Vaginal Agenesis Research Articles

PO-0911 Herlyn-werner-wunderlich Syndrome And Distal Vaginal Atresia. Two Particular Cases

Background and aims Mullerian anomalies are rare, causing malformations of the female reproductive system as the Herlyn-Werner-Wunderlich Syndrome (HWWS) and distal vaginal atresia. These patients are usually asymptomatic until menarche. There may be associated with renal and urinary tract anomalies. We describe two cases of young girls with occluded vagina associated with urethrovaginal fistula. Cases presentation The first patient is a 3-years-old asymptomatic girl with prenatal diagnosis of left renal agenesis. Ultrasonography, performed 6 months prior to consultation, identified an anechoic cyst in hypogastrium. On genital examination, distal vagina occluded by a protruding membrane over the vaginal introitus. Magnetic resonance revealed a left paravesical formation suggestive of hydrocolpos. By the transmembrane puncture, urine was aspired. Laparoscopy confirms uterus didelphys. Membrane resection enable two hemivaginas. Based on the association of these anomalies, the diagnosis of HWWS was made. Girl present constant loss of urine and cystoureterography performed reveals urethrohemivaginal fistula. The second patient is a 4-years-old girl with occluded vagina suggesting distal atresia. Saline injected through the membrane eliminates by urethra. Cystoureterography confirms urethrovaginal fistula. Conclusions The diagnoses of HWWS (uterus didelphys, unilateral low vaginal obstruction and ipsilateral renal agenesis) also known by OHVIRA and distal vaginal atresia in infancy and early childhood are unusual. The reported cases represent examples off premenarche diagnosis. In both patients, we detected urethrovaginal fistula, association not reported before in literature. Early diagnosis allows appropriate therapeutic management and prevents subsequent complications.

Laparoscopically Assisted Neovaginoplasty in Vaginal Agenesis: A Long-Term Outcome Study in 240 Patients

Study ObjectiveTo assess the long-term outcome of an optimized minimally invasive neovaginoplasty technique in vaginal agenesis. DesignCombined retrospective and prospective study. SettingUniversity hospital. Participants240 patients with congenital vaginal agenesis. InterventionsPatients with an indication for neovagina creation underwent laparoscopically assisted neovaginoplasty involving vaginoabdominal blunt perforation and intracorporeal traction using tension threads and an abdominally positioned extracorporeal traction device. Main Outcome MeasuresLong-term anatomic success, functional success compared with similar-aged controls, long-term complications, and incidence of human papilloma virus (HPV) infections. ResultsDuring median follow-up for 16 (range 11-141) months, mean functional neovaginal length remained stable at 9.5 cm in all patients, including those who had no sexual intercourse and had stopped wearing the vaginal dummy. Median dummy wearing time was 8.6 months. Time to epithelialization depended on the time of onset and frequency of sexual intercourse. At long-term follow-up, median total Female Sexual Function Index score was 30.0, comparable with similar-aged controls. No common long-term complications occurred. Four patients required cauterization of granulation tissue. 7/240 (2.9%) patients were HPV-positive with low- to high-grade squamous intraepithelial lesions, 3 patients reverting to HPV-negative status at long-term follow-up. ConclusionsOur technique creates a neovagina of adequate size and secretory capacity for normal coitus, requiring no prolonged dilation postoperatively, even in the absence of sexual intercourse. The procedure is fast, effective and minimally traumatic, has a very low long-term complication rate and provides very satisfactory long-term functional results.

Cervicovaginal anastomosis by Gore-Tex in Mullerian agenesis.

