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The obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome, a rare Mullerian duct anomaly, is a triad of uterine anomalies with ipsilateral renal agenesis and obstructed hemivagina. The aetiopathogenesis of this developmental anomaly is debatable, with several theories being postulated to explain its occurrence. We report two cases of this rare syndrome which were detected on imaging done for unrelated reasons. Case 1 is a 24-year-old woman who presented with primary infertility. After an incidental detection of the syndrome and ruling out a male factor, she has been planned for excision of the vaginal septum. Case 2 is a 47-year-old woman with OHVIRA detected during the evaluation of urolithiasis and is asymptomatic for the syndrome. The OHVIRA syndrome can be asymptomatic or may present as haematocolpos, pelvic pain and infertility which requires surgical intervention, after which successful pregnancies have been reported.

Aim To analyze the clinical characteristics and management of patients with complete septate uterus, double cervix, obstructed hemivagina, and ipsilateral renal agenesis. Methods This retrospective study reviewed the medical records of 17 patients with complete septate uterus, double cervix, obstructed hemivagina, and ipsilateral renal agenesis admitted to the Third Xiangya Hospital of Central South University between June 2007 and December 2019. Results The median age at surgery was 23 years. The most common presenting complaint was infertility. Seven (41.2%) patients were misdiagnosed previously. The obstruction was complete in five patients. All 17 patients underwent vaginoplasty, in which seven adolescent girls underwent vaginoscopic vaginal septum resections. Eight patients underwent hysteroscopic resections of the uterine septum for infertility or spontaneous miscarriage. Laparoscopy was performed in seven patients for specific indications, and only one patient was found to have pelvic endometriosis during the laparoscopy. During the follow-up, seven patients wished to conceive, and there were five living infants (four cesarean deliveries at term and one preterm vaginal delivery). Conclusion Complete septate uterus with double cervix, obstructed hemivagina, and ipsilateral renal agenesis is an variant of obstructed hemivagina and ipsilateral renal agenesis syndrome. Consequently, healthcare providers should be aware of this potential variant.

Study Objective To propose a “3O” (obstruction, ureteric orifice, and outcome) subclassification system associated with obstructed hemivagina and ipsilateral renal anomaly (OHVIRA). Design Retrospective case series. Setting Xiangya Hospital, Central South University, Changsha, Hunan, China. Participants A total of 26 women with obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) over a 9-year period. Interventions Not applicable. Main Outcome Measures In all cases, the obstruction, ureteric orifice, outcome and surgical strategy were reviewed. Results In our study, the “obstruction” category included 14 cases of blind hemivagina, 8 cases of buttonhole septum, 3 cases of cervical fistula, and 1 case of cervical atresia. A total of 25 patients with vaginal obstruction underwent resection of the vaginal septum. The patient with cervical atresia underwent a failed cervicoplasty, followed by hemi-hysterectomy. The “ureteric orifice” category included 24 cases of absent ureter with no orifice, as well as 2 cases of ureteric orifice emptying into the obstructed hemivagina. The 2 patients were treated with laparoscopic extirpation of the ectopic ureter and renal moiety. Regarding the “outcome” category, 5 patients with severe recurrent hematometra, hematosalpinx, and ovarian endometrioma underwent hemi-hysterectomy, salpingectomy, and cystectomy of the ovarian endometrioma. Both patients (1 with a septate uterus and 1 with a bicornuate uterus) who experienced recurrent abortion accepted uterine correction. Conclusion We provide new insights into the anatomical variants of this rare syndrome with the relevant surgical implications. Magnetic resonance imaging is the most useful tool in 3O diagnosis.

Aim To review the experience of a single tertiary center with obstructed hemivagina and ipsilateral renal agenesis (OHVIRA syndrome) and evaluate if MRI findings correlate with outcome. Methods Patients (n = 32) diagnosed with OHVIRA syndrome between 2001 and 2019 were analyzed. Presenting symptoms, age of menarche, age at operation, MRI findings, management, and outcome were reviewed. In sagittal MRI planes, distance from hematocolpos to perineum was measured. Measurements were compared among patients who underwent single-stage vaginoplasty and hemihysterectomy. Results Mean age at diagnosis and mean age of menarche was 16.8 ± 6.4 (10−33) and 12.8 ± 1.0 (10–15). Main presenting complaints were abdominal pain/dysmenorrhea. Eighteen anomalies (56.3%) were on right side. Twenty-eight had ipsilateral renal agenesis, 3 patients had normal renal anatomy and one had unilateral multicystic dysplastic kidney. In MRI, 21 patients had hematocolpos, 11 patients had both hematocolpos and hematometra. 28 patients underwent single-stage vaginoplasty and vaginal septum resection. One had hemihysterectomy due to sepsis at presentation. Three patients had hemihysterectomy due to proximal vaginal septum and impossibility of vaginoplasty. During follow-up, 7 cases (21.9%) had married and 5 of these (71.4%) were pregnant or had delivered. Five patients had reoperation during follow-up. MRI images of 19 patients indicated mean distances from hematocolpos to perineum whom underwent vaginoplasty or hemihysterectomy were 33.9 ± 18.1 mm (10–79 mm) and 87.3 ± 11.0 mm (80–100), respectively (p = .009). Conclusions Gold standard treatment of OHVIRA syndrome is single-stage vaginoplasty. Distance from hematocolpos to perineum in MRI may correlate with surgical outcome. Hemihysterectomy may be an alternative for extreme proximal vaginal septum or infectious complications.

Herlyn-Werner-Wunderlich syndrome, a rare Mullerian duct anomaly, includes a triad of uterine didelphys, obstructed haemivagina and ipsilateral renal agenesis. A 58-year-old woman with Herlyn-Werner-Wunderlich syndrome, reported of recurrent genital bleeding for 9 years, was finally diagnosed with endometrial cancer. She had a history of vaginal septum resection and nephrectomy of atrophic right kidney. MRI demonstrated uterine didelphys, a tumour filling the left uterus and a cyst on the right lateral side of the uterus. Robot-assisted hysterectomy, including bilateral salpingo-oophorectomy and pelvic lymphadenectomy, was performed. As the cyst communicated with the right cervix, but not with the urinary tract, a Gartner duct cyst was diagnosed. Uncertain diagnosis and delay of treatment in endometrial cancer may occur in patients with Herlyn-Werner-Wunderlich syndrome. We should preoperatively fully evaluate the anatomy of the uterus and surrounding tissues and plan surgical procedures, especially in patients with urogenital malformations.

