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Study Objective To review the currently available methods for surgical and nonsurgical neovaginal creation with an additional focus on situations requiring vaginal stretching, replacement, or augmentation and the management of vaginal stenosis or stricture. Design Review of the existing literature Methods PUBMED search with key words vaginal agenesis, neovaginal creation, vaginoplasty, vaginal stretching, vaginal augmentation, vaginal stenosis, outcomes of vaginoplasty, Mullerian anomalies, vaginal anomalies Results The need to perform vaginal stretching, replacement, or augmentation will vary depending on the underlying reproductive tract condition. Conclusions A variety of techniques have been described to performed vaginal stretching, replacement, or augmentation. There are risks and benefits to each technique. Vaginal stricture and stenosis remain difficult problems given the high rates of these complications in patients undergoing these procedures. Although several techniques can alleviate this problem, they rely on the thickness of the stenosis and the location of the stenosis within the vagina.

Treatment for children born with vaginal agenesis remains difficult, without a clear gold standard for tissue replacement. An autologous-engineered vaginal replacement would significantly improve quality of life for people born with this condition. The aim of this study was to critically review literature on the current state of tissue engineering for vaginal reconstruction in a pediatric population. An electronic literature search was conducted using PubMed for articles describing pediatric vaginal tissue engineering from January 2003 to December 2020. Nine studies met inclusion criteria and were reviewed. The model, methods, cell type and source, scaffold type, and time of analysis and evaluation were compared. Three studies used

Vaginal atresia is seen in genetic disorders such as Mayer–Rokitansky–Küster–Hauser (MRKH) syndrome, which can cause significant sexual dysfunction. Current treatments include surgical reconstruction or mechanical dilation of the vaginal canal. Mechanical dilation requires patients to be highly motivated and compliant while surgical reconstruction has high rates of complications. This study evaluated a novel vaginal expansion sleeve (VES) method as an alternative treatment for vaginal atresia. The proprietary cylindrical VES is a spring-like device consisting of polyethylene terephthalate helicoid trusses capped at each end with a fixed diameter resin cap for fixation within tissues. Following the development of the VES and mechanical characterization of the force–length relationships within the device, we deployed the VES in Sprague Dawley rat vaginas anchored with nonabsorbable sutures. We measured the VES length–tension relationships and post-implant vaginal canal expansion ex vivo. Vaginal histology was examined before and after implantation of the VES devices. Testing of 30 mm sleeves without caps resulted in an expansion force of 11.7 ± 3.4 N and 2.0 ± 0.1 N at 50% and 40%, respectively. The implanted 20 mm VES resulted in 5.36 mm ± 1.18 expansion of the vaginal canal, a 32.5 ± 23.6% increase (p = 0.004, Student t test). Histological evaluation of the VES implanted tissue showed a significant thinning of the vaginal wall when the VES was implanted. The novel VES device resulted in a significant expansion of the vaginal canal ex vivo. The VES device represents a unique alternative to traditional mechanical dilation therapy in the treatment of vaginal atresia and represents a useful platform for the mechanical distension of hollow compartments, which avoids reconstructive surgeries and progressive dilator approaches.

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.

Study Objective To investigate the clinical appearance and morphologic and ultrastructural aspects of the mucosa of the peritoneal neovagina after laparoscopic Davydov neovaginoplasty in patients with Mayer-Rokitansky-Kuster-Hauser syndrome. Design The study group was a prospective, observational, experimental cohort of cases treated in the same institution between 2015 and 2019. Patients were followed up at 3, 6, and 12 months after surgery and then every 12 months. Setting Single-center academic institution and teaching hospital in Milan, Italy. Patients Fifty-one consecutive subjects with clinical and imaging diagnosis of Mayer-Rokitansky-Kuster-Hauser syndrome surgically treated by the same team and postoperatively followed. Interventions All the subjects underwent the same standardized surgical procedure and thereafter were followed up at 3, 6, and 12 months after surgery and then every 12 months; a minimum follow-up of 12 months was achieved in all cases. Vaginoscopy and Schiller test were performed at each follow-up visit, and a biopsy specimen of the neovagina was obtained in a limited number of patients (6 out of 51) for light microscopy (LM) and scanning electron microscopy (SEM) analysis of the tissue. Measurements and Main Results In vaginoscopy, the neovaginal mucosa appeared homogeneous, smooth, and pink all along the neovaginal tract; the Schiller test detected iodine positivity at different degrees of extension upward from the hymenal ring, starting at 3 months postoperatively with almost complete positivity between 6 to 12 months in all cases. LM demonstrated adequate thickness and differentiation of the new mucosa along with the presence of glycogen storage; SEM revealed an ultrastructural surface appearance very close to normality. The main difference compared with a normal vagina was the reduced presence of vaginal mucosal folds. Conclusion Under different techniques (vaginoscopy, Schiller test, LM, and SEM), a minimum of 6 months after surgery, the peritoneal neovagina epithelium showed aspects comparable to the natal mucosa of the vagina.

Meyer Rokitamsky Kuster Hauser sendromlu 21 yaşındaki bir kadın amenore ve koitus sırasında vajinal penetrasyon sağlayamama şikayetleri ile başvurdu. Manyetik rezonans incelemede normal görünen overlerin yanında bilaterak rudimenter uterin kalıntılar ve uterus yokluğu tespit edildi. Laparoskopik olarak vajinal yolla yukarı itilmiş olan pelvik peritona ve vezikorektal boşluktaki vajinal apekse 3 cm'lik bir insizyon yapılarak kör vajinaya 2 parmak genişliğinde bir açıklık oluşturuldu. Ön ve arka viseral periton kenarları vajinal grasper yardımı ile yeni oluşturulmuş vajinal açıklıktan aşağı doğru çekilerek vajinal epitelyal sınıra dikildi. Purse string sütür tekniği ile sütüre edilerek yeni oluşturulmuş olan neovajinal açıklık kapatıldı. Hastanın postoperatif dönemi sorunsuz geçti. Hasta 3 ay boyunca geceleri vajinal silikon dilatatör kullandı ve takiben cinsel aktivitede bulunmasına izin verildi. Modifiye Davydov prosedürü diğer cerrahi yöntemlerle karşılaştırıldığında; düşük komplikasyon oranları ve düşük vajinal stenoz oranlarının yanında daha iyi kozmetik sonuçlar ve daha yüksek seksüel tatmin oranları olan etkili bir cerrahi işlemdir., A-21 year-old woman diagnosed with Meyer Rokitamsky Kuster Hauser syndrome has presented with a complaint of amenorrhea and failure to have vaginal penetration during coitus. On magnetic resonance imaging, bilateral rudimentary uterine remnants next to normal-appearing ovaries with absence of uterus have been detected. Laparoscopically, a 3 cm transverse incision was made pelvic peritoneum and vaginal apex to create a two-finger width opening to the blind vagina. Anterior and posterior visceral peritoneal edges have been grasped and sutured vaginally to the vaginal epithelial border. A continuous purse string stitch technique has been utilized to close the proximal part of the neovagina. The postoperative course of the patient was uneventful. The patient kept using a vaginal silicone dilatator every night for 3 months and she was allowed to have regular coital activity after then. The modified Davydov procedure is an efficient surgical technique with lower complication rates and vaginal stenosis rates.

