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A complete uterine septum, with a double cervix and vaginal septum, is a complex and rare congenital genital tract anomaly. The diagnosis is difficult and often challenging, requiring complex imaging investigations and diagnostic hysteroscopy. The benefit of hysteroscopic metroplasty for this uterine malformation is still the subject of dispute. However, the potential benefits of obtaining pregnancies and reducing the rate of abortions make this surgical method a desirable one. We present a series of three cases with U2bC2V1 malformation that were diagnosed via magnetic resonance imaging (MRI), in which hysteroscopic removal of the uterine septum and resection of the longitudinal vaginal septum were performed, with the preservation of the two cervixes. All patients became pregnant after the hysteroscopic intervention and reported an improvement in dyspareunia and dysmenorrhea. [ABSTRACT FROM AUTHOR]

Herlyn-Werner-Wunderlich syndrome, also known as obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), is a Müllerian duct anomaly. It is a rare clinical condition consisting of a duplicated uterus with an oblique vaginal septum that causes partial genital tract outflow obstruction. A urinary tract anomaly, most commonly renal agenesis, is usually present on the obstructed side. The diagnosis of genital tract outflow obstruction is often delayed due to the normal functioning of the unaffected side. The most frequent complications are dysmenorrhea, chronic pelvic pain, infection, infertility and endometriosis. This report describes a 17-year-old G0P0 patient with a history of severe dysmenorrhea and left-sided renal agenesis, who was admitted for complaints of foul vaginal discharge over the past 3 months that was unsuccessfully treated with antibiotics. Transrectal ultrasound revealed the presence of 2 separate hemicavities on transverse and longitudinal views. A cystic lesion with ground-glass opacities was detected between the bladder and a normal-appearing cervix, which was determined to be hematocolpos. The diagnosis of OHVIRA was made. This case highlights the importance of excluding a Müllerian anomaly in the presence of renal system abnormalities. Being aware of the type of anomalies, combinations and variants is crucial to determine the diagnosis and the best surgical approach. Ultrasound was an invaluable imaging exam to determine the type of anomaly and its complexity. Awareness of this syndrome and its variants will prevent misdiagnosis and will help to define the appropriate treatment for these patients.

A complete uterine septum, with a double cervix and vaginal septum, is a complex and rare congenital genital tract anomaly. The diagnosis is difficult and often challenging, requiring complex imaging investigations and diagnostic hysteroscopy. The benefit of hysteroscopic metroplasty for this uterine malformation is still the subject of dispute. However, the potential benefits of obtaining pregnancies and reducing the rate of abortions make this surgical method a desirable one. We present a series of three cases with U2bC2V1 malformation that were diagnosed via magnetic resonance imaging (MRI), in which hysteroscopic removal of the uterine septum and resection of the longitudinal vaginal septum were performed, with the preservation of the two cervixes. All patients became pregnant after the hysteroscopic intervention and reported an improvement in dyspareunia and dysmenorrhea.

Background & aim: A vaginal septum is a condition in which the wall of the tissue dividing vagina does not resolve completely. This complication can be transverse and longitudinal. Although the occurrence of a transverse vaginal septum is 2 per 100,000 births, the exact etiology of this anomaly remains still unidentified. In this study, a case of high transverse vaginal septum with vaginal and cervical agenesis is reported.Case report: A 13-year-old single and virgin girl presented with cyclic abdominal pain. On examination and magnetic resonance imaging, vaginal and cervical agenesis was diagnosed. Surgical therapy included an incision in the lower part of the uterus at the site of the bulging and the use of a hysterometer passing down the uterus. The cervix was not touched, cervical agenesis was diagnosed, and the patient underwent abdominal hysterectomy and bilateral tubal salpingectomy. The pathological results confirmed the cervical agenesis.Conclusion: It is recommended that in patients with high transverse vaginal septum, hysterectomy be performed for the treatment of cervical and vaginal agenesis. However, it may not be a suitable treatment choice for some cases, and it is better to decide based on the patient's condition.

