Management of congenital cervical atresia with vaginal aplasia: A case report and review of the literature.

INTRODUCTION: Vaginal agenesis is a rare congenital condition, with an incidence of 1 in 4500 female births. CASE REPORT: We present a clinical case of vaginal aplasia with cervical atresia in a 31-year-old woman with primary amenorrhea. We aim to report the diagnostic process and provide a comprehensive outline of different possible treatments. DISCUSSION: The most common etiology of these agenesis cases is Mayer-Rokitansky-Küster-Hauser syndrome associated with uterine aplasia. However, vaginal aplasia can occur in 9 % of cases where the uterus is present. During embryogenesis, the Müllerian ducts give rise to the fallopian tubes, uterus, and upper two-thirds of the vagina, while the lower portion of the vagina develops from the urogenital sinus. Vaginal aplasia arises from a failure in the development of the terminal portion of the paramesonephric ducts. Abdominal pain, especially periodic pain, is the most common symptom, followed by primary amenorrhea. MRI is considered the gold standard for the diagnosis and precise description of female genital tract anomalies. CONCLUSION: Total hysterectomy remains a preferred option for cases of complete vaginal atresia to mitigate the risk of cervical or vaginal stenosis, adhesions, and pelvic inflammation resulting from poor menstrual blood drainage.

A Cohort of 469 Mayer-Rokitansky-Küster-Hauser Syndrome Patients-Associated Malformations, Syndromes, and Heterogeneity of the Phenotype.

The Mayer-Rokitansky-Küster-Hauser syndrome is characterized by aplasia of the uterus and upper two-thirds of the vagina. While it can appear as an isolated genital malformation, it is often associated with extragenital abnormalities, with little still known about the pathogenetic background. To provide an overview of associated malformations and syndromes as well as to examine possible ties between the rudimentary tissue and patient characteristics, we analyzed a cohort of 469 patients with MRKHS as well as 298 uterine rudiments removed during surgery. A total of 165 of our patients (35.2%) had associated malformations (MRKHS type II). Renal defects were the most common associated malformation followed by skeletal abnormalities. Several patients had atypical associated malformations or combined syndromes. Uterine rudiments were rarer in patients with associated malformations than in patients without them. Rudiment size ranged from 0.3 cm3 to 184.3 cm3 with a mean value of 7.9 cm3. Importantly, MRKHS subtype or concomitant malformations were associated with a different frequency of uterine tissue as well as a different rudiment size and incidence of endometrial tissue, thereby indicating a clear heterogeneity of the phenotype. Further research into the associated molecular pathways and potential differences between MRKHS subtypes is needed.

Customised Vaginal Stent-The Phases in Management of Vaginal Agenesis in Mayer-Rokitansky-Küster-Hauser (MRKH) Syndrome.

Objective: Vaginal agenesis or atresia in females suffering from MRKH syndrome is more common and management involves both surgical and non-surgical approaches. Use of prefabricated stents to maintain the patency of the canal may not fit appropriately during the initial surgical phase and are not economical. This case report discusses a series of modifications in a custom-made vaginal dilator to improve the retention for expansion after surgical management of MRKH syndrome. Case report: A 28-year-old female diagnosed with MRKH syndrome with characteristic Mullerian agenesis was referred for customised vaginal stent. Customised surgical stent was fabricated with loops for orientation and retention, which was later modified into interim expansion and passive stent. Conclusion: The customisation of the vaginal stent, provision of a retentive loop that positioned the stent in the proper orientation, and gradual increase in the size of the stent, ensured dilatation in a patient with vaginal agenesis.

Balloon vaginoplasty as a minimally invasive method in the management of vaginal aplasia.

OBJECTIVE: The aim of this article is to describe the management of vaginal aplasia and to introduce minimally invasive surgical procedures for neovagina formation. METHODOLOGY: Literature review obtained from studies and papers dealing with the management of congenital vaginal aplasia. CONCLUSION: Vaginal aplasia is a rare congenital anomaly, often in coincidence with congenital defects of the uropoietic system. Management nowadays favors non-surgical or minimally invasive surgical methods for neovagina formation. Saman et al introduced a new method of neovagina formation, namely balloon vaginoplasty. The advantage of the surgical procedure is traction using a soft Foley balloon, determining both the length and width of the neovagina. The method uses the expansion of the natural vaginal mucosa without the need for dissection of the vesicorectal space. The soft Foley balloon does not cause erosion of the vaginal mucosa.

