Vaginal Agenesis Research Articles

Mullerian Agenesis in One of the Non-Identical Twins: A Case Report and Literature Review

Mullerin agenesis also referred as mullerian aplasia, Mayer-Rokitansky-Kuster-Hauser Syndrome (MRKH) or vaginal agenesis is the congenital anomaly of vertical fusion defect of mullerian system resulting in variable abnormalities of female reproductive tract with normal functioning ovaries. It is second most common cause of primary amenorrhea after gonadal agenesis. The occurrence of mullerian agenesis in twins is very rare. We present the case of one of the non-identical twins, 22 years female, with primary amenorrhea. Her elder twin sister had a normal female reproductive system. In our case, diagnosis was supported by presence of definitive primary amenorrhea with well-developed secondary sexual characteristics. Normal physiological hormonal levels ruled out the ovarian or pituitary pathology. Imaging studies like ultrasonography and magnetic resonance imaging (MRI) provided additional confirmation.

Ectopic Ureter Causing Hydrometra in a Case of Cervical Dysgenesis with Vaginal Agenesis

Ectopic Ureter Causing Hydrometra in a Case of Cervical Dysgenesis with Vaginal Agenesis

ACOG Committee Opinion No. 728: Müllerian Agenesis: Diagnosis, Management, And Treatment.

Müllerian agenesis, also referred to as müllerian aplasia, Mayer-Rokitansky-Küster-Hauser syndrome, or vaginal agenesis, has an incidence of 1 per 4,500-5,000 females. Müllerian agenesis is caused by embryologic underdevelopment of the müllerian duct, with resultant agenesis or atresia of the vagina, uterus, or both. Patients with müllerian agenesis usually are identified when they are evaluated for primary amenorrhea with otherwise typical growth and pubertal development. The most important steps in the effective management of müllerian agenesis are correct diagnosis of the underlying condition, evaluation for associated congenital anomalies, and psychosocial counseling in addition to treatment or intervention to address the functional effects of genital anomalies. The psychologic effect of the diagnosis of müllerian agenesis should not be underestimated. All patients with müllerian agenesis should be offered counseling and encouraged to connect with peer support groups. Future options for having children should be addressed with patients: options include adoption and gestational surrogacy. Assisted reproductive techniques with use of a gestational carrier (surrogate) have been shown to be successful for women with müllerian agenesis. Nonsurgical vaginal elongation by dilation should be the first-line approach. When well-counseled and emotionally prepared, almost all patients (90-96%) will be able to achieve anatomic and functional success by primary vaginal dilation. In cases in which surgical intervention is required, referrals to centers with expertise in this area should be considered because few surgeons have extensive experience in construction of the neovagina and surgery by a trained surgeon offers the best opportunity for a successful result.

Neovaginal Construction with Pelvic Peritoneum: Reviewing an Old Approach for a New Application.

Transgender reassignment surgeries have become a major topic of interest within the last decade. Although there are numerous surgical techniques for male-to-female reassignment, a physician must assess each patient's anatomy and past medical history to determine the most suitable technique. Additionally, patient preference is important. The neovaginal constructive technique most commonly used in male-to-female reassignment surgery is penile skin inversion, but various other techniques and tissues can also be used including pelvic peritoneum, buccal mucosa, and bowel. Surgical text descriptions were enhanced by creating new anatomical illustrations. Donor and recipient site anatomies, and the surgical technique leading to creation of the neovagina, are demonstrated in detail with relevant illustrations. A review of the literature concerning the anatomy, procedure development, and outcomes is presented. The pelvic peritoneum was originally used in neovaginal construction for females with vaginal agenesis as a result of MRKH syndrome. The use of this technique to create a neovagina in male-to-female transgender reassignment surgery has become appealing owing to the relative ease of the procedure, low complication rates, and overall high patient satisfaction. This technique offers a potential new choice for transgender male-to-female neovaginal construction, but further studies are needed to confirm its successful use in transgender surgery. Clin. Anat. 31:175-180, 2018. © 2017 Wiley Periodicals, Inc.