We present a simple surgical technique aiming to improve urine outflow through the common urogenital sinus in cloaca and facilitate drainage of existing hydrocolpos. The study included three cases of cloaca with associated hydrocolpos that were operated during the period 2022 through 2023. The patient is placed in the prone position for a standard posterior sagittal anorectoplasty. The distal rectal fistula is severed flush with the vagina/sinus leaving an open defect in the posterior wall of the vagina/sinus. The defect is then widened distally via a vertical incision (∼1 cm) through the posterior wall of the common urogenital sinus toward but not reaching the perineum. This vertical defect is then closed horizontally displacing the posterior vaginal wall downwards toward the perineum (posterior sinuplasty). The postoperative recovery was uneventful in the three cases. Adequate drainage of hydrocolpos was confirmed by imaging at follow-up, as well as improvement of upper urinary tract dilatation. In selected cases of cloaca, posterior sinuplasty is a simple procedure that can be applied during anorectoplasty to provide effective drainage of associated hydrocolpos.

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) is a rare malformation that not only causes severe menstrual cramps shortly after menarche but can also lead to endometriosis and infection in the future. We report a case of OHVIRA successfully managed by vaginoscopic excision of the vaginal septum. A 12‐year‐old virgin girl presented to our hospital with dysmenorrhea and lower abdominal pain. OHVIRA was diagnosed using magnetic resonance imaging. Vaginoscopic surgery was performed for drainage of hematocolpos and excision of the vaginal septum. Vaginoscopic excision of the vaginal septum was performed using a resectoscope, without a vaginal speculum. The procedure was completed safely without injuring the hymen. This is the first case report of successful excision of the vaginal septum by vaginoscopic surgery for OHVIRA in Japan. Vaginoscopic excision may be one of the effective options for the treatment of vaginal obstruction. [ABSTRACT FROM AUTHOR]

Double uterus (uterus didelphys) is the second least common congenital anomaly of the female genital tract resulting from failure of fusion of the two Müllerian ducts during embryological development, leading to duplication of the uterus and the cervix. The condition may be associated with abnormalities of the Wolffian duct such as ipsilateral renal agenesis. Presence of a double uterus increases obstetric complications. We report five cases of young women with uterus didelphys who were delivered by Caesarean Section for obstetric indications.

Introduction: Obstructive vaginal anomalies such as transverse septum and vaginal agenesis usually present with primary amenorrhea and pelvic pain. This study was performed with aim to evaluate the results of vaginal reconstructive surgery (vaginoplasty) in patients with vaginal agenesis (Rokitansky syndrome) and transverse vaginal septum.Methods: This cohort study was performed on all patients with vaginal agenesis and transverse vaginal septum who had underwent vaginoplasty in the pelvic floor disorders department of Imam Khomeini Hospital ,Tehran in 2014-2019. Basic information, clinical symptoms at admission, type of Mullerian anomaly, type of surgery, complications during surgery, vaginal re-stenosis and pregnancy were collected from patients' files and by phone calls and follow up visits after surgery. Central and dispersion indices were used to describe the data. Frequency distribution was presented in the form of tables.Results: 33 patients had transverse vaginal septum and 35 cases had vaginal and uterine agenesis (Rokitansky syndrome). In terms of early symptoms at admission, the most common symptoms were amenorrhea, pelvic pain, and inability to sexual intercourse or dyspareunia. In 11 cases (33.33%), vaginal re-stenosis occurred after correction of transverse vaginal septum, all of which occurred in the first 3 months after surgery due to incorrect use of vaginal dilator. Laparotomy was performed in 2 cases (6%) due to severe abdominal pain after initial surgery that was diagnosed as pelvic endometriosis. In the vaginal agenesis group, vaginal re-occlusion occurred in5 cases (14.28%) after the surgery.Conclusion: The most common complication after surgery was vaginal re-obstruction, so training the patient and emphasizing on the regular use of vaginal dilator is very important to prevent this complication.

As evidenced by the 2021 American Society for Reproductive Medicine Müllerian Anomaly Classification (ASRM MAC), there are numerous possible configurations of the female genitourinary system. Some anomalies place patients at higher risk of infertility, miscarriage, fetal malpresentation, and preterm labor. Correct characterization of Müllerian anomalies is critical for proper infertility treatment and pregnancy counseling. This case study of a 32-year-old nulliparous woman describes the radiographic modalities utilized in the diagnosis of a complex Müllerian anomaly. To characterize the Müllerian anomaly, the patient underwent a 2D transvaginal ultrasound (TVUS), saline infusion sonohysterogram (SIS) with 3D reconstruction, and second-opinion interpretation of a previous MRI. Radiographic diagnoses were conflicting among different modalities. While the saline infusion sonohysterogram suggested a bicornuate uterus, the MRI of the anomaly was interpreted as a uterine didelphys. Furthermore, TVUS and MRI indicated the presence of two cervices, while only one cervix was appreciated with SIS. Given the inconsistent interpretation of her anomaly, the patient was brought to the operating room. After direct visualization with exam and hysteroscopy, the patient was ultimately diagnosed with a uterine didelphys with a communication of endometrial cavities in the lower uterine segment, two cervices, a hypoplastic right vagina, and a longitudinal vaginal septum extending to the hymen. The patient was counseled that this anomaly is not expected to impact natural conception and would not require surgical resection. The patient went on to conceive spontaneously in the right uterine horn, with a plan for primary cesarean delivery. This case was of sufficient complexity that an accurate diagnosis was not made until physical exam and hysteroscopic visualization verified findings from MRI and ultrasonographic imaging. Appropriately describing a Müllerian anomaly according to the 2021 ASRM MAC is crucial, and multiple forms of imaging and physical examination may be necessary to accurately characterize uterine anomalies., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Valdez-Sinon et al.)

