Cervicovaginal Agenesis Research Articles

Long-Term Outcomes of the Restoration of Uterovaginal Continuity and Vaginoplasty—Utero-Colo-Neovaginoplasty—in Cervicovaginal Agenesis Using the Sigmoid Colon

Introduction and HypothesisCongenital cervicovaginal agenesis in the presence of a functional endometrium is a rare Müllerian anomaly. The management ranges from hysterectomy historically to various reconstructive procedures more recently. We report our experience with utero-colo-vaginoplasty in the management of this anomaly and its long-term follow-up.MethodsThe case records of all the patients with vaginal or cervicovaginal agenesis in our hospital from January 2002 to December 2019 were reviewed retrospectively. The patients were then called for an outpatient visit and examined in detail. The anatomical variations, surgical procedures and outcomes were recorded and analysed.ResultsSixteen patients aged 14 to 26 years were included during the study period. They presented with cyclical painful cryptomenorrhea. Magnetic resonance imaging (MRI) confirmed cervicovaginal or distal vaginal agenesis. All the patients underwent utero-colo-vaginoplasty. Intraoperative rectal injury led to post-operative faecal leak from the perineal wound in one patient in the post-operative period. Restoration of painless menstrual flow was possible in all 16 cases. Long-term complications were seen in 4 patients. These were stenosis of the perineal neovaginal orifice in 2 patients, obstruction at colo-uterine anastomosis in 1 patient and mucosal prolapse at the neovagina in 1 patient. Three of these patients needed secondary surgical procedures. Five were sexually active and reported consummation of penetrative intercourse. None of them had conceived.ConclusionIn our experience, utero-colo-vaginoplasty allows for regular painless menstruation and coitus with minimal long-term complications. The sole disadvantage is the failure to conceive.

Unification and orificing of two functional noncommunicating uterine horns through the created neovagina using peritoneum

ObjectiveTo demonstrate the surgical approach for Müllerian agenesis with bilateral uterine remnants containing functional endometrium. DesignStepwise demonstration of the technique with narrated video footage SettingA Tertiary University Hospital, Reproductive Surgery Unit. PatientAn 18-year-old adolescent was admitted to a tertiary university hospital with complaints of primary amenorrhea and cyclic pelvic pain. Physical examination and MRI scans suggested a complex Müllerian abnormality. The patient had uterine remnants with bilateral functional endometrium and cervico-vaginal agenesis. InterventionAn operation was planned to reconstruct her anatomy by providing a neovagina and anastomosing the uterine remnants. GnRH analogs were prescribed to suppress her menstruation until the procedure. The operation was performed in the third month after the initial diagnosis. A laparoscopy was conducted, revealing approximately 5x6 cm bilateral uterine horns with healthy adnexa.As the first step, a neovagina was created using a modified peritoneal pull-down technique, a standard approach in our clinic. A vaginal incision was made, and a blind vaginal dissection was performed to reach the peritoneum vaginally. Subsequently, an acrylic vaginal mold was inserted. The vaginal orifice was laparoscopically incised using ultrasonic energy with guidance from the inserted vaginal acrylic mold. The orifice was gradually dilated with larger molds. The entire pelvic peritoneum was dissected circularly, and the distal part of the dissected peritoneum was pulled down using four 2.0 Vicryl sutures at 0, 90, 180, and 270 degrees from the opened vaginal orifice. The uterine cavities of both remnants were incised, and two separate Foley catheters were placed in both cavities. A mold with a hole was used to insert the catheters through the vagina. Both catheters were secured in the cavities with Prolene sutures pulled up from the anterior abdominal wall.The next step involved uterine anastomosis. The uterine remnants were unified through continuous suturing, resulting in the formation of a normally shaped uterus. In the final step, the created uterus and neovagina were anastomosed. The patient received instructions on how to perform mold exercises and follow-up care. Main Outcome MeasureDescription of a laparoscopic management of a rare mullerian abnormality. ResultsThe postoperative MRI scan at one month revealed healed unified uterine cavities and vagina. The patient experienced spontaneous menstruation in the second month after surgery and now maintains regular menses with an approximately 9-10 cm functional vagina. Within three months after surgery, the VAS (visual analog scale) scores for chronic pelvic pain and dysmenorrhea decreased from 9 to 2-3. ConclusionsMüllerian abnormalities are exceptionally rare, and their spectrum is broad, making it challenging to identify an exact surgical method to restore functional anatomy. Therefore, a customized surgical approach should be designed for each patient based on their unique condition.

