Tubal replacement of cervix for treatment of type II vaginal atresia complicated with cervical dysplasia: a case report.

Vaginal atresia is a rare obstructive disease of the reproductive tract. It is characterized by the absence or underdevelopment of the vaginal canal and results in various clinical manifestations. Hysterectomy can physically and mentally burden young female patients with a congenital cervix and complete vaginal atresia. This report presents a case of type II vaginal atresia complicated by cervical dysplasia in a female patient >10 years of age. Our team opted to preserve the patient's uterus, innovated a fallopian tube transplantation technique, and performed cervicovaginal reconstruction using natural channels instead of the cervical canal. The patient experienced menarche within the first 2 weeks postoperatively, and follow-up at 6 months revealed no abnormalities.

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

INTRODUCTION AND HYPOTHESIS: Congenital 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. METHODS: The 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. RESULTS: Sixteen 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. CONCLUSION: In 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.

Cervical Septum Incision Adversely Impacts Clinical Outcomes in Women With Complete Uterine Septum and Duplicated Cervix.

STUDY OBJECTIVE: To investigate the reproductive outcomes of women with complete septate uterus and duplicated cervix who either did or did not receive cervical septum incision during hysteroscopic transcervical incision of the uterine septum. DESIGN: Retrospective study approved by the hospital ethics committee. SETTING: Obstetrics and Gynecology Hospital of Fudan University, Shanghai, China. PATIENTS: Women with complete septate uterus and duplicated cervix who underwent hysteroscopic transcervical incision of the uterine septum in Obstetrics and Gynecology Hospital of Fudan University between January 2008 and December 2020 (n = 105). INTERVENTIONS: Hysteroscopic incision of the septum. MEASUREMENTS AND MAIN RESULTS: Included patients were grouped according to whether or not cervical septum incision was performed. Reproductive outcomes including gravidity, abortion rate, preterm birth rate, full-term birth rate, premature rupture of membranes, and cervical incompetence were assessed. In the no incision group, the abortion rate (7.4%) was significantly lower than that of the incision group (27.6%, p = .01); the preterm birth rate (4.6%) was significantly lower than that of the incision group (36.8%); and the full-term birth rate (95.5%) exceeded that of the incision group (63.2%, p <.01). Incidence of premature rupture of membranes and cervical incompetence during pregnancy was higher in the incision group (15.8% and 10.5%, p <.01 and p = .03). CONCLUSION: Significantly improved reproductive outcomes were observed among patients with complete septate uterus and duplicated cervix whose cervical septum was preserved during the hysteroscopic transcervical incision of the uterine septum procedure.

Natural conception and term pregnancy after hysteroscopic incision of complete septate uterus with septate cervix: A case report and literature review.

OBJECTIVE: To discuss several techniques of hysteroscopic surgery for complete septate uterus. CASE REPORT: A 40-year-old female with unexplained primary infertility was diagnosed with complete septate uterus with septate cervix. Hysteroscopic incision of complete septate uterus was performed by using ballooning technique. The patient conceived naturally shortly after the operation and delivered a healthy, term infant. CONCLUSION: Hysteroscopic incision of complete septate uterus is a safe and prompt way of metroplasty. With the knowledge obtained from a pre-operative MRI, it can be completed without laparoscopy and the need for hospitalization.

[Concomitant extragenital malformations of female reproductive tract anomalies: analysis of 444 cases in Peking Union Medical College Hospital].

Objective: To analyze the incidence and clinical phenotype of the concomitant extragenital malformations in the patients with female reproductive tract anomalies. Methods: A retrospective study was conducted using clinical data of hospitalized patients diagnosed with uterine, cervical, or vaginal malformations from January 2003 to December 2022 in Peking Union Medical College Hospital. The malformations were classified according to American Society for Reproductive Medicine müllerian anomalies classification 2021, and in each type, the incidence and specific manifestations of concomitant extragnital malformations were analyzed. Results: A total of 444 patients were included. The overall incidence of concomitant extragenital malformations was 43.5% (193/444), including urinary system, skeletal system, and other system malformations. Renal malformations on the obstructed side were present in all patients with oblique vaginal septum syndrome (100.0%, 78/78). The total incidence of concomitant extragnital malformations was as high as 8/11 in uterus didelphys, 43.5% (10/23) in unicornuate uterus, 33.6% (79/235) in Mayer-Rokitansky-Küster-Hauser syndrome, 18.8% (6/32) in septate uterus and 18.5% (12/65) in cervical agenesis. Urinary system malformations (30.6%, 136/444) and skeletal system malformations (13.5%, 60/444) were the most common concomitant malformations in all types, in which, unilateral renal agenesis and scoliosis were the most common. Conclusions: Urinary and skeletal system malformations are important features of female reproductive tract anomalies. Urologic ultrasonography and spinal roentgenogram are recommended for all patients with female reproductive tract anomalies.