Background Herlyn-Werner-Wunderlich syndrome is a rare congenital anomaly characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. The most common presentation is abdominal pain, dysmenorrhea, and abdominal mass secondary to hematocolpos. We present the first case report on Herlyn-Werner-Wunderlich syndrome from Bangladesh. Case presentation A 15-year-old Asian girl presented with lower abdominal pain of 3 months’ duration. She had had menarche 3 months earlier and had a regular menstrual cycle with cyclical abdominal pain. Abdominal examination found a tender mass on the right iliac fossa. Further evaluation with ultrasound revealed distended endometrial cavity filled with complex fluid and nonvisualization of the right kidney. Pelvic magnetic resonance imaging showed absent right kidney and two separate endometrial stripes surrounded by endometrium and a muscular layer. The right endometrial cavity and cervix were distended with blood. This magnetic resonance imaging finding is consistent with Herlyn-Werner-Wunderlich syndrome with uterine didelphyis, right-sided hematometra resulting from obstructed hemivagina, and ipsilateral agenesis of the right kidney. The vaginal septum was resected for vaginoplasty. She was discharged 5 days after surgery and came for follow-up after 7 days. Vaginal examination revealed a healthy wound with no adhesion of the vaginal wall. She also informed us that she had started regular menstruation without any pain 30 days after the operation. Conclusion An unusual presentation of regular menstruation and nonspecific abdominal pain delays the diagnosis, which can lead to complications such as endometriosis and infertility. Awareness is required; otherwise, misdiagnosis clearly can occur.

Objective Herlyn-Werner-Wunderlich syndrome (HWW) is a rare congenital malformation of the urogenital tract due to a fusion failure in the Mullerian ducts. This anomaly consists of a didelphus uterus with obstructed hemivagina and sometimes associated with ipsilateral renal agenesis. The treatment of choice is surgical, it consists of a simple procedure of resection of the vaginal septum and drainage of the obstructed hemivagina and retained collections. Case report We report the case of a pregnancy in a 37-year-old woman with SHWW without resection of the vaginal septum. Conclusions The early detection is important due to the possible associated complications. Women with uterine defects are subject to an increased risk of complications in pregnancy and childbirth. Therefore, each case must be treated individually.

This report addresses and discusses two cases of uterine didelphys in pregnancy. The first case describes the diagnosis, management and subsequent pregnancies in a 28-year-old woman, para 2, with known didelphys uterus, left-obstructed hemi-vagina and ipsilateral renal agenesis. This uterine anomaly was diagnosed at 13 years of age, after pelvic imaging identified a haematocolpos and two uteri. To drain this haematocolpos, a hymenectomy was performed. In the second case, an incidental finding of uterine didelphys and vaginal septum in a 28-year-old primigravida is described. Both patients delivered healthy male infants at term via emergency and planned lower segment caesarean sections, indicating women with major uterine anomalies can have successful obstetric outcomes.

Objective To demonstrate a systematic approach for the hysteroscopic management of a patient with uterine septum with double cervix and a nonobstructive longitudinal vaginal septum (Class U2bC2V1 of the ESHRE/ESGE classification). Design A step-by-step demonstration of the technique with narrated video footage of three different cases. Setting University Hospital. Patient(s) We present three patients diagnosed with uterine septum with double cervix and a nonobstructive longitudinal vaginal septum (Class U2bC2V1 of the ESHRE/ESGE classification). Case 1 is a 31-year-old woman with class U2bC2V1 mullerian anomaly and primary infertility. Case 2 is a 29-year-old patient with class U2bC2V1 mullerian anomaly, infertility, and dyspareunia. Case 3 is a 32-year-old patient with class U2bC2V1 mullerian anomaly, infertility, and abnormal uterine bleeding. She also was diagnosed with a submucous leiomyoma. Intervention(s) Hysteroscopic management of this complex mullerian anomaly using miniaturized hysteroscopic instruments, including the mini-resectoscope, was performed. All three procedures were performed in the operating room with the patient under general anesthesia. No complications were encountered. Patients were discharged home the same day of the procedure after a short period of observation. The critical aspects of the procedure are highlighted. Main Outcome Measure(s) Description of the systematic approach with a detailed demonstration of the critical steps of the procedure. Result(s) Complete resolution of the vaginal septum with creation of a single uterine cavity was obtained in all three cases. Absence of intrauterine adhesion formation was confirmed. Conclusion(s) Due to the recent innovations in hysteroscopic equipment and improved surgical techniques, the hysteroscopic management of patients with complex mullerian anomalies using miniaturized instruments is a feasible and effective treatment option.

The ADAMTS (A Disintegrin and Metalloproteinase with Thrombospondin Motifs) enzymes are secreted metalloproteinases with major roles in development, morphogenesis, and tissue repair via the assembly and degradation of extracellular matrix (ECM). In this study, we investigated the role of ADAMTS18 in the development of the reproductive tract in female mice by phenotyping Adamts18 knockout (Adamts18-/-) mice. The results showed that Adamst18 mRNAs were abundantly expressed in vaginal epithelial cells and muscularis cells of the developing vagina. At the time of vaginal opening (5 weeks of age), about 41 % of Adamts18-/- females showed enlarged protrusions in the upper and middle parts of the vagina, reduced vaginal length, and simultaneously exhibited vaginal atresia. 6% Adamts18-/- females exhibited vaginal septum. Histological analyses revealed that the paired Mullerian ducts in ∼33 % female Adamts18-/- embryos failed to fuse at embryonic day 15.5 (E15.5) resulting in the formation of two vaginal cavities. Results of TUNEL assay and immunohistochemistry for caspase-3 showed that the number of apoptotic cells in the terminal portion of the vagina of 5-week-old Adamts18-/- females with vaginal atresia was significantly decreased. Adamts18-/- females also showed a significant decrease in serum estradiol E2 compared to age-matched Adamts18+/+ females. Results of qRT-PCR showed that the expression level of the anti-apoptosis gene Bcl-2 was significantly increased and that of the apoptosis-related gene Epha1 was decreased in the vagina of 5-week-old Adamts18-/- females. These results suggest that ADAMTS18 regulates vaginal opening through influencing the fusion of Mullerian ducts and apoptosis of vaginal cells in mice.

Obstructed Hemivagina and Ipsilateral Renal Anomaly syndrome is a rare obstructive uterovaginal anomaly involving both mesonephric and paramesonephric ducts. It usually presents after menarche with non-specific symptoms like pelvic pain, dysmenorrhea, or paravaginal mass and examination findings of paravaginal or pelvic mass. Because of non-specific symptoms and signs, the diagnosis is usually overlooked, which leads to complications like endometriosis, tubo-ovarian abscess compromising patient’s fertility, and quality of life. Therefore, in presence of these nonspecific clinical features along with imaging findings of uterine didelphys and unilateral renal agenesis, this syndrome should be considered in the diagnosis. We present a case of a 17-year-old lady with Obstructed Hemivagina and Ipsilateral Renal Anomaly syndrome, diagnosed by finding of paravaginal mass on examination and uterine didelphys with ipsilateral renal agenesis in USG and managed successfully by resection of vaginal septum. Keywords: ipsilateral renal anomaly;paravaginal mass;uterovaginal anomaly.