Successful Reproductive Outcome Following Challenging Surgical Management of Mullerian Anomalies

Background: Mullerian duct anomalies consist of a set of structural malforma-tions of reproductive organ resulting from abnormal development of the paramesonephric or Müllerian ducts. The prevalence of these anomalies ranges from 0.001 to 10% in the general population and from 8-10% in women with an adverse reproductive history. The aim of this study was to diagnose different type of mullerian anomalies and to evaluate the reproductive outcome that occurs as a result of surgical management of malformation of genital tract. Methods: A prospective observational study of mullarian anomalies and its diagnosis & reproductive outcome was observed by using data from women with congenital anomalies attended in OPD either with complains or incidental diagnosis with HSG,during laparoscopy at tertiary care center. Total 100 women with different types of mullarian anomalies were included in study. Results: Most common utero-vaginal anomaly seen in present study was septate uterus with on basis of clinical examination. Out of 100 patient (35)35% were diagnosed mullerian agenesis. Cervico vaginal agenesis (15)15%, Septate uterus 28(28%), Bicornaute uterus 7(7%), Transverse vaginal septum 5 % ,unicornuate 5%, Didelphi’s with longitudinal vaginal septum 2%, Microperforate Hymen 3(3%). Among all patients 26 patients were pregnant. Among primary amenorrhea 35 patient of mullarian agenesis after progressive non-surgical vaginal dilatation 22 patient had no coital difficulty. 5 patients were need vaginoplasty. Among 15 patients of cervicovaginal agenesis 6 become menstruating, 2 were pregnant, 6 patient failed anastomosis, needed total abdominal hysterectomy 3 patients on oral pill. Among 5 transverse vaginal septum, 5 having regular menestration among 2 become pregnant. Among other mullarian anamolies after challenging surgical management 22 patient become pregnant. Conclusion: Present study shows prevalence of congenital malformation of female reproductive tract is 0.83% at our .....

7918 Laparoscopic Repair of Cervico-Vaginal Agenesis in a Previously Failed Attempt of Mcindoe's Vaginoplasty: A Case Report

<h3>Study Objective</h3> A unique Laparoscopic approach for surgical repair of Cervico-vaginal agenesis. <h3>Design</h3> A case report with 6 months follow-up. <h3>Setting</h3> laparoscopy surgery with patient in lithotomy position under anaesthesia. <h3>Patients or Participants</h3> A 23-yrs old female came with primary amenorrhea with lower abdominal pain since 2 years. Her MRI was suggestive of: 1) upper 2/3rd vaginal aplasia with hematoma in the cervical canal 2) b/l hematosalpinx 3) b/l ovarian hemorrhagic cysts. McIndoe's Vaginoplasty was attempted 6months back which had failed. <h3>Interventions</h3> Laparoscopic cervico- vaginal agenesis repair was planned <h3>Measurements and Main Results</h3> The occurrence of cervical agenesis is quite uncommon (1:80 000–1:100 000) and only 39% of these cases simultaneously have vaginal agenesis. Initially, these cases were managed by hysterectomy or abdominal cervicovaginoplasty. Later, a minimally invasive approach came to light with the advent of laparoscopy and surgical expertise. Principles of surgery: 1. Laparoscopic management of Hematosalpinx and hemorrhagic cysts. 2. Dissection of UV fold and pushing the bladder down to expose 3-4 cm length of the vagina. 3. Dissection between cervix and vagina in the right plane. 4. Creating neo-vagina by dissection of the vaginal agenesis. 5. Vertical incision on the cervix and horizontal incision on the vagina Suturing techniques between cervix and vagina and placing a silicon catheter in cervical canal and neo-vagina. <h3>Conclusion</h3> Laparoscopic approach for cervico vaginal agenesis is better than vaginal approach with cutting cervix vertically for patency of cervical canal.

