Mayer-Rokitansky-Küster-Hauser Syndrome Research Articles

Etiology of primary amenorrhea: Experience from a tertiary care hospital in Western India

Objectives: The etiology of primary amenorrhea is varied and can involve different organ systems. Gonadal dysgenesis and Mullerian agenesis are two most common disorders contributing to it. However, there is no consensus about the prevalence of different etiologies among studies reported from different parts of the world. The present study was carried out to evaluate different etiological factors responsible for primary amenorrhea in Western India. Materials and Methods: This was a prospective observational study carried out at a tertiary care hospital in Maharashtra. Thirty cases of primary amenorrhea were evaluated over a period of 2 years and data were analyzed regarding etiology and various management options. Results: Fourteen out of 30 cases had Mullerian agenesis and gonadal dysgenesis was reported in five cases. No case of genital tuberculosis was reported in our study. Most cases of Mullerian agenesis were managed with vaginoplasty, whereas cases of gonadal dysgenesis were managed with cyclical hormonal therapy. Conclusion: Mullerian agenesis was the most common etiology of primary amenorrhea followed by gonadal dysgenesis in our study. Diagnosis of patients of amenorrhea should be meticulous and their management depends on the underlying etiology.

Agenesis of female internal reproductive organs, the Mayer- Rokitansky-Küster-Hauser syndrome

Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is the most common cause of uterine and uterine tubes absence/underdevelopment and the second most common cause of primary amenorrhea. It is characterized by a congenital agenesis of the uterine tubes, uterus, cervix, and upper part of the vagina. This study presents our 10 years of experience with the diagnostics and therapeutic management of patients with MRKH syndrome. We also focused on the description of anatomical deviations of the female reproductive organs. We studied a cohort of 16 patients hospitalized with MRKH syndrome between 2011-2020. We analysed the age at diagnosis, clinical signs and symptoms, diagnostic techniques, methods of neovagina creation, and anatomical anomalies of the female reproductive organs. The mean age of our patients at the time of diagnosis was 16.75 years. The most frequent clinical manifestations were primary amenorrhea (56.25 %), pelvic pain (31.25 %) and coitus-associated problems. We used two methods of neovagina creation. In six patients we used the non-surgical Frank's dilators method. The vaginal length at the beginning of the dilation was 1.5-2 cm. In 4 patients the vaginal length was under 1 cm, so we performed surgical neovagina creation using the laparoscopic Vecchietti method. An-other two patients could create the vagina via regular sexual intercourse. Based on magnetic resonance imaging and ultrasound examination, the following anomalies were observed: complete agenesis of the uterus (50 % of cases), fibrous band (25 %) and rudimentary uterus (25 %). Satisfactory results in the attempt to create a neovagina can be obtained by both non-surgical and surgical methods. Modern medicine provides many therapeutic measures, which make it possible for the affected women to have a normal sexual life (Tab. 1, Fig. 3, Ref. 37).

Fertility options in Mayer-Rokitansky-Küster-Hauser syndrome

Background: The Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is the most common cause of uterine aplasia, with a worldwide frequency of 1 in 4500 females. Although abundant literature is present regarding the different available methods for creating a neovagina in MRKH syndrome, the attention dedicated to these women’s reproductive potential remains insufficient. Methods: Online searches were carried out in PubMed database during November and December 2020. The search included a combination of the various terms (e.g., MRKH, vaginoplasty, uterus transplantation, infertility treatment, gestational surrogacy, etc.). The following inclusion criteria were used: (1) The articles should be written in English or French language. (2) The article should have been published by an official scientific organization. Results: Until few years ago, the only option for women with MRKH syndrome was legal adoption, now gestational surrogacy (GS) and uterine transplantation (UTx) have become new fertility options available to these patients. Discussion: GS is officially recognized in several countries and widely practiced. On the other hand, UTx, although the great recent technical improvements, is far from being an option for all women with MRKH syndrome secondary to its complexity and the necessary immunologic therapies.