Background: The ideal timing of genital surgery in differences/disorders of sex development (DSD) is controversial and differs according to the underlying type of DSD. Increasing numbers of persisting sinus as a result of delayed feminizing genitoplasty in DSD patients require interdisciplinary collaboration of pediatric surgeons/urologists and gynecologists. This study focusses on surgical techniques other than bowel vaginoplasties and results of gender assigning surgery in young adolescents. Methods: Data of adolescent and adult patients treated between 2015 and 2022 were analyzed retrospectively: underlying type of malformation, techniques of vaginoplasty, vaginal length and caliber, possibility of sexual intercourse, and temporary vaginal dilatation. Results: A total of 9 patients received a primary vaginoplasty at a median age of 16.75 years (range 10.3–29.25). The underlying anatomical conditions were persistent urogenital sinus (UGS) in 8 patients (3 patients with CAH, 2 patients with XY-DSD, 1 patient with cloacal malformation and missed UGS, 2 patients with UGS only). One patient had a MURCS association. Surgical techniques were total urogenital mobilization and perineal flap vaginoplasty in 4 patients, modified McIndoe vaginoplasty in 4 patients, and a laparoscopic vaginal pull-through in 1 patient. In a median follow-up of 45 months (2–84), all but 1 patient presented with physiological vaginal length and width. Conclusions: If possible, modern treatment concepts delay gender assigning surgery until the participation of the patient in the decision-making process is possible. Optimal treatment concepts are given by transfer of surgical techniques from pediatric urology/surgery by multidisciplinary teams. Techniques other than bowel vaginoplasties are favorable.

Increasing numbers of transgender patients are opting for gender-affirming care. Since pediatric and adolescent gynaecology (PAG) providers perform the majority of vaginoplasty procedures for developmental anomalies of the female reproductive tract (such as vaginal agenesis), this commentary supports the position that PAG providers should be involved in the pre- and postoperative care of trans women.

Highlights • Vaginal agenesis is a complex pediatric condition that lead to long-term urinary, gastrointestinal, and sexual dysfunction. • A meticulous physical examination and in-depth diagnostic investigations are necessary to determine the appropriate surgical management. • Reconstruction of neovagina can be retrieved from the ileum., Vaginal agenesis with anorectal malformations is a complex pediatric condition that adversely affects various physiological processes in the body. It may cause disturbances in defecation and urination, abnormalities in the urinary and gastrointestinal tract, dysfunction of the genital and reproductive organs, and sexual function disorders. The complexity in the surgical management of vaginal agenesis includes the selection of a functional reconstruction technique for anal and vaginal formation, timing of the reconstruction, and management of complications in the associated organ system. Herein, we describe a patient with Mayer–Rokitansky–Küster–Hauser syndrome accompanied by a rectovesical fistula. Other abnormalities, such as microcephaly, polydactyly, long urethral abnormalities resembling the male urethra, and complications in the kidney and urinary tract, were observed in the patient. The associated complications included recurrent urinary tract infections, urinary overflow incontinence, vesicoureteric reflux, hydroureter, and left renal hydronephrosis. The patient underwent posterior sagittal anorectoplasty surgery and vaginal reconstruction. The long-term vaginal physiological development of patients with this condition remains unknown.

Background Creating a functional neovagina is a practical therapeutic intervention for women with congenital vaginal agenesis and sexual needs. Although the incidence of neovaginal prolapse (NP) is low, it is inconvenient for patients and is a challenging problem for gynecologists. Case A 32-year-old woman who had undergone transabdominal sigmoid vaginoplasty 10 years previously at another hospital visited our clinic for evaluation and treatment of NP. Gynecological examination showed exstrophy of the vaginal apex, 4 cm beyond the hymen. Laparoscopic sacrocolpopexy was performed using a mesh. The mesh was sutured at the anterior wall and apex of the neovagina and suspended in the anterior sacral region without blood vessels. Summary and Conclusion Laparoscopic sacrocolpopexy might be an effective and safe treatment for NP.

Background. There is still no standardized treatment for vaginal agenesis; surgical repair using a minimally invasive technique is a good option, as it offers rapid results along with a fast postoperative recovery. Objective. To describe a new modified Vecchietti procedure by a mini-laparoscopic approach with intraoperative use of indocyanine green fluorescence technology for the creation of a neovagina in the setting of congenital vaginal agenesis. Methods. Retrospective review of all cases of congenital vaginal agenesis submitted to a mini-laparoscopic modified Vecchietti procedure with intraoperative use of indocyanine green (ICG) fluorescence (between June and September 2019). Patient relevant medical history, surgical technique, postoperative care, outcomes, and complications are described. Results. Four patients with Mayer–Rokitansky–Küster–Hauser syndrome (mean age: 19 years; mean preoperative vaginal length: 1.5 cm) were included in the study. The procedure was completed successfully and uneventfully in all patients. External traction device was removed 5–7 days after surgery and replaced by external vaginal silicone dilators. This technique was able to create 10–11 cm neovaginas in 1 week; six weeks after surgery all patients had an epithelized 10.5–12 cm length and 2.5–3 cm diameter neovagina. Complications consisted of one case of isolated fever at the ninth postoperative day, solved with empiric antibiotic treatment. Conclusion. The mini-laparoscopic modified Vecchietti approach, associated with ICG fluorescence imaging, is feasible and effective in the treatment of congenital vaginal agenesis, as it is a simple and safe procedure with promising outcomes.