Malformasi Genital Perempuan: Kasus di RSUP Dr. Sardjito Yogyakarta Tahun 2019

BackgroundReproductive tract malformations are rare in general population but are commonly encountered in women with infertility and recurrent pregnancy loss. Their true prevalence in the general population is not absolutely known mainly owing to methodological bias. Common uterine anomalies are important owing to their impact on fertility, and certain Mullerian malformations are particularly important because they cause serious clinical symptoms and affect woman’s quality of life. Identification of symptoms and timely diagnosis are an important key to the management of these defects. Although MRI being gold standard in delineating uterine anatomy, recent advances in imaging technology, specifically 3-dimensional ultrasound, achieve accurate diagnosis. Surgical management depend on the type of anomaly and its complexity and also involves multiple specialties; thus, patients should be referred to centres with experience in the treatment of complex genital malformationsObjectiveDetermined the description of cases of female genital malformation at Dr. Sardjito Hospital in 2019.MethodThis is descriptive study. The subject in this study were all new patient with female genital malformation at Obgyn Polyclinic Dr. Sardjito Hospital January-December 2019. Obtained 30 cases of female genital malformation.ResultThere is 30 cases of genital malformation in 2019. They were 4-44 years with mode at group 16-20 years. They consist of vaginal and cervicovaginal agenesis 14 cases, Mayer-Rokitansky-Küster-Hauser (MRKH) Syndrom 6 cases, hematometra of hemiuterine 4 cases, Herlyn-Werner-Wunderlich (HWW) syndrome 3 cases, imperforate hymen, didelphys uterus and genital ambiguous each 1 case. Five cases of cervicovaginal agenesis have been done sigmoid vaginoplasty, all of them have good sexual function after procedure. One case MRKH syndrome has been done sigmoid vaginoplasty with good sexual function after that. Two cases HWW syndrome have been surgical treatment that are vaginal septectomy and vaginal septectomy with laparascopic cystectomy.ConclusionThe correct knowledge of embryology of the genitourinary is essential for the understanding, study, diagnosis and management of genital malformations, especially complex ones and those that lead to reproductive problems. Surgical techniques for correcting genital malformations depend on the type of anomaly and complexity.

Three-dimensional Printer Molds for Vaginal Agenesis: An Individualized Approach as Conservative Treatment.

The aim of this study was to evaluate the use of vaginal molds, made with three-dimensional (3D) printing, for conservative treatment through vaginal dilation in patients with vaginal agenesis (VA). A total of 16 patients with a diagnosis of VA (Mayer-Rokitansky-Küster-Hauser syndrome, total androgen insensitivity syndrome, and cervicovaginal agenesis) from the Federal University of São Paulo were selected. Device production was performed in a 3D printer, and the polymeric filament of the lactic polyacid (PLA) was used as raw material. A personalized treatment was proposed and developed for each patient. There were 14 patients who reached a final vaginal length of 6 cm or more. The initial total vaginal length (TVL) mean (SD) was 1.81(1.05) and the final TVL mean (SD) was 6.37 (0.94); the difference, analyzed as 95% confidence interval (95% CI) was 4.56 (5.27-3.84) and the effect size (95% CI) was 4.58 (2.88-6.28). The 3D printing molds for vaginal dilation were successful in 87.5% of the patients. They did not present any major adverse effects and offered an economical, accessible, and reproducible strategy for the treatment of VA.

Successful laparoscopic sigmoid transposition for cervicovaginal agenesis in the presence of a functioning uterus: A case series.