Congenital vaginal atresia: A report of 39 cases in a regional Obstetrics and Gynecology Hospital.

To investigate the clinical course and management of congenital vaginal atresia. This retro-spective analysis included patients with congenital vaginal atresia treated from March 2004 to August 2014 at the Obstetrics and Gynecology Hospital of Fudan University. Thirty-nine patients were included in this study. Their average age was 16.87±2.2 years when they came to our hospital. Totally, 51% of the patients had isolated congenital vaginal atresia with a normal cervix, whereas the others had either cervical atresia or imperforate hymen. The primary presenting signs and symptoms included primary amenorrhea (71.8%), periodic abdominalgia (41.0%), abdominal pain (36.0%), dyspareunia (10.3%), menstrual disorders (5.1%), and pelvic mass (5.1%). Ultrasound and magnetic resonance imaging (MRI) were effective inspection methods for the screening of urogenital tract-associated anomalies. Vagi-noplasty mainly included simple vagina reconstruction with insertion of a mold (n=22) and split-thickness skin grafting (n=4). In 64% of surgical patients, normal menstrual bleeding was achieved. Four of the patients subsequently became pregnant and delivered at term. Primary amenorrhea, periodic abdominalgia and abdominal pain are the main reasons for the post pubertal patients to visit doctors. Surgical methods can successfully provide these patients an opportunity for subsequent conservative management, can result in normal menstrual bleeding, resolve cyclic pelvic pain, and provide some po-tential for fertility.

Ultrasound-guided introital drainage of pyometrocolpos

Abstract Pyometrocolpos can be caused by congenital malformations such as distal vaginal atresia and imperforate hymen. Patients usually present with obstructive urinary tract infections, acute kidney injury, or sepsis. Percutaneous drainage of the infected fluid can help treat the patient; however, recurrence is of concern. In this case report, we present a case of a child with recurrent pyometrocolpos due to distal vaginal atresia despite initial percutaneous drainage. To our knowledge, this is the first report of ultrasound-guided introital drainage of pyometrocolpos with relief of symptoms obviating the need for repeat drainage or immediate surgery.

A modified neo-vagina procedure in a low resource urogynecological unit: a case report of a 21\xa0year old with Mayer-Rokitansky-K\xfcster-Hauser (mrkh) Syndrome operated at Mbarara referral hospital, Southwestern Uganda

BackgroundAlthough vaginal agenesis as may occur in Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a rare condition, it is associated with not only anatomical problems but also serious psychological and social problems like painful sexual intercourse, primary amenorrhea and infertility. Surgery, which is aimed at reconstruction of a vagina of adequate length and width to serve the function, is the main method of treatment. Many methods for vaginal reconstruction have been described but each has its complications and limitations. The most commonly preferred procedure for treating this condition is the McIndoe vaginoplasty which involves dissection into the recto-vesical space, inserting two split thickness skin grafts folded over a mold in this newly created space and regular dilatation of the neovagina postoperatively to avoid stenosis. However surgeons with this expertise in this part of the world are rare to find and where they are available, the special molds on which to fold the skin grafts into the neovaginal space are not readily available.Case presentationA 21-year-old female with vaginal agenesis was operated on using a modification of the McIndoe procedure using a cylinder of a 60cm3 syringe as a vaginal mold/form and kept in place. We left a Foley in place for 10 days and we did a dye test after removing the syringe to ensure that there was no leakage resulting from fistula formation.ConclusionThe operation was successful and on subsequent monthly reviews of the patient, she has a patent functional vagina of about 9 cm in length at 8 months after the operation with resumption of sexual intercourse.

