to hematometra or hematocolpos. We present a 15-year-old unmarried patient with an unusual case of OHVIRA syndrome suffering from dysmenorrhea and painful mass in suprapubic region. We described the role of imaging modalities in diagnosis of the Herlyn-Werner Wunderlich syndrome with a review of literature. On USG and MRI, she had right renal agenesis with compensatory hypertrophy of the left kidney, didelphic uterus with an obstructed hemi-vagina on right side which led to marked distention of ipsilateral cervix and proximal vagina in the form of hematometrocolpos. OHVIRA syndrome can present early or late, depending on the type. In patients with uterine and vaginal abnormalities, a work-up for associated renal anomalies should be performed. The choice imaging modalities for the diagnosis of OHVIRA syndrome are ultrasound and MRI. Knowing the imaging findings of this rare condition is crucial for early diagnosis in order to prevent complications which may lead to endometriosis and infertility.
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22
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Uno AT, Mukaisho KI, Hitosugi M. Dysmenorrhea due to undiagnosed obstructed hemi-vagina and ipsilateral renal anomaly syndrome can become a cause of suicide. Environ Health Prev Med 2022; 27:8. [PMID: 35288492 PMCID: PMC9093615 DOI: 10.1265/ehpm.21-00043] [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] [Indexed: 11/23/2022] Open
Abstract
Background A Japanese woman in her early twenties had committed suicide, jumped from a 25-meter high bridge into a lake. She had been suffering from severe dysmenorrhea and general fatigue monthly. Results A forensic autopsy revealed indications of a bicorporeal uterus, obstructed hemi-vagina, and ipsilateral renal agenesis, which lead to a diagnosis of obstructed hemi-vagina and ipsilateral renal anomaly (OHVIRA) syndrome. On the right side of the uterus, an enclosed cavity composed of black clots was observed. Histological findings suggested that her endometrium was in the early proliferative phase, implying that she was in the menstrual phase just before her death. She may have been suffering from severe lower abdominal pain from the increased pressure of the closed uterus cavity. Conclusions This case indicates that dysmenorrhea from undiagnosed OHVIRA syndrome can possibly lead to a suicide attempt. In Japan, because suicide was the leading cause of death for people aged 15 to 39 in 2019, preventive measures for suicide should be promoted. The present case also suggests that intervention for dysmenorrhea may prevent this in adolescent woman.
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Affiliation(s)
- Akari Takaya Uno
- Department of Legal Medicine, Shiga University of Medical Science
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23
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Jang E, So KA, Kim B, Lee AJ, Kim NR, Yang EJ, Shim SH, Lee SJ, Kim TJ. Delayed diagnosis of imperforate hymen with huge hematocolpometra: A case report. World J Clin Cases 2021; 9:8901-8905. [PMID: 34734073 PMCID: PMC8546805 DOI: 10.12998/wjcc.v9.i29.8901] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/09/2021] [Revised: 07/20/2021] [Accepted: 08/27/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Imperforate hymen is a rare obstructive anomaly of the female reproductive tract. It is associated with complications, such as cyclical abdominal pain, urinary retention, and pelvic mass.
CASE SUMMARY A 13-year-old girl presented several times to the emergency room with lower abdominal pain for a year. She received conservative treatment, such as pain control, at each visit. She visited our gynecological clinic for worsening pain, and a 14-cm hematocolpos was found on ultrasonography. She was finally diagnosed with an imperforate hymen with hematocolpometra. Hymenectomy was performed, which resulted in event-free regular cyclical menstruation.
CONCLUSION Imperforate hymen should be considered in a premenarcheal adolescent girl with periodic abdominal pain.
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Affiliation(s)
- Eunbi Jang
- Department of Obstetrics and Gynecology, Konkuk University School of Medicine, Seoul 05030, South Korea
| | - Kyeong A So
- Department of Obstetrics and Gynecology, Konkuk University School of Medicine, Seoul 05030, South Korea
| | - Bomin Kim
- Department of Obstetrics and Gynecology, Konkuk University School of Medicine, Seoul 05030, South Korea
| | - A Jin Lee
- Department of Obstetrics and Gynecology, Konkuk University School of Medicine, Seoul 05030, South Korea
| | - Nae Ri Kim
- Department of Obstetrics and Gynecology, Konkuk University School of Medicine, Seoul 05030, South Korea
| | - Eun Jung Yang
- Department of Obstetrics and Gynecology, Konkuk University School of Medicine, Seoul 05030, South Korea
| | - Seung-Hyuk Shim
- Department of Obstetrics and Gynecology, Konkuk University School of Medicine, Seoul 05030, South Korea
| | - Sun Joo Lee
- Department of Obstetrics and Gynecology, Konkuk University School of Medicine, Seoul 05030, South Korea
| | - Tae Jin Kim
- Department of Obstetrics and Gynecology, Konkuk University School of Medicine, Seoul 05030, South Korea
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24
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Khashoggi AA, Hakami NA, Alghamdi MA, Ayed HY, Alqarni AD, Alshahrani TJ, Alshamrani AM, Alayyaf AS, Almansour RA, Alolyan SA, Alotaibi AA, Alabdulltif SK, Obaidi EA, Akkasi HA, Alshammari M. Hematocolpos: An Unusual Cause of Right Iliac Fossa Pain. Cureus 2021; 13:e18835. [PMID: 34804690 PMCID: PMC8593911 DOI: 10.7759/cureus.18835] [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] [Accepted: 10/17/2021] [Indexed: 11/05/2022] Open
Abstract
Acute appendicitis is the most frequent indication for emergency abdominal surgery in childhood. Despite being a common condition, the diagnosis of acute appendicitis can be challenging. Ultrasound examination remains the imaging tool of choice for the diagnosis of acute appendicitis. We report the case of a nine-year-old girl who presented with acute abdominal pain in the right lower quadrant accompanied by nausea and vomiting. Physical examination revealed right iliac fossa rebound tenderness. Ultrasound examination showed a dilated blind-ended structure in the right iliac fossa conferring the diagnosis of acute appendicitis. The patient underwent a CT scan which made the diagnosis of imperforate hymen with hematocolpos. The patient underwent hymenotomy and her symptoms showed complete resolution. Imperforate hymen with hematocolpos is a rare congenital genital tract anomaly. The case highlights the importance of appropriate genital examination in pediatric patients. In the present case, the patient might have undergone an unnecessary abdominal surgery if the correct diagnosis was not established.
