Uterovaginal Anomalies Research Articles

Developmental uterovaginal anomalies and histologic findings in transgender patients receiving gender-affirming hysterectomies: A large case series.

To describe the incidence of uterovaginal anomalies and histologic findings in transgender and nonbinary (TGNB) patients seeking hysterectomies. All patients receiving gender-affirming hysterectomies between 2013 and 2023 were retrospectively reviewed. Primary outcomes included uterovaginal anomalies and histological findings. Multivariable logistic regressions were performed to evaluate relationships between variables of interest and whether they predict findings of uterovaginal anomalies, inactive endometrium, adenomyosis, leiomyoma, endometriosis, and cervical atrophy. 278 patients received hysterectomies at an average age of 29.2 ± 8.3 years. Seven patients (2.5%) were found to have a developmental anomaly, including two bicornuate uterus (0.7%), two unicornuate uterus (0.7%), one septate uterus (0.4%), and two vaginal septum (0.7%). 60 patients (21.6%) were found to have inactive endometrium and 26 patients (9.4%) had cervical atrophy. Although 262 patients (94.2%) were on testosterone therapy, hormone duration was not a significant predictor of any uterine findings. This study describes uterovaginal anomalies in a large cohort of patients receiving gender-affirming hysterectomies. Although long-term testosterone use is commonly believed to be associated with endometrial and cervical atrophy, this study shows no such association.

Modern methods of surgical treatment of obstructive genital malformations in girls

Congenital anomalies of female genital tract in children and adolescents are more commonly detected at the age of menarche with several symptoms caused by obstruction. These diseases are rare, but without timely diagnostics and management they can cause a number of serious complications. This article is aimed to review modern approaches to diagnostics and management of this pathology and to highlight some aspects that require further research. A literature search was conducted using the following databases: MEDLINE, Google Scholar, eLIBRARY, international guidelines and clinical recommendations. Key words that were used for search were: «congenital malformations», «distal vaginal atresia», «imperforated hymen», «OHVIRA», «transverse vaginal septum», «hematocolpos», «obstructive uterovaginal anomalies», «surgical treatment in pediatrics». International clinical trials which had been analysed by the authors were included in the review. They have explored the current literature related to the following obstructive malformations: distal vaginal atresia, transverse vaginal septum, imperforated hymen and Herlin–Werner–Wunderlich syndrome. Main conclusions are: further studies with a larger sample of patients, longer follow-up, long-term outcomes and effective preventive measures against complications. Another unresolved question is the age which can be recommended for surgical treatment of obstructive malformations. Potentials for developing new algorithms of earlier diagnostics are also among research priorities.

FRI413 Hormonal Menstural Suppression For Menstrual Obstruction: A Retrospective Review Of Patients With Complex Obstructed Uterovaginal Anomalies Who Underwent Surgical Management In A Tertiary Referral Centre

Abstract Disclosure: H.I. Learner: None. S.M. Creighton: None. S.A. Clarke: None. Aims: To describe the use of hormonal menstrual suppression in patients undergoing surgery for complex utero-vaginal anomalies with menstrual obstruction. Background: Obstructed menstruation can present with painful primary amenorrhea or progressive dysmenorrhea due to unilateral obstructing duplication utero-vaginal anomalies. Menstrual suppression is essential to relieve pain during investigation and surgical planning. The off licence use of progestogens, hormonal contraception and GNRH analogues is common but there is little literature describing their best use in this context. Methods: This was a retrospective case series review of patients undergoing surgical management of complex menstrual obstruction in a tertiary referral centre between 2016-2022. Data collected on age, diagnosis, symptoms, duration of symptoms, surgery, the type of endometrial suppression used and associated side effects.Patients with partial obstruction were excluded. Results: Eighty-four patients were identified from the surgical database. The median age at time of surgery was 16 years (range 11-36). Unilateral obstruction was managed in 53/84 and complete obstruction in 31/84. Diagnoses included obstructed hemi-vagina (32/84), unicornuate uterus with functional uterine remnant (19/84), transverse vaginal septum (15/84), cervical/vaginal agenesis (9/84), and Mullerian agenesis with functional uterine remnant (5/84). Symptom duration information was available in 74/84 with a median of 10 months(0-252 months). Changes to endometrial suppression treatment were required in most patients, with 50/84 (40%) remaining on their original prescription. The average number of treatment regimes was 2 (0-7). Menstrual suppression at time of surgery was Norethisterone 35/84 (42%), Continuous combined oral contraceptive pill18/84 (21%), Medroxyprogesterone 11/84 (13%), GNRH 10/84 (12%), Desogestrel 6/84 (7%), and Depo-Provera 4/84 (5%). Tibolone was used as add-back in five patients on GNRHa for more than 6 months duration of use (4/5) or hypoestrogenic side-effects (1/5) . Conclusion Menstrual suppression for pain relief is an essential component in management for complex uterovaginal anomalies. Suppression should be started at initial presentation but there is limited guidance for clinicians as to what and how to prescribe. Our study has demonstrated that this can lead to chaotic prescribing, unnecessary pain and multiple treatment changes. Presentation: Friday, June 16, 2023