This study investigated the long-term outcomes of laparascopic sigmoid cervicovaginoplasty in patients with congenital cervicovaginal atresia with a functioning uterus. In this case series study, seven patients with cervicovaginal atresia with a functioning uterus underwent laparoscopic sigmoid cervicovaginoplasty surgery between 2016 and 2020. Mean follow-up duration was 25.9months (2-48 months). All of the patients had regular menstrual cycles. The average length of the vagina was 8.9cm (7.6-10.5cm). In one patient, proximal stenosis of neovagina was observed 12 months after surgery. The mean age and body mass index of the patients were 18 years (12-27 years) and 19.7 (17.6-22.4), respectively. The average time period between the initiation of disease symptoms and the operation was 52.28 months (2-156 months). There were no postoperative short-term complications. We did not perform hysterectomy in our patients. None of our patients complained of signs of low anterior resection syndrome. Two patients had sexual activities without dyspareunia, postcoital bleeding, or malodorous vaginal discharge. Sigmoid cervicovaginoplasty is a safe and effective procedure with satisfactory long-term outcomes. This surgery eliminates the psychological burden of hysterectomy in these patients. Through preserving the uterus, patients may have a chance of possible future pregnancy if abdominal cerclage is performed.

Minimally invasive vaginal approach to the uterovestibular anastomosis for cervicovaginal aplasia: a case series and review of literature.

Congenital cervicovaginal agenesis (CVA) with functioning endometrium is an extremely rare mullerian anomaly. Genital tract patency and fertility preservation are the major challenges in cases of CVA. With the advances in surgical techniques, management has shifted from a radical approach-like hysterectomy towards a more conservative approach of uterine conservation. In the present study, we report our experience in managing four cases of congenital CVA with complete vaginal atresia, which were treated with a simple minimally invasive vaginal approach for UVA without using graft for neovagina creation and studied the long-term anatomic and functional result of uterovestibular anastomosis (UVA) in patients with CVA. A literature review was performed for congenital complete vaginal atresia (≤ 2cm blind vagina), with a functioning uterus. The experience and results of this 4-year study are in accordance with the prior studies with 97% (33/34) success rate, where vestibular mucosa was used for the UVA. Findings of this study should encourage more gynaecologists to learn and recreate a complete vaginal approach, as no special surgical equipments are needed. Hysterectomy should only be reserved for cases, where repeated anastomosis attempts fail. This minimally invasive technique should be preferred over canalization procedures and graft, as it is associated with a higher success rate with least complications and recurrence. Conservative end-to-end anastomosis with a completely vaginal approach should be offered as the primary treatment option for CVA with total vaginal atresia.

Partial Mullerian Agenesis with Renal and Skeletal Anomalies: An Observational Study of MURCS Association

Background: Müllerian agenesis is a disorder of genital development characterized by absence of the vagina, an absent or hypoplastic uterus, and normal or hypoplastic fallopian tubes. Typically, the ovaries are entirely normal, although one or both also may be undescended, hypoplastic, or associated with an inguinal hernia. Affected patients often also have urologic anomalies (15-40) % (unilateral renal agenesis, ectopic or horseshoe kidney, and duplication of the collecting systems) and skeletal malformations (5-10) %, (e.g., hemiverterbrae and scoliosis, or the Klippel-Feil syndrome). The cause is unknown, although some cases are associated with chromosomal translocations or occur in familial aggregates, suggesting a genetic basis. Logically, müllerian agenesis might be attributed to an activating mutation in the gene encoding AMH or its receptor, causing excess AMH activity, but none have been identified. Aim: The aim of this study was to analyze and present rare cases of partial mullerian agenesis combined with renal and/or skeletal malformations, their causes, clinical features, diagnosis and treatment along with review of literatures. Methods: A retrospective observational study was conducted in the department of Bangabandhu Sheikh Mujib Medical University. All four cases presented with similar type of presentation and were diagnosed as partial Mullerian aplasia (cervico-vaginal agenesis) with other associated anomalies (renal, skeletal or cardiac anomalies) ie: MURCS association. Results: We analysed four cases of mullerian agenesis with renal and/or skeletal abnormalities presented with primary amenorrhoea with cyclical lower abdominal pain. Subsequently these patients were ascertained to have cervicovaginal agenesis with renal and skeletal abnormalities by physical examination and relevant investigations. Two (50%) patients had renal anomalies in the form of renal agenesis and one small malrotated kidney while 100% patients had skeletal abnormalities. One (25%) patient had congenital cardiac anomaly, one (25%) patient had history of cleft lip and cleft palate and one (25%) patient had history of imperforated anus at birth. Evaluation included clinical examination, whole abdominal ultrasound, skeletal survey and karyotyping along with some supportive investigations. Conclusion: The management is varied but multidisciplinary approach, psychological and social supports are the key points of managing such cases.