* Abbreviations: MFCC: : Maternal Fetal Care Center MRI: : magnetic resonance imaging VSD: : ventricular septal defect A 12-week fetal sonogram in a 22-year-old gravida 1 para 0 pregnant woman was suggestive of multiple anomalies. She had previously been known to have a congenital uterine anomaly with both a vaginal septum and uterine septum that were removed 3 years before the current pregnancy. She had 2 cervices with 1 uterine cavity and normal bilateral fallopian tubes at the time of this spontaneous pregnancy. Her initial course was uncomplicated, but fetal ultrasonography at 12 weeks’ gestation showed a large anterior wall defect spanning both the chest and abdomen, with concern for possible ectopia cordis. The fetus was noted to have restricted movement within a subjectively small amniotic sac. Cell-free fetal DNA screening was low risk and amniocentesis was not performed. She was referred to the Maternal Fetal Care Center (MFCC) at 17 weeks’ gestation for further evaluation and counseling. Imaging at the MFCC at 17 weeks and 3 days of gestation included fetal ultrasonography, magnetic resonance imaging (MRI), and fetal echocardiography. Imaging findings included the following: a small portion of the cardiac apex was outside the chest, a small anterior diaphragmatic hernia, a large abdominal wall defect with liver and bowel herniation, and significant thoracic kyphosis (Fig 1). In addition, a complete amnion-chorion separation was noted (Fig 2, ultrasound scan; Fig 3, MRI). Fetal echocardiography showed a ventricular septal defect (VSD) with a mildly hypoplastic pulmonary valve and partial ectopia cordis with a small sternal defect with left cardiac ventricle herniation. Figure 1. Prenatal T2 magnetic resonance imaging at 17 weeks’ gestation demonstrating herniated liver (solid green arrow) and small herniation of the apex of the heart (dashed green arrow) through a diaphragmatic defect. Evisceration of the small bowel (solid yellow arrow) and colon (dashed yellow arrow) are also visualized. Demonstration of kyphoscoliosis (red arrow). Figure 2. Ultrasound image demonstrating …

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.

Study Objective To evaluate the outcomes of a new hysteroscopic technique for the treatment of a longitudinal vaginal septum. Design A retrospective study. Setting Arbor Vitae Center for Endoscopic Gynecology, Rome, Italy. Patients Thirty women who underwent hysteroscopic treatment for a vaginal septum. Intervention All enrolled patients underwent pelvic examination, hysteroscopy, and 2-dimensional ultrasound. In case of a diagnosis of a partial or complete uterine septum, magnetic resonance imaging or 3-dimensional ultrasound were performed, and surgical procedures were performed after diagnostic laparoscopy was used to better visualize the uterine fundus. All patients were treated with the technique using vaginal septum as uterine septum or the same methodology applied for a uterine septum. Measurements and Main Results The procedure was successfully accomplished in all patients, and longitudinal vaginal septa were totally resected. There were no perioperative complications, and in only 1 case, vaginal bleeding occurred because of an erroneous identification of the excision line that was easily remedied with coagulation by resectoscope. Dyspareunia has improved or completely disappeared in all patients. Conclusion The technique of vaginal septum as uterine septum for the resectoscope of a longitudinal vaginal septum appears to be safe and effective with good outcomes, even in terms of dyspareunia.

This study is to evaluate the efficacy and safety of vaginoscopic incision of oblique vaginal septum in adolescents with Obstructed hemi-vagina and ipsilateral renal agenesis (OHVIRA) syndrome. It is about Fourteen adolescents with OHVIRA syndrome managed by vaginoscopic incision of the oblique vaginal septum using a “No-touch” technique over an 8-year period. In all fourteen adolescents with OHVIRA the oblique vaginal septum was incised successfully without any intraoperative complications. Postoperative pain was unremarkable and each patient’s symptoms resolved postoperatively. The 3-month postoperative follow up office vaginoscopy revealed that the vaginal septum had not reformed nor was any vaginal stenosis noted. Vaginoscopic incision of the oblique vaginal septum using a “No-Touch” technique is a safe, minimally invasive, and effective approach for treating OHVIRA syndrome in adolescents with hematocolpos. This technique may be utilized to minimize disruption to the undeveloped vaginal wall and postoperative pain while providing excellent surgical visualization throughout the procedure.

Introducción. El síndrome de Herlyn-Werner-Wünderlich (HWW) es una asociación de tres alteraciones anatómicas urogenitales de baja incidencia. Las alteraciones müllerianas son poco comunes y suelen ser hallazgos incidentales, por lo que se encuentran subdiagnosticadas y poco se conoce su génesis o su relación con tener más probabilidad de expresión en el lado derecho. Reporte de caso. Se trata de una paciente de 17 años quien consultó por dolor abdominal hipogástrico de fuerte intensidad ligado a sangrado fétido. En el examen físico se encontró tabique vaginal y en las imágenes complementarias se evidenció útero didelfo y agenesia renal, diagnosticando así síndrome HWW. Igualmente, se documentó piómetra y hematocolpos que se manejaron con terapia de antibióticos; dada la adecuada respuesta al tratamiento, se dio egreso hospitalario con seguimiento por consulta externa, pues el manejo definitivo no se pudo proporcionar durante la estancia hospitalaria. Discusión. Una causa de abortos a repetición es la presencia de síndrome HWW; sin embargo, al ser correctamente diagnosticado, se puede dar un tratamiento definitivo que disminuya las perdidas concepcionales y colecciones hematopurulentas urogenitales. Conclusiones. El síndrome HWW es una malformación poco común que aumenta el riesgo de padecer cuadros infecciosos ginecológicos o colecciones hemáticas. El tratamiento definitivo consiste en el manejo quirúrgico del tabique vaginal que mejora la calidad de vida y disminuye el riesgo de morbilidades asociadas a la patología.

Summary Introduction Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare female urogenital tract malformation. Study objective To present 10 patients with OHVIRA treated at the clinical center. To perform a systematic review of OHVIRA case series related to the prevalence of anatomical variants, surgical interventions and endometriosis, and to compare them with our case series. Materials and methods Medical records from 10 OHVIRA patients treated between 2016 and 2020 were retrospectively reviewed. For the systematic review, PubMed and Web of Science were used to search for relevant studies. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were strictly followed. Results The most common anatomical variant includes left obstructed hemivagina (50.7%) with isolated hematocolpos or hydrocolpos (55.9%), uterus didelphys (82.9%), and ipsilateral renal agenesis (92.2%). Vaginal septectomy was the most common surgical approach (86.5%). Hemivaginectomy (2.2%), hemihysterectomy (4.2%), or total hysterectomy (0.7%) were also performed in several patients. Some subjects required salpingectomy (3.3%) or oophorectomy (1.8%). 7.5% of patients, mainly infants, did not require surgery due to the spontaneous resolution of hydrocolpos. Endometriosis was fortuitously found in 13.6% of the selected cases who underwent laparoscopy or laparotomy. Discussion The most common variant of OHVIRA includes isolated hematocolpos and a thick vaginal septum between adjacent hemivaginas. Endometriosis was present in approximately 14% of OHVIRA patients, but this number is probably underestimated. Routine laparoscopy is not required. However, all patients need further monitoring due to a higher risk of endometriosis. Based on the analyzed studies and our case series, vaginal septectomy is a sufficient surgical technique to relieve symptoms and prevent possible complications in most OHVIRA patients.