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Affiliation(s)
- Alya A Khashoggi
- College of Medicine, King Saud Bin Abdulaziz University for Health Sciences, Riyadh, SAU
| | | | | | - Hussam Y Ayed
- College of Medicine, King Khalid University, Abha, SAU
| | - Ali D Alqarni
- College of Medicine, Ibn Sina National College for Medical Studies, Jeddah, SAU
| | - Talal J Alshahrani
- College of Medicine, Ibn Sina National College for Medical Studies, Jeddah, SAU
| | | | - Abdallah S Alayyaf
- College of Medicine, Prince Sattam Bin Abdulaziz University, Al-Kharj, SAU
| | - Raghad A Almansour
- College of Medicine, Imam Mohammad Ibn Saud Islamic University, Riyadh, SAU
| | | | | | | | | | - Hatim A Akkasi
- College of Medicine, Majmaah University, Al Majmaah, SAU
| | - Malak Alshammari
- College of Medicine, Imam Abdulrahman Bin Faisal University, Dammam, SAU
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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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Kueppers J, Wehrli L, Zundel S, Shavit S, Stahr N, Szavay P. OHVIRA-syndrome in a newborn. JOURNAL OF PEDIATRIC SURGERY CASE REPORTS 2021. [DOI: 10.1016/j.epsc.2021.101859] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/28/2022] Open
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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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Tanitame K, Tanitame N, Urayama S, Ohtsu K. Congenital anomalies causing hemato/hydrocolpos: imaging findings, treatments, and outcomes. Jpn J Radiol 2021; 39:733-740. [PMID: 33840015 PMCID: PMC8338850 DOI: 10.1007/s11604-021-01115-7] [Citation(s) in RCA: 23] [Impact Index Per Article: 5.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/08/2021] [Accepted: 03/30/2021] [Indexed: 12/28/2022]
Abstract
Hemato/hydrocolpos due to congenital urogenital anomalies are rare conditions discovered in neonatal, infant, and adolescent girls. Diagnosis is often missed or delayed owing to its rare incidence and nonspecific symptoms. If early correct diagnosis and treatment cannot be performed, late complications such as tubal adhesion, pelvic endometriosis, and infertility may develop. Congenital urogenital anomalies causing hemato/hydrocolpos are mainly of four types: imperforate hymen, distal vaginal agenesis, transverse vaginal septum, and obstructed hemivagina and ipsilateral renal anomaly, and clinicians should have adequate knowledge about these anomalies. This article aimed to review the diagnosis and treatment of these urogenital anomalies by describing embryology, clinical presentation, imaging findings, surgical management, and postoperative outcomes.
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Affiliation(s)
- Keizo Tanitame
- Department of Diagnostic Radiology, Hiroshima Prefectural Hospital, Minami-ku, Ujinakanda, Hiroshima, 734-8530, Japan.