Retroperitoneal 'uterus-like mass' of cervical origin.

We report a rare case of a retroperitoneal uterus-like mass communicating with the endocervix, which presented as abdominal pain and bloating associated with severe irregular vaginal and postcoital bleeding. Our patient did not have any structural abnormalities of the urogenital system or otherwise, which makes a müllerian defect unlikely in our case. Based on the diagnostic criteria for the choristoma, that theory would be excluded here as the mass communicated with the endocervix. This strengthens the theory of metaplasia, under the effect of oestrogen and accelerated by the hyperoestrogenic state of pregnancy as the most likely postulate for our patient. Although the uterus-like mass is not commonly reported, it should be considered as a possible differential for pelvic masses.

Long-Term Follow-Up after Laparoscopic Uterovestibular Anastomosis in Patients with Cervical Atresia and Complete Absence of the Vagina.

BACKGROUND: The surgical treatment of girls with cervical atresia and complete absence of the vagina remains a problem because of the rarity of cases and the controversial study results. OBJECTIVE: To describe the surgical technique and long-term results of laparoscopically assisted uterovestibular anastomosis in patients with cervical atresia and complete absence of the vagina STUDY DESIGN: Sixteen consecutive patients with cervical atresia and complete absence of the vagina were conservatively treated with laparoscopically assisted uterovestibular anastomosis in 2 tertiary care referral centers. The follow-up assessments included clinical examination, determination of the presence and quality of sexual intercourse, and vaginoscopy. RESULTS: All patients underwent laparoscopically assisted uterovestibular anastomosis. No perioperative complications occurred. The mean follow-up period was 8 ± 3.2 years. In all patients, the length of the neovagina was greater than 4 cm at 1 year after the surgery and approximately 6 cm after 2 years. After the start of sexual intercourse, the neovagina exceeded 7 cm in length in 2 of the 11 sexually active patients. At 12 months after the surgery, iodine-positive epithelium was present in all patients and was maintained over time. The continuity of the neovagina, neocervix, and uterine body was maintained without further interventions in 15 of the 16 patients. During the follow-up, 11 patients were sexually active, 5 were married, 4 were seeking conception, and 2 had spontaneous pregnancy. CONCLUSIONS: Laparoscopically assisted uterovestibular anastomosis seems to be a safe and effective treatment for patients with cervical atresia and complete absence of the vagina, at least in terms of the recovery of menstrual function and sexual activity.

Utero-vaginal anastomosis in cases of cervical malformations: long-term follow-up and fertility challenges.

PURPOSE: To study the long-term results of utero-vaginal anastomosis in cases of cervical malformations. METHODS: This is a retrospective cohort study. Nine patients presented with cryptomenorrhea due to cervical malformations (5 patients with cervical agenesis and vaginal aplasia, 2 patients with cervical agenesis and upper vaginal aplasia, and two patients with cervical dysgenesis in form of cervical obstruction). Five patients had utero-vaginal anastomosis (UVA) with McIndoe vaginoplasty. Four patients had UVA without vaginoplasty. Follow-up was done by transabdominal and/or transvaginal ultrasound monthly for the first 3 months then every 6 months thereafter for a duration that ranged from 15 to 82 months. The main outcome measures are achieving menstruation, dysmenorrhea, pelvic inflammatory disease (PID), needed interventions after primary surgery, infertility, and pregnancy rate. RESULTS: Nine (100%) patients achieved menstruation, one (12%) experienced severe dysmenorrhea, two (22%) had PID, seven (78%) needed dilatation of the anastomosis site, three (33%) needed reoperation, nine (100%) had primary infertility, two (28.5%) achieved clinical pregnancy, and only one (14%) ended by live birth. CONCLUSION: Conservative surgery for cervical malformation is a promising choice for relieving the obstructive symptoms. Regular dilatation is recommended. Pregnancy is a remote hope that is hindered by many challenges.

Reconstructive surgery for congenital atresia of the uterine cervix: a systematic review.