First-Line Therapy for Vaginal Atresia. Conservative Treatment vs Surgical Techniques: Quandaries Looking at Numbers.

Although it has been clearly stated that vaginal dilation must be considered the first-line treatment for clinical conditions characterized by an absent or hypoplastic vagina, mainly Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, a great number of scientific papers on surgical vaginal reconstructions are reported every year. This wide variety of surgical techniques (more than 10) are recognized and performed worldwide, making it difficult to compare results and define an evidence-based approach. Standardized treatment should be considered even more important in the pediatric and adolescent population for the implications offered by the uterus transplantation scenario.

Modified balloon vaginoplasty for high position vaginal atresia.

INTRODUCTION: The first-line approach for the management of distal vaginal atresia involves a pull-through vaginoplasty. If the proximal vagina is 3 cm or more from the introitus, the risk of stenosis is high, and an interposition graft may be necessary. We describe a safe, low-cost, and accessible approach for distal vaginal atresia ≥ 3 cm that we call the "modified balloon vaginoplasty" and validate the technical feasibility and anatomical outcomes. METHODS: Ten patients who underwent modified balloon vaginoplasty were retrospectively evaluated. Age, symptoms at presentation, length of atresia, operation time, and postoperative complications were analyzed. RESULTS: All the cases were successfully performed without any intraoperative morbidity. The postoperative complications included one case of stenosis ring in the distal vagina because not right used vagina model. All the girls had regular menstruation and were satisfied with the surgical outcome. CONCLUSION: Modified balloon vaginoplasty allows further distention of the distal vagina or thinning of the septum, which may decrease the risk of stenosis, is a beneficial choice for patients with distal vaginal atresia ≥ 3 cm.

[Mayer-Rokitansky-Küster-Hauser syndrome: a cause of primary amenorrhea: about a case]. / Le syndrome de Mayer-Rokitansky-Küster-Hauser comme cause d'aménorrhée primaire: à propos d'un cas.

Mayer-Rokitansky -Küster -Hauser syndrome (MRKH) is a rare cause of primary amenorrhea. It is defined as congenital aplasia of the uterus and of the upper two thirds of the vagina in women with normal development of secondary sexual characteristics. Diagnosis is essentially based on magnetic resonance imaging (MRI). We here report the case of a 17-year-old girl presenting with primary amenorrhea with well developed secondary sexual characteristics. Laboratory tests revealed normal ovarian function as well as gonadotropic axis. Pelvic ultrasonography and magnetic resonance imaging revealed complete uterine agenesis of the uterus, the upper two-thirds of the vagina and left kidney, allowing to confirm the diagnosis of Mayer-Rokitansky-Küster-Hauser syndrome type II. This study highlights the importance of suspecting MRKH in young patients with well-developed sexual characteristics and any type of primary amenorrhea as well as of performing MRI in order to detect any specific sign of this disorder.

Winter syndrome: about an uncommon case report.

BACKGROUND: Congenital genital tract outflow obstruction may occur at different levels and with different clinical presentations. Winter syndrome was first described in 1968 as an association of renal, genital and middle ear anomalies. This syndrome is characterized by autosomal recessive transmission, unilateral or bilateral renal hypoplasia, distal vaginal atresia, and moderate to severe conductive hearing loss with malformation of the ossicles. The diagnosis is usually made when symptoms of obstruction are obvious. It presents most commonly with primary amenorrhea in a girl with a normal XX genotype, ovarian and hormone function; and cyclical abdominal pain. Ultrasound confirm the physical examination, revealing the presence of a normal uterus and cervix, normal ovaries and fallopian tubes, and a large hematocolpos. CASE PRESENTATION: This case reports Winter syndrome in a 14-year-old girl which vaginal atresia was managed by a trans perineal vaginal pull through. CONCLUSIONS: Winter syndrome is a rare congenital condition whose clinical picture is that of an adolescent girl with primary amenorrhea and cyclic pelvic pain due to vaginal atresia, varying degrees of renal dysgenesis and deafness due to malformation of the ossicles of the middle ear. Diagnosis is based on clinical examination and imaging. Magnetic resonance imaging allows assessing the importance of atresia and thus guiding surgical management. The goals of surgical intervention are to provide relief from pain, ensure normal sexual intercourse and to preserve fertility. A thorough knowledge of embryology, pre-operative imaging with MRI and clinical examination is essential to plan an appropriate surgical management.