| | - Nobuko Tanitame
- Department of Radiology, Hiroshima City Hiroshima Citizens Hospital, Hiroshima, Japan
| | - Saiko Urayama
- Department of Obstetrics and Gynecology, Hiroshima Prefectural Hospital, Hiroshima, Japan
| | - Kazuhiro Ohtsu
- Department of Maternal and Child Health Research Center, Hiroshima Prefectural Hospital, Hiroshima, Japan
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Wdowiarz K, Skrajna A, Reinholz-Jaskólska M. Diagnosis and treatment of Herlyn-Werner-Wunderlich syndrome: a case report. PRZEGLAD MENOPAUZALNY = MENOPAUSE REVIEW 2021; 20:52-56. [PMID: 33935621 PMCID: PMC8077801 DOI: 10.5114/pm.2021.104034] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 06/17/2020] [Accepted: 08/20/2020] [Indexed: 11/30/2022]
Abstract
INTRODUCTION Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital anomaly characterized by uterus didelphys with blind hemivagina and ipsilateral renal agenesis. It is one of the rare anomalies of the genitourinary system and is usually identified in the first few years after menarche. CASE REPORT An 18-year-old patient presented with painful menses for 1 year and was suspected for vaginal haematoma. Symptoms appeared 5 years after menarche. DISCUSSION Magnetic resonance imaging of the pelvis revealed a congenital anomaly of the genital tract. Two steps of surgical management were performed. First, a hysteroscopic incision of the vaginal septa and evacuation of vaginal haematoma were performed. Subsequently, the vaginal septum was partially excised during laparohysteroscopy. CONCLUSIONS Ipsilateral renal agenesis was confirmed by ultrasound during hospitalization, and the diagnoses of obstructed hemivagina and ipsilateral renal anomaly syndrome were established.The aim of this study was to present the diagnostic and therapeutic approaches in HWW syndrome, and to draw attention to the challenges in its diagnosis, which can coexist with multiple complications.
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Affiliation(s)
- Kinga Wdowiarz
- Department of Obstetrics and Gynaecology, Praski Hospital, Warsaw, Poland
| | - Adrianna Skrajna
- Department of Obstetrics and Gynaecology, Praski Hospital, Warsaw, Poland
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Jhirwal M, Singh P, Sharma C, Khera P. Herlyn-Werner-Wunderlich (HWW) syndrome with kyphoscoliosis: a rare urogenital anomaly in a teenage girl. BMJ Case Rep 2021; 14:14/3/e238688. [PMID: 33753378 PMCID: PMC7986945 DOI: 10.1136/bcr-2020-238688] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
Abstract
The obstructed haemivagina with ipsilateral renal agenesis (OHVIRA) syndrome also known as Herlyn-Werner-Wunderlich syndrome, a rare congenital malformation in females, results due to developmental abnormality in the müllerian duct. We are reporting a case of a 14-year-old girl who presented with pain and a lump in abdomen for the last 5 months. The MRI abdomen and pelvis confirmed the diagnosis of OHVIRA syndrome. The patient underwent haematocolpos and haematometra drainage, followed by vaginal septum resection.
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Affiliation(s)
- Manisha Jhirwal
- Obstetrics & Gynecology, AIIMS Jodphur, Jodhpur, Rajasthan, India
| | - Pratibha Singh
- Obstetrics & Gynecology, AIIMS Jodphur, Jodhpur, Rajasthan, India
| | - Charu Sharma
- Obstetrics & Gynecology, AIIMS Jodphur, Jodhpur, Rajasthan, India
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Kozłowski M, Nowak K, Boboryko D, Kwiatkowski S, Cymbaluk-Płoska A. Herlyn-Werner-Wunderlich Syndrome: Comparison of Two Cases. INTERNATIONAL JOURNAL OF ENVIRONMENTAL RESEARCH AND PUBLIC HEALTH 2020; 17:E7173. [PMID: 33007989 PMCID: PMC7579596 DOI: 10.3390/ijerph17197173] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 09/08/2020] [Revised: 09/27/2020] [Accepted: 09/28/2020] [Indexed: 11/20/2022]
Abstract
BACKGROUND Herlyn-Werner-Wunderlich Syndrome is a rare malformation syndrome characterized by uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis. Symptoms appear most often after menarche and are secondary to hematocolpos. We compare clinical symptoms, diagnosis and treatment of two patients, a 13-year-old and a 17-year-old. CASE REPORT Despite the non-uniform clinical symptoms, it should be noted that in both patients, the 13-year-old and the 17-year-old, hematocolpos, which was probably the cause of lower abdominal pain, was diagnosed with ultrasound. The diagnosis was complemented by laparoscopy, which determined the diagnosis of malformation of uterus didelphys with obstructed hemivagina. The patients had a history of kidney agenesis, which, after gynecological diagnosis, turned out to be ipsilateral. In the 13-year-old, agenesis was diagnosed by uroscintigraphy, while in the 17-year-old it was diagnosed by urography. Incision and drainage of the residual vagina was performed in the course of therapeutic management. In both cases, the clinical situation required a repeated widening of the orifice. CONCLUSIONS Lower abdominal pain accompanying hematocolpos suggested Herlyn-Werner-Wunderlich Syndrome (HWWS) as the cause of symptoms. 3D transvaginal ultrasound enabled the determination of a congenital uterine defect with high probability, although inconclusive cases required confirmation by laparoscopy. Incision of the blocked vagina and drainage of hematocolpos were the key components of treatment. The treatment of HWWS is a multi-step process.