To determine usage of polytetrafluoroethylene, Gore-Tex, as a stent in patients with congenital cervicovaginal agenesis. Follow-up study. Tertiary center referral hospital. Eight patients with Mullerian agenesis who had congenital cervical agenesis, associated with partial or complete vaginal aplasia. Laparoscopic uterovaginal anastomosis with placement of a polytetrafluoroethylene stent for reconstruction of cervical agenesis. In cases associated with vaginal agenesis, modified McIndoe vaginoplasty was done concomitantly. Eight patients with cervical agenesis who had partial and complete vaginal aplasia underwent vaginoplasty. Mean (SD) follow-up after surgery (3 years old). All patients except three of them, (62.5 %) experienced relief of abdominal pain and regular menstruation. Due to ascending infection three of them (37.5 %) needed reoperation. So, they underwent laparotomy. Two of these three patients had total abdominal hysterectomy and one case tolerated resection of right obstructed side of didelphys uterus, then she became pregnant spontaneously. Four out of these eight cases were married and the others were single. One out of our married patients conceived three times. Her first pregnancy was aborted. She delivered two alive babies after inserting transabdominal Shirodkar suture in her other pregnancies. Uterovaginal anastomosis assisted laparoscopy with polytetrafluoroethylene (Gore-Tex) is effective and hopeful to relieve patients' symptoms, menstruation regularity, and saving fertility. If ascending infection threatening health happens, hysterectomy will be recommended.

Non-Obstructive Müllerian Anomalies

BackgroundThe nonobstructive group of anatomic variants involving the reproductive tract includes vaginal agenesis as well as the congenital anomalies of the vagina and uterus, occurring without pain during the pubertal years. ObjectiveThe objective is to discuss the non-obstructive morphologic variations in anatomy of the uterus and vagina. DesignSystematic review using the GRADE system. ResultsThese congenital anomalies are not associated with abnormalities of the external genitalia and therefore may be missed on routine physical examination. When these anomalies do cause symptoms they may be as minor as difficulty with menstrual hygiene or more significant such as primary amenorrhea, dyspareunia, recurrent pregnancy loss, and reproductive complications. ConclusionsWomen with non-obstructive reproductive tract anomalies present at various ages due to the asymptomatic nature or late symptom onset of certain conditions. An MRI is the gold standard in evaluation of such conditions to aid in confirming the müllerian variant. Each condition requires careful counseling because obstetric and gynecologic risks and consequences may differ. Treatment is individualized in cases of uterovaginal agenesis with both nonsurgical and surgical options available for neovagina creation. In cases of uterine or vaginal septae, the treatment timing may vary depending on patient history. Finally, in cases of non-obstructive communicating uterine horns, the risk of ectopic pregnancy is high in the remnant horn. Should a pregnancy occur in this small underdeveloped horn, therefore, excision is recommended.

Rectovestibular fistula with vaginal atresia: our experience and a proposed course of management.

Rectovestibular fistula with coexisting vaginal atresia poses a surgical dilemma with regard to the timing and type of reconstruction. We present our experience and suggest an appropriate course of management. Seven patients with rectovestibular fistula and coexisting vestibular atresia were operated in our hospital during January 2004 through December 2013. The details of their bowel, menstrual and sexual functions were recorded. Five of the seven patients who underwent anoplasty in childhood presented to us in their teens with primary amenorrhea and cyclical abdominal pain. All five had sigmoid colon neovaginoplasty. Four of these had the uterus or its remnants anastomosed to the neovagina. All four have regular menstrual cycles. One patient is sexually active and has satisfactory sexual function. The bowel function in all the five patients is good. The remaining two patients presented in their infancy and had the anorectovestibular fistula left as the neovagina. The recto-sigmoid was pulled down to form the neoanus. Both these patients have bowel incontinence. We recommend the rectovestibular fistula be used as the neoanus and not as the neovagina. Delayed bowel vaginal replacement has excellent results and allows for optimal assessment of functioning uterine body or remnants.