Management of three cases of pyometrocolpos due to distal vaginal atresia in infants

Children with pyometrocolpos due to distal vaginal atresia may present as acutely ill, with severe obstructive uropathy and septicemia. In such patients the clinical course is markedly improved by urgent drainage of the infected cystic mass, and a temporary drainage procedure is required to allow local findings of infection to subside before definitive surgery. We present a 3 cases one at 2 months and two cases at 4- months of age old females with pyometrocolpos two of them with the above-mentioned clinical course and one of them with typical presentation of Mayer Rokitansky Kuster Houser syndrome (MRKH syndrome) ( is characterized by Mullerian duct structures agenesis, vaginal atresia being the commonest variant) ,presenting and having association of anorectal malformation, Mullerian duct agenesis and renal anomaly , all received urgent laparotomy and drainage through the upper abdominal wall transverse incision (was performed in spite of the high risks of complex drainage procedures and general anesthesia). This drainage did result in dramatically improved clinical status, and findings of local inflammation resolved within 4-6 weeks, which facilitated a later successful definitive surgical procedure.

Clinical features and surgical procedures of congenital vaginal atresia—A retrospective study of 67 patients

ObjectiveTo explore the characteristics of congenital vaginal atresia, further improve its classification, and therefore help the clinical diagnosis and treatment of congenital vaginal atresia. MethodsThis was a retrospective study of 67 patients with congenital vaginal atresia (from March 1984 to March 2015). Clinical and surgical characteristics were analyzed. ResultsFor lower vaginal atresia, 25 patients successfully underwent vaginoplasty at the lower portion of the vagina. For complete vagina atresia, 25 patients with type i cervical atresia were treated with artificial vaginoplasty+tracheloplasty, and all showed no dysmenorrhea within six months after surgery. Four patients with type ii cervical atresia and two patients with type iii cervical atresia successfully underwent hysterectomy+artificialvaginoplasty. Two patients with type iv cervical atresia underwent combined abdominoperineal artificial vaginoplasty+tracheloplasty. One patient with upper vaginal atresia successfully underwent hysterectomy via the narrow segment of the cervix. Three patients with top vaginal atresia had no dysmenorrhea after transvaginaltracheloplasty. ConclusionThis study suggests two new categories of vaginal atresia (upper vaginal atresia and top vaginal atresia), which could be used as a reference for treatment of this condition. Appropriate treatments were performed using a personalized approach and satisfactory results were achieved.

Providers' Experiences with Vaginal Dilator Training for Patients with Vaginal Agenesis

Study ObjectiveTo examine providers' experiences with vaginal dilator training for patients with vaginal agenesis. Design and SettingAnonymous electronic survey. ParticipantsMembers of the North American Society for Pediatric and Adolescent Gynecology. Interventions and Main Outcome MeasuresHow providers learn about vaginal dilator training, common techniques, and methods used for patient training, assessment of patient readiness, common patient complaints, issues leading to early discontinuation. ResultsThere were a total of 55 completed survey responses of which 31 respondents (56%) had been in practice for more than 10 years. Forty-nine were gynecologists (89%), 20 had completed a fellowship in pediatric and adolescent gynecology (36%), and 6 were reproductive endocrinologists (11%). Thirty-one respondents had first learned about vaginal dilator training through lectures (56%) whereas only 9 through mentorship and fellowship (16%). According to respondents, the most common issue leading to early discontinuation was lack of patient motivation and readiness (n = 42; 76%). The most common complication was pain or discomfort (n = 45; 82%). More than half of respondents determined dilator therapy was successful when patients reported comfortable sexual intercourse (n = 30; 55%) and 65% (n = 35) did not delineate any restrictions to initiation of sexual intercourse. Most respondents (87%) requested further vaginal dilator training at either a clinical meeting (n = 26; 47%) or with a training video (n = 22; 40%). ConclusionOur study in an experienced cohort of pediatric gynecology providers highlights the need for further research and training on vaginal dilation education.

Vaginoplasty with Uterovaginal Anastomosis for partial Vaginal and Complete Cervical Agenesis: A Rare Müllerian Anomaly

ABSTRACT Congenital absence of cervix is a rare condition that occurs in 1 in 80,000 to 100,000 births. Here, we report a case of a 20-year-old, married woman who presented to the gynecology outpatient department with primary amenorrhea and complaints of cyclical left-sided lower abdominal pain and difficult coitus for 2 years. Magnetic resonance imaging report suggested unicornuate uterus with hematometra and cervical agenesis. On laparotomy, unicornuate globular uterine horn with absent cervix was present on the left side and separate solid rudimentary horn was found on right lateral pelvic wall. Left adnexa was normal, whereas right ovary was enlarged with minimal hydrosalpinx. Vaginoplasty with uterovaginal anastomosis was performed after draining the hematometra and successful restoration of cyclical menses was achieved on follow-up. How to cite this article Mishra VV, Nawal R, Aggarwal R, Nanda S, Bandwal P, Gandhi K. Vaginoplasty with Uterovaginal Anastomosis for partial Vaginal and Complete Cervical Agenesis: A Rare Müllerian Anomaly. J South Asian Feder Obst Gynae 2017;9(3):274-276.