Double Pedicle Artery Rotation Sigmoid Vaginoplasty for Vaginal Aplasia Management.

Sigmoid vaginoplasty has been popular for neovagina reconstruction in vaginal aplasia. The most common surgical complication was vaginal stenosis caused by inadequate vascularization and tension because of graft length. Therefore, ischemia ensued and disrupted wound healing. The selection of double pedicle artery rotation sigmoid vaginoplasty is expected to reduce this problem. Five patients from April to December 2016 were diagnosed with vaginal aplasia; 4 had history of neovagina stenosis. These patients underwent sigmoid vaginoplasty with double pedicle artery rotation. No complications occurred during or after the procedure. Assessment postsurgery was conducted at 1 year. These results suggest that double pedicle artery rotation sigmoid vaginoplasty is a safe and acceptable technique for management of vaginal aplasia. The procedure decreased tension inside vascular pedicles as a result of maintaining abundant vascularization supply. Consequently, this procedure could avert graft necrosis, leakage, and severe stenosis. All of the patients exhibited regular menstrual cycle and satisfactory sexual activity. The outcomes were excellent with remarkable anatomical and functional results.

An alternative approach to vaginal dilation in patients with Meyer-Rokitanski-Küster-Hauser syndrome: two case reports.

Vaginal dilation, currently considered as the first-line therapy for vaginal aplasia in patients with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, is a safe and effective treatment that aims to create a functional neovagina. However, rigid vaginal dilators classically described in the literature usually cause physical discomfort and side effects that can lead to vaginal necrosis. Here, we present two cases of MRKH syndrome patients with vaginal agenesis whose main complaint was the inability to have sexual intercourse with their partners. Considering unavailability of acrylic dilators and previous studies reporting good responses with the use of silicone dilators in women with post-radiotherapy vaginal stenosis, the medical team and patients opted for creation of a neovagina through the daily use of silicone vaginal dilators. Patient 1 developed an 8-cm vagina after 6 months of treatment and had a satisfactory sex life with her partner. Patient 2 developed a 7-cm vagina and reported significant symptom improvement. None of the patients developed side effects after the treatment. The use of inexpensive and easily accessible silicone vaginal dilators may be an effective and noninvasive alternative with few side effects for women with vaginal agenesis, particularly in the developing countries.

Experiences of a multistep process with medical and psychological interventions for patients with congenital uterine aplasia to achieve motherhood: the Gothenburg-Tübingen collaboration.

Congenital uterine aplasia, also known as Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS) is a condition associated to a non-functional uterus in the presence of functional ovaries. In a setting where surrogacy is illegal (or not accepted) and adoption is the only alternative, neovaginoplasty and subsequent uterus transplantation (UTx) can provide a route to motherhood for women with MRKHS. This review article describes a multistep process by which patients with MRKHS can achieve motherhood with their own biological child. This process involving a careful clinical diagnosis, psychological counselling, assessment of eligibility for neovagina creation and UTx, the surgical treatment, fertility treatment, and long-term follow-up was developed at the Tübingen University Hospital and in close collaboration with Sahlgrenska Academy, University of Gothenburg, Sweden, where the basic experimental and clinical groundwork for UTx was laid and the first-ever UTx procedure was performed.

Surgery is not superior to dilation for the management of vaginal agenesis in Mayer-Rokitansky-Küster-Hauser syndrome: a multicenter comparative observational study in 131 patients.