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Affiliation(s)
- Mateusz Kozłowski
- Department of Gynecological Surgery and Gynecological Oncology of Adults and Adolescents, Pomeranian Medical University, 70-111 Szczecin, Poland; (K.N.); (D.B.); (A.C.-P.)
| | - Katarzyna Nowak
- Department of Gynecological Surgery and Gynecological Oncology of Adults and Adolescents, Pomeranian Medical University, 70-111 Szczecin, Poland; (K.N.); (D.B.); (A.C.-P.)
| | - Dominika Boboryko
- Department of Gynecological Surgery and Gynecological Oncology of Adults and Adolescents, Pomeranian Medical University, 70-111 Szczecin, Poland; (K.N.); (D.B.); (A.C.-P.)
| | - Sebastian Kwiatkowski
- Department of Obstetrics and Gynecology, Pomeranian Medical University, 70-111 Szczecin, Poland;
| | - Aneta Cymbaluk-Płoska
- Department of Gynecological Surgery and Gynecological Oncology of Adults and Adolescents, Pomeranian Medical University, 70-111 Szczecin, Poland; (K.N.); (D.B.); (A.C.-P.)
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Abstract
Congenital gynecologic anomalies result from interruption of embryologic development of the female reproductive tract. The anomalies may be hymenal, vaginal, cervical, or uterine. The impact of these anomalies is variable: some are asymptomatic, incidental findings that require no intervention, others require simple surgical management, while some complex anomalies may require a multidisciplinary approach with extensive surgical expertise for optimal outcomes. Uterovaginal anomalies may occur in isolation or in association with other malformations, such as renal anomalies. The origin, presentation, evaluation and treatment of these conditions are reviewed here.
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Fascilla FD, Olivieri C, Cannone R, De Palma D, Manosperta F, Costantino AS, Carugno J, Vicino M, Cicinelli E, Bettocchi S. In-office Hysteroscopic Treatment of Herlyn-Werner-Wunderlich Syndrome: A Case Series. J Minim Invasive Gynecol 2020; 27:1640-1645. [PMID: 32320799 DOI: 10.1016/j.jmig.2020.04.013] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/04/2020] [Revised: 03/26/2020] [Accepted: 04/07/2020] [Indexed: 10/24/2022]
Abstract
Herlyn-Werner-Wunderlich syndrome (HWWs) is a rare congenital malformation of the female urogenital track characterized by a triad consisting of didelphys uterus, obstructed hemivagina, and ipsilateral renal agenesis. We report 5 consecutive cases of patients diagnosed with HWWs treated in our center. Imaging studies with 2-dimensional/3-dimensional ultrasound and abdominopelvic magnetic resonance imaging were obtained to confirm the diagnosis. The treatment consisted of a 1-step surgical in-office hysteroscopic incision of the vaginal septum using 5 Fr hysteroscopic bipolar electrodes. At the follow-up visit, between 1 and 2 months after the initial procedure, the patients underwent a diagnostic vaginoscopy with excision of exceeding vaginal tissue if needed, performed with a vaginal hysteroscopic approach. In-office hysteroscopic treatment of patients diagnosed with HWWs is a safe and effective, minimally invasive treatment modality that provides symptomatic relief and preserves fertility, avoiding the cost and risks of the use of general anesthesia in an operating room setting. We recommend shifting the management of this challenging condition to the office setting.
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Affiliation(s)
- Fabiana Divina Fascilla
- Unit of Obstetrics and Gynecology, Di Venere Hospital, Bari Local Health Authority (Drs. Fascilla and Vicino)
| | - Claudiana Olivieri
- II Unit of Obstetrics and Gynecology, Department of Biomedical Sciences and Human Oncology, Policlinico of Bari, (Drs. Olivieri, Cannone, De Palma, Costantino, and Cicinelli)
| | - Rossella Cannone
- II Unit of Obstetrics and Gynecology, Department of Biomedical Sciences and Human Oncology, Policlinico of Bari, (Drs. Olivieri, Cannone, De Palma, Costantino, and Cicinelli)
| | - Domenico De Palma
- II Unit of Obstetrics and Gynecology, Department of Biomedical Sciences and Human Oncology, Policlinico of Bari, (Drs. Olivieri, Cannone, De Palma, Costantino, and Cicinelli)
| | - Francesco Manosperta
- Student at Medical School of the Faculty of Medicine, University "Aldo Moro" of Bari, Italy (Dr. Manosperta)
| | - Alfredo Sigfrido Costantino
- II Unit of Obstetrics and Gynecology, Department of Biomedical Sciences and Human Oncology, Policlinico of Bari, (Drs. Olivieri, Cannone, De Palma, Costantino, and Cicinelli)
| | - Jose Carugno
- Obstetrics, Gynecology and Reproductive Sciences Department, Minimally Invasive Gynecology Unit, University of Miami, Miller School of Medicine, Miami, Florida (Dr. Carugno)
| | - Mario Vicino
- Unit of Obstetrics and Gynecology, Di Venere Hospital, Bari Local Health Authority (Drs. Fascilla and Vicino)
| | - Ettore Cicinelli
- II Unit of Obstetrics and Gynecology, Department of Biomedical Sciences and Human Oncology, Policlinico of Bari, (Drs. Olivieri, Cannone, De Palma, Costantino, and Cicinelli)
| | - Stefano Bettocchi
- Inter-Departmental Project Unit of "Minimal-Invasive Gynecological Surgery," Policlinico of Bari (Dr. Bettocchi) University of Bari Aldo Moro, Bari, Italy.