OBJECTIVE To demonstrate the advantage of using aqueous vaginal contrast and scheduled hematocolpos with magnetic resonance imaging (MRI) to improve the delineation of gynecologic anatomy and to recommend that this modality be considered in patients with complex mullerian anomalies. DESIGN Video demonstration of MRI adjuncts to improve visualization of gynecologic anatomy. SETTING Academic Hospital. PATIENT(S) A patient with obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) who presented for definitive surgical management. INTERVENTION(S) OHVIRA is a unilateral obstructed mullerian anomaly that presents typically after menarche with progressively worsening dysmenorrhea caused by progressive distension of the obstructed hemivagina and uterine horn. The definitive treatment for this anomaly is resection of the unilateral obstruction. When the obstructed hemivagina is within close proximity to the patent hemivagina, vaginal septum resection should be performed to relieve the obstruction successfully. However, when the obstructed hemivagina and uterine horn are not adjacent to the patent hemivagina, a simple septum resection is not feasible and there is a high rate of restenosis if anastomosis is attempted. In this case, laparoscopic removal of the obstructed uterine horn, fallopian tube, cervix, and vagina should be considered as an alternative approach to resolving the obstruction. A surgical approach can be recommended only once the surgeon has a clear understanding of the patient's pelvic anatomy and the magnitude of the obstruction. In the presented case, a 17-year-old patient with OHVIRA presented for definitive surgical management. While on hormonal suppression, a pelvic MRI was performed that identified a uterus didelphys with a left hemiuterus and cervix communicating with a patent vagina. The right hemiuterus and cervix were measured 2.5 cm from the patent vagina. However, because of hormonal suppression, the vaginal cavity was decompressed, making it very difficult to discern the relationship between the two uteri and vaginas. To better determine whether vaginal septum resection to relieve the obstruction was feasible, norethindrone was discontinued to allow menstrual blood to fill the obstructed hemivagina followed by a subsequent pelvic MRI with aqueous vaginal contrast to fill the patent vagina with contrast gel to improve the visualization of the decompressed vaginal cavities. MAIN OUTCOME MEASURE(S) Advantage of aqueous vaginal contrast and scheduled hematocolpos with MRI to image pelvic anatomy in a patient with a complex mullerian anomaly to guide surgical decision-making. RESULT(S) The addition of vaginal aqueous contrast clearly delineated the course and caliber of the patent vagina and its relationship to the obstructed hemivagina, now filled with blood. The inferior margin was in closer proximity to the patent vagina, but with only a very narrow segment (

The coexistence of imperforate hymen and vaginal septum is rare and their ability to mimic malignant manifestations have not been frequently reported. This current case report describes a 13-year-old girl that presented with cyclic abdominal pain for 6 months. She was found to have a huge mass via abdominal plain film X-ray and sonography, with inexplicably high levels of serum carcinoembryonic antigen, cancer antigen (CA)-19-9 and CA-125. Pelvic computed tomography imaging disclosed two huge cystic lesions in the uterine and upper vaginal cavities. Surgical intervention conformed the diagnosis of a concurrent imperforate hymen and transverse vaginal septum, echoing the imaging findings of haematocolpometra. Her tumour marker levels gradually returned to normal after surgery. This rare case of concomitant imperforate hymen and transverse vaginal septum highlights that haematocolpometra, a benign disease that might mimic malignancy, should be taken into consideration in any adolescent females with an abdominal mass and amenorrhoea to ensure an early diagnosis and timely appropriate management.

Gel sonovaginography is a new way of assessing local cervical and vaginal disorders, in which regular transvaginal sonography is known to have limitations. In gel sonovaginography, 20 mL of ultrasound gel is instilled into the vagina, followed by examination with a transvaginal transducer. In a study involving 28 women with known or suspected disorders such as cervical and vaginal cancer, cervical polyps, vaginal septa, and deep infiltrating endometriosis, a substantial improvement in visualization and assessment of local lesions and structures was noted with gel sonovaginography. This simple technique appears to be valuable for accurate diagnosis of local cervical and vaginal disorders.

Objective To demonstrate various types of longitudinal vaginal septa (LVS), their classification, and the surgical management of typical and unique morphologic conditions of LVS. Design Video presentation of clinical appearance and surgical techniques for treatment of LVS. Setting University hospital and two private. Patient(s) Representative cases from 121 consecutive women treated from 2013 to 2018 with LVS as a part of complex uterovaginal malformations or in isolated forms with [1] typical morphologic configuration of LVS, [2] rarer variants, or [3] specific anatomic restrictions. Intervention(s) Resection of LVS performed as a main surgical procedure in cases with didelphys and bicornuate uterus in symptomatic women and as a part of corrective surgery of complete septate uterus. The three main nonsuturing techniques used were speculoscopy and septum excision using three different electrosurgical modalities; speculoscopy with laparoscopic devices; and vaginoscopy with hysteroscopic instruments. Main Outcome Measure(s) Clinical appearance and suggested classification, feasibility of surgery, and perioperative and anatomic results in a short follow-up period (3 months). Result(s) We identified distinct types of longitudinal vaginal septa. Considering clinical appearance, we suggest classification of LVS based on four main features: [2] completeness of vaginal division: partial and complete type; [2] the symmetricity: symmetric and asymmetric position (with dominant left and right side); [3] association with the cervix: merged and isolated forms; and [4] concomitant vaginal openings: normal, and narrow openings: vaginal stenosis and hymen persistent ( Fig. 1 ). Vaginoscopic techniques by hysteroscope were successful in atraumatic treatment of women with substantial anatomic restrictions, and all of the presented techniques can be effectively used for typical LVS. However, vessel-sealing systems allow for bloodless surgery in contrast with other methods. This study was based on previously acquired data during large prospective study approved by the local ethics committee, and written informed consent to participate in the prospective study and permit publishing anonymous data regarding the medical images, videos of procedures, and results was obtained from all patients. Conclusion(s) A new classification of longitudinal vaginal septum allows better characterization compared with the currently available classification systems. Different surgical modalities are discussed with their respective advantages and disadvantages. Vaginoscopic incision using resectoscope is a reasonable alternative for women with an intact hymen and vaginal stenosis. The impact of vaginal septum resection on obstetric, reproductive, and sexual outcomes should be assessed in randomized controlled trials and large well-designed studies.