BACKGROUND: Vaginal agenesis in Mayer-Rokitansky-Küster-Hauser syndrome can be managed either by various surgeries or dilation. The choice still depends on surgeon's preferences rather than on quality comparative studies and validated protocols. OBJECTIVE: We sought to compare dilation and surgical management of vaginal agenesis in Mayer-Rokitansky-Küster-Hauser syndrome, in terms of quality of life, anatomical results, and complications in a large multicenter population. STUDY DESIGN: Our multicenter study included 131 patients >18 years, at least 1 year after completing vaginal agenesis management. All had an independent gynecological evaluation including a standardized pelvic exam, and completed the World Health Organization Quality of Life instrument (general quality of life) as well as the Female Sexual Function Index and Female Sexual Distress Scale-Revised (sexual quality of life) scales. Groups were: surgery (N = 84), dilation therapy (N = 26), and intercourse (N = 20). One patient was secondarily excluded because of incomplete surgical data. For statistics, data were compared using analysis of variance, Student, Kruskal-Wallis, Wilcoxon, and Student exact test. RESULTS: Mean age was 26.5 ± 5.5 years at inclusion. In all groups, World Health Organization Quality of Life scores were not different between patients and the general population except for lower psychosocial health and social relationship scores (which were not different between groups). Global Female Sexual Function Index scores were significantly lower in the surgery and dilation therapy groups (median 26 range [2.8-34.8] and 24.7 [2.6-34.4], respectively) than the intercourse group (30.2 [7.8-34.8], P = .044), which had a higher score only in the satisfaction dimension (P = .004). However, the scores in the other dimensions of Female Sexual Function Index were not different between groups. The Female Sexual Distress Scale-Revised median scores were, respectively, 17 [0-52], 20 [0-47], and 10 [10-40] in the surgery, dilation therapy, and intercourse groups (P = .38), with sexual distress in 71% of patients. Median vaginal depth was shorter in dilatation therapy group (9.6 cm [5.5-12]) compared to surgery group (11 cm [6-15]) and intercourse group (11 cm [6-12.5]) (P = .039), but remained within normal ranges. One bias in the surgery group was the high number of sigmoid vaginoplasties (57/84, 68%), but no differences were observed between surgeries. Only 4 patients achieved vaginas <6.5 cm. Delay between management and first intercourse was 6 months (not significant). Seventy patients (53%) had dyspareunia (not significant), and 17 patients all from the surgery group had an abnormal pelvic exam. In the surgery group, 34 patients (40.5%) had complications, requiring 20 secondary surgeries in 17 patients, and 35 (42%) needed postoperative dilation. In the dilation therapy group, 13 (50%) needed maintenance dilation. CONCLUSION: Surgery is not superior to therapeutic or intercourse dilation, bears complications, and should therefore be only a second-line treatment. Psychological counseling is mandatory at diagnosis and during therapeutic management.

Uterovaginal Anastomosis for Cases of Cryptomenorrhea Due to Cervical Atresia with Vaginal Aplasia: Benefits and Risks.

STUDY OBJECTIVE: The objective of this study was to assess short-term benefits and risks of utero-vaginal anastomosis done for cases of cryptomenorrhea due to cervical atresia with vaginal aplasia. DESIGN: Prospective study. SETTING: Surgical procedures were done between December 2013 and September 2015 at the department of Obstetrics and Gynecology, Cairo University Hospital. PARTICIPANTS: Five patients who had cryptomenorrhea due to cervical atresia associated with vaginal aplasia were included. INTERVENTIONS: Utero-vaginal anastomoses were performed in 2 stages; a stage of McIndoe vaginoplasty and a stage of excision of the atretic cervical tissue and anastomosing the uterus to the neovagina. Follow-up was done by gynecological and ultrasound examination in a duration ranged from 12 to 36 months. MAIN OUTCOME MEASURES: Occurrence of regular menstrual flow and relief of the severe cyclic pain. RESULTS: All patients had relief of the severe cyclic pain. Four patients had regular menstrual flow. One patient developed occlusion of the track after 1 year and needed dilatation once. Three patients developed low vaginal stenosis without occlusion of the track. One patient had rectal injury repaired without causing postoperative morbidity. CONCLUSION: Uterovaginal anastomosis is a promising conservative management option for cervical atresia with vaginal aplasia, which has benefits but is not free of risks. Long-term follow-up is still needed to judge its feasibility. We recommend performing McIndoe vaginoplasty as a starting stage before the anastomosis preferably in a separate setting.