Successful Staged Management of Cervico Vaginal Agenesis- Seven Cases

<h3>Study Objective</h3> Cervicovaginal Agenesis with a functional Uterine Corpus, poses a unique challenge. Earlier, hysterectomy was the resultant treatment as initial canalization attempts reported fatal ascending infections and failures. We report our successful staged approach to create a neovagina for its coital function and save the functional uterine corpus for its menstrual and reproductive functions. <h3>Design</h3> retrospective single center experience. <h3>Setting</h3> tertiary referral center. <h3>Patients or Participants</h3> Seven girls confirmed to have Cervicovaginal Agenesis with hematometra after clinical and MRI evaluation. Five had associated endometrioma. <h3>Interventions</h3> They were planned for staged management. At the first stage, they underwent laparoscopic assessment of pelvis, correction of endometrioma and creation of neovagina. All had an anomalous uterine corpus. Hematosalphinx and endometriosis were treated when noted. Neovagina was created in four by conventional Mc Indoe's vaginoplasty, one needed a simple Frank's method. Girls were taught self vaginal dilatation in between for 3 months to obtain a wide and long neo vaginal lumen with mature epithelial lining. They were given GnRH to be symptom free in between. At the second stage once the neovagina matures girls underwent laparoscopic utero-neovaginal anastomosis. After preparing both the lower uterine segment and neovaginal apex for at least 0.5 cm, anastomosis was done using 0 barbed sutures. A silicone Foley stent was used in the initial period. To make it a tension free anastomosis, ligaments were released to mobilize the corpus down, carefully preserving uterine vascularity. After 3 months of cyclical pills a hysteroscopic assessment of the anastomosis and endometrial cavity was done. <h3>Measurements and Main Results</h3> On long follow-up, now all have established menstrual cycles. Two are married, having vaginal intercourse presently considering fertility options. <h3>Conclusion</h3> The staged procedure helps relieve immediate symptoms, manage endometriosis, directly assess the corpus, reduces ascending infection at first and then help the neovaginal graft mature and pliable for a successful anastomosis at second stage.

Unique minimally invasive management of cervicovaginal agenesis

A 10-year-old premenarchal girl presented with worsening abdominal pain for 3 months. She had undergone laparotomy twice at 1 and 9 years of age for urogenital sinus and subacute intestinal...

Percutaneous Intrauterine Device Placement: A Solution for Hematometra Due to Cervicovaginal Agenesis

Müllerian duct underdevelopment results in agenesis and atresia of the vagina, cervix, or uterus, which can lead to hematometra and hematometrocolpos. Management includes vaginal-cervical serial dilation and/or surgery and menstrual suppression with endovaginal levonorgestrel intrauterine device (IUD) placement. However, these options are not feasible in vaginal agenesis because there is no external tract. This report describes the percutaneous placement of an IUD to treat hematometra due to cervicovaginal agenesis.

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.