Background: Transverse vaginal septum is a rare mullerian anomaly. Patients with a complete transverse vaginal septum generally complain of primary amenorrhea. Here, we presented a rare case of high transverse vaginal septum with abdominal mass during infancy in addition to chronic pelvic pain and cyclic hematuria during adulthood.Case presentation: The patient was a 26-year-old female with complain of severe abdominal and pelvic pain with significant weight loss. She had a history of surgery for correction of hydrometrocolpos (fluid accumulation in the uterus and vagina) because of transverse vaginal septum at infancy period. She had no gynecologic follow-up until the age of 26 years. At the last surgical evaluation, diffuse pelvic endometriosis with advanced metastatic colon cancer was noted.Conclusion: After diagnosis of transverse vaginal septum during infancy period, detailed counseling should be performed about the other treatment and follow-up which is needed to prevent severe complications that could affect the quality of life.

We present a case of a 12.5-year-old girl who has suffered from recurrent urinary tract infections for many years but has never undergone a detailed diagnostic process. Only as a teenager did she complain of acute pain in her lower abdomen and it turned out that her genital organs had not properly developed. She had an obstructive defect in the reproductive tract. When there was a significant amount of discharge collected in the lumen of the genital tract and the organs had distended, acute pain appeared, which allowed us to make the diagnosis. In the diagnostic process, transperineal ultrasonography turned out to be extremely helpful, allowing us to establish the type and thickness of the obstruction. The patient underwent excision of transverse vaginal septum, and postoperative silicon dilators were used to prevent the recurrence of the obstruction. There was no recurrence of urinary infection or complications during the 11 months of follow-up.

Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA), or Herlyn-Werner-Wunderlich syndrome, is a rare Mullerian duct anomaly. Several associations of OHVIRA with other anomalies are being reported. A 13-year-old girl reported with acute onset abdominal pain. Patient was operated on in the past for type IV congenital pouch colon (all stages complete). She was diagnosed with hematometrocolpos and underwent tube vaginostomy. Further workup was suggestive of OHVIRA, which was subsequently managed with surgical repair. Delineation of reproductive anomalies in female patients with anorectal malformations is of paramount importance. OHVIRA syndrome should be considered at a high index of suspicion in female patients with a solitary functioning kidney. Nonspecific symptoms in an adolescent female can lead to erroneous judgment, leading to unnecessary investigations, which, if not well managed in time, can be detrimental to fertility. [ABSTRACT FROM AUTHOR]

Background: Transverse vaginal septum is a rare mullerian anomaly. Patients with a complete transverse vaginal septum generally complain of primary amenorrhea. Here, we presented a rare case of high transverse vaginal septum with abdominal mass during infancy in addition to chronic pelvic pain and cyclic hematuria during adulthood. Case presentation: The patient was a 26-year-old female with complain of severe abdominal and pelvic pain with significant weight loss. She had a history of surgery for correction of hydrometrocolpos (fluid accumulation in the uterus and vagina) because of transverse vaginal septum at infancy period. She had no gynecologic follow-up until the age of 26 years. At the last surgical evaluation, diffuse pelvic endometriosis with advanced metastatic colon cancer was noted. Conclusion: After diagnosis of transverse vaginal septum during infancy period, detailed counseling should be performed about the other treatment and follow-up which is needed to prevent severe complications that could affect the quality of life. [ABSTRACT FROM AUTHOR]

