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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 (

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.

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.

Objective To evaluate the safety and efficacy of hysteroscopic septum resection of the complete septate uterus with cervical duplication in patients with recurrent spontaneous abortions or infertility. Design Prospective consecutive clinical study. Setting University hospital for obstetrics, gynecology, and reproductive medicine. Subject(s) Twenty-five patients with a complete septate uterus, cervical duplication, and history of recurrent spontaneous abortions (13 cases) or infertility (12 cases) were included. Intervention(s) Hysteroscopic septum resection and sparing the double cervix using a bougie served as a means of orientation and blockage of internal cervical os. Main Outcome Measure(s) Intraoperative and postoperative complications, and postoperative anatomic identification of the uterus. Result(s) No intraoperative and postoperative complications were encountered. Postoperatively, ultrasound revealed minor fundal septal remnants in 7 (31.8%) of 22 patients receiving the ultrasound detection, and no residual septum in the other 15 cases (68.2%). Conclusion(s) By using a bougie technique, hysteroscopic correction of complete septate uterus with cervical duplication and sparing the double cervix can be performed successfully.

Objective To determine if sectioning of the cervical septum in hysteroscopic metroplasty of the complete uterine septum is associated with intraoperative bleeding, cervical incompetence, and secondary infertility. Design Multicenter, randomized, controlled clinical trial. Setting University hospitals. Patient(s) Twenty-eight women with a diagnosis of complete uterine septum who had a history of pregnancy wastage or infertility. They were randomized into two groups: group A underwent metroplasty including section of the cervical septum; group B underwent the same procedure with preservation of the cervical septum. Intervention(s) Hysteroscopic metroplasty was performed for all patients in the two groups. Main Outcome Measure(s) Operating time, distending media deficit, total distending media used, intraoperative bleeding, complications, and reproductive outcome. Result(s) Operating times were 36.40 ± 10.67 minutes and 73 ± 14.40 minutes in group A and group B, respectively. Distending media deficit was 456.66 ± 165.68 mL in group A, while in group B it was 673.84 ± 220.36. Two cases of pulmonary edema and three cases of significant bleeding (>150 mL) were seen in group B. The cesarean section rate was significantly higher in group B. There were no significant differences in the reproductive outcome in the two groups. Conclusion(s) Resection of the cervical septum during hysteroscopic metroplasty of complete uterine septum makes the procedure safer, easier, and less complicated than the procedure with preservation of the cervical septum. This procedure is recommended for all cases of complete uterine septum.

Objective To review clinical implications, reproductive outcome, and long-term consequences in women with complete septate uterus and longitudinal vaginal septum. Design A retrospective study. Setting A university hospital. Patient(s) Sixty-seven patients who had a complete septate uterus including the cervix and a longitudinal vaginal septum. Intervention(s) The longitudinal vaginal septum was incised in 36 cases and metroplasty for uterine septum was undertaken in 4 patients. Main Outcome Measure(s) Fertility, outcome of pregnancies, possible late consequences, and presence of other anomalies. Result(s) Eight (15.7%) of 51 women attempting pregnancy had primary infertility of nonuterine causes. Forty-nine women not undergoing metroplasty produced 115 pregnancies, abortion rate 27%, preterm delivery 12%, and live birth rate 72%. Only five women with no metroplasty had only miscarriages and 44 women at least one delivery. One of four women undergoing metroplasty delivered preoperatively and three after metroplasty. During the follow-up period endometriosis was observed in two (3%) of 61 cases and two patients had borderline ovarian tumors. Kidney abnormalities were found in 11 (20%) of 55 patients studied, double ureter being the most common. Conclusion(s) Complete septate uterus with longitudinal vaginal septum is not associated with primary infertility, and pregnancy may progress successfully without surgical treatment. The results do not support elective hysteroscopic incision of the septum in asymptomatic patients or before first pregnancy.

