Müllerian Anomalies: Presentation, Diagnosis, and Counseling.

Müllerian anomalies represent a complex collection of developmental defects occurring in up to 5% of the general population. They are increasingly more common in individuals with infertility (8.0%) and in those with a history of pregnancy loss (13.3%); they have the highest prevalence in individuals with a history of both (24.5%). A wide spectrum of anomalies can occur based on the stage at which müllerian development ceases in utero, ranging from mild (eg, a partial uterine septum) to severe, with complete absence of the cervix, uterus, and fallopian tubes (eg, müllerian agenesis). The components of the reproductive tract involved and, importantly, whether an obstruction of the tract is involved correlates with the timing of presentation, the constellation of associated symptoms, and the necessity for either medical or surgical management. Individuals, regardless of the severity of the defect, should be counseled on the gynecologic, reproductive, and obstetric risks associated with their specific müllerian anomaly to minimize adverse sequela and outcomes. We will review the clinical presentation, diagnostic evaluation, and clinical counseling of individuals with müllerian anomalies.

"Can't Touch This!": Laparoscopic management of an obstructed uterus didelphys before and after treatment for pelvic inflammatory disease.

OBJECTIVE: To describe the laparoscopic management of an obstructed uterus didelphys before and after treatment for pelvic inflammatory disease. To compare the appearance of pelvic organs during active infection with their appearance after washout and appropriate antibiotic treatment, emphasizing the importance of knowing when to abort a procedure. DESIGN: Video demonstration of surgical and medical management considerations during a complex pelvic surgery. Visualization of tissue healing that occurs with appropriate antibiotic treatment. SETTING: Academic Center. PATIENT: A patient who presents for definitive surgical management of a uterus didelphys with an obstruction at her right hemicervix. Her presentation is complicated by a tubo-ovarian abscess. INTERVENTION: A uterus didelphys is classically defined as two hemiuteri with duplicated cervices with or without a longitudinal vaginal septum. Uterus didelphys may have an obstruction and/or communication between the two uterine horns, in which case patients may present with complications such as cyclic pelvic pain from hematometra or genital tract infection. This is a case report of a 14-year-old G0 who presented to the emergency department with two weeks of vaginal bleeding, severe diffuse abdominal pain, and malodorous vaginal discharge. Transabdominal ultrasound and a magnetic resonance imaging of the pelvis established a new diagnosis of a uterus didelphys with an obstruction at her right hemicervix and a fistulous tract connecting her right and left hemiuteri at the level of the internal cervical os. She was also found to have a 3 cm left ovarian cyst and a new finding of congenital absence of her right kidney. Patient was administered ceftriaxone, doxycycline, and metronidazole antibiotics as treatment of presumed pelvic inflammatory disease but experienced minimal improvement after 24 hours. The decision was made to proceed with surgical intervention. A survey of the pelvis revealed significant inflammation, friable peritoneum, and endometriosis. The uterine horns in didelphic configurations were visualized. The fimbriae at the left fallopian tube were notably splayed out, swollen, and inflamed. There was a notable large mass in the location where the ovarian cyst had been previously described on imaging. A large amount of purulent material was expressed when compressed, consistent with a tubo-ovarian abscess. The infection likely originated from the menstrual blood collection at the right obstructed cervix that ascended through the communication between the right and left hemiuteri. The pelvis was irrigated thoroughly. At this point, the decision was made to stop the procedure, pursue antibiotic treatment, and resolve the active infection before correcting her complex müllerian anomaly. Patient continued on her antibiotic course, which included piperacillin-tazobactam, while hospitalized, followed by a five-day course of amoxicillin-clavulanate. She was also placed on medroxyprogesterone acetate for menstrual suppression. MAIN OUTCOME MEASURE: Advantage of allowing time for antibiotic treatment and tissue healing before repair of a complex müllerian anomaly. RESULT: With antibiotic treatment, she recovered well postoperatively with resolution of her pain. Three months later, she returned to the operating room for definitive surgical management of her obstructed uterine didelphys. On laparoscopy, there was a significant improvement in tissue quality. Most notably, the fimbriae of the left fallopian tube were no longer inflamed. We proceeded with the planned correction of the complex müllerian anomaly. After resection of the right uterine horn, the fistula tract was identified and also resected. The defect in the right hemicervix was closed over, reinforcing the medial side of the left hemicervix. She had an uncomplicated postoperative recovery, and menses resumed without pain. CONCLUSIONS: The presented case provides unique insight into the tissue healing that occurs before and after antibiotic treatment. Knowing when to stop, especially in the setting of an active infection, is extremely important for performing a procedure safely, minimizing harm, and allowing for robust tissue repair. It is also important to optimize modifiable preoperative factors before correcting a complex müllerian anomaly. Assessing and reassessing the situation during a complex pelvic surgery is essential, especially in the setting of a complex müllerian anomaly where the preoperative examination and imaging may not be definitive.