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Munley JA, Taylor JA. Diagnosis and surgical management of Herlyn-Werner-Wunderlich syndrome. JOURNAL OF PEDIATRIC SURGERY CASE REPORTS 2020. [DOI: 10.1016/j.epsc.2019.101364] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/25/2022] Open
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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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Lee KH, Hong JS, Jung HJ, Jeong HK, Moon SJ, Park WH, Jeong YM, Song SW, Suk Y, Son MJ, Lim JJ, Shin JI. Imperforate Hymen: A Comprehensive Systematic Review. J Clin Med 2019; 8:jcm8010056. [PMID: 30621064 PMCID: PMC6352236 DOI: 10.3390/jcm8010056] [Citation(s) in RCA: 40] [Impact Index Per Article: 6.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/19/2018] [Revised: 12/29/2018] [Accepted: 01/03/2019] [Indexed: 12/28/2022] Open
Abstract
Imperforate hymen (IH) is an uncommon congenital anomaly of the female genital tract, with the hymen completely obstructing the vaginal opening. Despite the simple diagnosis and treatment of IH, missed or delayed diagnosis is often a clinical problem owing to its low incidence, nonspecific symptoms, or insufficient physical examination. The aim of this study is to identify the characteristics, clinical presentations, treatment modalities, and outcomes of imperforate hymen patients. In this study, a literature search of PubMed databases was performed for sources published up to 3 July 2018 for English-language studies with the term “imperforate hymen”. The literature review identified 251 citations and 155 articles (143 case reports, 12 case series) containing 253 patients who were finally included (two papers were not written in English). Among 236 postnatal patients, the mean age of the patients was 10.7 ± 4.7 years. Abdominal pain (54.2%), urinary retention (20.3%), abnormal menstruation (14.0%), dysuria (9.7%), increased urinary frequency (5.1%), severe presentation of renal failure (n = 5, 2.1%), and urinary tract infection (n = 1, 0.4%) were presented. Most patients diagnosed with the condition underwent surgical treatment (83.5%), most of whom were treated via a hymenotomy (35.2%) and hymenectomy (36.4%), and the use of prophylactic antibiotics were only used in 7 patients. There were no differences in outcomes between two surgical methods. In addition, 141 (59.7%) patients showed improvement and 5 deceased patients were not related to IH or the operation itself; Complications, such as vaginal adhesion, were only noted in 6.6% of patients. In addition, among 17 cases of newborns with a diagnosis of IH before birth, hymenectomy (n = 5, 29.4%) and hymenotomy (n = 9, 52.9%) were the main treatment modalities and showed improved prognosis in 52.9% of newborns. Because IH diagnosis is easy and postsurgical prognosis is good, clinicians should carefully examine every female patient at birth. IH should be considered regarding adolescent girls with abdominal pain, lower back pain, or urinary retention, and perform appropriate physical examinations of the genital introitus. In addition, accurate diagnosis as IH, not misdiagnosing as vaginal septum or agenesis, is important to prevent severe complications such as stricture and ascending infection.
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Affiliation(s)
- Keum Hwa Lee
- Department of Pediatrics, Yonsei University College of Medicine, Yonsei-ro 50, Seodaemun-gu, C.P.O. Box 8044, Seoul 120-752, Korea.
- Division of Pediatric Nephrology, Severance Children's Hospital, Seoul 03722, Korea.
| | - Ji Sun Hong
- Yonsei University Wonju College of Medicine, Wonju 26426, Korea.
| | - Hyuk Jun Jung
- Yonsei University College of Medicine, Seoul 120-752, Korea.
| | - Hyun Ki Jeong
- Yonsei University College of Medicine, Seoul 120-752, Korea.
| | - Seo Jin Moon
- Yonsei University College of Medicine, Seoul 120-752, Korea.
| | - Woo Hyun Park
- Yonsei University College of Medicine, Seoul 120-752, Korea.
| | - Yoon Mi Jeong
- Yonsei University College of Medicine, Seoul 120-752, Korea.
| | - Seung Won Song
- Yonsei University College of Medicine, Seoul 120-752, Korea.
| | - Yongjune Suk
- Yonsei University College of Medicine, Seoul 120-752, Korea.
| | - Min Ji Son
- Yonsei University College of Medicine, Seoul 120-752, Korea.
| | - Jae Jung Lim
- Yonsei University College of Medicine, Seoul 120-752, Korea.
| | - Jae Il Shin
- Department of Pediatrics, Yonsei University College of Medicine, Yonsei-ro 50, Seodaemun-gu, C.P.O. Box 8044, Seoul 120-752, Korea.
- Division of Pediatric Nephrology, Severance Children's Hospital, Seoul 03722, Korea.
- Institute of Kidney Disease Research, Yonsei University College of Medicine, Seoul 03722, Korea.