Objective: To describe and summarize the early diagnosis, treatment and outcomes of obstructed hemivagina and ipsilateral renal anomaly (OHVIRA).Methods: Data from patients with OHVIRA treated at the study centre over the last decade were analysed retrospectively, including demographic characteristics, symptoms, diagnosis, treatment and outcomes. Relevant published literature was also reviewed in this study.Results: In total, 11 cases were diagnosed with OHVIRA over the study period, ranging in age from 3 to 14 years (median 11 years). Four cases were asymptomatic, four had abdominal pain and three had urinary symptoms. All 11 cases underwent vaginoplasty. Ureteral bladder re-implantation (n = 1), dysplastic nephrectomy (n = 1), separation of labia minora adhesions (n = 1), perineoplasty (n = 1) and urethroplasty (n = 1) were performed. Cases were followed up for 2 months to 8 years (median 5 years) with good follow-up outcomes.Conclusions: Based on the cases in this study and the relevant literature, ultrasound appears to be an effective method for the diagnosis of OHVIRA. Experienced ultrasonographers are able to achieve a high corrected diagnosis rate. The differential diagnosis of OHVIRA should be considered by paediatric surgeons, urologists, emergency physicians and gynaecologists when they identify patients with didelphys uterus or renal agenesis. Early surgery can reduce the risk of potential genital tract infection and gynaecological complications. [ABSTRACT FROM AUTHOR]

In November 2011, a 61-year-old woman was diagnosed with squamous cell carcinoma (SCC) of the cervix in a uterus didelphys with vaginal septum. The patient was diagnosed with Fédération Internationale de Gynécologie et d’Obstétrique (FIGO) stage IIB because of infiltration to the left parametrium without infiltration to the pelvic wall. The patient was treated with external-beam radiotherapy (EBRT) and brachytherapy (BT), using concomitant chemotherapy with cisplatin. A total of 50 Gy were delivered (2 Gy/fraction/day) to the pelvis, with a central shield after 40 Gy. The patient was treated four times with BT (6 Gy × 4 fractions), with tandem and ovoid applicators inserted once to the left side; tandem to the left side and ovoid bilaterally were inserted twice; and tandem to the right side and ovoid bilaterally were inserted once. Six years and 8 months after the start of treatment, the patient had had no relapse or severe late adverse effects. For accurate diagnosis and optimal treatment of the uterus didelphys, careful interview and pelvic examination at initial diagnosis of a patient are very important.

A total of 26 mice were observed with vaginal septa and 10 of them progressively developed into vaginal atresia. Among these vaginal atresia mice, 6 mice eventually suffered from uterus hydrometrocolpos syndrome (HMCPS) and polycystic ovary syndrome (PCOS) and the others were cured with surgical intervention once the vaginal orifices were found closed. Imaging and pathological observation showed that vaginal atresia led to cervix's lipoid secretion and uterine effusion. Moreover, Serum biochemical examination and ELISA test revealed significantly dramatic drop (about 50%) in blood triglycerides (P<0.01) and double increase in aromatase (P<0.01) in vaginal atresia mice. These findings give gained variable insight into further studies and provide vital guide for therapy on vaginal atresia. [ABSTRACT FROM AUTHOR]

Currently, there is no commercially available soft vaginal mold designed for reconstructive surgeries for congenital vaginal anomalies. Stricter operating room regulations discourage the use of makeshift molds from foams and gloves. A colpo-pneumo-occluder balloon is designed to maintain pneumoperitoneum after colpotomy in laparoscopic hysterectomies and is approved for use in vaginal surgeries. A 17-year-old girl with a congenital transverse vaginal septum experienced recurrent obstruction and hematocolpos. We successfully used a colpo-pneumo-occluder balloon as a vaginal mold during postoperative care. Its size and design make this device ideal for use in vaginal reconstructive surgeries in adolescents. The laparoscopic colpo-pneumo-occluder, a sterile vaginal device, is appropriate to use as an adjustable, soft vaginal mold for correction of congenital and acquired vaginal anomalies. [ABSTRACT FROM AUTHOR]

Transverse vaginal septum is a rare female genital tract anomaly which may occur exclusive of any concomitant anomalies. We report the case of a pre menercheal girl who presented at our resource constrained center with primary amenorrhoea and cyclic abdominal pain. We performed a resection of the transverse vaginal septum and followed it up with the introduction of a Foley catheter in the vagina. After 4 weeks of discharge, no septal closure was present, and the menstrual flow was normal.