Study objective To show our technique for hysteroscopical resection of a complete uterine septum. Design Step-by-step description of the procedure using pictures and video (educational video). The video was approved by our hospital's ethical committee. Setting Uterine malformations represent a rare yet usually asymptomatic condition that can be associated with poor obstetric outcomes. The ESGE-ESHRE classification is widely accepted for the description of female genital tract anomalies. Treatment of uterine septum should be considered if fertility is desired, being hysteroscopic resection the gold-standard procedure. Patient A patient with an U2bC2V1 malformation according to the ESHRE-ESGE classification was treated with hysteroscopy. Intervention The procedure was performed in an operative room under general anesthesia using a 9mm hysteroscope with a bipolar cutting loop. The surgical technique was started with the resection of the vaginal septum with monopolar electrosurgery until the visualization of both cervix. A Foley probe was placed in one uterine hemicavity and afterwards hysteroscopy on the other hemicavity was performed. Transrectal ultrasound guidance was used to identify the limits of the septum and therefore increase the safety of the procedure. Resection of the septum was started in its upper part until the Foley probe was seen, then continuing downwards until internal cervix orifice was reached. In the hysteroscopic follow-up after 3 months, we visualized a small residual septum that was resected to fully restore the uterine cavity and improve the patient's obstetric outcomes. Main results The procedure was completed without complications and the 2nd look hysteroscopy showed a normal uterine cavity. Conclusion The combination of real time US-guidance and the placement of an intrauterine balloon through one cervix may increase safety during the procedure as it allows a clear identification of both uterine cavities and septum borders while resection is performed.

The management of the obstructed hemivagina and ipsilateral renal anomaly, or OHVIRA syndrome, requires vaginal access compromising sometimes the hymenal integrity. A vaginoplasty is the most commonly performed procedure in such scenarios. However, the necessity to maintain the hymen integrity may limit this technique. In this case report, we describe a safe and successful laparoscopy-assisted resection for the management of a high vaginal septum while maintaining an intact hymen.

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.

Background The objective of this study was to report 2 cases of the combined congenital anomalies of complete vaginal atresia and partial cervical agenesis, and highlight the limitations of magnetic resonance imaging for definitive initial diagnosis, and consequently the importance of early definitive management, to avoid life-threatening sepsis. Herein we provide a retrospective case audit of two patients with congenital abnormalities between 2005 and 2013 who were treated in a quaternary statewide pediatric and adolescent gynecology center. Cases Two patients with the combined congenital anomalies of complete vaginal agenesis and partial cervical agenesis highlight the difficulties encountered with the limitations of magnetic resonance imaging in accuracy of diagnosis, as well as development of life-threatening sepsis that requires hysterectomy. Both patients were initially imaged as having distended endometrial cavities and cervical canals with what was thought to be an obstructive upper vaginal septum and absent lower vagina. Both required initial neovagina creation, however the cervices were never clinically or surgically visualized. Summary and Conclusion Partial cervical agenesis is a relatively rare form of Mullerian abnormality which, if not diagnosed and definitively treated early, can have significant morbidity and mortality. Although magnetic resonance imaging is the diagnostic imaging gold standard for Mullerian abnormalities, it is important to recognize the limitations of this modality, the potential sequelae of these limitations, and to appreciate the importance of early accurate diagnosis and treatment of this condition. Importantly, if the imaging diagnosis does not completely correlate with the clinical and surgical findings, then a high suspicion of complete or partial cervical agenesis is prudent, because the consequences of nondefinitive early treatment can be life-threatening and potentially fatal.

Children with anorectal malformations (ARM) constitute a significant group within a pediatric surgery practice. It is important with female cases of anorectal malformations to consider the association of gynecologic anomalies, especially at the time of the definitive repair. However, it is critical to consider the association of such gynecologic anomalies when caring for patients with a cloacal anomaly. If not recognized, an opportunity to diagnose and treat such anomalies may be missed with the possibility of negative implications for future reproductive capacity. With the knowledge of the associated anomalies and long-term sequelae, surgeons can provide better care for girls and important counseling for parents. Knowledge of reproductive related issues in females with cloaca allows the pediatric surgeon an opportunity both, to provide optimal surgical management in infancy, childhood, and into young adulthood and to collaborate medically and surgically with an experienced gynecologist in patients with more complex anatomic variations. Appropriate counseling for patients and families about potential reproductive concerns that may develop many years after the definitive surgical repair allows preparation and planning to preserve future fertility.

Aim To evaluate the diverse presentations of Herlyn–Werner–Wunderlich (HWW) syndrome on magnetic resonance imaging (MRI) and its surgical correlations. Materials and methods From 2014 to 2019, a total of 40 patients with HWW syndrome underwent pelvic MRI. All MRI images were reviewed by two experienced radiologists in consensus. Uterine anomalies, obstruction sites, laterality and communications, urinary anomalies, and other abnormal findings were recorded. MRI findings were correlated with ultrasonography and surgical results. Results On MRI, the uterus was didelphic in 25 (62.5%), septate in nine (22.5%) and bicornuate in six cases (15%); an ipsilateral vaginal septum was found in 30 cases (75%), and ipsilateral cervical obstruction (obliterated cervical os) was found in 10 cases (25%). Twenty-seven patients underwent surgical treatment. MRI diagnoses of obstruction sites correlated completely with the surgical results. Although obstruction communications could not be found with ultrasonography, MRI identified nine cases of cervical communication, but failed to detect the five cases of surgically confirmed fistula on the vaginal septum. Moreover, MRI identified nine cases of ipsilateral ureteral remnants and/or paravaginal cystic structures, all with insertion into the obstructed vagina or cervix, and one case of ipsilateral ureterocele. Conclusions HWW syndrome can present as different combinations of uterine anomalies, unilateral cervico-vaginal obstruction, and ipsilateral renal anomalies; ipsilateral paravaginal cystic structures and/or ureter remnants inserting into the obstructed vagina or cervix can occasionally be seen. MRI can provide a comprehensive preoperative evaluation and better identification of obstruction sites, but seems insensitive for detecting vaginal communications in HWW syndrome.

Transverse vaginal septum is a rare female genital tract anomaly, and little is described about its surgical treatment. We report the case of a patient who wished to preserve hymenal integrity due to social and cultural beliefs. We performed a vaginoscopic resection of the septum under laparoscopic view, followed by the introduction of a Foley catheter in the vagina, thus preserving the hymen. After 12 months of follow-up, no septal closure was present, and the menstrual flow was effective. Vaginoscopic hysteroscopy is an effective method of vaginal septum resection, even in cases in which hymenal integrity must be preserved due to social and cultural beliefs.Septo vaginal transverso é uma anomalia rara do trato genital feminino, e pouco é descrito sobre o tratamento cirúrgico. Relatamos o caso de uma paciente que desejava preservar a integridade do hímen devido a crenças sociais e culturais. Realizamos ressecção vaginoscópica do septo sob visão laparoscópica, seguida da introdução de um cateter de Foley na vagina, preservando assim o hímen. Após 12 meses de acompanhamento, não havia fechamento do septo, e o fluxo menstrual era eficaz. A histeroscopia vaginoscópica é um método eficaz de ressecção dos septos vaginais, incluindo os casos em que a integridade do hímen deve ser mantida devido a crenças sociais e culturais.