Objective Congenital Mullerian duct malformations are a challenging group of conditions for surgeons and need surgical experience and skill. Accordingly, the aim of this study is to present the diagnosis, surgical management, and clinical implications of congenital uterovaginal abnormalities. Methods Between 1980 and 2015, 8 patients with congenital uterovaginal abnormalities were diagnosed. In one patient a unique case of an unusual horseshoe shaped double uterus communicating via a transverse canal along with agenesis of the cervix and vagina was noted, and utero-vaginal agenesis was diagnosed in 6 patients. Complete androgen insensitivity syndrome with its female phenotype associated with bilateral testicular tissue in the inguinal canal with an accompanying absence of the ovaries, uterus, uterine tubes, vagina, and an imperforate hymen, was diagnosed in one patient. Clinical examination of all the patients revealed well-developed secondary sexual characteristics. A modified McIndoe vaginoplasty procedure was the surgical treatment common to all patients to treat vaginal agenesis. The surgery was performed by a consultant (Dr. K.G. Paul) using the standardized surgical technique. Results An unusual Mullerian duct anomaly, uterus bicornisacollis, was successfully corrected by uteroplasty and a new cervix was constructed. Complete vaginal agenesis was corrected by a modified McIndoe vaginoplasty technique. None of the patients had any significant post-operative complications. Conclusion Knowledge of congenital uterovaginal abnormalities diagnosed in this study is essential for surgeons, clinical anatomists, radiologists, and morphologists who may increase the success of their diagnostic evaluations and surgical approaches to the region.

Objective To investigate the clinical characteristics and outcomes of pelvic abscess after cervicovaginal canalization for congenital cervical and vaginal agenesis. Cases Four patients who had pelvic abscess after cervicovaginal canalization for congenital cervical and vaginal agenesis are reported. The mean onset time of pelvic abscess after primary canalization was 67.7 months. Three patients presented with pelvic endometriosis. Three patients underwent cervical catheter or intrauterine device placement, with a mean time of 62 months. All patients underwent hysterectomy and pelvic abscess removal, and no recurrence of pelvic abscess or formation of pelvic pseudocysts was observed during the follow-up. Conclusion Pelvic abscess after cervicovaginal canalization was mainly due to re-obstruction of the neocanal, the risk of which may increase when surgery is combined with prolonged cervical stent placement and pelvic endometriosis. Hysterectomy should be suggested once pelvic abscess is diagnosed.

Cervicovaginal atresia with a functional uterus is rare. There are no established surgical methods to treat this condition, and only a few reports have been published on surgical techniques. Furthermore, postoperative complications, such as restenosis, often require reoperation. A 19-year-old woman was pointed out cervical hypoplasia and referred to our hospital for further examination and treatment. A pelvic examination revealed that the vagina had a slight recession with a blind end. Transrectal ultrasound and pelvic magnetic resonance imaging revealed congenital vaginal agenesis and cervical hypoplasia. Elective surgery was performed after reshaping the vagina. A radical surgery was performed 10 months later after sufficient self-dilation by using Frank's technique in an outpatient setting. At first, we approached by laparoscopically to correct autologous peritoneum and to bladder detach, then the cervical canal was identified. Next, a skin biopsy punch was used several times to hollow out the cervical tissue to shape and expand the cervical canal. A catheter was then placed in the uterus and autologous peritoneum was wrapped around it and fixed to the cervical canal. The catheter was removed 6 weeks postoperatively, and the patient continued dilating her vagina until she was able to have sexual intercourse, and then stopped the self-dilation. Eight months postoperatively, the patient did not report any menstrual irregularities. It is important to make corrections to prevent restenosis of the vagina and cervical canal and prevent the symptoms from recurring. Make use of autologous peritoneum as graft onto the cervical canal is effective method for the treatment of cervicovaginal atresia.

Our objective was to provide a minimally invasive neovaginoplasty technique to construct a nearly physiologic vagina to facilitate sexual functioning and appropriate vaginal length in patients with congenital vaginal agenesis. This retrospective study at a tertiary care hospital comprised 52 patients with congenital vaginal agenesis because of Mayer-Rokitansky-Kuster-Hauser syndrome or androgen insensitivity syndrome presented for vaginal reconstruction. Modified McIndoe vaginoplasty was done in all patients between 2010 and 2018 using a vaginal mold created with glove, nonadherent petroleum gauze, and Interceed absorbable adhesion barrier (Ethicon, Johnson & Johnson, Somerville, NJ) that was placed in the neovagina space created between the bladder and rectum for 7 days. Operative details, complications, length and width of the neovagina, and functional outcome were evaluated. The mean operation time was 35 minutes. The mean length of the constructed neovagina was 8.4 cm × 3.4 cm at 6 weeks follow-up. Epithelialization was completed by 4 to 6 months. All patients reported satisfactory sexual activity with no pain and good mucosal sensitivity. This modified neovaginoplasty technique is easy to perform, involves painless postoperative dilatations as the cornerstone of treatment, and results in adequate secretion, allowing lubrication and acceptable physiologic results.

The vagina is a membranous muscular tube that connects the vulva and uterus. Congenital or congenital abnormalities in the form of complete or partial absence of the vagina (vaginal agenesis). Patients who experience vaginal agenesis have less frequency, namely 1 in 4000 births, 1 in 4000 to 10,000 births (ACOG). Meanwhile in Dr. Cipto Mangunkusumo Jakarta from 1995 to 1999, an average of 10-12 cases per year. It has been reported the case of a female patient aged 14 years who entered the Gynecology Ward Dr. M. Djamil Padang on January 25, 2014 at 11.00 WIB with a delivery from the RSMJ gyn clinic with a diagnosis of transverse vaginal septum + hematometra + hematotrachelos + hematokolpos planned for a septal incision. After performing the operative action, ± 500 cc of blackish brown liquid was successfully removed.Keywords: Agenesis Vagina, TVS, Tranverse Septum Vagina, Case Report

Various vaginoplasty procedures have been developed for patients with Mayer–Rokitansky–Küster–Hauser (MRKH) syndrome. Here, we describe a novel laparoscopic vaginoplasty procedure, known as the Kisu modification, using a pull-down technique of the peritoneal flaps with additional structural support to the neovaginal apex using the incised uterine strand in patients with MRKH syndrome. Ten patients with MRKH syndrome (mean age at surgery: 23.9 ± 6.5 years, mean postoperative follow-up period: 17.3 ± 3.7 months) underwent construction of a neovagina via laparoscopic vaginoplasty. All surgeries were performed successfully without complications. The mean neovaginal length at discharge was 10.3 ± 0.5 cm. Anatomical success was achieved in all patients, as two fingers were easily introduced, the neovagina was epithelialized, and the mean neovaginal length was 10.1 ± 1.0 cm 1 year postoperatively. No obliteration, granulation tissue formation at the neovaginal apex, or neovaginal prolapse was recorded. Five of the 10 patients attempted sexual intercourse and all five patients were satisfied with the sexual activity, indicating functional success. Although the number of cases in this case series is few, our favorable experience suggests that the Kisu modification of laparoscopic vaginoplasty procedure is an effective, feasible, and safe approach for neovaginal creation in patients with MRKH syndrome.