46. Management of Hematocolpos in Lower Vaginal Atresia by Image-Guided Drainage with Interventional Radiology: A Case Series

<h3>Background</h3> Obstructive uterovaginal anomalies such as lower vaginal atresia (LVA), can lead to hematocolpos (HC) and subsequent pain at presentation. Definitive management of long LVA requires vaginoplasty with diligent postoperative dilation to prevent vaginal stenosis. However, many adolescents are not emotionally mature enough to adhere to this. Menstrual suppression and delayed vaginoplasty is recommended, however pain symptoms may require drainage of HC. We present a novel approach to management of symptomatic HC via image-guided percutaneous drainage by Interventional Radiology (IR). <h3>Case</h3> Case 1: 14 yo with small bladder and stress urinary incontinence presented with pelvic pain and was found to have a solitary uterus, 6 cm LVA, and a large HC on MRI. She declined vaginoplasty and opted for IR drainage. Ultrasound (US)-guided transabdominal percutaneous uterine access was obtained and tissue plasminogen activator (tPA) was infused through a drain to thin the HC fluid to allow complete aspiration. Norethindrone acetate (NAT) was initiated for menstrual suppression. Cases 2 and 3: 10 yo presented with abdominal pain and found to have a solitary uterus, 5.3 cm LVA, and a large HC on MRI. Menstrual suppression with NAT failed to control her pain; after counseling, she declined vaginoplasty and opted for IR drainage. US-guided percutaneous access was obtained, tPA infused to thin the HC, and pigtail drain placed to assist with further decompression. Cone beam CT verified the patient's anatomy and confirmed drain position. Drain was removed post-operative day 1. Symptomatic re-accumulation of HC occurred after 15 months of menstrual suppression and a repeat US-guided drainage was performed with complete decompression; GnRH agonist arm implant was placed for long-term menstrual suppression. Case 4: 15 yo with complex history including urogenital sinus, renal transplant, and urinary diversion was admitted to the hospital with pelvic pain. MRI suggested a dilated bicornuate uterus with HC and 6 cm LVA. She was counseled on management options, and elected IR drainage. US-guided, percutaneous drainage of the uterine cavity was performed with complete decompression of HC. Cone beam CT confirmed uterine anatomy after drainage. NAT was initiated for long-term menstrual suppression. Table 1 provides a detailed summary of each case. <h3>Comments</h3> We present a novel approach to management of symptomatic HC from LVA via image-guided percutaneous drainage of HC by IR. This simple outpatient procedure can successfully relieve symptoms and allow for menstrual suppression to serve as a temporizing measure until patients are mature enough to undergo definitive surgery for various obstructive uterovaginal anomalies.

MANAGEMENT OF ENDOCRINE DISEASE: Diagnosis and management of primary amenorrhea and female delayed puberty.