Novel technique of neovagina creation with uterine serosa in the treatment of vaginal agenesis associated with mullerian agenesis

Abstract Objective Our aim was to create a neovagina with the least surgical morbidity and the best functional outcome. Materials and methods We hereby describe a new technique (Lee's neovaginoplasty) using a combined laparoscopic and vaginal approach in the creation of a neovagina using the uterine serosa layer from the rudimentary uterus and the peritoneum as a graft to line the vagina. This procedure was performed in three patients who were followed-up for a duration of 4 months to 2 years. Vaginal dilation was maintained with a vaginal mold daily for 3 months and three to four times a week thereafter. Results Adequate vaginal length of 6-7 cm and width of 2.5 cm was achieved postoperatively. There were no surgical complications and postoperative recovery was fast. Vaginal examination 1 month later showed healthy vaginal tissue with no necrosis or infection. Long-term follow-up did not show any shortening or stenosis of the vagina. Patients were able to have satisfactory sexual intercourse with no pain. Conclusion The laparoscopic-vaginal approach of using a uterine serosa and peritoneal graft for creation of a neovagina is a simple and effective approach with minimal surgical morbidity that can create a passageway for satisfactory intercourse.

Long-term outcomes of vaginoplasty with autologous buccal micromucosa.

To evaluate the technical feasibility and anatomical and functional outcomes of one-stage vaginoplasty with autologous buccal micromucosa. We retrospectively reviewed our experiences with 38 patients with vaginal agenesis treated with primary surgery from June 2006 to April 2012. All patients underwent transvestibular vaginoplasty with autologous buccal micromucosa. We describe the details of this technique and evaluate the long-term anatomical, functional, and sexual outcomes. A total of 38 patients (33 with Mayer-Rokitansky-Kuster-Hauser syndrome and five with complete androgen insensitivity syndrome) were included in the present study. The mean operative time was 86.4 minutes (range 75-120 minutes). The mean blood loss was 68.5 mL (range 40-80 mL). At a mean follow-up of 33.5 months (range 15-76 months), the mean depth of the neovagina was 8.5±0.66 cm (range 7-10 cm), the mean circumference was 12.3±1.24 cm (range 10-15 cm), and the mean volume was 100±8 mL (range 85-120 mL). By histologic examination, we found the neovaginal mucosa was stratified nonkeratinized squamous mucosa and had secretory function. The mean female sexual function index score of the 32 sexually active patients was 28.8±2.1. No spouse reported discomfort during intercourse. The mean postoperative dependence on the vaginal stent was 15±2.1 months (range 9-20 months). Transvestibular vaginoplasty with autologous buccal micromucosa is an effective and feasible approach for patients with Mayer-Rokitansky-Kuster-Hauser syndrome and complete androgen insensitivity syndrome. The procedure has satisfactory long-term anatomical and functional results. III.

An Atypical Presentation of Vaginal Agenesis

Complete failure of the vaginal plate to canalize results in vaginal agenesis. Vaginal agenesis may occur as an isolated defect secondary to failure of the urogenital sinus to develop the caudal aspect of the vagina,1 or it may occur with associated degrees of Mullerian aplasia. Regardless of the etiology, patients with vaginal agenesis and a functional endometrium often present with primary amenorrhea and cyclic abdominal pain related to hematometra.1-2,4-9 Co-existing urologic and skeletal abnormalities are also common.3,10,11

Vaginal agenesis treated by coital dilatation in 20 patients

Vaginal agenesis treated by coital dilatation in 20 patients

Monti's Principle in the Treatment of Congenital Uterovesical Fistula

Congenital uterovesical fistula is rare and generally associated with genital tract abnormalities derived from mullerian ducts or urogenital sinus. Management is usually challenging, and it involves vaginal reconstruction. A 15-year-old female patient presented with a 2-year history of cyclical hematuria. Investigation revealed a bicornuate uterus and complete vaginal agenesis associated with congenital uterovesical fistula. The fistula was repaired, and a neovagina was created using Monti's technique. Postoperative recovery was uneventful with normal voiding and initiation of regular menstruation through the neovagina. We discuss the options of vaginal reconstruction and stress the advantages of the technique used in this case.