Vaginal agenesis is a common congenital anomaly in females and is most commonly associated with Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome. These patients can be treated with both surgical and nonsurgical procedures. Prefabricated as well as customized vaginal stents are used for the reconstruction and maintenance of neovagina. This case report explains the fabrication of customized tissue conditioner-reinforced acrylic vaginal stent for the treatment of a 20-year-old female having vaginal agenesis associated with MRKH syndrome. This vaginal stent with resilient surface provides a relatively easy, simple, and cost-effective alternative for the treatment of vaginal agenesis. It is also associated with increased compliance and comfort to the patient.

Mayer-Rokitansky-Kuster-Hauser syndrome (MRKHS) is the major cause of vaginal agenesis. Vaginoplasty with oxidized cellulose has been used by gynecologists as a surgical option in vaginal agenesis; however, it is not very widespread among pediatric surgeons. A case of MRKHS who underwent vaginoplasty with oxidized cellulose is reported here.

Background Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, also referred to as Müllerian aplasia, is a congenital disorder characterized by aplasia of the uterus and upper part of the vagina in females with normal secondary sex characteristics and a normal female karyotype (46,XX). Main body The diagnosis is often made during adolescence following investigations for primary amenorrhea and has an estimated prevalence of 1 in 5000 live female births. MRKH syndrome is classified as type I (isolated uterovaginal aplasia) or type II (associated with extragenital manifestations). Extragenital anomalies typically include renal, skeletal, ear, or cardiac malformations. The etiology of MRKH syndrome still remains elusive, however increasing reports of familial clustering point towards genetic causes and the use of various genomic techniques has allowed the identification of promising recurrent genetic abnormalities in some patients. The psychosexual impact of having MRKH syndrome should not be underestimated and the clinical care foremost involves thorough counselling and support in careful dialogue with the patient. Vaginal agenesis therapy is available for mature patients following therapeutical counselling and education with non-invasive vaginal dilations recommended as first-line therapy or by surgery. MRKH syndrome involves absolute uterine factor infertility and until recently, the only option for the patients to achieve biological motherhood was through gestational surrogacy, which is prohibited in most countries. However, the successful clinical trial of uterus transplantation (UTx) by a Swedish team followed by the first live-birth in September, 2014 in Gothenburg, proofed the first available fertility treatment in MRKH syndrome and UTx is now being performed in other countries around the world allowing women with MRKH syndrome to carry their own child and achieve biological motherhood. Conclusion Several advances in research across multiple disciplines have been made in the recent years and this kaleidoscopic review provides a current status of various key aspects in MRKH syndrome and provides perspectives for future research and improved clinical care.

Cervical agenesis or dysgenesis is a rare congenital anomaly. The patients usually present with primary amenorrhoea, primary infertility, and cyclical abdominal pain or with a history of prior surgeries like hymenectomy, vaginoplasty, or adhesiolysis for endometriosis along with well developed secondary sexual characters. We present a case of 15 years old girl with cervical dysgenesis and proximal vaginal agenesis, who presented with severe cyclical abdominal pain. She was managed with cervicovaginal canalization by coring and drilling technique done by vaginal approach with simultaneous laparoscopic guidance. Being a rare type of developmental anomaly of the female genital tract, no standard treatment for type-2 cervical dysgenesis has been established. The patient was followed up for 18 months during which she reported to be having regular menstruation.

Introduction and importance Congenital or hypoplasia vaginal agenesis is a very rare condition caused by the failure of developmental Mullerian ducts. The prevalence is 0.001%–0.025% populations. This condition often misdiagnosed because the symptom does not appear. Acute symptoms such as abdominal pain may occur due to the obstruction of retrograde menstrual flow. In this case, we presented a case complex management of vaginal atresia with pyosalpinx, hematometra and bilateral hematosalpinx. Presentation of case A 12 years old teenager, non-sexually active, complained cyclic abdominal pain that worsening in seven months before admission. Patient never had menstrual blood flow during her life. Patient was diagnosed with hematometra, hematocolpos, bilateral hematosalpinx and distal vaginal agenesis. Amnion graft neovagina was performed. Five days after surgery, patient started to have fever. On the seventh days after surgery, amnion graft was removed. The next two days patient still had fever. Because of continuous fever, patient was test of COVID 19. The result was positive. On the eleventh days after the first surgery, patient complained abdominal pain VAS 3–4. Patient was diagnosed with pyosalpinx by ultrasound examination. Laparotomy was done performing adhesiolysis, bilateral salpingectomy, and omentectomy. Discussion In our case vaginal reconstruction surgery from vaginal approach has been done without management of the bilateral hematosalpinx because the consideration of small caliber of bilateral hematosalpinx. But then complications were developed when vaginal canal was opened, bilateral hematosalpinx were transformed into bilateral pyosalpinx and continue to developed into bilateral tubal abscess. We assume during this process, the bacteria from vagina could fastly infecting the blood and transformed it into pus and grew until tubal abscess. Conclusion The surgical intervention in vaginal agenesis must be considered as a treatment and not only focus on the reconstruction. Laparoscopy or laparotomy may offered as options for combination treatment with vaginal approach reconstructive surgery for vaginal agenesis with obstruction complications such as hematometra and hematosalpinx to prevent the worst condition like ascending infection or misdiagnosed other severe conditions., Highlights • Secondary pyosalpinx after reconstructive surgery of vaginal agenesis patient with bilateral hematosalpinx: a rare case • Secondary pyosalpinx as a complication of bilateral hematosalpinx in vaginal agenesis patient • Postoperative evaluation is important in patient with vaginal agenesis and bilateral hematosalpinx.