The authors discuss the incidence, the embryological development, the classification, the presentation and the treatment options of this rare reproductive tract abnormality. In the past, the treatment proposed almost unanimously was hysterectomy and subsequent construction, when necessary, of a neovagina. In recent decades, numerous experiences of conservative therapies have accumulated that allow the restoration of menstrual function and in some rare cases even the achievement of a pregnancy. However, complications associated with recanalization of the cervix frequently resulted in the need for repeated surgery, risk of serious and sometimes fatal ascending infection. This review aims to analyze the most recent and significant experiences of conservative surgery in this field to provide an accurate picture of the various techniques and their outcomes, especially from the point of view of fertility. Conservative surgery should now be considered as the first-line treatment option. On the other hand, it is not possible to draw conclusions on the superiority of one technique over another among the various conservative options. This would require large series with adequate follow-up, which unfortunately are not available.

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

BACKGROUND: 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. CASE PRESENTATION AND REVIEW OF THE LITERATURE: 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 (≤ 2 cm 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. CONCLUSIONS: 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.

Conservative surgical repair in cervical atresia associated with partial or complete absence of the vagina.

OBJECTIVE: To describe the surgical technique of laparoscopically assisted uterovaginal/vestibular anastomosis in patients with cervical atresia associated with partial or complete absence of the vagina. DESIGN: Surgical video article. Local institutional review board approval and written permission from the patients were obtained. SETTING: Tertiary referral center. PATIENT(S): The surgical video presents surgical correction in 3 different patients with cervical agenesis. The first patient, aged 14 years, had a normoconformed uterus and total absence of the vagina. The second patient, aged 12 years, demonstrated a left unicornuate uterus and partial absence of the vagina. The third patient, aged 13 years, displayed a right unicornuate uterus and total absence of the vagina. INTERVENTION(S): Laparoscopic time and perineal time. During laparoscopy, the entire abdominopelvic cavity was assessed to evaluate the uterine morphology and size to exclude anomalies such as hematometra. The adnexa and adhesions were evaluated and any endometrial flare-ups were treated appropriately. A laparoscopic ultrasound probe was used to evaluate the size and location of the endometrial cavity. In cases with total absence of vaginas, an H-shaped incision in the hymenal dimple allowed a larger area of available tissue for the anastomosis. A tunnel was then created by blunt finger dissection between the bladder and rectum. Simultaneously, the uterus was pushed caudally by an assistant while the operator grasped it from below using an internal probe. A circular myometrial incision at the uterine caudal body allowed to reach the endometrial cavity and open it. The edges of the uterine cavity were then anastomized with the edges of the hymenal incision. In cases with partial absence of vaginas, the creation of the tunnel between the vagina and rectum was not necessary and the open uterus was anastomosed with the margins of the vaginal dome, engraved on the guide of a metal dilator. All patients received broad-spectrum antibiotics (i.e., cephalosporins of the last available generation) on the day before surgery and on the day of surgery. MAIN OUTCOME MEASURE(S): Intraoperative anatomic and ultrasound data, neovaginal length, and recovery of menstrual function 180 days after surgery. RESULT(S): The surgical procedure was successful in all cases. No major complications were recorded, and in particular, no bladder or rectal injuries occurred. No stenosis of the neocervix was recorded. The main hospital stay of the patients was 3.5 ± 1.5 days. In each case, the neovagina developed gradually over time after surgery because of the upward traction action exerted by the uterus through its natural ligament apparatus (cardinal ligaments and ovarian vessels). This fact eliminated the requirement for the use of a mold after surgery. At the 15-week follow-up, vaginoscopy was performed, with mucus observed at the site of uterovaginal anastomosis in all cases. None of the patients developed infection after surgery because of the avoidance of molds or pessaries and the natural mucus production. Six months after surgery, the length of the neovagina was >4 cm in all 3 cases. CONCLUSION(S): Laparoscopic-assisted uterovaginal/vestibular anastomosis may be considered the treatment of choice for patients with cervical atresia associated with partial or complete absence of the vagina.

The surgical strategies of vaginoplasty for vaginal agenesis patients with or without functional uterus.

BACKGROUND: Vaginal agenesis is a rare condition worldwide. Most reported cases were accompanied by the absence of uterus or uterine hypoplasia; for patients with functional endometrium, hysterectomy was most likely to be conducted to lower postoperative complications. OBJECTIVE: Based on our successful experience in vaginoplasty with autologous buccal mucosal, the purpose of this article is to discuss the surgical strategies in the reconstruction of neovaginal for vaginal agenesis patients with functional uterus and cervical hypoplasia. METHODS: The uterus was preserved in our procedure, and the cervicoplasty was performed to connect the uterine cavity with the neovagina. After the vaginal cavity was formed, the cervix was confirmed and fixed. With the assistance of laparoscope, the direction and angle of the cervix and the uterine body were observed and confirmed. An incision was made in cervix to connect the uterine cavity, and a Foley's catheter was inserted. The newly formed opening of cervix and neovagina was covered by autologous buccal mucosal. RESULTS: The connection between neovagina and cervix uteri was successfully conducted in patient with functional uterus. Unimpeded and regular menstrual was achieved, and the cyclic abdominal pain was disappeared. No complications were observed. CONCLUSION: For patients without functional uterus, vaginoplasty with autologous buccal mucosal can be conducted. However, fertility-preserving surgery should be the primary choice in patients with functional endometrium. It can be concluded from our experience that the utero-vaginal connection with the assistance of laparoscope and the use of autologous buccal mucosa is a promising way to achieve ideal outcomes.