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.

Intensity-modulated radiotherapy (IMRT) is considered the preferred option in squamous cell canal cancer (SCAC), delivering high doses to tumor volumes while minimizing dose to surrounding normal tissues. IMRT has steep dose gradients, but the technique is more demanding as deep understanding of target structures is required. To evaluate genital marginal failure in a cohort of patients with non-metastatic SCAC treated either with IMRT or 3DCRT and concurrent chemotherapy, 117 patients with SCAC were evaluated: 64 and 53 patients were treated with IMRT and 3DCRT techniques, respectively. All patients underwent clinical and radiological examination during their follow-up. Tumor response was evaluated with response evaluation criteria in solid tumors v1.1 guideline on regular basis. All patients’ data were analyzed, and patients with marginal failure were identified. Concomitant chemotherapy was administered in 97 and 77.4% of patients in the IMRT and 3DCRT groups, respectively. In the IMRT group, the median follow-up was 25 months (range 6–78). Progressive disease was registered in 15.6% of patients; infield recurrence, distant recurrence and both infield recurrence and distant recurrence were identified in 5, 4 and 1 patient, respectively. Two out of 64 patients (3.1%) had marginal failures, localized at vagina/recto-vaginal septum and left perineal region. In the 3DCRT group, the median follow-up was 71.3 months (range 6–194 months). Two out of 53 patients (3.8%) had marginal failures, localized at recto-vaginal septum and perigenital structures. The rate of marginal failures was comparable in IMRT and 3DCRT groups (χ2 test p = 0.85). In this series, the use of IMRT for the treatment of SCAC did not increase the rate of marginal failures offering improved dose conformity to the target. Dose constraints should be applied with caution—particularly in females with involvement of the vagina or the vaginal septum.

Objectives To demonstrate the hymen-sparing management of a blind hemivagina in obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome with the use of transrectal ultrasound (TRUS)–guided vaginoscopic septoplasty supported by pre- and postoperative diagnostics with the use of a novel ultrasound technique: 3-dimensional saline-solution infusion contrast sonovaginocervicography (3D-SVC) with virtual speculoscopy. Design Video presentation of surgical and ultrasound techniques. Setting University hospital and two private centers. Patient(s) We are demonstrating four cases with blind hemivagina as a component of OHVIRA syndrome and varying level and features of obstruction including: 1) hemihydrocolpos; 2) hemihematocolpos; 3) "old blood" deposits in small hemivagina; and 4) narrow hymenal opening. Interventions(s) The patients were diagnosed preoperatively by means of 3D-SVC with the use of TRUS. Surgery was planned according to available data from ultrasound and 3D-SVC, and the place of incision of the vaginal septum and blinded hemivagina with cervix were performed with the use of TRUS guidance. Wide septal incision was performed with the use of a monopolar or bipolar resectoscope with needle Collin electrode, and after incision the occult second of double cervix or part of septate cervix was visualized, and the septum was excised with the use of a loop electrode. In narrow hymenal opening, a small diagnostic sheath was used for wide septal incision. Anatomic results in the vagina were assessed with the use of 3D-SVC 2 months after surgery. Main Outcome Measure(s) Agreement between imaging from preoperative diagnostics with the use of 3D-SVC and intraoperative findings, and anatomic (hymenal integrity, obstruction, status of vagina and cervix) and clinical outcomes (pain). Result(s) In these four cases, 3D-SVC accurately recognized the morphology of blind hemivagina, oblique vaginal septa, and double or septate cervix. Successful minimally invasive wide septoplasty with preservation of hymen were performed with the use of hysteroscope and TRUS guidance. Concomitant laparoscopy was performed if endometriosis and hematosalpinx were present. No peri- or late postoperative complications occurred. Patients were discharged within 3 hours or within 12 hours in case of laparoscopy. Anatomic results were optimal (lack of septum) or suboptimal (wide opening) after septum resection and incision, respectively, without recurrence of obstruction according to 3D-SVC. Pain was not noticed 2 months after the primary surgery. Conclusion(s) 3D-SVC is a useful and accurate technique in diagnosis, surgery planning, and postoperative assessment in women with blind hemivagina and intact hymen. TRUS-guided vaginoscopic septoplasty is a reasonable alternative to traditional vaginal surgery and allows hymen preservation.

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

Study Objective To compare a case series of the obstructed hemivagina, ipsilateral renal anomaly and uterine didelphys triad with the literature, with a focus on a subset of patients with cervical aplasia. Design, Setting, and Participants A retrospective case series was conducted of all patients with the triad managed between 2005-2013 at a tertiary center for adolescent gynecology. Results Similarities in this cohort, compared to the literature, included heterogeneity of presentation, presence of endometriosis, and asymmetry of affected side. Notable differences included 1 patient with a 16p11.2 microdeletion and 2 patients with subsequent unilateral cervical aplasia. All patients underwent magnetic resonance imaging for diagnosis. Vaginal septum division was performed in 8 cases and excision in 1 case. Both cases with cervical aplasia ultimately underwent hemi-hysterectomy, and highlight the implications of this rare variant in regards to its existence, limitations of magnetic resonance imaging in this context, and suggestions for improvement in diagnosis and management. Conclusion The complexity of these cases, especially the evolving manifestation of cervical aplasia postoperatively, illustrates the need to recognize limitations in imaging and divergence in definitive management.

Purpose of investigation To describe a novel approach for longitudinal vaginal septum (LVS) resection. Materials and Methods: Two cases of young girls with a uterus didelphys and a longitudinal vaginal septum. The technique consisted in grasping the vaginal septum with a laparoscopic 33-cm long bipolar cutting forceps, five-mm in diameter, and divided it to its midportion towards the two cervices. Results In both cases, the procedure was straightforward, uncomplicated, completed within three minutes and the patients were discharged four hours later. It was associated with minimal blood loss, short recovery time, absence of local ischemia, and optimum healing process. Conclusion The authors believe that surgical safety, efficacy and operative result make bipolar cutting forceps a tailored option for LVS resection.