Puberty is the period of transition from childhood to adulthood characterized by the attainment of adult height and body composition, accrual of bone strength and the acquisition of secondary sexual characteristics, psychosocial maturation and reproductive capacity. In girls, menarche is a late marker of puberty. Primary amenorrhea is defined as the absence of menarche in ≥ 15-year-old females with developed secondary sexual characteristics and normal growth or in ≥13-year-old females without signs of pubertal development. Furthermore, evaluation for primary amenorrhea should be considered in the absence of menarche 3 years after thelarche (start of breast development) or 5 years after thelarche, if that occurred before the age of 10 years. A variety of disorders in the hypothalamus-pituitary-ovarian axis can lead to primary amenorrhea with delayed, arrested or normal pubertal development. Etiologies can be categorized as hypothalamic or pituitary disorders causing hypogonadotropic hypogonadism, gonadal disorders causing hypergonadotropic hypogonadism, disorders of other endocrine glands, and congenital utero-vaginal anomalies. This article gives a comprehensive review of the etiologies, diagnostics and management of primary amenorrhea from the perspective of pediatric endocrinologists and gynecologists. The goals of treatment vary depending on both the etiology and the patient; with timely etiological diagnostics fertility may be attained even in those situations where no curable treatment exists.

Congenital genital tract obstruction as a challenge for functioning reproductive health: a study from a tertiary care center in Nepal

Aims: To find out the uterovaginal anomalies associated with congenital genital tract obstruction and successes achieved in its management.&#x0D; Methods: This was a descriptive study done at department of Obs/Gyn at Tribhuvan University Teaching Hospital from April 2014 to April 2018. Types of genital tract obstruction, surgical treatment, success of management and surgical complications were studied.&#x0D; Results: Of 35 cases 16 (46%) consisted of adolescents aged 11-15 yrs. Majority cases of GTO was due to vaginal septum 12(34%): longitudinal 4(33%), mostly transverse vaginal septum (TVS) 8(67%): upper TVS 4 and lower TVS 4,one among each associated with concurrent Imperforate hymen (IH). Other GTO consisted of only IH cases 9 (26%), vaginal agenesis 7 (21%), non- communicating rudimentary horn 4 , non-communicating right cornua 1and cervical stenosis 1(3%). Resection of septum performed in 12 cases of vaginal septum. hymenectomy in 8 and rudimentary horn excision in 4 cases. Restenosis was common complication in six cases.&#x0D; Conclusions: Adolescent age of 11-15 years is the common age of presentation of genital tract anomaly with vaginal septum the most common. Pre-surgical assessment is the key to successful surgical outcome.&#x0D; Keywords: adolescent, congenital, genital tract obstruction, resection

Surgical Correction and Rehabilitation of Patients with Congenital Uterovaginal Anomalies and Concomitant Genital Endometriosis

Study Objective To evaluate the results of surgical treatment and rehabilitation in patients with uterovaginal anomalies and concomitant external genital endometriosis. Design Prospective cohort study. Level II-1. Setting Federal State Budget Institution «Research Center for Obstetrics, Gynecology and Perinatology» Ministry of Healthcare of the Russian Federation. Patients or Participants Since 2013 to 2019 we examined 198 patients with various uterovaginal anomalies. The 1st group included 105 patients with concomitant endometriosis, the 2d consisted of 93 patients without endometriosis. Interventions Surgical treatment of uterovaginal anomalies was made according to the anatomical type of malformation, clinical manifestations and reproductive outcomes. In cases with concomitant endometriosis we made excision and coagulation of endometriotic lesions. Measurements and Main Results The prevalence of primary infertility was significantly higher in 1st group- 55.5%, compared with the 2d group - 25.3% (p = 0.0057). The index of dysmenorrhea was significantly higher in 1-st group - 53.3%, compared with the control group -36, 6% (p = 0.0179). The frequency of external genital endometriosis was 53%. In 20% of patients with aplasia of the uterus and vagina without functional rudimental horns endometriotic lesions were visualized on pelvic peritoneum. Conclusion The laparoscopy revealed no significant differences in the incidence of endometriosis among patients with and without outflow obstruction of menstrual blood. Sampson's theory of retrograde menstruation doesn't explain the development of endometriosis in all patients with uterovaginal anomalies. Comprehensive treatment, including surgical correction of the anatomic type of malformation, with incision and coagulation of endometriotic lesions helped to get pregnancy in 60% of patients.

Uterovaginal Anomalies: A Guide for the Generalist Obstetrician-Gynecologist.

Congenital gynecologic anomalies result from interruption of embryologic development of the female reproductive tract. The anomalies may be hymenal, vaginal, cervical, or uterine. The impact of these anomalies is variable: some are asymptomatic, incidental findings that require no intervention, others require simple surgical management, while some complex anomalies may require a multidisciplinary approach with extensive surgical expertise for optimal outcomes. Uterovaginal anomalies may occur in isolation or in association with other malformations, such as renal anomalies. The origin, presentation, evaluation and treatment of these conditions are reviewed here.