47. Presentation and Management of Uterine Didelphys with Unilateral Cervico-Vaginal Agenesis: a Multi-Center Case Series

47. Presentation and Management of Uterine Didelphys with Unilateral Cervico-Vaginal Agenesis: a Multi-Center Case Series

Neovagina creation using sigmoid colon in vaginal agenesis: a case report and review of literature

Mullerian duct anomalies are rare and can present with abnormalities in upper vagina, cervix, uterus, and fallopian tubes. The exact incidence of cervicovaginal agenesis is unknown and there are very few cases recorded in the literature. Authors report a 12-year-old girl referred from gynaecologist with chronic cyclical abdominal pain with underdeveloped, geniatilia and developed secondary sexual characters. Radiological investigations showed hypoplastic cervix with hematometra and left hemato salpinx. A definitive repair with creation of a neovagina using a sigmoid colon segment was performed in a single stage. Many techniques are described for reconstruction of cervicovaginal canal. Use of colon in creation of a neovagina is described by authors. Here we report a case of complete vaginal agenesis presenting with Hematometra-Hematosalpinx where a neovagina was created using a segment of sigmoid colon. Single staged surgery for neovagina creation using segment of sigmoid colon offers a promising and safe alternative for cervicovaginal agenesis.

Laparoscopic Hysterectomy with Cervicovaginal Agenesis

Video Objective We present a laparoscopic surgical approach for hysterectomy in Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome with cervicovaginal agenesis. Laparoscopic approaches for removal of uterine remnants have been published with only reports of laparotomy described for hysterectomy with cervicovaginal agenesis. Setting We discuss the diagnosis, management considerations and preoperative planning for definitive surgical treatment of a patient with MRKH with cervicovaginal agenesis presenting with hematometra and pain. Patient initially presenting with primary amenorrhea and pain, with previous medical treatment to achieve amenorrhea. Interventions Laparoscopic hysterectomy with bilateral ureterolysis and uterine artery ligation in a patient with cervicovaginal agenesis. Conclusion We show that laparoscopic hysterectomy is a viable and safe option in patients with cervicovaginal agenesis. The importance of preoperative imaging to rule out associated anomalies and assist in surgical planning is stressed. We highlight necessary alterations in surgical technique to overcome the lack of traction, identify potential anomalous vasculature and ultimately maintain hemostasis, clear delineation of surgical anatomy, and avoid injury. Tissue extraction through laparoscopic in bag morcellation is performed thus avoiding mini laparotomy altogether.

Uterus Dysplasia Associated with Cervico-Vaginal Agenesis.

Müllerian ducts can form upper parts of normal female reproductive system and any failure in ductal fusion may result in to müllerian duct anomalies (MDA). We present a case of MDA and a uterus dysplasia with no evidence of cervical or upper vaginal tissue. This case showes the role of magnetic resonace imaging (MRI) on MDA diagnosis and urges the need for a unified reliable and practical classification more compatible with clinical practice.

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.

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.

Diagnostic imaging and cataloguing of female genital malformations.

To help physicians and radiologists in the diagnosis of female genito-urinary malformations, especially of complex cases, the embryology of the female genital tract, the basis for Müllerian development anomalies, the current classifications for such anomalies and the comparison for inclusion and cataloguing of female genital malformations are briefly reviewed. The use of the embryological system to catalogue female genito-urinary malformations may ultimately be more useful in correlations with clinical presentations and in helping with the appropriate diagnosis and treatment. Diagnostic imaging of the different genito-urinary anomalies are exposed, placing particular emphasis on the anomalies within group II of the embryological and clinical classification (distal mesonephric anomalies), all of them associated with unilateral renal agenesis or dysplasia. Similarly, emphasis is placed on cases of cervico-vaginal agenesis, cavitated noncommunicated uterine horns, and cloacal and urogenital sinus anomalies and malformative combinations, all of them complex malformations. Diagnostic imaging for all these anomalies is essential. The best imaging tools and when to evaluate for other anomalies are also analysed in this review.Teaching points• The appropriate cataloguing of female genital malformations is controversial.• An embryological classification system suggests the best diagnosis and appropriate management.• The anomalies most frequently diagnosed incorrectly are the distal mesonephric anomalies (DMAs).• DMAs are associated with unilateral renal agenesis or renal dysplasia with ectopic ureter.• We analyse other complex malformations. Diagnostic imaging for these anomalies is essential.