Objective To describe a unique congenital mullerian anomaly. Design Case report. Setting A university-based reproductive endocrine center. Patient(s) Five reproductive-age, nulligravida patients who underwent clinical, radiologic, and surgical work-up. Intervention(s) Retrospective review of prior medical records and studies. Main outcome measure(s) Definition of abnormal pelvic anatomy. Result(s) Five patients from a university-based, reproductive endocrine center were found to have cervical duplication with a longitudinal vaginal septum, uterine septum, and a normal fundus. The patients most often presented initially to their primary obstetrician–gynecologists with symptomatic complaints secondary to their vaginal septums. Diagnoses were obtained with physical examinations, ultrasound imaging, hysterosalpingograms, magnetic resonance imaging, and surgical evaluation. Conclusion(s) These findings call into question the classic hypothesis of unidirectional (caudal to cranial) mullerian development and supports an alternative embryologic hypothesis of Muller et al., which states that fusion and resorption begins at the isthmus and proceeds simultaneously in both the cranial and caudad directions. The high number of cases reported here might be due to the increased accessibility and accuracy of such imaging modalities as magnetic resonance imaging. These patients will be followed longitudinally so that it can be determined whether this anomaly affects fertility and so that the optimal treatment plans can be developed.

Objective To report the hysteroscopic management, preserving hymen integrity, of a vaginal septum in a virginal patient with uterus didelphys and obstructed hemivagina as a short and safe alternative to the conventional method. Design Case report. Setting University hospital. Patient(s) A 13 year-old girl with uterus didelphys and obstructed hemivagina. Intervention(s) Hysteroscopic septum resection. Main Outcome Measure(s) Hysteroscopic resection, preserving hymen integrity, of a vaginal septum in uterus didelphys and obstructed hemivagina in an adolescent patient. Result(s) The vaginal septum was resected safely in the patient, and hymen integrity was preserved. The cyclic dysmenorrhea and hematometra disappeared, and the patient was free of dysmenorrhea after 1 year of follow-up. Conclusion(s) Hysteroscopy, preserving hymen integrity, provides excellent visualization in adolescents and is an easy, quick, convenient, and effective tool in the management of obstructed hemivagina.

Magnetic resonance imaging is considered the gold-standard imaging technique in cases of Mullerian and vaginal anomalies, however, vaginal delineation often proves difficult, owing to the fact that vaginal walls are normally collapsed and in close proximity. Instilling gel through the introitus allows for better depiction of the distal vagina.

Objective To present the experience of a large referral center with patients with the rare but specific syndrome of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. Design, Patients Between 1953 and 1991, 15 patients with the syndrome were evaluated by the authors; each patient's records were carefully reviewed. Main Outcome Measures For each patient, the clinical course, specific anatomic findings, treatment(s) offered, and obstetrical outcome are presented. Results The specificity of the association of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis was confirmed by our series, the largest in the literature to date. The findings suggest a specific developmental anomaly of the mullerian ductal system, probably secondary to a wolffian duct anomaly. The most common clinical presentation was that of the onset of pelvic pain and/or dysmenorrhea shortly after menarche, in association with the finding of a vaginal/pelvic mass. When a communication between the two sides existed, symptoms of abnormal vaginal discharge and bleeding were common. Optimal outcome appears to have occurred for patients initially managed using a conservative surgical approach, i.e., simple vaginal septum resection combined with conservative treatment of associated endometriosis. Obstetrical outcome in our patients was similar to that in patients with the more common combination of uterus didelphys and sagittal vaginal septum. Conclusion A greater awareness of the syndrome of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis should lead to its prompt diagnosis, allowing for early and appropriate surgical intervention as well as decreased long-term morbidity.

Objective To review a case of concomitant longitudinal uterocervicovaginal septum and imperforate hymen. Setting A university-based tertiary care center. Patient(s) A 14-year-old girl with a septum spanning the length of the uterus, cervix, and upper two-thirds of the vagina, as well as an imperforate hymen. Intervention(s) Hymenotomy with subsequent vaginal septoplasty. Main Outcome Measure(s) NA. Result(s) NA. Conclusion(s) Anatomic anomalies of the reproductive tract may be found in tandem and when suspected, a full evaluation including advanced imaging to clearly delineate the exact anatomy, should be performed before proceeding with surgical correction.