Complex clinical and echographic assessment of the peculiarities of developmental anomalies of uterus and vagina in children and adolescents

The objective. To assess the structure of independent and combined forms of anomalies in the development of the uterus and/or vagina in children and adolescents, justifying the need for ultrasound screening in this age group.Materials and methods. The study involved 146 patients with malformations of the uterus and/or vagina at the age from 10 to 20 years. All patients underwent a clinical examination, which included anamnestic data, gynecological examination, and ultrasound examination. The calculation of indicators of the diagnostic information content of ultrasound was carried out taking into account the sensitivity, specificity and accuracy of the method.Results. In the course of the study, it was found that the largest proportion of all anomalies of the internal genitals was uterine malformations (37,0%), complete doubling of the uterus and vagina (17,1%), vaginal aplasia (15,1%). Genetic or chromosomal pathology was detected in 36 patients (24,7%), in the remaining 110 patients (75,3%) a multifactorial nature of the disease was assumed.Attention is drawn to the fact that 48 patients (32.8%) with uterine and/or vaginal malformations, who did not present complaints, were identified during the primary ultrasound screening, which indicates the effectiveness of this method in examining girls and adolescents. In 12 children (8,25%) in addition to abnormalities in the development of the uterus and/or vagina, ultrasound revealed tumors and tumor-like diseases of the uterine appendages. Also, 57 malformations of other organs and systems were revealed in 49 (33,6%) examined patients, and 8 (5,5%) had concomitant defects. In most cases, in 99 patients (67,8%) with anomalies in the development of internal genitalia, the anamnesis was aggravated by the factors of their parents (burdened heredity, disease, occupational health, etc.)Conclusion. As a result of a comprehensive clinical examination of patients with congenital anomalies in the development of internal genitalia, the diagnosis was clarified, the share of hereditary and some environmental factors in the manifestation of this pathology was revealed, which is one of the common gynecological diseases that are characterized by a variety of forms of anomalies, clinical symptoms and a high percentage of diagnostic errors. At puberty, the use of echography to clarify the state of the internal genital organs is of great practical importance. Non-invasiveness, absence of psychogenic traumatization, safety and high information content make it possible not only to diagnose congenital pathology, but also to use this method as a dynamic observation of the state of the uterus and/or vagina in girls and adolescents, which is very important when choosing rational methods for correcting this pathology

National survey of primary amenorrhea and relevant conditions in Japan.

In Japan, most of the patients with primary amenorrhea or related conditions, such as delayed menarche, are diagnosed by pediatricians or gynecologists; accordingly, the number of the patients and the ratio of the causes were unclear. To clarify them, we conducted a nationwide survey in both the departments for the first time. We sent a questionnaire about the patients with chief complaint of no menarche whose first visit was from January 2015 to December 2017, to 596 training institutions for specialist physicians of the Japan Society of Obstetrics and Gynecology and 152 facilities to which councilors of the Japanese Society for Pediatric Endocrinology belong. We received replies from 283 (37.8%) institutions. During the 3 years, 1043 patients first visited pediatrics or gynecology for no menarche. In 303 patients under 16 years old at the first visit, 177 (58.4%) patients had menarche by the age of 16. Of them, 41 (13.5%) patients had menarche spontaneously. Among 308 patients aged 16 to 17 at the first visit, 216 patients were 18 years or older at the survey. Of them, 124 (57.4%) patients had menarche by the age of 18, and 21 (9.7%) of them had menarche spontaneously. The causes of amenorrhea were detected in 462 patients. Abnormal karyotype including Turner syndrome was the most common at 122 (26.4%), followed by Mullerian agenesis at 73 (15.8%). The first national survey revealed the number and causes of primary amenorrhea and related conditions. This report will provide better information for clinicians.