Antibody microarray analysis of amniotic fluid proteomes in women with cervical insufficiency and short cervix, and their association with pregnancy latency length.

INTRODUCTION: This study aimed to investigate amniotic fluid (AF) proteins that were differentially expressed between patients with cervical insufficiency (CI) and asymptomatic short cervix (SCX, ≤ 25 mm), and whether these proteins could be predictive of spontaneous preterm birth (SPTB) in these patients. METHOD: This was a retrospective cohort study of 129 singleton pregnant women with CI (n = 80) or SCX (n = 49) at 17 to 26 weeks who underwent amniocentesis. An antibody microarray was used to perform comparative proteomic profiling of AF from matched CI (n = 20) and SCX (n = 20) pregnancies. In the total cohort, an ELISA validation study was performed for 15 candidate proteins of interest. Subgroup analyses of patients with CI and SCX were conducted to evaluate the association between the 15 proteins and SPTB at < 32 weeks of gestation. RESULTS: Eighty-six proteins showed intergroup differences. ELISA validation confirmed significantly higher levels of AF EN-RAGE, IL-8, lipocalin-2, MMP-9, S100A8/A9, thrombospondin-2, and TNFR2 in patients with CI than in those with SCX. Multivariable analysis showed that increased AF levels of EN-RAGE, S100A8/A9, and uPA were independently associated with SPTB at < 32 weeks in patients with CI; whereas in patients with SCX, high AF levels of APRIL, EN-RAGE, LBP, and TNFR2 were independently associated with SPTB at < 32 weeks. CONCLUSIONS: Multiple AF proteins show altered expression in patients with CI compared with SCX controls. Moreover, several novel mediators involved in inflammation were identified as potential biomarkers for predicting SPTB after the diagnosis of CI and SCX. These results provide new insights into target-specific molecules for targeted therapies to prevent SPTB in patients with CI/SCX.

Complete uterine septum involving cervix with unilateral external cervical os stenosis: diagnosis and management.

BACKGROUND: Congenital external cervical os stenosis is a form of Mullerian duct abnormality which is rare in virginal adolescents. CASE: Pre-operatively, two non-sexually active adolescents of 12 and 14 years of age, with history of light menses and severe lower abdominal pain for 2 and 4 months respectively, were diagnosed with an obstructed, oblique vaginal septum syndrome. However, at operative vaginoscopy, a single cervix with a duplicated cervical os and unilateral cervical stenosis was discovered, confirming the diagnosis of a complete uterine septum involving the cervix and unilateral cervical stenosis. They were treated by expanding the affected external cervical os while leaving the hymen intact. A year following the treatment, follow up results found no recurrence of symptoms. SUMMARY AND CONCLUSION: Operative vaginoscopy using a hysteroscope is crucial and recommended for the diagnosis and treatment of congenital external cervical os stenosis in virginal adolescents.

ASRM müllerian anomalies classification 2021.

There are many proposed classification systems for müllerian anomalies. The American Fertility Society (AFS) Classification from 1988 has been the most recognized and utilized. The advantages of this iconic classification include its simplicity, recognizability, and correlation with clinical pregnancy outcomes. However, the AFS classification has been criticized for its focus primarily on uterine anomalies, with exclusion of those of the vagina and cervix, its lack of clear diagnostic criteria, and its inability to classify complex aberrations. Despite this classification and others, the wide range of müllerian anomalies is still largely unknown and confusing to many providers. Consequently, müllerian anomalies may go undiagnosed for extended periods, receive inappropriate or inadequate surgical interventions, and result in persistent issues such as pain or loss of reproductive function. The American Society for Reproductive Medicine Task Force on Müllerian Anomalies Classification was formed and charged with designing a new classification. The Task Force set goals for a new classification and chose to base it on the iconic AFS classification from 1988 because of its simplicity and recognizability, while expanding and updating it to include all categories of anomalies. In addition, this was recognized as an opportunity to raise awareness of this area of medicine, educate providers and learners, and promote patient advocacy. Presented here is the new American Society for Reproductive Medicine Müllerian Anomalies Classification 2021.