Congenital vaginal agenesis, davydov procedure: A case report and review of literature

Congenital vaginal agenesis, also called Mullerian agenesis or Rokitansky–Mayer–Kuster–Hauser syndrome, is a rare anomaly of the female genital tract. The affected individuals present with primary amenorrhea, varying degree of uterovaginal anomalies, infertility, and other associated renal, spine, and skeletal malformations. Embryologically, the vagina is formed from the fusion of the two embryonic structures: the upper part from the Mullerian duct system and the lower third from the urogenital sinus. Mullerian agenesis is caused by embryologic underdevelopment of the Mullerian duct, with resultant agenesis or atresia of the vagina, uterus, or both. We report the case of a 25-year-old woman who presented with complaints of delayed onset of menses and inability to have sexual intercourse with her partner. She had well-developed secondary sexual characteristics. She had never experienced penetrative sexual intercourse despite being married for 2 years. A general physical examination revealed a young woman of normal stature. She had well-developed breast (Tanner stage 5). The clitoris and pudendal cleft were normal. There was normal external urethral meatus with blind ended vagina of 2 cm long. A digital rectal examination revealed good sphincteric tone with no uterus or cervix palpated. A diagnosis of congenital uterovaginal anomaly was made. A buccal smear done revealed XX karyotype. She was counseled about her condition. Consents for surgery and media release were sought and obtained. A Davydov procedure for neovaginal construction was made and her postoperative condition was satisfactory. Congenital vaginal agenesis is a rare condition with profound effects on the affected individuals. Therefore, proper counseling on future fertility options and the need for neovaginal construction for improved sexual satisfaction should never be overemphasized among others.

A New Perspective on the Problem of Concomitant Endometriosis and Genital Malformations: Surgical and Embryogenetic Aspects

Study Objective To optimize the results of surgical treatment and rehabilitation of patients with genital tract malformations combined with external genital endometriosis. Design Prospective study. Level II. Setting Department of Operative Gynecology, V.I. Kulakov National Medical Research Center for Obstetrics, Gynecology and Perinatology, Ministry of Healthcare of Russian Federation. Patients or Participants 453 patients with utero-vaginal malformations underwent surgery from 2013 to 2018. Based on the presence of endometriotic foci, two groups of patients were created: group 1 included 240 patients with uterine and vaginal malformations and external genital endometriosis; group 2 included 213 patients with uterine and vaginal malformations without concomitant endometriosis. Interventions All patients underwent surgical treatments using laparocopic and hysteroscopic approaches for correction of congenital malformations and endometriosis excision. Measurements and Main Results The indications for surgical treatment of patients with utero-vaginal anomalies included primary infertility in 41.5%, secondary infertility in 18.6%, recurrent miscarriages in 26.2%, dysmenorrhea in 45.4%, menorrhagia in 23.2%, pain syndrome in 17.2%, and dyspareunia in 8.1% cases. External genital endometriosis was diagnosed in 53.0% of these patients. We revealed no significant difference in the incidence of endometriosis in patients with utero-vaginal anomalies with or without the obstruction to the menstrual flow. Conclusion Implantation theory cannot explain the development of endometriosis in all patients with utero-vaginal malformations. Therefore, all pathogenetic theories must be considered in the investigation of possible mechanisms of endometriosis development in patients with genital tract malformations.

Functional (Dynamic Contrast-Enhanced) MRI Assists in the Evaluation of Blood Perfusion in Myometrium and Intrauterine Septi of Women with Symmetric Uterine Anomalies