Introduction and importance We reported a case of common channel anomaly complicated with vaginal agenesis and rectal stone, which is a long-term complication of PSARP, a combination of very rare conditions with high morbidity and especially difficult treatment. Presentation of case A 15-year-old female presented with a chief complaint of cyclic abdominal pain. The patient had no history of menstruation before. Physical examination showed a common channel. The diameter of the common channel was approximately 1 cm and 0.3 cm in length. A sound (±2 mm thickness) was inserted to the small opening between the urethra and anal mucosa with the length of the canal 6 cm. At the end of the opening, a stone-like structure was felt. The management of this case was abdominal hysterectomy with right salpingectomy and stone evacuation. Discussion Cloacal malformation is thought to be associated with vaginal agenesis since both malformations are considered to have a similar pathophysiologic background. Treatment can be performed using posterior sagittal combined with laparotomy approach. After surgery, patients will need a long-term follow-up since the conditions may be associated with many possible urologic and gynecologic comorbidities, including recurrent urinary tract infections, hematosalpinx, and vesicourethral reflux, including stone formation. Conclusion Our case was considered one of the most complex common channel anomalies because it was complicated with a rare condition called vaginal agenesis and rectal stone after PSARP. A multidiscipline approach was necessary., Highlights • Common channel anomaly with vaginal agenesis and rectal stone after PSARP is a complex rare condition. • Requiring multiple diagnostic modalities • Multidiscipline approach was needed.

Congenital vaginal agenesis is a common condition with an instance of 1 in 5000 females. It is usually associated with Mayer-Rokitansky-Kuster Hauser syndrome. Such anomalies have a high impact on the physiology and psychology of patients. A simple approach for the hollowing of the customized vaginal stent prosthesis has been described for the case of Mayer-Rokitansky-Kuster-Hauser syndrome.

This video reviews technical considerations for performing a modified McIndoe vaginoplasty with skin graft. A 24-year-old female was referred for management of vaginal agenesis. She had unsuccessfully tried vaginal dilation and was interested in vaginal canal creation. A 10 × 20-cm split-thickness skin graft was harvested from the buttock and secured to a condom-covered rubber-sponge mold. The vaginal dissection was initially performed with electrocautery and sharp dissection to enter the plane between the bladder anteriorly and the rectum posteriorly. Then, blunt dissection using a finger, surgical sponges, and retractors was performed to open the space to the level of the peritoneal reflection. With the dissection completed, the graft-covered mold was inserted and secured with labial stay sutures. During the second stage of the procedure, roughly 14 days later, the mold was removed, the graft assessed, and the distal edges secured. A polyethylene mold was then used as the wound continued to heal. The patient had an uncomplicated perioperative course. She had excellent take of her skin graft, with 10 cm vaginal length and adequate vaginal caliber. Vaginoplasty with a split-thickness skin graft is an excellent surgical option for vaginal canal creation in patients with vaginal agenesis.

The purpose of this study was to explore the long-term follow-up of treatment of congenital partial vaginal agenesis using the Wharton-Sheares-George technique. The technique was performed on 52 patients with congenital partial vaginal atresia from January 2009 to December 2017. As a result, the mean operating time of the Wharton-Sheares-George technique procedure was 25.6 ± 2.2 min. The mean estimated blood loss was 16.7 ± 4.7 mL. The average length of stay in hospital for the patients was 2.3 ± 0.2 days. There were no intraoperative and postoperative complications. The median duration of follow-up was 58 months. All of the patients experienced a resumption of menses. The mean vaginal length at 3 months was 7.3 ± 0.8 cm. Vaginal stenosis and adhesion did not occur in any cases during the follow-up. Twenty patients had sexual activities and the mean value of the FSFI questionnaire was 31.2 ± 4.0. Six cases became pregnant. There were four cases who each delivered one child and two cases who both delivered two children.IMPACT STATEMENT

Vaginal dilator therapy is used to increase vaginal length for vaginal agenesis, to increase vaginal width for vaginal narrowing, and to prevent or treat stenosis after vaginal surgery. Although it is an effective therapy, many reproductive health providers have had little training on how to guide patients through this therapy. The purpose of this review is to educate providers on how to assess patient readiness and how to support patients through the process of vaginal dilation.

We present two cases of congenital vaginal agenesis with functional uterine corpus, manifesting with periodic lower abdominal pain and hematometra in adolescence. Both patients were successfully treated with the creation of neovagina and neocanal structures to discharge menstrual blood; this may also facilitate the preservation of fertility. Both cases were characterized by degrees of congenital vaginal agenesis, whether short or completely absent, with no communication between the uterine cavity and external genitalia, as confirmed by physical examination and imaging. We surgically reconstructed a neovagina with the modified McIndoe's procedure, using an artificial skin graft, and canalized to the caudal portion of the uterine cavity. Although redilatation of the neocanal was required, no patient suffered severe infection in postoperative course and both now exhibit regular menstruation. Although hysterectomy has classically been the preferred treatment for such cases, recent technical progression enables treatment of such diseases with conservative and minimally invasive surgery, in a safe manner.

STUDY QUESTION Do sexual functioning, sexual esteem, genital self-image and psychological and relational functioning in women with Mayer–Rokitansky–Küster–Hauser (MRKH) syndrome differ from a comparison group of women without the condition? SUMMARY ANSWER In comparison to controls, women with MRKH with a non-surgically or surgically created neovagina did not differ in psychological and relational functioning but reported lower sexual esteem and more negative genital self-image, intercourse-related pain, clinically relevant sexual distress and sexual dysfunction, with sexual esteem levels strongly associated with sexual distress and sexual dysfunction. WHAT IS KNOWN ALREADY Studies on sexual functioning measured with standardized questionnaires in women with MRKH syndrome compared with women without the condition have yielded contradictory results. Factors associated with sexual functioning in this patient population have rarely been investigated. STUDY DESIGN, SIZE, DURATION Between November 2015 and May 2017, 54 women with MRKH syndrome with a neovagina and 79 age-matched healthy women without the condition were enrolled in this case–control study. PARTICIPANTS/MATERIALS, SETTING, METHODS All participants had to be at least 18-years old and had to live in a steady heterosexual relationship. Women with MRKH syndrome were asked to participate by their (former) gynecologists at three university hospitals and by MRKH peer support group. Controls were recruited via advertisement in local newspapers and social media. Standardized questionnaires were administered to assess sexual functioning, sexual esteem, genital self-image and psychological and relational functioning. MAIN RESULTS AND THE ROLE OF CHANCE Women with MRKH syndrome with a surgically or non-surgically created neovagina reported significantly more pain during intercourse (P LIMITATIONS, REASONS FOR CAUTION Given the nature of the study focusing on sexual functioning, a potential selection bias cannot be excluded. It is possible that those women with the most severe sexual and/or psychological disturbances did or did not choose to participate in our study. WIDER IMPLICATIONS OF THE FINDINGS The study results add new data to the very limited knowledge about psychosexual functioning of women with MRKH syndrome and are of importance for more adequate counseling and treatment of these women. STUDY FUNDING/COMPETING INTEREST(S) The research was financially supported by the Dutch Scientific Society of Sexology (Nederlandse wetenschappelijke Vereniging Voor Seksuologie). The funding was unrestricted, and there was no involvement in the conduct of the research. There are no conflicts of interest to declare.