Background Vaginal aplasia occurs in 1 in 5,000-10,000 female live births. In this report, we evaluated a novel dual-force vaginoplasty technique for treatment of 11 patients with segmental vaginal aplasia. Technique The principle of the approach is to thin the atretic part between two counteracting forces. The instrument was inserted laparoscopically into the proximal hematocolpos. Two balloon catheters, one for drainage and one for traction, were threaded over the inserter. The traction catheter was then threaded over a silicon tube, leaving the balloon in the proximal portion of the vagina and connecting across the vaginal septum to a fenestrated Teflon olive, which was positioned against the distal surface of the vaginal septum. This created a dual "pushing and pulling" force across the septum, which, over 3-4 days, pulls the upper vaginal pouch down while the vaginal dimple is pushed up. The aplastic segment becomes thin and easy to dilate and permits achievement of vaginal patency. The drainage of the hematocolpos is predominantly through the balloon catheter so postoperative wound management is facilitated. Experience Eleven menarchal girls were diagnosed with segmental vaginal aplasia. The dual-force vaginoplasty was performed on each and was tolerated well with no operative complications. They all reported establishment of the menstrual cycle and significant improvement of pain during follow-up. Conclusion Creation of a dual pushing-pulling force on the atretic vaginal segment is a feasible short procedure for management of segmental vaginal aplasia.

A girl born in 2006 has been under close surveillance in our pediatric urology unit since birth. Besides cloacal malformation, she has associated spinal cord dysraphism, uterus didelphys, and a vaginal septum.

Objective To describe the clinical characteristics of patients with Herlyn-Werner-Wunderlich syndrome (HWWS) and explore the relationship between an obstructive genital abnormality and pelvic endometriosis. Design Case series. Setting University hospital. Patient(s) A total of 94 women with Herlyn-Werner-Wunderlich syndrome (HWWS). Intervention(s) Retrospectively reviewed charts of patients diagnosed with HWWS and follow-up contact. Main Outcome Measure(s) Data for each patient regarding demographics, presenting symptoms, concomitant complications, and anatomic variations were obtained from operation notes and clinical records. Result(s) The occurrence of pelvic endometriosis was 19.15% (18/94) in patients with HWWS. All ovarian endometrial cysts (100%) occurred ipsilateral to the vaginal septum. Pelvic endometriosis was significantly more frequent in patients with complete hemivaginal obstructions (10/27, 37.0%) than those with incomplete obstructions (8/67, 11.9%). Conclusion(s) One-fifth of patients with HWWS were susceptible to pelvic endometriosis, and all of the ovarian endometriotic cysts were ipsilateral to the vaginal septum. Pelvic endometriosis in adolescents appeared to be related to the obstructed genital abnormality. Vaginal septum resection should be the first step in treatment and surgery has an important role in the treatment of endometriosis and pelvic adhesion.

Aim To outline the anatomical variations of obstructive reproductive tract anomalies (ORTA) using magnetic resonance imaging (MRI) and its role in preoperative evaluation. Materials and methods MRI and treatment of 21 paediatric patients with ORTA were reviewed and analysed. MRI findings were correlated with ultrasound and surgical findings. Results Patients presented in two distinct ways: primary amenorrhoea with cyclic pelvic pain, or progressive dysmenorrhoea. MRI showed haematocolpos, haematocervix, haematometra, and/or haematosalpinx; it also provided detailed information regarding uterine morphology, ipsilateral kidney absence, and endometriosis. Diagnosis at MRI of the obstruction sites correlated completely (100%) with the surgical diagnosis. Obstruction occurred at different levels of the genital tract, and surgical treatment was given based on the obstruction sites. One patient underwent excision of the hymen tissue for imperforate hymen. Four cases of lower vaginal atresia were treated with vaginoplasty. Three patients with typical Herlyn–Werner–Wunderlich (HWW) syndrome underwent resection of the vaginal septum, and one patient with concurrent post-partum placenta increta was treated accordingly; one patient with atypical HWW syndrome had the left uterus resected. There were 11 cases of cervical agenesis or cervicovaginal dysgenesis, eight of which were complicated with uterine anomalies, and in all cases the uterus was removed. Among the 10 obstructive cervical anomalies, there were three cases of cervical agenesis and seven cases of cervical dysgenesis, including five obliterated cervical os (cervical obstruction), one cervical fibrous cord, and one cervical fragmentation. Conclusion ORTA can occur from the hymen to the lower segment of the uterus and requires surgical intervention. The preoperative evaluation is vital to guide proper surgery. MRI, with its imaging advantages, is the imaging technique of choice to assess the obstructed sites and complicated anomalies of ORTA.

Introduction. Uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis represents a complex congenital anomaly, also known in the literature as Herlyn-Werner-Wunderlich (HWW) syndrome. Case Outline. A 16­year­old patient presented with secondary amenorrhoea and abdominal pain. Her history revealed anorexia nervosa; she had menarche at the age of 14; her menstrual cycles were regular, with progressive dysmenorrhoea. Nine months after the explorative laparotomy performed at the regional healthcare center there was no a definitive diagnosis. Pelvic examination showed a paravaginal pelvic mass located on the right side. Magnetic resonance imaging of the abdomen and pelvis confirmed the diagnosis of HWW syndrome. Transvaginal excision and marsupialisation of the vaginal septum were performed. Regular menstrual cycles were resumed after four years following the treatment of anorexia nervosa. Twelve years after the operation, the patient conceived spontaneously; pregnancy developed in the left non­obstructed uterus. She underwent Cesarean section at the 37th gestational week and gave birth to a healthy female infant. Conclusion. HWW syndrome is an uncommon cause of abdominal pain and progressive menstrual discomfort shortly after menarche. It is therefore essential to maintain a high index of suspicion of the existence of this syndrome in such cases, since prompt and adequate treatment prevents the development of complications and allows for preservation of the reproductive potential of both hemiuteri. [Projekat Ministarstva nauke Republike Srbije: Celularni i molekularni patogenetski mehanizmi poremecaja reprodukcije i genitalnih organa u prevenciji, dijagnostici i terapiji podprojekat, br. 41021: Tretmani antenatalnih hidronefroza]

The aim of this study was to evaluate the outcome of pregnancies in women who had uterine malformation and surgically treated obstructed hemivagina with ipsilateral renal agenesis.The study group comprised 21 women with malformed uterus (12 didelphic, 6 septate and 3 bicornuate uterus). All of them had a history of surgical excision of the longitudinal vaginal septum caused obstructed hemivagina and ipsilateral renal agenesis. All pregnancies and possible surgical interventions were evaluated during the follow-up period (median 13.2 years).Thirteen out of 21 women attempting pregnancy conceived. They produced 22 pregnancies, 17 (77 %) were contralateral to the treated obstructed hemivagina and unilateral renal agenesis. The median interval between surgical treatment of obstructed hemivagina and the first pregnancy was 10.5 years. Twenty (91 %) pregnancies ended in delivery of a living infant. Preeclampsia (14 %), preterm delivery (36 %), high frequency (38 %) of fetal breech presentation and the cesarean section rate (67 %) were found.Accurate diagnosis and appropriate surgery to open an obstructed hemivagina in adolescence assure fertility. Preterm birth is associated with malformed uterus and unilateral renal agenesis may predispose to preeclampsia.