Laparoscopically assisted cervical canalization and neovaginoplasty in a woman with cervical atresia and vaginal aplasia.

Cervical atresia is a Müllerian duct system anomaly, and it is often associated with vaginal aplasia. We report the case of a 17-year-old girl who presented with primary amenorrhea and cyclical abdominal pain, and was diagnosed with cervical atresia and vaginal aplasia that were treated laparoscopically. Laparoscopically assisted cervical canalization and neovaginoplasty were performed to relieve dysmenorrhea and allow for sexual intercourse and fertility. We did not use a bowel segment, skin, or peritoneum as a graft for the neovaginoplasty. To prevent adhesions and promote epithelialization, we used an estrogen-containing cream. Moreover, we did not use a vaginal mold. The patient is free of cervical stenosis and able to have intercourse. Long-term follow-up is necessary to ensure a future pregnancy and childbirth.

Comparison of small intestinal submucosa graft with split-thickness skin graft for cervicovaginal reconstruction of congenital vaginal and cervical aplasia.

STUDY QUESTION: What is the difference in vaginal-length gain and resumption of menstruation following cervicovaginal reconstruction using split-thickness skin (STS) graft versus small intestinal submucosa (SIS) graft for patients with congenital vaginal and cervical aplasia? SUMMARY ANSWER: No difference was found in the number of patients who resumed menstruation between the two groups; however, significantly greater vaginal-length gain was found in the STS group. WHAT IS ALREADY KNOWN: Hysterectomy and vaginoplasty are typically recommended for patients without a cervix or without a sufficient cervix to avoid postoperative re-obstruction. Advances in surgical techniques have enabled the use of autologous tissues or heterologous biological grafts for reconstructive procedures, allowing patients undergoing these procedures to preserve the possibility of conception. STUDY DESIGN, SIZE, DURATION: This was a retrospective study of 26 women who were diagnosed with congenital vaginal and cervical aplasia with a functional endometrial cavity and underwent cervicovaginal reconstruction using STS or SIS grafts between January 2012 and October 2015 at the Obstetrics and Gynecology Hospital of Fudan University, Shanghai, China. PARTICIPANTS/MATERIALS, SETTING, METHOD: 15 women underwent cervicovaginal reconstruction using SIS graft and 11 underwent cervicovaginal reconstruction using STS graft. Clinical characteristics, perioperative data, resumption of menstruation, vaginal stenosis, length of the neovagina, vaginal-length gain, stricture of the cervix and body image were postoperatively assessed. MAIN RESULTS AND THE ROLE OF CHANCE: At a median follow-up of 21 (2-46) months, all but one of the patients experienced relief of abdominal pain and resumed menstruation. Re-obstruction of the cervix occurred in only one patient in the SIS group. The SIS group reported significantly higher body image scores and cosmetic satisfaction. Although the two groups had a similar vaginal length before surgery, the vaginal-length gain was significantly greater in the STS group (4.9 ± 1.7 cm in the SIS group versus 7.5 ± 0.7 cm in the STS group, P = 0.004) and the neovagina length at 6 months post-operation was significantly shorter in the SIS group (6.5 ± 0.7 cm in SIS the group versus 8.0 ± 0.5 cm in the STS group, P < 0.0001). LIMITATIONS, REASONS FOR CAUTION: Due to a lack of long-term follow-up, patient satisfaction with sex life, pregnancy rates and outcomes after the two approaches require further evaluation. WIDER IMPLICATIONS OF THE FINDINGS: Combined laparoscopic and vaginal cervicovaginal reconstruction using SIS or STS graft is a safe and effective treatment for preserving uterus function in women with congenital vaginal and cervical aplasia. However, SIS graft must still be improved to achieve satisfactory vaginal length. STUDY FUNDING/COMPETING INTERESTS: This study was supported by the Chinese National Nature Sciences Foundation (grant number 81471416) and the National Key Clinical Faculty Construction Program of China. No competing interests are declared. TRIAL REGISTRATION NUMBER: N/A.