Systematic review of sexual function and satisfaction following the management of vaginal agenesis

Historically, sexual satisfaction following the management of vaginal agenesis was assessed subjectively. Standardized sexual function questionnaires are being used more frequently as instruments to accurately and more objectively assess the subjective nature of sexual outcomes as part of a more holistic approach to the care of women with vaginal agenesis. Articles concerning the management of vaginal agenesis were systematically reviewed, with specific focus on those that discussed functional outcomes, sexual satisfaction and psychosomatic outcomes, and in particular attempted to measure these outcomes. A total of 6,691 articles on vaginal agenesis were identified, with 106 of these reporting sexual satisfaction and psychosomatic outcomes. Only 1 randomized control trial (RCT) was identified, the remaining articles being made up of case series or case reports. Only 17 articles used standardized objective assessment of sexual satisfaction. While the bowel technique had the longest vaginal length at 12.87 cm, it had the most number of complaints of dyspareunia (4.8%), stenosis (10.5%) and the lowest average subjective sexual satisfaction. The Davydov method used standardized sexual function assessments most frequently. This technique had a higher average score than both the bowel vaginoplasty technique in the only RCT and the Vecchietti method in a prospective assessment. Overall, the management of vaginal agenesis requires a multidisciplinary approach to fully support these patients from initial diagnosis, through management decision-making and long-term follow-up, through transition to adulthood.

Hymen Tearing in the Treatment of Vaginal Agenesis

Rupture of the hymen before marriage is a significant problem for a woman and her family. The purpose of this article is to present a case report where hymen membrane perforation was required for vaginal agenesis in a 13-year-old adolescent, identify psychosocial issues related to the torn hymen in the Turkish culture, discuss gender inequality affecting health care of women with a torn hymen, identify the impact of a torn hymen on a Turkish woman's social status, and discuss health care implications for the woman and her family. The girl could not understand the situation and experienced feelings of guilt. The parents displayed an intense reaction of shock and denial after being informed about the disorder and treatment process and felt a sense of hopelessness about the future of their daughter. Nurses are expected to fulfill their training, counseling, and advocacy roles about this issue.

Acellular porcine small intestinal submucosa graft for cervicovaginal reconstruction in eight patients with malformation of the uterine cervix

Can surgical reconstruction of the cervix and vagina in patients be achieved using an acellular porcine small intestinal submucosa (SIS) graft? Our experiences of combined laparoscopic and vaginal cervicovaginal reconstruction using an SIS graft in eight patients were positive, with successful reconstruction and no complications, cervical stenosis or vaginal stenosis. In patients with agenesis and dysgenesis of the uterine cervix and vagina, surgical reconstruction of the internal genitalia is a challenging problem for gynecologists. Hysterectomy with the creation of an artificial vagina was the treatment of choice in the 1990s. Recently, conservative management has been gradually adopted to avoid extirpation of the uterus, including the canalization techniques, the uterovaginal anastomosis and the reconstruction of cervical and vaginal agenesis with some autologous tissues. This prospective observational study from January 2012 to March 2013 included 8 patients aged 10-18 years with malformation of the cervix (1 with cervical agenesis, 1 with a cervical body consisting of a fibrous band and 6 with obstruction of the cervical os) and vagina (4 with complete vaginal aplasia and 4 with a 1-3 cm long vaginal pouch) diagnosed by physical examination and magnetic resonance imaging. Eight patients underwent combined laparoscopic and vaginal cervicovaginal reconstruction using an SIS graft during the end of menstruation. A T-shaped intrauterine device connected with a 14-French Foley catheter was inserted into the uterine cavity to keep the newly created cervix patent, and then a permanent lower uterine cerclage was placed. Patients were assessed post-operatively at 1, 2, 4, 6, 12 and 15 months, and data on menstruation and the morphology of the neovagina and cervix were recorded. The mean ± SD age of the patients was 14.5 ± 2.8 (10-18) years. All patients had a history of cyclic abdominal pain, and the average delay in diagnosis was 4.5 ± 4.0 (0-12) months. One patient had a previous history of unsuccessful attempt at canalization and two post-operative hematometra drainages before referral. The mean operating time was 201 ± 67 (120-330) min, with a mean estimated blood loss of 157 ± 154 (30-500) ml. The first case was converted to laparotomy, and the others were successfully completed. None of the patients had a complication or required blood transfusion. All the patients showed resumption of menstruation. The patients were followed for 8 ± 4 (4-15) months, and no cervical or vaginal stenosis occurred in any of the cases. The sample size of this study was small. A larger study that compared this method with previous techniques regarding the complication and success rates would increase the value of the study. A combined laparoscopic and vaginal cervicovaginal reconstruction with an SIS graft is a potential alternative to the management of congenital agenesis and dysgenesis of uterine cervix and vagina. The work was supported by National Key Clinical Faculty Construction Program of China. No competing interests are declared.