Pediatric granular cell tumor of vulva: A rare benign tumor

We present a rare case of a school-aged girl with granular cell tumor of the vulva. The patient presented with a right labial swelling of unknown duration. Initial diagnostic workup with ultrasound suggested a gonadal structure in the right labia, which was read as ovarian herniation through the Canal of Nuck into the right labia majora. Given concerns for a concurrent Mullerian dysgenesis syndrome predisposing the patient to ovarian herniation, the patient was scheduled for a diagnostic laparoscopy and hernia repair with possible excision of vulvar mass. Laparoscopy revealed normal reproductive anatomy with two visualized normal appearing pelvic ovaries. Subsequent excision of the mass was determined to be granular cell tumor by pathology. There are fewer than 20 reported cases of pediatric vulvar granular cell tumors in the literature and no reports of granular cell tumors presenting as ovarian structure on imaging. This case highlights the importance of including this rare tumor in the differential diagnosis for vulvar mass which may be misread as ovary on ultrasound. • Granular cell tumors of the vulva are extremely rare, especially in children. • Ultrasound imaging of the tumor may present as ovarian structures. • Clinicians should include granular cell tumors within the differential for a vulvar mass. • In children wide local excision is curative and follow up is necessary for minimum of two years due to high recurrence rates.

Association of the rectovestibular fistula with MRKH Syndrome and the paradigm shift in the management in view of the future uterine transplant

Uterine transplantation in Mayer-Rokitansky-Küster-Hauser (MRKH) patients with absolute uterine function infertility have added a new dimension and paradigm shift in the management of females born with rectovestibular fistula coexisting with vaginal agenesis. The author reviewed the relevant literature of this rare association, the popular and practical classifications of genital malformations that the gynecologists use, the different vaginal reconstruction techniques, and try to know what shall serve best in this small cohort of these patients lest they wish to go for uterine transplantation in future.

English

BACKGROUND Mayer-Rokitansky-Küster-Hauser Syndrome or MRKH Syndrome is a rare condition and is the second most common cause of primary amenorrhea, comprising of vaginal atresia (upper two thirds), rudimentary uterus, normal fallopian tubes, ovaries, broad and round ligaments. The spectrum of uterine anomalies (hypoplasia or duplication) include a partial lumen to a bicornuate or septate uterus with obstruction (unilateral or bilateral). The incidence is 1 in 4500 - 5000 female live births, presenting with primary amenorrhoea. The secondary sexual characteristics, external genitalia, ovaries and karyotype are normal. There are two types - the first type is the isolated form and the second type also termed as MURCS association [Müllerian duct aplasia, renal dysplasia-agenesis, hydronephrosis, horseshoe kidney and cervicothoracic anomalies such as fused vertebrae, scoliosis etc.]. Initial assessment with ultrasound scan of abdomen and pelvis followed by MRI study of the abdomen and pelvis are the imaging modalities of choice. METHODS This is a case series of 9 female patients who had presented to the Department of Obstetrics & Gynaecology and the Department of Radiodiagnosis from July 2019 to June 2020, aged between 15 and 20 years with a chief complaint of anxiety due to primary amenorrhoea. Following a thorough clinical, gynaecological and biochemical evaluation (levels of FSH, LH and 17 beta oestradiol), radiological examination (ultrasound and MRI - abdomen and pelvis) was conducted. RESULTS In our study, out of a total of nine cases, six cases were MRKH Type I and three were MRKH Type 2. All the nine cases presented with primary amenorrhoea, normal secondary sexual characteristics (except one case with ectopic atrophic ovaries) and normal external genitalia. Available hormonal profile was unremarkable. Uterus was not palpable on PV and per speculum examination. Along with the above features, when features of only hypoplastic / infantile / rudimentary / absent uterus with hypoplastic / absent upper two thirds of vagina, normal pelvic ovaries or ectopic inguinal ovaries was present, a diagnosis of MRKH Type–I was given. With additional features of renal abnormalities or skeletal system abnormalities, a diagnosis of MRKH Type–II was given. CONCLUSIONS MRKH syndrome is a condition caused due to the failure of fusion of Müllerian duct derivatives. It affects 1 in 4500 - 5000 female live births. It is a class I Mullerian duct anomaly including vaginal atresia, uterine anomalies & malformations of the upper urinary tract. There are two types in this. USG and MRI of the abdomen and pelvis are helpful in imaging this condition. KEYWORDS MRKH Syndrome, Ultrasound, MRI, MURCS Association

Laparoscopic vaginal bead pull-through vaginoplasty technique using dental prosthesis material.