Approaches to Female Congenital Genital Tract Anomalies and Complications

Objective: Female congenital genital tract anomalies may appear with quite confusing and deceptive complications. This study aims to evaluate the difficulties in diagnosis and treatment of female congenital genital tract anomalies that frequently present with complications. Summary: During a 10-year period, we evaluated 20 female patients with congenital genital tract anomalies aged between 3 days and 16 years. All patients were retrospectively analyzed in terms of the results of diagnostic studies, surgical intervention, and treatment. Methods: Ultrasonography and magnetic resonance imaging revealed hydromucocolpos or hematocolpometra, imperforate hymen, distal vaginal atresia, didelphys uterus, an obstructed right hemivagina, uterovaginal atresia, a unicornuate uterus with a noncommunicating rudimentary horn, a vesicovaginal fistula, a utero-rectal fistula, intraabdominal collection, and a vaginal calculus. Results: Two patients had Mayer-Rokitansky-Küster-Hauser syndrome and 6 patients had obstructed hemivagina and ipsilateral renal anomaly syndrome. Definitive surgical interventions were hymenotomy, vaginal pull-through, vaginovaginostomy, and vesicovaginal fistula repair using a transvesical approach. In conclusion, female congenital genital tract anomalies may appear with a wide range of complications. Conclusions: There is a potential to do significant harm, if the patient's anatomic problems are not understood using detailed imaging. Revealing the anatomy completely and defining the complications that have already developed are critical to tailor the optimal treatment strategies and surgical approaches.

Simple neovaginoplasty using spontaneous regeneration ability of labial and vestibular flap in patients with Müllerian agenesis.

Objectives:This study is aimed to introduce a simple neovaginoplasty procedure without significant complications using the spontaneous regenerative ability of labial and vestibular advancement flaps in patients with Müllerian agenesis.Materials and methods:Prospectively collected data of 5 patients with vaginal agenesis due to Müllerian duct abnormality who underwent neovaginoplasty using labial and vestibular advancement flaps were retrospectively reviewed. Operative details, perioperative outcome, complications, length and width of the neovagina, and the postoperative sexual activity were evaluated.Results:The mean operation time was 48 min (range 30–60 min) and the duration of follow-up ranged from 7 to 50 months. The mean length of the neovagina was 9.6 cm × 3.5 cm and 10.8 cm × 3.5 cm at hospital discharge and at final follow-up, respectively. No significant complications occurred during or after surgery. Epithelialization was completed by 8–20 months and the time to first sexual intercourse ranged from 3 weeks to 27 months and none of the patients experienced any intercourse-related difficulties.Conclusion:Our neovaginoplasty technique using labial and vestibular advancement flap is simple, safe, minimally invasive and effective while avoiding the morbidity associated with other grafting techniques.

Evaluation of amnion in creation of neovagina in women with Mayer-Rokitansky-Kuster-Hauser syndrome

To assess the outcome of amnion vaginoplasty in cases of vaginal agenesis due to Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome managed at the authors' institution. Retrospective study. Tertiary care hospital. Fifty women with MRKH who underwent neovaginoplasty. Modified McIndoe's vaginoplasty was done in all the patients, using human amnion graft. Functional status assessed by Female Sexual Function Index, anatomic status (length and width of neovagina), and epithelialization of vagina. Mean (±SD) vaginal length after surgery was 8.2 ± 1cm. Mean vaginal length at 6-month follow-up in sexually active patients was significantly longer as compared with the patients who were not sexually active after surgery (8.4 ± 1.04cm vs. 6.6 ± 2.4cm). Mean Female Sexual Function Index score was 30.8 ± 2.1. Vaginal biopsy showed complete epithelialization of vaginal mucosa. In a developing nation like India, McIndoe's method with amnion graft seems to be a promising option owing to its low cost, easy availability, and safety, ease of the procedure not requiring any special instrument, physiologic outcome with respect toepithelialization of the vagina without hair growth, and satisfying functional outcome.