Study Objective Successful reproductive outcomes of patients with uterovaginal anomalies remain in the range of 25-37%, despite applying minimally invasive surgical corrections and assisted reproductive methods. The objective of our study is to introduce functional/dynamic enhanced MRI evaluation of the vascular circulation of the myometrium and intrauterine septum in patients with symmetric uterine anomalies. Design Prospective cohort study. Setting National Medical Research Center for Obstetrics, Gynecology and Perinatology, Moscow, Russia. Patients or Participants 213 patients of patients with symmetric uterine anomalies were evaluated between 2015 and 2017 at our department. Interventions Surgical correction of genital malformations was performed in accordance with clinical manifestations: obstruction of menstrual outflow, abdominal pain, infertility, miscarriage, sexual problems. The new method of functional/dynamic contrast enhanced (DCE) MRI was employed in order to estimate uterine blood perfusion in symmetric uterine malformations. Dynamic monitoring of serial images evaluated the blood supply in the myometrium and septum by digital diagram and color-mapping. Measurements and Main Results The functional MRI was performed in 42 patients requiring surgery, and 30 patients managed without surgical correction. Microcirculation was reduced by over 32 % in most patients with sub-septate uterus, and in 46% of patients with complete uterine septum, increasing the need for hysteroresectoscopic metroplasty in these patients. Histopathologic evaluation of the resected septi identified the presence of significant uterine dysmorphogenesis, vascular malformations, and deep myometrial fibrosis. The asymmetric perfusion of the duplicate uterus was detected in 85% of patients. Successful pregnancy progressed in hemi-uteri with better perfusion results (p Conclusion Functional/dynamic-enhanced MRI identifies the degree of blood perfusion in the myometrium and intrauterine septi of women with symmetric uterine anomalies and may assist in development of surgical and assisted reproductive strategies in the management of these patients.

Management of Acute Obstructive Uterovaginal Anomalies: ACOG Committee Opinion Summary, Number 779.

Obstructive uterovaginal anomalies may present after puberty with amenorrhea, dysmenorrhea, pelvic pain, recurrent vaginal discharge, or infertility. The evaluation of a patient with a suspected obstructive reproductive anomaly should include a detailed medical history, physical examination, and imaging. The genital examination is critical to differentiate a patient with an imperforate hymen from a patient with labial adhesions, urogenital sinus, transverse vaginal septum, or distal vaginal atresia. Pelvic ultrasonography is the initial imaging method recommended for a patient with cyclic pain and amenorrhea or a patient with persistent dysmenorrhea. It is important to note that diagnosis of a uterine or vaginal anomaly by imaging before puberty can be challenging and misleading because of the small size of the prepubertal uterus and the lack of endometrial stimulation and menstrual distention of the vagina. Consultation with a radiologist experienced with imaging of uterovaginal anomalies may be helpful to determine the most accurate diagnosis. In general, obstructive vaginal and uterine anomalies are not surgical emergencies, and the complexities of these conditions are best managed by gynecologic care providers familiar with the surgical management of these conditions. Given the high risk of stenosis and complications associated with transverse vaginal septum, distal vaginal atresia, and cervical atresia, referral to a center with expertise in the management of these anomalies is paramount. The best long-term outcome is achieved with a complete evaluation, clear understanding of the anomaly, mobilization of appropriate surgical resources, sufficient preoperative counseling, and planned surgical intervention.

Management of Acute Obstructive Uterovaginal Anomalies: ACOG Committee Opinion, Number 779.

Obstructive uterovaginal anomalies may present after puberty with amenorrhea, dysmenorrhea, pelvic pain, recurrent vaginal discharge, or infertility. The evaluation of a patient with a suspected obstructive reproductive anomaly should include a detailed medical history, physical examination, and imaging. The genital examination is critical to differentiate a patient with an imperforate hymen from a patient with labial adhesions, urogenital sinus, transverse vaginal septum, or distal vaginal atresia. Pelvic ultrasonography is the initial imaging method recommended for a patient with cyclic pain and amenorrhea or a patient with persistent dysmenorrhea. It is important to note that diagnosis of a uterine or vaginal anomaly by imaging before puberty can be challenging and misleading because of the small size of the prepubertal uterus and the lack of endometrial stimulation and menstrual distention of the vagina. Consultation with a radiologist experienced with imaging of uterovaginal anomalies may be helpful to determine the most accurate diagnosis. In general, obstructive vaginal and uterine anomalies are not surgical emergencies, and the complexities of these conditions are best managed by gynecologic care providers familiar with the surgical management of these conditions. Given the high risk of stenosis and complications associated with transverse vaginal septum, distal vaginal atresia, and cervical atresia, referral to a center with expertise in the management of these anomalies is paramount. The best long-term outcome is achieved with a complete evaluation, clear understanding of the anomaly, mobilization of appropriate surgical resources, sufficient preoperative counseling, and planned surgical intervention.