Objective:Many reconstructive surgical procedures have been described for vaginal agenesis. Almost all are surgically challenging, multistage, time-consuming or leave permanent scars on the abdomen or skin removal areas. The aim of this study was to introduce a simple and cheaper approach for laparoscopic vaginal bead-pull through.Materials and Methods:In this retrospective study, we report a total of six patients with congenital absence of vagina [Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome] who were treated with a laparoscopic vaginal bead pull-through technique between 2018 till 2019 with a dental prosthesis material.Results:Six patients with MRKH syndrome were treated with a laparoscopic vaginal bead pull-through technique. None of the women had any previous treatment. The mean age at the time of surgery was 18.7±3.1 years and mean body mass index was 25 (range, 19-38) kg/m2. None of the patients had any additional malformations. In all patients, normal external genitalia and complete vaginal agenesis were observed during examination. The mean duration of surgery was 72 (range, 55-95) minutes. All patients were discharged on the 3rd postoperative day. No intraoperative complications were encountered. All patients had their urinary catheters removed within 12 hours after surgery. The mean vaginal length at discharge was 10 (range, 8-13) cm and all patients had adequate vaginal diameter, allowing introduction of three fingers easily. At the 12th postoperative month, the mean vaginal length was 9.2±1.6 cm. All patients had complete epithelization. All the women were sexually active one year after surgery.Conclusion:The laparoscopic vaginal bead pull-through technique using dental prosthesis material can provide satisfactory results with shorter surgical time and lower cost.

The Endometrial Transcription Landscape of MRKH Syndrome.

The Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome (OMIM 277000) is characterized by agenesis of the uterus and upper part of the vagina in females with normal ovarian function. While genetic causes have been identified for a small subset of patients and epigenetic mechanisms presumably contribute to the pathogenic unfolding, too, the etiology of the syndrome has remained largely enigmatic. A comprehensive understanding of gene activity in the context of the disease is crucial to identify etiological components and their potential interplay. So far, this understanding is lacking, primarily due to the scarcity of samples and suitable tissue. In order to close this gap, we profiled endometrial tissue of uterus rudiments in a large cohort of MRKH patients using RNA-seq and thereby provide a genome-wide view on the altered transcription landscape of the MRKH syndrome. Differential and co-expression analyses of the data identified cellular processes and candidate genes that converge on a core network of interconnected regulators that emerge as pivotal for the perturbed expression space. With these results and browsable access to the rich data through an online tool we seek to accelerate research to unravel the underlying biology of the syndrome.

La transplantation utérine : essor de la technique chirurgicale, nouvelles indications. État de l’art avec revue de la littérature

ObjectivesThe aim of this review is to summarize the development of UT on worldwide and to develop the new questions posed by this technique in 2020. MethodsAccording to the PRISMA model, via Pubmed, we searched for publications containing the keywords: uterus transplantation; UT and cryopreservation from 2000 to 2020. ResultsAt least 76 UTx have been carried out around the world and 19 healthy babies were born. The main indication remains the uterine agenesis (MRKH Syndrome>85% cases) then the history of hysterectomy (hemorrhage of the delivery or cervical cancer) and the non-functional uterus (Asherman's syndrome, diffuse adenomyosis). The 2 types of donors (living and deceased) are developed representing respectively 75% and 25% of the TU; the success rate in terms of return of rules is better in the living donor group and is 79% vs 68% in the deceased donor group. The choice of donor type must take into account the constraints of both procedures. Surgical complications (grade III) for the donor are estimated to be 14% mainly represented by ureter wounds. Technical simplifications concerning the venous return of the graft but also the carrying out of robot-assisted surgery would reduce the operating time for the donor and facilitate the collection process. ConclusionTU is a complementary alternative to GPA and adoption allowing patients to be surrogates, legal and biological of the baby. An extension of the indications to patients with non absolute uterine infertility is in the process of democratization.