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Successful preterm pregnancy in a rare variation of Herlyn-Werner-Wunderlich syndrome: a case report. BMC Pregnancy Childbirth 2018; 18:498. [PMID: 30558561 PMCID: PMC6296152 DOI: 10.1186/s12884-018-2133-2] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/16/2018] [Accepted: 12/03/2018] [Indexed: 11/10/2022] Open
Abstract
Background Herlyn–Werner-Wunderlich syndrome (HWWS) is an uncommon congenital anomaly of the female urogenital tract, characterised by uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. We reported the difficult pregnancy course complicated by an extremely rare and unique case of this syndrome associated with ectrodactyly, a clinical combination never described in literature. Case presentation A 28- year-old nulliparous woman previously diagnosed for HWWS associated with ectrodactyly of the right foot and with a history of abdominal left hemi-hysterectomy, ipsilateral salpingectomy, vaginal reconstruction when she was an adolescent. She suffered from threats of abortion in the first trimester, recurrent urinary tract infections during all pregnancy. At 33 weeks + 5 days of gestational age, she was hospitalized for premature rupture of the membranes and uterine contractions and a caesarean section was performed because of breech presentation. Postpartum period was complicated by a pelvic abscess resolved with parental antibiotic therapies. Conclusions Our literature review shows an unusual aspect in our case: HWWS is not classically associated with skeletal anomalies. Moreover, the most frequent urogenital side affected is the right, not left side as in this woman. Preterm spontaneous rupture of membranes and fetal abnormal presentation represent frequent complications and probably post-caesarean infections are related to pregnancies in the context of this syndrome.
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Smith AL, Kolon TF, Canning DA, Weissbart SJ. Partially Obstructed Longitudinal Vaginal Septum Presenting in Adulthood With Complaint of Urinary Incontinence. Urology 2018; 124:302-305. [PMID: 30471368 DOI: 10.1016/j.urology.2018.11.018] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/28/2018] [Revised: 11/05/2018] [Accepted: 11/13/2018] [Indexed: 11/26/2022]
Abstract
A longitudinal vaginal septum can be obstructive or nonobstructive. We report on an adult woman who presented with involuntary fluid loss per vagina and had a partially obstructive longitudinal vaginal septum. A 36-year-old nulliparous female presented with malodorous, clear, leakage per vagina that she described as "urinary incontinence." Examination revealed a fluid-filled fluctuant anterior vaginal wall with a draining sinus. Imaging revealed a solitary right kidney with duplicated ectopic fluid-filled ureters inserting into a partially obstructed left hemivagina with a longitudinal vaginal septum. A longitudinal vaginal septum may present in adulthood with the complaint of urinary incontinence.
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Affiliation(s)
- Ariana L Smith
- University of Pennsylvania, Division of Urology, Philadelphia, PA.
| | - Thomas F Kolon
- University of Pennsylvania, Division of Urology, Philadelphia, PA
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Jia G, Chai W, Cui M, Wen Y, Cui L, Gong F. A case report on Herlyn-Werner-Wunderlich syndrome with spontaneous abortion. Medicine (Baltimore) 2018; 97:e12004. [PMID: 30200079 PMCID: PMC6133451 DOI: 10.1097/md.0000000000012004] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/14/2018] [Accepted: 07/19/2018] [Indexed: 11/25/2022] Open
Abstract
INTRODUCTION Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare congenital abnormality of the urogenital tract characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. It is usually diagnosed after menarche, with a clinical presentation of dysmenorrhea, recurrent abdominal pain, and irregular menses. However, it is rare to diagnose it during pregnancy, subsequently resulting in spontaneous abortion. CASE PRESENTATION A 22-year-old Chinese woman with HWWS whose left uterine pregnancy underwent spontaneous abortion presented with a right perforated obstructed hemivagina and right renal agenesis. The right vaginal septum was resected and the hematocolpos was drained, thereby relieving lower abdominal pain and preserving future fertility. CONCLUSION Co-presentation of unilateral renal agenesis and uterus didelphys should encourage clinicians to rule out HWWS. Early diagnosis and subsequent treatment can avoid possible serious complications.