Outcomes in patients undergoing robotic reconstructive uterovaginal anastomosis of congenital cervical and vaginal atresia.

ObjectiveTo introduce our experience of robotic surgery of reconstructive uterovaginal anastomosis and operative outcomes in congenital cervical and vaginal atresia patients.MethodsClinical observation and follow‐up of four patients with congenital cervical and vaginal atresia who underwent robotic reconstruction of cervix and vagina by SIS (small intestinal submucosa, SIS) graft.ResultsAverage patient age was 13.8 ± 2.2. Patients complained of severe periodic abdominal pain. Diagnosis was made according to clinical characteristics, physical examination, MRI and classified by ESHRE/ESGE system. All patients underwent reconstruction of cervix and vagina by uterovaginal anastomosis by SIS graft. Average operation time was 232.5 ± 89.2 min, average blood loss was 225.0 ± 95.7 mL. After surgery, all patients have regular menstruation without pain. Average follow up was 12 months, average vagina length was 8.9 ± 0.3 cm, average vagina width was 2.9 ± 0.1 cm.ConclusionRobotic assisted reconstruction of cervix and vagina is feasible from our experience, enlarged cases and additional studies are required.

Erratum: Menstrual, Sexual, and Obstetrical Outcomes after Vaginal Replacement for Vaginal Atresia Associated with Anorectal Malformation.

Background The authors of this article became aware of significant differences in the management of two females with a rectovestibular fistula and associated vaginal agenesis. In one patient, a sigmoid colovaginoplasty was performed at the time of the posterior sagittal anorectoplasty (PSARP), and the other underwent repair of the rectovestibular fistula with a primary PSARP, but the surgeons elected to delay the timing of vaginal reconstruction. We decided to review the literature, to establish if recommendations could be made to optimize the management of these children based on current evidence. Methods A literature review was conducted to determine the management and long-term outcomes in patients with an anorectal malformation and associated vaginal atresia. Specific gynecological outcomes assessed were menstrual egress and adequacy of the vaginal replacement for penile–vaginal intercourse. Results Eighty-eight cases were included in the review. Age at diagnosis had a bimodal distribution: 0 to 5 years, 56%; >10 years, 37%. Vaginal atresia was recognized before the operation in 45 patients. Types of vaginal atresia encountered were: (a) distal vaginal atresia (n = 17), (b) vaginal agenesis with absent Mullerian development (n = 47), (c) vaginal agenesis with variable Mullerian development (n = 17), and (d) cervico-vaginal agenesis (n = 7). Types of vaginal replacement used were sigmoid colovaginoplasty (n = 26), distal rectal fistula as neovagina (n = 30), terminal ileum (n = 5), vaginal pull-through (n = 9), and others/unknown (n = 8). Two patients followed a perineal dilatation program and nine patients await reconstruction. Median follow-up was 18 months (6 weeks to 31 years). Long-term menstrual outcomes were reported in 18 (21%) patients. Sexual function was reported in 10 (11%) patients. Three pregnancies were reported but none resulted in live births. Conclusion Vaginal atresia is often missed in association with ARMs. Management should be in collaboration with pediatric gynecologists. Due to a lack of long-term outcome data, no definite conclusion can be drawn for the best technique, tissue, or timing of vaginal replacement. The opportunity to perform vaginal replacement in conjunction with the rectal repair may be worth considering because of a shallower pelvis, nonscarred tissue planes, and the excellent surgical exposure.