Neovaginal Prosthesis - Hallmark in the Management of Patients with MRKH Syndrome – A Case Report

Neovaginal Prosthesis - Hallmark in the Management of Patients with MRKH Syndrome – A Case Report

Uterine Conserving Surgery in Patient With Cervicovaginal Agenesis: A Case Report

Congenital absence of the vagina with variable uterine development known as Mullerian agenesis or Mayer-Rokitansky-Kuster-Hauser syndrome. Cervicovaginal agenesis in the presence of normal uterus is very rare. Conservative surgery has recently been suggested in patients with congenital cervicovaginal atresia in order to relieve the symptoms and maintain fertility. A 13-year-old female referred to a pelvic floor clinic, because of primary amenorrhea and severe cyclic pelvic pain. Ultrasonography revealed a large amount of blood accumulation in the uterine cavity, and also, the cervicovaginal agenesis was reported. Both ovaries were normal. A neovagina was created by dissection of the space between the bladder and rectum. Under ultrasonography guidance, two Pezzer catheters were inserted between uterine ending and neovagina, so the catheters kept the canal patent, a soft mould was applied to prevent the vaginal stricture. Cervicovaginal agenesis, accompanied by normal functional endometrium, is a rare but challenging Mullerian anomaly in the case of surgical treatment. One of the successful conservative treatment in a fully educated patient is the vaginal reconstruction and uterovaginal anastomosis by stenting and continues the application of vaginal mould.

Sigmoid vaginoplasty in Mayer-Rokitansky-Kuster-Hauser syndrome

IntroductionMayer-Rokitansky-Kuster-Hauser syndrome (MRKH) is characterized by congenital aplasia of the uterus and upper 2/3 of the vagina, with normal female secondary sexual characteristics and a normal karyotype (46, XX). The frequency is 1/4500–5000 female births. The aim of this study was to report the management of MRKH syndrome with sigmoid vaginoplasty.Patients and methodThis study included 4 patients recruited over a 4-year period from February 2016 to January 2019. MRKH syndrome was retained in the presence of normal secondary sexual characteristics with normal external genitalia associated with vaginal aplasia and uterine agenesis. The approach was a laparotomy and a perineal approach under general anesthesia. The procedure involved the removal of a sigmoidal colonic graft that was anastomosed with the vaginal dimple.ResultsThe average age was 23 years. All patients had consulted for primary amenorrhea, infertility, and/or difficulties in sexual intercourse. The diagnosis of MRKH type 1 was retained in all patients. The average length of the vagina was 3.25 cm before surgery and 13.63 cm after surgery. The postoperative outcomes were uneventful in 3 patients. One patient developed anastomotic stenosis that was successfully treated with vaginal dilation for 2 weeks. The average postoperative follow-up was 30 months.ConclusionIn the context of a low-resource setting, sigmoid transposition represents a good procedure to treat vaginal aplasia and restore a satisfactory sexual activity to patients with MRKH type 1.

Living-Donor Uterus Transplantation: Pre-, Intra-, and Postoperative Parameters Relevant to Surgical Success, Pregnancy, and Obstetrics with Live Births.