Purpose. To determinate transvaginal scan (TVS) accuracy in the preoperative evaluation of deep endometriosis in a large cohort of patients with subsequent laparoscopic assessment. Methods. A retrospective study was performed in a tertiary referral center for endometriosis. Transvagi- nal scan reports were retrieved from an electronic database of all patients who underwent laparoscopy for pelvic pain or infertility in 2009. The accuracy of TVS was assessed for 10 different sites of pelvic endo- metriosis. Results. Four hundred twenty women were included in the study. Sensitivity and specificity of TVS were 61% and 99%, respectively, for bladder en- dometriosis, 52% and 96% for endometriosis of recto- vaginal septum, 65% and 99% for rectum endometrio- sis, and 69% and 98% for endometriosis of the sig- moid colon. Conclusions. TVS appears to be useful for the detection of endometriosis located in the bladder and involving the sigmoid colon, the rectovaginal septum, and the rectum. V

Introduction A significant number of internationally adopted children have congenital birth defects. As a specialist center for colorectal diagnoses, we evaluate such children with an anorectal malformation (ARM) and have found that a significant number need a reoperation. Knowledge of the common complications following ARM surgery has led us to develop treatment algorithms for patients with unknown past medical and surgical history, a situation typically encountered in the adopted population. Methods The results of investigations, indications, and rate of reoperation were assessed for adopted children with an ARM evaluated between 2014 and 2016. Results 56 patients (28 males) were identified. 76.8% required reoperative surgery. Mislocation of the anus outside the sphincter complex was seen in 50% of males and 39.3% of females. Anal stricture, rectal prolapse, retained vaginal septum, and a strictured vaginal introitus were also common. Conclusion The reoperative surgery rate in the internationally adopted child with an ARM is high. Complete, systematic evaluation of these children is required to identify complications following initial repair. Development of mechanisms to improve the primary surgical care these children receive is needed.

Background We report on an unusual presentation of Herlyn-Werner-Wunderlich syndrome in two 11-year-old girls within a year of menarche. The setting was a training and research hospital. Case We present two patients in the pubertal period with cyclic abdominal pain and urinary incontinence who received hysteroscopic septal resection. Menstrual flow was resumed and the complaints of incontinence were eliminated after the hysteroscopic resection of the vaginal septum. Summary and Conclusion Overflow incontinence was completely resolved after septum resection in two patients. The risk of stricture is high in Herlyn-Werner-Wunderlich syndrome if the septum is partially excised to open the obstruction, whereas the risk of stricture is low if a complete or wide excision is performed. In the presence of abdominal pain and urinary incontinence in puberty, Herlyn-Werner-Wunderlich syndrome must be considered in the differential diagnosis, and a detailed evaluation of the urinary system and pelvic anatomy must be performed.

BACKGROUND: Herlyn-Werner-Wunderlich syndrome is defined by uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. We report the presentation, radiographic findings, and outcomes of three patients with Herlyn-Werner-Wunderlich syndrome who underwent surgical management at a tertiary care academic medical center. CASES: Two patients were diagnosed by magnetic resonance imaging and one by ultrasonography. All three underwent successful transvaginal septoplasty and drainage of the hematocolpos and hematometra. One required septoplasty revision with temporary vaginal stent placement. CONCLUSION: Herlyn-Werner-Wunderlich syndrome is a rare condition. The most urgent concern is the obstruction of menstrual effluent. Unlike an isolated transverse vaginal septum, the vaginal septum with Herlyn-Werner-Wunderlich syndrome is parallel to the patent vaginal canal, less pliable, and closer to the cervix. These anatomical differences result in pronounced distortion with significant diagnostic and surgical challenges.

Background Urinary retention is rare in adolescence. Here I describe such a case in a patient who had transected her urethra in a motor vehicle accident at 3 years of age. The presumptive diagnosis of urethral stricture was proven incorrect. Objectives Both to present a complicated and unique case whose history pointed strongly to one diagnosis initially but turned out very differently than expected; and to give an example of the use of bedside ultrasonography in the Emergency Department as a diagnostic tool in an unusual circumstance. Case Report A 13-year-old girl was evaluated for 3 days of urinary retention. Her history of pelvic fracture and urethral transection in childhood suggested a urethral stricture. Her work-up revealed, however, a vaginal stricture and resultant hematocolpos compressing the urethra. Upon surgical excision, the stricture was confirmed to be scar tissue, rather than a coincidental congenital septum. Conclusion Hematocolpos is an uncommon but well-described cause of urinary retention in premenarchal females. This is a difficult diagnosis in any circumstance, and this patient’s complex history complicated the process. It was the bizarre bedside ultrasound that prompted further work-up than simple urethral catheterization and urologic follow-up, which her urologist had suggested over the phone. This patient’s case is unique in the literature.

Background Congenital vaginal obstruction commonly presents after puberty and presentation in infancy is uncommon. Due to lack of awareness, diagnosis may be missed in infants, sometimes with disastrous consequences. Method A retrospective review of 9 neonates and infants treated for congenital vaginal obstruction in 16 years. Results The patients were aged 2 days to 4 months (median 28 days) at presentation. The main presenting feature was a lower abdominal mass, producing intestinal and urinary obstruction in 7 patients. In 2 patients, other conditions were suspected and diagnosis made only intraoperatively and in one patient, diagnosis was incidental during treatment for another condition. The cause of vaginal obstruction were low transverse vaginal septum 6, imperforate hymen 2, high transverse vaginal septum one. Associated anomalies were present in 5 patients (persistent cloaca 3, Hirschsprung disease 1, polydactyly 1). Low vaginal septum was treated by incision and drainage of hydrometrocolpos, imperforate hymen by hymenotomy and drainage of hydrometrocolpos and high vaginal septum by excision. There was recurrence of hydrometrocolpos in one patient who had low vaginal septum, requiring repeat surgery. One patient, in whom diagnosis was missed, died from overwhelming infection. Short term follow-up was good. Conclusion Congenital vaginal obstruction is an uncommon presentation in this setting. Missing the diagnosis can lead to disastrous consequences. A high index of suspicion is required for prompt recognition and treatment.

Uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) or Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital urogenital anomaly. A 13-year-old female presented with acute abdominal pain and dysmenorrhea. Ultrasonography and magnetic resonance imaging showed uterus didelphys, hematometrocolpos, obstructed hemivagina, and right renal agenesis. Hemivaginal septal resection and anastomosis between the obstructed hemivagina and the normal vagina was tried, but it was not possible. Unilateral hysterectomy was performed. HWW syndrome may present with acute abdomen and is usually treated with vaginal septum resection and drainage of the hematometrocolpos.