Diagnostic dilemmas in management of neonatal hydrometrocolpos: A crucial role of magnetic resonance imaging

Neonatal hydrometrocolpos is a rare abnormality and can present as an abdominal mass, urinary tract obstruction, anuria, hydroureteronephrosis, constipation or respiratory distress. Hydrometrocolpos in neonate can be due to imperforate hymen, vaginal atresia, vaginal septum, urogenital sinus or even cloaca. Clinical examination and routine ultrasonography cannot differentiate causes of hydrocolpos in all such cases. Surgical approach depends upon the underlying cause of hydrometrocolpos. We report an unusual case of neonatal hydrometrocolpos in which it was extremely difficult to differentiate urogenital sinus from imperforate hymen or vaginal atresia. The patient underwent magnetic resonance imaging (MRI) which clearly showed the imperforate hymen and was helpful in planning management of the case. Hence, there is the definitive role of MRI in the differential diagnosis of hydrometrocolpos and is equally helpful in making surgical decisions.

Pyocolpos with infected right ovarian cyst: A rare case report

Pyocolpos is a rare clinical finding in vaginal atresia. Pyocolpos is cystic dilatation of the vagina due to accumulation of pus due to genital tract obstruction. The causes of obstruction can be imperforate hymen, transverse vaginal membrane, and rarely vaginal atresia. We present a case of 18-year-old married women Go Po+o, admitted with severe pain and swelling of the lower abdomen for 3 months. She had undergone laparotomy with vaginoplasty for vaginal atresia with hematocolpos and pyoperitoneum in 2009 and had refused mould insertion. On laparotomy, she was found to have pyocolpos and infected right ovarian cyst, which was probably due to re-obstruction of vagina. Right ovarian cystectomy followed by subtotal hysterectomy was performed. Post-operative period was uneventful. Following vaginoplasty, follow up and regular vaginal dilatation are essential to avoid complications involving re-obstruction and ascending genital infection.

Partial Vaginal Agenesis with Transverse Vaginal Septum

ABSTRACTPartial vaginal agenesis with transverse vaginal septum is rare malformation of female genital system. The reported incidence of vaginal atresia is 1:4000 to 5000 live female birth. The incidence of septum in upper one-third is 46%, middle 35%, lower 19%. Transverse vaginal septum occurs due to defect in vertical fusion of mullerian ducts during embryogenesis. Vaginal atresia occurs due to failure of canalization of sinovaginal bulbs.We are presenting such a rare case of partial vaginal agenesis with transverse vaginal septum.The aim of this presentation is to make the consideration of transverse vaginal septum and vaginal atresia in young girls with cryptomenorrhea and to emphasize on is meticulous evaluation and diagnosis with counselling of a minor along with parents of future reproductive outcome along with surgical difficulties anticipation and difficulties post vangioplasty.How to cite this articleMuralidhar L, Venkatesh S, Pandey P. Partial Vaginal Agenesis with Transverse Vaginal Septum. Int J Infertil Fetal Med 2014;5(3):110-112.

New laparoscopic peritoneal pull-through vaginoplasty technique.

BACKGROUND:Many reconstructive surgical procedures have been described for vaginal agenesis. Almost all of them are surgically challenging, multi-staged, time consuming or leave permanent scars on abdomen or skin retrieval sites.AIM:A new simple technique using laparoscopic peritoneal pull-through in creation of neo vagina has been described.MATERIAL AND METHODS:Total of thirty six patients with congenital absence of vagina (MRKH syndrome) were treated with laparoscopic peritoneal pull through technique of Dr. Mhatre between 2003 till 2012. The author has described 3 different techniques of peritoneal vaginoplasty.RESULTS:This technique has given excellent results over a period of one to seven years of follow-up. The peritoneal lining changes to stratified squamous epithelium resembling normal vagina and having acidic Ph.CONCLUSION:Apart from giving excellent normal vaginal function, as the ovary became accessible per vaginum three patients underwent ovum retrieval and pregnancy using surrogate mother, thus making this a fertility enhancing procedure.