Vaginoplasty in vaginal agenesis associated with MRKH syndrome with tabularized peritoneal pull-through

In this paper, a 16 year old girl who was diagnosed as a case of Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, underwent a combined laparotomy-peritoneal approach to create a neovagina by using tubularized peritoneal graft with uneventful postoperative period. Vaginal dilation was maintained with a vaginal mould daily for six months and three to four times weekly thereafter. She was followed-up after 2 and 4 weeks in the first month and three monthly for a duration of six months. On second follow-up, adequate vaginal length of 6-7 cm and width of 2.5-3 cm were achieved with healthy vaginal tissue. Hence, the laparotomy-peritoneal approach of using a peritoneal graft for creations of a neovagina can be an effective approach with minimal surgical morbidity to create a passageway for satisfactory intercourse.

Vaginoplasty with an Autologous Buccal Mucosa Fenestrated Graft in Two Patients with Vaginal Agenesis: A Multidisciplinary Approach and Literature Review

Here we describe the procedure and outcomes of a multidisciplinary approach to vaginoplasty using autologous buccal mucosa fenestrated grafts in 2 patients with vaginal agenesis. This procedure resulted in anatomic success, with a functional neovagina with good vaginal length and caliber and satisfactory sexual function capacity and well-healed buccal mucosa. There were no complications, and the patients were satisfied with the surgical results. We conclude that the use of a single fenestrated graft of autologous buccal mucosa is a simple, effective procedure for the treatment of vaginal agenesis that results in an optimally functioning neovagina with respect to vaginal length, caliber, and sexual capacity.

Primary Amenorrhea - A One Year Review

Objective:Primary amenorrhea occurs in about 0.1-2% of reproductive age group women. The aim of this study was to evaluate the etiology and management of patients with primary amenorrhea in a tertiary referral centre. Material and Methods:The study was performed using 13 complete medical records of women with primary amenorrhea who attended the Department of Obstetrics and Gynecology, Maxcure Suyosha Hospital, Hyderabad, India between May 2015 and May 2016. Out of the 13 cases, 8 had 46XX Mullerian agenesis (MRKH syndrome), 4 had 46XY Androgen Insensitivity Syndrome (AIS) and 1 had 45XO/46XY Turner mosaic syndrome. Neovagina formation was done in 8 cases, bilateral gonadectomy was done in 5 cases and bilateral salpingectomy in 3 cases by laparoscopic methods. Results:Minimally invasive removal of gonads was accomplished with less morbidity to patients and without any complications. Histopathology of gonads revealed a sertoli cell pattern in 3 cases and gonadoblastoma in 2 cases. Successful application of surgical technique of neovagina creation using Interceed was achieved in patients with vaginal agenesis. Conclusion:Management of primary amenorrhea is a multi–modal approach with the type of intervention based on the etiology. A thorough evaluation to identify the cause for amenorrhea and timely surgical intervention would produce a better cosmetic and functional result and address the psychosocial issues associated with this condition.

Three-Dimensional (3D) Sonography in Gynecology

In Chang-Gung Memorial Hospital, Lin-Kou, three-dimensional (3D) sonography with power Doppler angiography had been applied in clinical evaluation since 2002. The technique can be utilized in many complicated conditions, and we had cumulative experiences in mainly three circumstances. The first one was to evaluate Mullerian duct anomaly such as HWW syndrome, uterine septum, and vaginal agenesis. The second was to assist differenciating benign from malignant tumors in uterus and ovaries. Finally, abnormal placentation such as placenta accreta was evaluated to predict the obstetric outcome. Currently, over five hundred cases of uterine anomaly and over one thousand cases of uterine tumor were collected. With all these experience, we summarized several diagnostic criterias for vessel characteristics in uterine and ovarian malignancy. Marked increased sensitivity and specificity were obtained in comparison with traditional two-dimensional sonography. Furthermore, we could provide precise and real-time images of congenital Mullerian duct anomaly and adjustable to fit individual angle of uterus with 3D sonography. MRI is also utilized for diagnosis of Mullerian duct anomaly. However, MRI has angle limitation that may affect the accurate diagnosis. It is also more expensive then 3D sonography. In our experiences, 3D sonography is a useful modality in diagnosis of Mullerian anomaly and can provide more details for treatment strategy.