Uterine cervical Naboth cyst is a common benign disease of gynecology, and it is caused by the damage to the cervix like childbirth, inflammation and artificial abortion. The diameter of the small cervical cysts varies from a few millimeters to 4 centimeters, which can be single or multiple. Most patients do not have self-conscious symptoms. Big cysts are rare and patients may have discomforts during sexual intercourses. Clinically preoperative identification of the large uterine cervical Nabothian cysts is very important. Differentiation from cervical malignant disease can help avoid expanding the scope of surgery and improve the postoperative qualities of lifes of patients. Large Naboth cysts are easily confused with mullerian duct cysts and cervical malignant adenomas before operation. Ultrasound examination has no absolute diagnostic value. The etiology of large cervical Naboth cysts is unclear. A case of pregnancy to term delivery with complete mediastinal uterus is reported. Whether there is a relationship between the incidence of large uterine Naboth cysts and uterine congenital malformations remains to be studied. [ABSTRACT FROM AUTHOR]

Introduction: The proportion of vaginal anomalies is generally 10% of all female reproductive tract anomalies. This study aimed to determine the characteristics and quality of life of patients with vaginal septum carried out operatively using the vaginal approach. Methods: This was a descriptive study with a retrospective approach. The characteristics of subjects were collected using secondary data from medical records from January 2014 to December 2018, while quality of life of subjects was measured by WHOQOL questionnaire. Results: Among fourteen patients with vaginal septum who performed an operative method with the vaginal approach, the most age distribution was <20 years (n=10, 71.4%). The majority of patients were single (n=10, 71.4%). There were 8 patients (57%) having menstruation. The most accompanying condition was hematometra-hematocolpos (n=5, 35.71%). There were 3 patients (21.42%) who had repaired. Most patients (n=13, 92.86%) did not report postoperative complaints. There were 11 patients (78.6%) having a good or satisfied quality of life. The mean results of the Physical Health domain were 55.42±9.112, Psychological domain was 66.21±9.374, Social Relationship domain was 63.00±12.949, and Environment domain was 61.85±6.981. Conclusion: Most patients with vaginal septum was less than 20 years, and the quality of life of was quite good, with only the physical aspect having a lower value. [ABSTRACT FROM AUTHOR]

Objective: Vaginal or uterine outlet obstruction leads to hematocolpos or hematometra. Detection of the etiology of this entity is important to guide adequate surgical management and thereby avoid complications and to preserve fertility. The aim of this study was to evaluate obstructing vaginal malformations in adolescent girls presenting with hematocolpos or hematometra with MR imaging. Materials and methods: A hospital based prospective study was conducted in a tertiary care centre from September 2015 to October 2016. The study included 17 adolescent females who were evaluated with MRI. Result: Of 17 adolescent female with vaginal or uterine outflow obstructive anomalies with hematocolpos or hematometra, where 6 patients (35.3%) had HWWS, 6 patients (35.3%) had imperforate hymen, 2 patients (11.8%) had transverse vaginal septum, 1 patient each (5.9%) had cervico-vaginal atresia, unicornuate uterus and communicating rudimentary Uterine horn. MRI revealed hematocolpos in 15 patients (88.2%), hematometra in 13 patients (76.5%), endometriotic ovarian cysts in 6 patients (35.3%) and hematosalpnix in 3 patients (17.6%). Conclusion: Early radiological diagnosis of the cause of vaginal or uterine outflow obstruction is important to guide adequate surgical management which if undertaken promptly helps to avoid complications due to reflux from vaginal or uterine outflow obstruction.

The ADAMTS family comprises 19 secreted metalloproteinases that cleave extracellular matrix components and have diverse functions in numerous disease and physiological contexts. A number of them remain ‘orphan’ proteases and among them is ADAMTS18, which has been implicated in developmental eye disorders, platelet function and various malignancies. To assess in vivo function of ADAMTS18, we generated a mouse strain with inactivated Adamts18 alleles. In the C57Bl6/Ola background, Adamts18-deficient mice are born in a normal Mendelian ratio, and are viable but show a transient growth delay. Histological examination revealed a 100% penetrant eye defect resulting from leakage of lens material through the lens capsule occurring at embryonic day (E)13.5, when the lens grows rapidly. Adamts18-deficient lungs showed altered bronchiolar branching. Fifty percent of mutant females are infertile because of vaginal obstruction due to either a dorsoventral vaginal septum or imperforate vagina. The incidence of ovarian rete is increased in the mutant mouse strain. Thus, Adamts18 is essential in the development of distinct tissues and the new mouse strain is likely to be useful for investigating ADAMTS18 function in human disease, particularly in the contexts of infertility and carcinogenesis.