Unilateral Functional Uterine Horn with Non Functioning Rudimentary Horn and Cervico-Vaginal Agenesis: Case Report

Developmental anomalies involving Mullerian ducts are one of the most fascinating disorders in Gynaecology. The incidence rates vary widely and have been described between 0.1-3.5% in the general population. We report a case of a fifteen year old girl who presented with primary amenorrhea and lower abdomen pain, with history of instrumentation about two months back. She was found to have abdominal lump of sixteen weeks size uterus. On examination vagina was found to be represented as a small blind pouch measuring 2-3cms in length. A rectovaginal fistula (2x2 cms) was also observed. Ultrasonography of abdomen revealed bulky uterus (size 11.2x6 cm) with 150 millilitre of collection. Adiagnosis of hematometra with iatrogenic fistula was made. Vaginal drainage of hematometra was done which was followed by laparotomy. Peroperatively she was found to have a left side unicornuate uterus with right side small rudimentary horn. Left fallopian tube and ovary showed dense adhesions and multiple endometriotic implants. Both cervix and vagina were absent. Total abdominal hysterectomy was done and rectovaginal fistula repaired. The present case is reported due to its rarity as it involved both mullerian agenesis with cervical and vaginal agenesis along with disorder of lateral fusion. This is an asymmetric type of mullerian duct development in which arrest has occurred in different stages of development on two sides. DOI: http://dx.doi.org/10.3329/bjms.v13i1.17500 Bangladesh Journal of Medical Science Vol. 13 No. 01 January2014: 101-104

Mckusick-kaufman syndrome: diagnosis and management.

A female newborn, a product of consanguineous marriage born by caesarian section delivery with a birth weight of 3.75 kg, was admitted to our hospital with abdominal distension. Antenatal ultrasound showed congenital heart disease and a pelvic mass. On examination, she had lower abdominal mass emanating from the pelvis. There was polydactly and syndactly of the right hand and polydactly of the right foot. There was also an anterior ectopic anus. Cardiovascular work up revealed common atrio-ventricular (A-V) canal with single atrium, moderate patent ductus arteriosus and severe A-V regurgitation. Abdominal ultrasound and CT-scan showed hydrometrocolpos with bilateral hydronephrosis and left perinephric fluid collection (Fig. 1). Surgery revealed hydrometrocolpos secondary to a low vaginal atresia. An abdomino-perineal vaginal pull through was performed. Postoperative period was uneventful.

Mayer-rokitansky-kuster-hauser syndrome associated with severe inferior vena cava stenosis.

Precis. The postoperative course of a neovagina creation procedure in a young woman with Meyer-Rokitansky-Kuster-Hauser syndrome was complicated, despite prophylaxis, by extensive pelvic deep venous thrombosis secondary to unsuspected severe inferior vena cava stenosis. Background. Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is characterized by congenital vaginal agenesis and an absent or rudimentary uterus in genotypical females. Malformations of the inferior vena cava (IVC) are not commonly associated with MRKH syndrome. We report a case of a patient with MRKH syndrome with severe IVC stenosis that was diagnosed when the patient presented with extensive pelvic deep venous thrombosis (DVT) during the postoperative course of a neovagina creation. Case. A 19-year-old female underwent a McIndoe procedure. Despite DVT prophylaxis, extensive pelvic DVT of the femoral vein was diagnosed on postoperative day 7. Therapeutic anticoagulation was initiated, and pharmacological and mechanical thrombolysis were performed. During these procedures, a hypoplastic IVC was noted. Conclusion. MRKH syndrome can be associated with IVC malformations, which constitute an anatomical risk factor for postoperative DVT.