Uterus transplantation (UTx) can provide a route to motherhood for women with Mayer–Rokitansky–Küster–Hauser syndrome (MRKHS), a congenital disorder characterized by uterovaginal aplasia, but with functional ovaries. Based on our four successful living-donor transplantations and two resulting births, this analysis presents parameters relevant to standardizing recipient/donor selection, UTx surgery, and postoperative treatment, and their implementation in routine settings. We descriptively analyzed prospectively collected observational data from our four uterus recipients, all with MRKHS, their living donors, and the two newborns born to two recipients, including 1-year postnatal follow-ups. Analysis included only living-donor/recipient pairs with completed donor/recipient surgery. Two recipients, both requiring ovarian restimulation under immunosuppression after missed pregnancy loss in one case and no pregnancy in the other, each delivered a healthy boy by cesarean section. We conclude that parameters crucial to successful transplantation, pregnancy, and childbirth include careful selection of donor/recipient pairs, donor organ quality, meticulous surgical technique, a multidisciplinary team approach, and comprehensive follow-up. Surgery duration and blood vessel selection await further optimization, as do the choice and duration of immunosuppression, which are crucial to timing the first embryo transfer. Data need to be collected in an international registry due to the low prevalence of MRKHS.

Urogynecology

Urogynecology

A Mayer-Rokitansky-Kuster-Hauser patient with leiomyoma and dysplasia of neovagina: a case report

BackgroundMost patients with congenital uterus and vaginal aplasia (i.e., Mayer–Rokitansky–Kuster–Hauser [MRKH] syndrome) have rudimentary pelvic uterine structures that contain smooth muscle. Although leiomyomas and dysplasia of vaginal mucosa are relatively common in the general population, they are rare in MRKH patients. Data on the vulnerability of neovaginas to HPV-associated dysplasia are limited.Case presentationA rare case of an MRKH patient with two gynaecological conditions detected during long-term gynaecological follow-up is presented. At the age of 21, the patient was treated for HPV-associated neovaginal dysplasia. At the age of 47, a pelvic leiomyoma was detected with transvaginal ultrasound and confirmed with magnetic resonance imaging.ConclusionA Pap smear or human papillomavirus testing is indicated in sexually active MRKH women. Uterine rudiments contain smooth muscle, which facilitates the development of oestrogen-dependent diseases, such as leiomyomas and adenomyosis. Although magnetic resonance imaging is recommended in cases of a pelvic mass, easily attainable and cost-efficient transvaginal ultrasound offers high diagnostic accuracy in patients with a surgically created neovagina and is suitable for the patients’ follow-up. Guidelines for the gynaecological follow-up of MRKH patients are warranted.

Laparoscopic Evaluation of Primary and Secondary Subfertility

Background: Prevalence of subfertility in industrialized countries has been quoted as 20%,and seems to be on the rise. Traditional way to assess the uterine cavity, tubal structure andtubal patency was hysterosalphingography but it is now been largely superseded bylaparoscopy and hysteroscopy. With the objective of this study was to highlight the role oflaparoscopy in establishing the diagnosis of primary and secondary female subfertility anddifferent therapeutic procedure done.
 Method: This cross sectional study was conducted in the Subfertility and ReproductiveMedicine Unit of Gynae and Obstetrics department of Dhaka Medical College Hospital, Dhakafrom January 2015 to June 2018. Total 4256 sub fertile patients attended the Infertility OPD.Out of these 215 patients were selected for laparoscopy. Those patients who hadcontraindication for laparoscopy were excluded from study. Detailed laparoscopic findingswere recorded.
 Results: Out of 125 selected sub fertile patients 136 (63.26%) patients were in primarysubfertility group while 79 (36.74%) patients were in secondary subfertility group. In primarysubfertility group(n=136), most common laparoscopic finding was PCO in 44 (32.34%) patientsfollowed by peritubal and periovarian adhesions in 24 (17.65%) patients, Bilateral tubalblock in 23(16.91%) patients, Endometriosis in 15(11.03%), Unusual tortuous and lengthytube in 15(11.03%) patients, fibroid in 11(8.09%) patients, Mullerian agenesis and hypoplasiain 3(2.21%) cases. No visible abnormality found in 22(16.18%) cases.The commonest finding by laparoscopy in patients with secondary infertility were PCO in 18(22.78%) patients and peritubal and periovarian adhesions in 18 (22.78%) patients, followedby Bilateral tubal block in 12(15.19%) patients, fibroid uterus in 12(15.19%) patients,Endometriosis in 10(12.66%), Genital tuberculosis in 3(1.40%) cases. No visible abnormalityfound in 12(15.19%) cases.Various laparoscopic procedure like Ovarian drilling. Adhesiolysis, ovarian cystectomy, cystpuncture, salphingostomy, Myomectomy.
 Conclusion: Laparoscopy helped us to detect and treat important clinical condition in bathprimary & secondary sub fertility.
 Bangladesh J Obstet Gynaecol, 2018; Vol. 33(2) : 143-148