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Affiliation(s)
- Guifeng Jia
- Department of Gynecology and Obsterics, The First Hospital of Jilin University, Changchun, Jilin, China
| | - Wei Chai
- Department of Gynecology and Obsterics, The First Hospital of Jilin University, Changchun, Jilin, China
| | - Miao Cui
- Eastgene Laboratory, Flushing, New York
| | - Yan Wen
- Department of Gynecology and Obsterics, The First Hospital of Jilin University, Changchun, Jilin, China
| | - Lifeng Cui
- Department of Gynecology and Obsterics, The First Hospital of Jilin University, Changchun, Jilin, China
| | - Fengyan Gong
- Department of Gynecology and Obsterics, The First Hospital of Jilin University, Changchun, Jilin, China
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Friedman MA, Aguilar L, Heyward Q, Wheeler C, Caldamone A. Screening for Mullerian anomalies in patients with unilateral renal agenesis: Leveraging early detection to prevent complications. J Pediatr Urol 2018; 14:144-149. [PMID: 29459133 DOI: 10.1016/j.jpurol.2018.01.011] [Citation(s) in RCA: 33] [Impact Index Per Article: 4.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/14/2017] [Accepted: 01/09/2018] [Indexed: 10/18/2022]
Abstract
BACKGROUND Mullerian anomalies have a known association with renal agenesis yet, to date, there are no formal recommendations for screening women with certain renal anomalies for associated genital tract disorders. OBJECTIVE The objective of this study is to review current data regarding the association between renal and Mullerian anomalies, and propose screening recommendations. STUDY DESIGN A comprehensive review of the literature was performed to identify relevant articles using the keywords "unilateral renal agenesis," "renal anomalies," and "Mullerian anomalies." RESULTS Over 30% of patients with unilateral renal agenesis have an associated Mullerian anomaly. However, diagnosis is frequently delayed in this population until after menarche when complications of retrograde menstruation with obstructive anomalies lead to significant problems including endometriosis, pelvic inflammatory disease, and infertility. No clear guidelines exist for communication among the antenatal sonographer, the obstetrician, the parents, and the child's pediatrician, which creates a barrier to effective screening and follow-up. Further, no current guidelines exist for screening women with certain renal anomalies for Mullerian anomalies. DISCUSSION The complications of Mullerian anomalies are easily preventable if identified early. We propose new guidelines for education and screening for Mullerian anomalies in patients with unilateral renal agenesis (URA) and multicystic dysplastic kidney (MCDK) to guide providers, patients, and parents on proper identification and management (Table). CONCLUSIONS Screening young women with URA and MCDK for Mullerian anomalies has the potential to prevent long-term complications from untreated obstructive malformations. Identification of unilateral renal agenesis on antenatal ultrasound must be clearly articulated with parents and the child's pediatrician so that proper screening can be performed before menarche. Pelvic sonography is a low-cost, high-yield screening tool to identify these anomalies.
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Affiliation(s)
- M Alexandra Friedman
- Brown University, Department of Obstetrics and Gynecology, The Warren Alpert Medical School, Providence, RI, USA.
| | - Liza Aguilar
- Brown University, Division of Pediatric Urology, Hasbro Children's Hospital, The Warren Alpert Medical School, Providence, RI, USA
| | - Quetrell Heyward
- Brown University, The Warren Alpert Medical School, Providence, RI, USA
| | - Carol Wheeler
- Brown University, Department of Obstetrics and Gynecology, Division of Reproductive Endocrinology and Infertility, The Warren Alpert Medical School, Providence, RI, USA
| | - Anthony Caldamone
- Brown University, Division of Pediatric Urology, Hasbro Children's Hospital, The Warren Alpert Medical School, Providence, RI, USA
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Noviello C, Romano M, Nino F, Martino A, Cobellis G. Clinical and radiological findings for early diagnosis of Herlyn-Werner-Wunderlich syndrome in pediatric age: experience of a single center. Gynecol Endocrinol 2018; 34:56-58. [PMID: 28562147 DOI: 10.1080/09513590.2017.1332178] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 10/19/2022] Open
Abstract
OBJECTIVE The authors present their experience in the management of pediatric patients with Herlyn-Werner-Wunderlich syndrome (HWWS) considering clinical classification and anatomical characteristics of the malformation. METHODS All the data of the patient presented at our Pediatric Surgery Unit from February 2010 to August 2015 were collected. According to the type of malformations, patients were divided in 3 groups: A (completely obstructed hemivagina), B (incompletely obstructed hemivagina), and C (communication between the duplicated cervices). RESULTS Six patients were treated in the study period. The mean age was 9 years (2 months-15 years). According to the characteristics of the HWWS, we had 5 patients in group A, 1 in group B, and none in group C. One-stage surgical treatment was performed in all cases of complete obstruction, but in one case a second look was necessary for a better resection of the septum. At a mean follow-up of 18 months all patients were symptoms free. CONCLUSIONS Prognosis of this malformation is good in case of early diagnosis and treatment. We suggest that when a renal agenesia is diagnosed, the patient needs an ultrasonographic follow-up of the contralateral kidney but also of the genital tract to find each minimal abnormalities, furthermore, a MRI scanning before the onset of menstruation can be necessary.