Background: Mullerian anomalies are of many types, and it is very difficult to classify them in a simple method and plan reconstructive surgery. The aims, objective and the reconstructive surgical approach varies according to the level of vaginal agenesis as seen in our experience of management of 11 cases. The difficulties in the perception and interpretation of the surgical anatomy on magnetic resonance imaging can be minimized by placing a soft vaginal mold inside the lower developed vaginal segment in cases with transverse vaginal septum. Methods: Retrospective observational study. Results: All the operated 10 adult patients showed good created vaginal space which helped in creating good bonding in between the couples to maintain the integrity of marriage. Better successful reconstructive surgical planning by placing a soft vaginal mould in the vagina during MRI scan helps in understanding the level of septum in our single operated case of transverse vaginal septum and given excellent postoperative result. Conclusion: The impact of surgery on family life and sexual profile of the patient postsurgery on long-term has been mentioned on rare occasion. The preoperative counseling of the couples helps in better postoperative outcome in terms of psychological and sexual satisfaction of the partners and in the treatment of primary amenorrhea and infertility.

Mayer-Rokitansky-Kuster-Hauser (MRKH) occurring in association with an anorectal malformation (ARM) is rare but well described (Wester et al., 2012) [1]. In some patients with MRKH and ARM, a rectovestibular fistula may be left in situ as a neovagina (Levitt et al., 1998; Ein and Stephens, 1971) [2,3]. Previously, adenocarcinomas arising in vaginal reconstructions have been reported, however neuroendocrine tumors (NET) have not been previously described in this setting (Steiner et al., 2002; Schober, 2007) [4,5]. We describe the case of a patient with MRKH associated with ARM that developed a neuroendocrine tumor of the neovagina constructed in infancy from a rectrovestibular fistula. She was immunosuppressed following a living related kidney transplant at age 11. At age 20, vaginal bleeding was noted. A 1.5 cm neuroendocrine tumor was found on the anterior vaginal wall and she underwent a complete full-thickness transvaginal resection. The management considerations for neuroendocrine tumors of the neovagina are discussed.

Introduction: Vaginal agenesis is a congenital disorder, which can be managed by nonsurgical dilation or surgical reconstruction of the vagina. The sigmoid vaginoplasty procedure is a popular approach, which pulls down part of the sigmoid colon to form a neovagina. One complication of this procedure is introital stenosis. Patient concerns: A 55-year-old woman presented to the outpatient general surgery department with severe, persistent abdominal pain. The patient was diagnosed with congenital absence of uterus and vagina, and a sigmoid vaginoplasty was performed 34 years ago. Diagnosis: A pelvic MRI and an abdominal enhanced CT scan were performed, finding that the uterus was absent, and the os of the vagina was closed, forming a closed loop full of fluid. Introital atresia and closed loop of neovaginal colon conduit were diagnosed. Interventions: Based on our conclusions and the patient's consent we surgically removed the neovagina. Outcomes: After surgery, the abdominal pain was relieved, and the patient reported full recovery during a 6-month follow-up appointment. Conclusion: Introital stenosis is one of the long-term complications of sigmoid vaginoplasty procedure. Introital stenosis, leading to introital atresia, is rare but may occur. Surgical removal of neovagina can relieve the pain in patients who do not have the demand of sexual intercourse.

Anorectal malformation with rectovestibular fistula associated with vaginal agenesis is rare. We report on a child in whom this combination was diagnosed at the age of 1 year. After creation of a divided descending colostomy, we chose to leave the rectum-rectovestibular fistula to function as a neovagina, while the sigmoid colon was relocated via modified posterior sagittal anorectoplasty. The colostomy was closed 6 months after the main surgery. After a follow-up of 3 years, the patient is continent for stool and urine. The introitus of the neovagina appears wide and easy to explore. We conclude that our surgical approach may be a good option for these children.

Background: Vaginal agenesis is one form of abnormality found in the uroginecology with a prevalence of 1: 4000 births. Various reconstruction techniques, both non-surgical and surgical, have been introduced, one of which is sigmoid vaginoplasty. Research on the complications and outcomes of sigmoid vaginoplasty procedure is still limited. Objective: To assess surgical complications, sexual and menstrual function outcomes of sigmoid vaginoplasty procedure. Method: This is a prospective study. A total 11 patients with a variety of genital tract malformations have been performed for sigmoid vaginoplasty during January 2017 to January 2019. Data are described descriptively-analytically. All patients were assessed for surgical complications, menstrual and sexual function after surgery. Result s and Discussion : A total of 10 cases (90.9%) were diagnosed with vaginal agenesis and 1 case (9.1%) was diagnosed with Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome. Durante surgery complications are severe bleeding and rectum injury, occurred in 2 cases (18.2%). Postoperative complication is surgical wound dehiscence, occurred in 2 cases (18.2%). All patients who have uterus (100%) showed good menstrual function outcomes. Of the 3 married patients, all patients (100%) showed good postoperative sexual function outcomes (FSFI score 27-30.4). Conclusion: The sigmoid vaginoplasty procedure is an effective procedure for patients with agenesis vaginal. This procedure has low surgical complications with good outcomes of menstrual and sexual function. Keywords: Sigmoid vaginoplasty; vaginal agenesis; menstrual function; sexual function.