Isolation and Identification of Cell Cultures from the Endometrium and Endometriotic Tissue of Women with Uterovaginal Anomalies

Objective: To investigate the immunophenotype of cultured mesenchymal stromal cells isolated from eutopic and ectopic endometrium in women with uterovaginal anomalies.

Herlyn-Werner-Wunderlich Syndrome: A Mini-review

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital malformation syndrome that is characterized by a triad of uterine didelphys, blind hemivagina, and ipsilateral renal agenesis. There is a wide variety of phenotypic presentation which is recognized as a spectrum of disease rather than a separate entity. The exact incidence and pathogenesis of HWW syndrome are yet to be investigated. While this disease typically involves adolescent girls who present with abdominal pain or a pelvic mass that is secondary to hematocolpos, nowadays, a majority of potential patients with HWW are being prenatally screened for renal anomalies. Therefore, it is recommended to search for uterovaginal anomalies whenever a multicystic dysplastic kidney or the absence of a kidney is noted in a newborn female, and the role of pediatric nephrologists has become ever more important for early recognition of the disease. Key words: Müllerian duct anomaly, renal agenesis, hemivagina, Uterus didelphys, female genital anomalies

Magnetic resonance imaging in diagnosis and classification of uterovaginal congenital anomalies

Uterine congenital anomalies are clinically relevant because these are associated with increased incidences of infertility and menstrual disorder. These are frequently not diagnosed at birth. These anomalies usually diagnosed at child-bearing age when reproductive malfunction arise. When normal mullerian duct development interrupted at any stage, it results in mullerian abnormality. Magnetic resonance imaging (MRI) has an excellent role in evaluation of mullerian duct anomalies. Ultrasonography is the primary investigation; however, MRI is an excellent noninvasive investigation for accurate evaluation of uterine congenital anomalies. MRI is a very good modality to evaluate the vaginal malformation, which usually difficult to evaluate by ultrasound.

158 - Surgical Treatment and Rehabilitation of Patients with Congenital Uterovaginal Anomalies and Concomitant Endometriosis

Study Objective: To evaluate the results of surgical correction and rehabilitation in patients with uterovaginal anomalies and concomitant endometriosis.

Comprehensive Approach to the Diagnosis, Surgical Correction and Rehabilitation of Patients with Uterovaginal Anomalies in Combination with Endometriosis

Comprehensive Approach to the Diagnosis, Surgical Correction and Rehabilitation of Patients with Uterovaginal Anomalies in Combination with Endometriosis

Classification and Surgery of Uterovaginal Anomalies

Classification and Surgery of Uterovaginal Anomalies

New theory of uterovaginal embryogenesis

ABSTRACTBackground: The explanation of uterine and vaginal embryogenesis in humans still poses many controversies, because it is difficult to assess early stages of an embryo. The literature review revealed many disagreements in Mullerian theory, inciting some authors to propose new embryological hypotheses. In the original Mullerian theory: the paramesonephral ducts form the Fallopian tubes, uterus and vagina; the mesonephral ducts regress in female embryos. Aims: The aim of this article is to investigate the development of Mullerian ducts in humans, using comparative analysis of fundamental embryological theory and various utero-vaginal anomalies. Material and methods: Between 1998 and 2015, 434 patients with various uterovaginal malformations had been operated on at the Scientific Centre of Obstetrics Gynaecology and Perynatology in Moscow. The anatomies of the uterovaginal malformations in these patients were diagnosed with ultrasound and MRI and then verified during surgical correction by laparoscopy. Results: A systematic comparison of uterovaginal malformations to those in the literature has allowed us to formulate a new theory of embryonic morphogenesis. The new theory is significantly different: ovary, ovarian ligamentum proprium, and ligamentum teres uteri derive from gonadal ridges; Fallopian tubes and vagina completely develop from mesonephral ducts. The uterus develops in the area of intersection between the mesonephral ducts with gonadal ridges by the fusion of the two. Conclusions: The new theory may to induce future embryological studies. The hypothetic possibility that the ovary and endometrium derive from the gonadal ridges could be the key to understanding the enigmatic aetiologies of extragenital and ovarian endometriosis.