Objective: To describe a technique for treating hematocolpos and hematometra in patients with uterus didelphys and unilateral imperforate vagina involving the use of resectoscopy under ultrasonographic control. Design: Case report. Setting: University hospital. Patient(s): A 13-year-old girl with uterus didelphys with unilateral hematometra, hematocolpos, and ipsilateral renal agenesis. The girl complained of severe abdominal pain, which appeared with each of her menses. Intervention(s): The intervention was performed by a vaginoscopic approach to preserve the integrity of the hymen. The first incision on the vaginal wall was performed in correspondence with the hematocolpos under continuous ultrasonographic guidance with the use of a straight resectoscopic loop. Resection of the vaginal septum was continued with the use of an angled resectoscopic loop until almost complete excision of the septum was achieved. Main Outcome Measure(s): Clinical, echographic, and vaginoscopic findings before the operation and 2 and 6 months after the operation. Result(s): The surgical procedure was easy to perform. Almost complete excision of the septum was achieved with just a few passages of the resectoscope. Complete drainage of both the hematocolpos and the hematometra was confirmed by ultrasonography. The postoperative period was completely uneventful. Clinical and vaginoscopic evaluations 6 months after the operation confirmed the integrity of the hymen, the complete resolution of clinical symptoms, and the persistence of a large communication between the two vaginas. Conclusion(s): Resectoscopic excision under ultrasonographic guidance of the vaginal septum in a girl with uterus didelphys with unilateral hematometra and hematocolpos was effective and easy to perform, and it fully respected the integrity of the reproductive system.

Objective To report a rare uterine anomaly consisting of a normal uterus, a double cervix, and a double vagina. Design Case report. Setting University hospital. Patient(s) A 28-year-old nulligravida patient referred for evaluation of primary infertility and a suspected mullerian anomaly. Intervention(s) Clinical and surgical evaluation of the anomaly. Main Outcome Measure(s) Description and treatment for a rare uterine anomaly and a subsequent literature search. Result(s) Successful resection of vaginal septum and subsequent pregnancy. Conclusion(s) This extremely rare anomaly is not explained by classic embryologic teachings, and it does not fit into the classification system currently used to describe mullerian anomalies.

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

Objective To report a rare uterine anomaly of a septate uterus, double cervix, and double vagina. Design Case report. Setting A university-based reproductive endocrinology center. Patient(s) A 20-year-old nulligravida with primary infertility. Intervention(s) Clinical examination and surgical resection of a vaginal septum with hysteroscopic examination of both hemicavities. Main Outcome Measure(s) Description and treatment for a rare uterine anomaly and a subsequent literature search. Result(s) Successful resection of vaginal septum and subsequent spontaneous pregnancy. Conclusion(s) This rare anomaly is not explained by classic embryologic teaching and is not described by the classification system currently used to describe mullerian anomalies.

We report a full-term delivery after resection of a longitudinal vaginal septum in a patient with a septate uterus and cervical duplication.

Objective To describe a case of 45,X/46,XY mixed gonadal dysgenesis complicated by malignancy with possible metastasis. Design Case report. Setting University hospital. Patient(s) A 15-year-old female with primary amenorrhea, short stature, and a vaginal septum. Intervention(s) Resection of transverse vaginal septum and laparoscopic bilateral gonadectomy. Result(s) The patient had dysgerminoma arising from gonadoblastoma in the left gonad and gonadoblastoma in the right gonad. No normal gonadal tissue could be identified. Postoperative computed tomography scan results were suspicious for lung metastases, but the patient opted for conservative management without chemotherapy. Conclusion(s) Mixed gonadal dysgenesis involves inherent malignancy risk and complex psychosocial issues, which necessitate a multidisciplinary approach to diagnosis and treatment.