Creation of a neovagina in a patient with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome and previously corrected rectovestibular fistula concomitant with imperforate anus.

BACKGROUND: Congenital absence of uterus and vagina (CAUV) when associated with anorectal malformations is usually diagnosed and repaired in infancy at the time of anorectoplasty. Long-term observations of patients are scarce and do not justify early vaginal reconstruction. Question arises whether creation of a neovagina can be safely and successfully performed when the patient is mature. CASE: The patient, diagnosed with MRKH syndrome at 16 years of age, underwent repair of rectovestibular fistula and imperforate anus ("cut-back" procedure, temporal sigmostomy and sagittal anterior anorectoplasty) in infancy. At 18, modified Wharton vaginoplasty was performed with a good anatomico-functional outcome. SUMMARY AND CONCLUSIONS: Early repair of anorectal malformation and postponed vaginal reconstruction seem to be a viable option for patients with congenital rectovestibular fistula and anal atresia concomitant with CAUV.

Successful isthmo-neovagina anastomosis after Davydov's colpopoiesis in Mayer-Rokitansky-Küster-Hauser syndrome patients with a functional rudimentary uterine horn.

The aim of these case reports is to present the feasibility of isthmo-neovagina anastomosis after Davydov's colpopoiesis in patients with Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) with a rudimentary uterine horn. Ten MRKH patients were treated surgically with laparoscopic Davydov's vaginoplasty between 2006 and 2012. Two of these patients were found to have a functional rudimentary uterine horn. These patients underwent a 2-step surgical intervention: (1) Davydov's colpopoiesis with a combined laparoscopic and perineal approach and (2) isthmo-neovagina anastomosis with laparotomy in the 2 cases with a uterine horn in a second surgical step 4 and 6 months after the initial procedure, respectively. The main outcome measures were the perioperative and postoperative details, the vaginal length during follow-up, the postoperative initiation of intercourse, and the postoperative menstrual function. Isthmo-neovagina anastomosis was uneventful, and there were no early postoperative complications. The mean hospitalization duration was 9.5 days. Anastomosis was successful in both patients. The mean follow-up was 42 months, and the mean vaginal length was measured 9.5 cm; both patients reported normal intercourse and normal menstrual function. Anatomic restoration of the genital tract in these patients appears to have been successful and led to functional menstruation and restitution of the patients' sex life.

Laparoscopically assisted neovaginoplasty in vaginal agenesis: a long-term outcome study in 240 patients.

STUDY OBJECTIVE: To assess the long-term outcome of an optimized minimally invasive neovaginoplasty technique in vaginal agenesis. DESIGN: Combined retrospective and prospective study. SETTING: University hospital. PARTICIPANTS: 240 patients with congenital vaginal agenesis. INTERVENTIONS: Patients 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 MEASURES: Long-term anatomic success, functional success compared with similar-aged controls, long-term complications, and incidence of human papilloma virus (HPV) infections. RESULTS: During 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. CONCLUSIONS: Our 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.

Simplificando a neovaginoplastia na síndrome de Mayer-Rokitansky-Küster-Hauser / Simplifying vaginoplasty in Mayer-Rokitansky-Küster-Hauser syndrome

Embora sua descrição diste da primeira metade do século XIX, talvez pela sua raridade, o tratamento da Síndrome de Mayer-Rokitansky-Küster-Hauser (MRKH) ainda hoje constitui um desafio. Neste artigo, após breve revisão teórica das características da síndrome, discutimos as técnicas propostas para criação de uma neovagina. Apresentamos a seguir um relato de caso de paciente de 39 anos submetida à Neovaginoplastia de Creatsas, que, pela sua simplicidade, rapidez de recuperação, baixa morbidade e excelentes resultados iniciais, parece ser a opção ideal para as pacientes brasileiras.

Although its description dates from the first half of the 19th century, the treatment of Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome remains a challenge, perhaps because of its rarity. In this paper, after a brief theoretical review of the characteristics of the syndrome, the proposed techniques for creating a neovagina are discussed. Then we report the case of a 39-year-old patient submitted to Creatsas neovaginoplasty, which owing to its simplicity, fast recovery, low morbidity and excellent early results, seems to be the ideal choice for Brazilian patients.