For women with congenital vaginal agenesis and sexual needs, creating a functioning neovagina is a practical therapeutic schedule. Colpopoiesis using the sigmoid intestine were more reported in the literatures. Though the incidence of neovaginal prolapse (NP) is relatively low (3-8%), it does bring great inconvenience to patients, and expose gynecologists to tough challenges. Hereon, we report a case of NP 10 years after sigmoid colon vaginoplasty that was successfully repaired by laparoscopic sacro-colpopexy with a mesh, hoping to provide meaningful evidence for this rare situation.

An observational, retrospective study was completed to compare the results of the PACIENA clinical trial (using the modified McIndoe technique) with a historical control group of nine patients who were operated on at San Juan University Hospital (1992&ndash, 2015) using the classic technique. The PACIENA clinical trial included seven patients with vaginal agenesis who were operated on at two reference sites (May 2017&ndash, May 2018) using a neovaginal polylactic acid (PLA) prosthesis (PACIENA&reg, prosthesis) and avoiding the use of a skin graft. The results illustrate a reduction in the length of surgery, 86.43 &plusmn, 4.75 min in the group with no skin graft compared to 155.56 &plusmn, 28.44 in the control group (p &lt, 0.05), and reduction in the length of hospitalization time. Differences were also registered in the length of the neovagina, the average being 8.93 &plusmn, 1.42 cm for cases and 6.56 &plusmn, 1.13 cm for controls, with no differences in neovaginal epithelialization times or in the satisfaction of sexual relations occurring between groups. The modification of the classical McIndoe technique using the neovaginal PACIENA&reg, prosthesis appears to be successful, obtaining good clinical results with shorter surgery and hospitalization times.

An observational, retrospective study was completed to compare the results of the PACIENA clinical trial (using the modified McIndoe technique) with a historical control group of nine patients who were operated on at San Juan University Hospital (1992–2015) using the classic technique. The PACIENA clinical trial included seven patients with vaginal agenesis who were operated on at two reference sites (May 2017–May 2018) using a neovaginal polylactic acid (PLA) prosthesis (PACIENA® prosthesis) and avoiding the use of a skin graft. The results illustrate a reduction in the length of surgery, 86.43 ± 4.75 min in the group with no skin graft compared to 155.56 ± 28.44 in the control group (p < 0.05); and reduction in the length of hospitalization time. Differences were also registered in the length of the neovagina, the average being 8.93 ± 1.42 cm for cases and 6.56 ± 1.13 cm for controls, with no differences in neovaginal epithelialization times or in the satisfaction of sexual relations occurring between groups. The modification of the classical McIndoe technique using the neovaginal PACIENA® prosthesis appears to be successful, obtaining good clinical results with shorter surgery and hospitalization times.

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.

Background Vaginal agenesis, a rare condition, is treated by various surgical techniques to achieve neovaginal reconstruction. The main difference between the approaches lies in the graft material used to cover the newly formed cavity. Objectives The purpose of this retrospective study was to describe the surgical procedure and outcomes of autologous buccal mucosal grafting in neovaginal reconstruction. Methods Sixteen patients with vaginal agenesis admitted to our department between January 2016 and January 2019 were included in our study. A reconstruction procedure, described in detail here, involving autologous buccal mucosa as graft material was successfully conducted in all of the patients. Long-term anatomic and functional outcomes were evaluated. Results The blood loss during operation was estimated to be 15 to 20 mL in all cases. No rectal or bladder injury occurred. The buccal mucosal wound completely healed 10 to 14 days after the operation. All patients had a well-formed neovagina 8 to 10 cm in length, with a mean diameter of >3 finger-breadths. Conclusions The application of autologous buccal mucosa in neovaginal construction is a simple procedure. Autologous buccal mucosa is an ideal material to achieve excellent cosmetic and functional results in patients with vaginal agenesis. Level of Evidence: 4

Mayer–Rokitansky–Küster–Hauser syndrome causes absence or underdevelopment of uterus and vagina, but women’s subjective experience remains understudied. This systematic review was conducted to examine the psychological and health-related quality-of-life outcomes of Mayer–Rokitansky–Küster–Hauser syndrome. In total, 22 articles identified through electronic search matched the inclusion criteria and were included in our review. Mayer–Rokitansky–Küster–Hauser syndrome may be associated with psychological symptoms and impaired quality of life, but especially with poor sexual esteem and genital image. Women may experience difficulties managing intimacy and disclosing to partners. Mothers may be perceived as overinvolved, with consequent negative emotions in women with the disease.

The Mayer-Rokitansky-Kuster-Hauser syndrome (MRKH) constitutes the second most common cause of primary amenorrhea, after gonadal dysgenesis. It is a congenital absence of the vagina with variable uterine development, which is a result of Mullerian duct agenesis or hypoplasia. The incidence for vaginal agenesis is 1 in 5000 (range 1 per 4000 to 10,000 females). We describe two cases of women diagnosed with MRKH syndrome who underwent laparoscopic Davydov-Moore vaginoplasty. Our video demonstrates the succesful operation which is composed of dissection of the rectovesical space, abdominal mobilization of the peritoneum to create the vaginal fornices, and attachment of the peritoneum to the introitus. Treatment of this syndrome should be multidisciplinary and individualized to the patient. It includes a combination of psychosocial support and correction of the anatomic abnormality.

An observational, retrospective study was completed to compare the results of the PACIENA clinical trial (using the modified McIndoe technique) with a historical control group of nine patients who were operated on at San Juan University Hospital (1992-2015) using the classic technique. The PACIENA clinical trial included seven patients with vaginal agenesis who were operated on at two reference sites (May 2017-May 2018) using a neovaginal polylactic acid (PLA) prosthesis (PACIENA(R) prosthesis) and avoiding the use of a skin graft. The results illustrate a reduction in the length of surgery, 86.43 +/- 4.75 min in the group with no skin graft compared to 155.56 +/- 28.44 in the control group (p < 0.05); and reduction in the length of hospitalization time. Differences were also registered in the length of the neovagina, the average being 8.93 +/- 1.42 cm for cases and 6.56 +/- 1.13 cm for controls, with no differences in neovaginal epithelialization times or in the satisfaction of sexual relations occurring between groups. The modification of the classical McIndoe technique using the neovaginal PACIENA(R) prosthesis appears to be successful, obtaining good clinical results with shorter surgery and hospitalization times.