Objective To demonstrate a rare and unusual presentation of transverse vaginal septum (TVS). Design Case report. Setting Department of Obstetrics and Gynecology, Ataturk University, Erzurum, Turkey. Patient(s) A woman with complaint of primary infertility presenting TVS. Intervention(s) After comprehensive examination, primary infertility associated with mullerian duct anomaly was surgically treated. Main Outcome Measure(s) Primary infertility and anatomic anomaly. Result After surgical intervention, the patient with primary infertility caused by asymptomatic TVS was successfully conceived. Conclusion(s) Anatomic congenital defects may contribute to primary infertility. Suspicious evaluation of all primary infertile couples is needed, because a rare asymptomatic anatomic congenital defect could lie behind the infertility.

Objective To present a case of mullerian defects associated with other anomalies, including ipsilateral renal agenesia, duplication of inferior vena cava, high-riding aortic bifurcation, and intestinal malrotation. Design Case study. Setting Academic medical center. Patient(s) A 16-year-old woman with mullerian defects associated with vascular and intestinal anomalies. Intervention(s) Excision of vaginal septum. Main Outcome Measure(s) Improvement of symptoms caused by obstructed mullerian defect. Result(s) After an incision on the vaginal septum, a large amount of chocolate-colored fluid was drained, and the dimensions of the right uterus were diminished. Then the vaginal septum was excised completely; we saw a second distinct cervical portion. Conclusion(s) Uterus didelphys with obstructed hemivagina is almost always associated with renal agenesis ipsilateral to the obstruction. Inferior vena cava duplication associated with mullerian anomalies is an extremely rare condition. In addition, the presented case also had a high-riding aortic bifurcation and intestinal malrotation. To the best of our knowledge, this is the first case to include all of these anomalies.

Objective To describe the first case of a leiomyoma localized in the septum of a septate uterus that had cervical duplication and a longitudinal vaginal septum. Design Case report. Setting A university hospital. Patient(s) A 43-year-old patient with primary infertility and complaint of menorrhagia. Intervention(s) Laparotomy, adhesiolysis, myomectomy, and resection of the vaginal septum in the first operation. Hysteroscopic resection of the complete uterine septum in the second operation. Main Outcome Measure(s) Magnetic resonance imaging findings and two-step repair of the anomaly. Cervical length at second trimester. Result(s) Restoration of normal anatomy was achieved after correct diagnosis and a two-step operation procedure. A normal cervical length was measured at 26 weeks of gestation. Conclusion(s) Large leiomyoma at rare localizations in the presence of uterine anomalies is a diagnostic challenge, but with available diagnostic technology and appropriate operations, successful results can be obtained.

The reproductive potential of the malformed uterus is assessed, with emphasis on problems of vertical and lateral fusion. An obstructive transverse vaginal septum, which appears to result from a rare autosomal recessive gene, can be encountered in infancy or may not manifest symptoms until the onset of menstruation when menstrual blood accumulates. Hysterectomy is the recommended treatment, except in rare cases where there is only partial failure of the cervix to develop or there is a very short distance between the vagina and the endometrial cavity. Only 1 case of successful reproduction has been documented among women with this condition. Pregnancies have been reported in instances of partial transverse vaginal septum; however, postpartum pyometra and pyocolpos can develop, requiring emergency surgical drainage. Symptoms in women with obstructed lateral fusion are related to the site of obstruction. Reproduction may occur after removal of the vaginal septum in women with a uterus didelphys with a double vagina and low vaginal obstruction. Ectopic pregnancies have been reported inw women with an obstructed rudimentary horn. Unilateral obstruction is almost always accompanied by absence of the ipsilateral kidney, suggesting that bilateral obstruction is associated with bilateral kidney agenesis with consequent nonviability of the developing embryo. Reproduction appears to be somewhat compromised by infertility, pregnancy wastage, and premature labor in patients with either a didelphic or a unicornuate uterus. The bicornuate uterus causes only minimal reproductive problems, while the septate uterus is almost always associated with reproductive failure. Examination under anesthesia or laparoscopy may be required to distinguish between these 2 types of double uterus. Excision of the septum by wedge is the recommended operative treatment of a septate uterus. After this procedure, 77% of patients in 1 series had a term delivery. 73% of all pregnancies following the surgery were carried to term.