Vaginal Reconstruction in Patients with Mayer-Rokitansky-Küster-Hauser Syndrome-One Centre Experience.

Background and Objectives: The Mayer–Rokitansky–Küster–Hauser syndrome is a congenital condition in which patients are born with vaginal and uterus agenesis, affecting the ability to have a normal sexual life and to bear children. Vaginal reconstruction is a challenging procedure for plastic surgeons. The aim of this study is to report our experience in the management of twelve patients with congenital absence of the vagina due to the MRKH syndrome. Materials and Methods: We performed a retrospective study on 12 patients admitted to the Plastic Surgery Department of the Clinical Emergency Hospital “Prof. Dr. Agrippa Ionescu”, Bucharest, Romania, for vaginal reconstruction within a period of eleven years (January 2009–December 2019). All patients were diagnosed by the gynaecologists with vaginal agenesis, as part of the Mayer–Rokitansky–Küster–Hauser syndrome. The Abbe‘–McIndoe technique with an autologous skin graft was performed in all cases. Results: The average age of our patients was 20.16 (16–28) years. All patients were 46 XX. The average surgical timing was 3.05 h (range 2.85–4h). Postoperative rectovaginal fistula was encountered in 1 patient. Postoperative average vaginal length was 10.4 cm (range 9.8–12.1 cm). Regular sexual life was achieved in 10 patients. Conclusion: Nowadays, there is no established standard method of vaginal reconstruction. In Romania, the McIndoe technique is the most applied. Unfortunately, even if the MRKH syndrome is not uncommon, less and less surgeons are willing to perform the procedure to create a neovagina.

Long Term Anatomical and Functional Results of Modified McIndoe Vaginoplasty with Amnion Graft in Patients with Congenital Vaginal Aplasia Compared with a Control Group

Background: Vaginal aplasia is a rare congenital anomaly with different surgical techniques suggested for vaginal reconstruction.McIndoe vaginoplasty is an easy one of these surgical methods with a low morbidity rate, but its long-term results need to be further investigated. Therefore, the female genital anatomy and sexual function was examined after performing McIndoe vaginoplasty in patients with mullerian anomaly or androgen insensitivity syndrome. Materials and Methods: In this historical cohort study, the data of 25 patients undergoing McIndoe vaginoplasty with amniotic graft from 2006 to 2017 at four selected hospitals of Shiraz affiliated with Shiraz University of Medical Sciences in Iran were extracted from medical records. Then, the patients were called to refer for physical examination and fill the Female Sexual Function Index (FSFI) questionnaire. In addition, 31 women of the same age range and without a sexual problem, vaginal surgery, or delivery were selected as the control group. Finally, the results of vaginal measurements and FSFI scores of the two groups were compared together. Results: Theaverage vaginal length of the case and control groups were 5.60±2.38 and 8.47±1.31 cm, respectively. Furthermore, the mean proximal vaginal diameter in case and control groups were measured 2.94±0.92, and 4.12±0.70 cm, respectively (P<0.001). The mean FSFI score of the case group was 12.81±7.87, and 24.19±2.90 for the control group (P<0.001). The vaginal indices and FSFI of the patients using mold routinely were still lower than the control group (P<0.05) although theyhad a larger vagina (P<0.001). Conclusion: Vaginal reconstruction using McIndoe vaginoplasty with amniotic graft failed to provide normal vagina function and anatomy for patients with congenital vaginal aplasia in long-term follow-up.