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Affiliation(s)
- Carmine Noviello
- a Pediatric Surgery Unit , Salesi Children's Hospital, Università Politecnica delle Marche , Ancona , Italy
| | - Mercedes Romano
- a Pediatric Surgery Unit , Salesi Children's Hospital, Università Politecnica delle Marche , Ancona , Italy
| | - Fabiano Nino
- a Pediatric Surgery Unit , Salesi Children's Hospital, Università Politecnica delle Marche , Ancona , Italy
| | - Ascanio Martino
- a Pediatric Surgery Unit , Salesi Children's Hospital, Università Politecnica delle Marche , Ancona , Italy
| | - Giovanni Cobellis
- a Pediatric Surgery Unit , Salesi Children's Hospital, Università Politecnica delle Marche , Ancona , Italy
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Lui CT, Chan TWT, Fung HT, Tang SYH. A Retrospective Study on Imperforate Hymen and Haematometrocolpos in a Regional Hospital. HONG KONG J EMERG ME 2017. [DOI: 10.1177/102490791001700503] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022] Open
Abstract
Objective To find out the characteristics and presentations of imperforate hymen in the local population in Hong Kong and to assess if diagnosis of imperforate hymen made in the emergency department can reduce time to operation and length of hospital stay. Design Retrospective study. Setting A regional public hospital in Hong Kong. Patients We retrospectively collected data of all patients with the diagnosis of imperforate hymen (ICD 9 coding 752.42) from the period of January 1999 to June 2009. Demographics of the patients, their presenting symptoms and signs, the diagnostic process, investigation findings and time of operation were recorded and analysed. Results Fifteen cases of imperforate hymen were reported during that period. All were adolescent girls aged from 10 to 15 years. A total of 13 patients presented to the accident and emergency department (AED). Seven patients had the diagnosis made in the AED and 2 patients received bedside pelvic ultrasound. More than half of them (8 patients) presented with acute retention of urine. Other presentations included lower abdominal pain, constipation, lower abdominal mass, and protruding introital mass. Most cases diagnosed in the AED (5 out of 7) were admitted to the gynaecology ward while the others were admitted to the surgical or urology wards. Five out of the 7 cases diagnosed in the AED received operation within 24 hours; whereas only 1 out of the 6 cases with the diagnosis made after admission had operation within 24 hours. The difference was statistically significant (p=0.035, Fisher's exact test). The mean length of stay of the group diagnosed in the AED was 1.9 days while the mean length of stay for the group diagnosed after admission was 4.2 days. Conclusion Early diagnosis of imperforate hymen and haematometrocolpos in the AED for adolescent girls with primary amenorrhoea could have positive impact on proper admission to the gynaecology ward, prompt operation and shorter length of stay in hospital.
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Hsu HW, Teng YH, Yeh CB, Ho SW. Young Girl With Abdominal Pain. Ann Emerg Med 2017; 70:e39-e40. [DOI: 10.1016/j.annemergmed.2017.04.025] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/23/2017] [Indexed: 11/29/2022]
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Zhang H, Qu H, Ning G, Cheng B, Jia F, Li X, Chen X. MRI in the evaluation of obstructive reproductive tract anomalies in paediatric patients. Clin Radiol 2017; 72:612.e7-612.e15. [DOI: 10.1016/j.crad.2017.02.002] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/08/2016] [Revised: 01/23/2017] [Accepted: 02/02/2017] [Indexed: 10/20/2022]
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Cosgrove P, Kahlden K, Barr L, Sanchez J. Obstructed hemivagina with ipsilateral renal agenesis (OHVIRA) syndrome with imperforate anus. JOURNAL OF PEDIATRIC SURGERY CASE REPORTS 2016. [DOI: 10.1016/j.epsc.2016.06.015] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022] Open
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Löllgen RM, Sabo J, Mettler A, Liniger B, Berger S. Unique Presentation of Hematometrocolpos Mimicking Cauda Equina Syndrome: Severe Back Pain and Urinary Incontinence in an Adolescent Girl. J Emerg Med 2016; 51:e19-23. [DOI: 10.1016/j.jemermed.2016.01.031] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/05/2015] [Revised: 12/16/2015] [Accepted: 01/22/2016] [Indexed: 12/18/2022]
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48
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Lai TS, Wong JW, Carney ME. Robotic Assisted Resection of a Non-Communicating Uterine Horn Ectopic Pregnancy. J Gynecol Surg 2016. [DOI: 10.1089/gyn.2015.0127] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/12/2022] Open
Affiliation(s)
- Tiffany S. Lai
- Department of Obstetrics, Gynecology, and Women's Health, John A. Burns School of Medicine, University of Hawaii, Honolulu, HI
| | | | - Michael E. Carney
- Department of Obstetrics, Gynecology, and Women's Health, John A. Burns School of Medicine, University of Hawaii, Honolulu, HI
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Obstructed hemivagina with pyocolpos: An unusual presentation after delivery. PERINATOLOGÍA Y REPRODUCCIÓN HUMANA 2016. [DOI: 10.1016/j.rprh.2016.06.001] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022] Open
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50
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Shah P, Sindhu P, Shah F, Shah P, Patel R. Herlyn–Werner–Wunderlich Syndrome: Clinical Presentation and Outcome After Repeat Surgery. J Gynecol Surg 2016. [DOI: 10.1089/gyn.2015.0041] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/13/2022] Open
Affiliation(s)
- Pragnesh Shah
- Department of Obstetrics and Gynecology, Jyoti Hospital and Minimally Invasive Surgery Centre, Ahmedabad, Gujarat, India
| | - Preeti Sindhu
- Department of Obstetrics and Gynecology, Jyoti Hospital and Minimally Invasive Surgery Centre, Ahmedabad, Gujarat, India
| | - Foram Shah
- Department of Obstetrics and Gynecology, B.J. Medical College, Ahmedabad, Gujarat, India
| | - Parul Shah
- Department of Obstetrics and Gynecology, Jyoti Hospital and Minimally Invasive Surgery Centre, Ahmedabad, Gujarat, India
| | - Ronak Patel
- Department of Radiology, Maulana Azad Medical College, New Delhi, India
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