Neovagina surgery in patients with vaginal agenesis is rare. No consensus exists regarding the best surgical technique. The aims of the current study were to show a new Thiel-embalmed cadaveric model to teach the surgical steps for different techniques of neovagina surgery and to evaluate opinions of this surgical teaching procedure. Four techniques&mdash, modified McIndoe, Vecchietti, Davydov, and vulvoperineal pediculated flaps&mdash, were recorded using an external camera and/or laparoscopic vision during their execution in a dissection room on &ldquo, feminized&rdquo, male cadavers. To determine the opinion of this teaching model, we designed an anonymous online survey that was available to participants via a computer application. After watching the video, more than 92% of participants agreed that feminized male cadavers were an excellent procedure for teaching these surgical techniques. Before watching this video, the most employed techniques were the McIndoe and Vecchietti procedures. After watching the video, modified McIndoe and vulvoperineal flaps were preferred by participants because they were considered to be easier to perform. It was considered that this model was useful for training neovagina techniques and, moreover, it should be recommended before techniques were performed on a real patient. Further investigation is needed to validate this model.

Background To evaluate the feasibility and clinical outcomes of vaginoplasties using a neovaginal polylactic acid prosthesis made with 3-dimensional (3D) printing technology as an intraneovaginal mould. Methods This was an interventionist, prospective, and multicentre clinical pilot investigation of a sanitary product (PACIENA prosthesis®) aiming to recruit and operate on 8 patients over 6 months with a follow-up period of 6 months. Only six patients with Rokitansky syndrome and one patient with Morris syndrome (7 patients in total) were operated on in two university hospitals: “La Fe”, Valencia (H1) and “Arrixaca”, Murcia (H2). Interventions: Extensive surgical dissection of a defined space between the urethra and bladder in the front and of the rectum in the back as well as insertion of the PACIENA prosthesis® covered with Interceed® were performed. After 12 days, the prosthesis was changed to the silicone-covered version for daily application. Results In the 6 patients with Rokitansky syndrome (86%), the primary endpoint (satisfactory vaginal outcome in terms of appearance, function, and sensation without relevant additional morbidity) was achieved, although only 2 patients (28%) were sexually active at the end of 6 months of follow-up. The patient with Morris syndrome withdrew from the study after 1 month. Patients without bacterial colonization showed positive Schiller tests at 1 month, and subsequent biopsies showed adequate keratinization and epidermization. Epithelization and iodopositivity were delayed in the patients who developed inflammatory granulomas. Conclusions Good anatomical and functional results can be achieved with the PACIENA prosthesis® for vaginoplasties without skin grafts. However, adequate patient selection and education, good surgical techniques and haemostasis, postoperative support, and prevention of bacterial colonization are important. Trial registration This clinical study was approved by the Ethical Clinical Investigation Committee of San Juan University Hospital on September 27, 2016, to be conducted in the participating centres; it was authorized by the Spanish Agency of Medicines and Health Products (AEMPS) on April 24, 2017 (exp. no. 585/16/EC), to be carried out in that hospitals.

Vaginal dilation is first line therapy for vaginal agenesis. No major urologic complications have even been described. We present the management and successful outcome of immediate repair for urethral trauma in a patient with history of congenital anomalies managed with vaginal dilation. Proper exposure is difficult, but urologic repair can be achieved with or without concomitant vaginal repair. Keywords: Vaginal dilation, Vaginal agenesis, Urethrovaginal fistula, Urethral disruption

The treatment of vaginal aplasia in patients suffering from MRKH syndrome is primarily based the Frank method: lengthening of the vaginal cupule using vaginal dilators. The efficiency of that treatment has been proven but the duration required to obtain a satisfying result is poorly documented. We have realized a retrospective and observational mono-centered study to define the required duration of treatment. The median duration of treatment for the 39 patients treated using the Frank method was 168 days or 5.5 months (ranging from 1.6 to 58.8 months). The frequency of the dilation sessions appears to be one of the decisive technical factors (ideally 3 sessions per day). Obtaining a sufficient compliance of the patients is an important obstacle, which requires adequate information, a consistent follow-up and a psychological care.; La prise en charge de l’aplasie vaginale chez les patientes atteintes du syndrome de MRKH repose en première intention sur la méthode de Frank : allongement de la cupule vaginale par l’utilisation de bougies vaginales. L’efficacité de ce traitement a été montrée mais la durée nécessaire à l’obtention d’un résultat satisfaisant est peu documentée. Nous avons réalisé une étude mono centrique rétrospective et observationnelle visant à définir la durée de traitement nécessaire. La durée médiane de traitement pour les 39 patientes traitées par méthode de Frank était de 168 jours soit 5,5 mois (1,6 mois à 58,6 mois). Un des facteurs techniques déterminants semble être la fréquence des séances de dilatation (idéalement 3 par jour). Une barrière importante est représentée par l’obtention d’une bonne adhésion des patientes au traitement par une information suffisante, un suivi rapproché et une prise en charge psychologique.

La agenesia vaginal es una entidad poco frecuente que puede presentarse en forma aislada o acompañada de otras anomalías congénitas. El desconocimiento de esta asociación puede retardar su diagnóstico, causar complicaciones indeseadas y comprometer el resultado del tratamiento. Se describen tres casos clínicos con agenesia vaginal y malformación anorrectal, cuyos diagnósticos no fueron realizados en el momento del nacimiento. Aunque la coexistencia de anomalías vaginales y anorectales sean poco frecuentes, los médicos responsables de su tratamiento deben ser conscientes de su existencia y practicar examen perineal cuidadoso.

Congenital pouch colon is a rare anorectal malformation of unknown embryogenesis in which all or part of the large intestine is replaced by a pouch-like dilatation that usually communicates with the urogenital tract via a fistula. This condition is more common among males in North Indian population and is associated with various anomalies such as uterovaginal malformations. It has been classified into five types that are showing a shift over the years towards less severe types such as type 4. We report a case of type 4 congenital pouch colon with anal agenesis without fistula and lower vaginal agenesis in a 29-week-old female fetus diagnosed during autopsy. The absence of a fistula and association with vaginal agenesis in a female fetus, which gets overlooked even in live births, makes it a unique case. Early prenatal diagnosis and awareness about this condition are essential for appropriate management and favorable surgical outcome.