Reproductive surgery: revisiting its origins and role in the modern management of fertility.

For years, reproductive surgery was the mainstay of reproductive care. With the evolution and ultimate success of in vitro fertilization (IVF), reproductive surgery became an adjuvant therapy, indicated mainly for severe symptoms or to enhance success rates with assisted reproductive technologies. As success rates for IVF have plateaued, and emerging data rekindles the enormous benefits of surgically correcting reproductive pathologies, there is renewed interest among reproductive surgeons in reviving research and surgical expertise in this area. In addition, new instrumentation and surgical techniques to preserve fertility have gained traction and will solidify the need to have skilled reproductive endocrinology and infertility surgeons in our practice.

Data-driven commentary on SARS-CoV-2 infection, vaccination, and fertility.

A recent study by Wesselink et al. (Am J Epidemiol. 2022 Jan 20;kwac011. doi: 10.1093/aje/kwac011. Online ahead of print) adds to the growing body of research finding that vaccination for coronavirus disease 2019 (COVID-19) is safe for individuals either seeking pregnancy or who are pregnant. The study's authors found no effect of COVID-19 vaccination on fecundity in a population of individuals with no known infertility who were attempting conception. The finding reinforces the messaging of the American Society for Reproductive Medicine COVID-19 Task Force, the aim of which is to provide data-driven recommendations to individuals contemplating pregnancy in the face of the COVID-19 pandemic. As safe and effective COVID-19 vaccines became available, and with an increasing number of studies showing a heightened risk of severe disease during pregnancy, an important role of the Task Force is to encourage vaccination during the preconceptual window and in early pregnancy. The Task Force supports ongoing research to address gaps in knowledge about safe and effective therapies and preventive measures for individuals contemplating pregnancy and during pregnancy. Such research will help optimize care for reproductive-age individuals in the face of current and future health crises.

Data-Driven Commentary on SARS-CoV-2 Infection, Vaccination, and Fertility.

A recent study by Wesselink et al. (Am J Epidemiol. 2022;191(8):1383-1395) adds to the growing body of research finding that vaccination for coronavirus disease 2019 (COVID-19) is safe for individuals either seeking pregnancy or who are pregnant. The study's authors found no effect of COVID-19 vaccination on fecundity in a population of individuals with no known infertility who were attempting conception. The finding reinforces the messaging of the American Society for Reproductive Medicine COVID-19 Task Force, the aim of which is to provide data-driven recommendations to individuals contemplating pregnancy in the face of the COVID-19 pandemic. As safe and effective COVID-19 vaccines became available, and with an increasing number of studies showing a heightened risk of severe disease during pregnancy, an important role of the Task Force is to encourage vaccination during the preconceptual window and in early pregnancy. The Task Force supports ongoing research to address gaps in knowledge about safe and effective therapies and preventive measures for individuals contemplating pregnancy and during pregnancy. Such research will help optimize care for reproductive-age individuals in the face of current and future health crises.

A framework approach for hysteroscopic uterine septum incision: partial and complete.

OBJECTIVE: To demonstrate safe and efficient techniques for hysteroscopic partial and complete uterine septum incisions with radiofrequency electrosurgery. Review of these techniques may be particularly helpful for a surgical trainee or a less experienced hysteroscopic surgeon. DESIGN: Video instruction of the hysteroscopic uterine septum incision techniques. SETTING: Academic hospital setting. PATIENT(S): One patient with a partial uterine septum and 1 patient with a complete uterine septum and a duplicated cervix (2 distinct external cervices) (1). INTERVENTION(S): Hysteroscopic partial and complete uterine septum incisions with a 7-mm unipolar knife electrode. Importantly, the demonstrated techniques can be performed using any hysteroscopic cutting instrument with which the surgeon is comfortable. MAIN OUTCOME MEASURE(S): Surgical techniques that can be used to safely and efficiently incise a uterine septum and determine when the incision is complete. RESULT(S): For a partial uterine septum, surgical techniques include uterine septum shortening, uterine septum thinning, and measurement of the residual septum length with the operating instrument to determine when the incision is complete. Visualization of the tubal ostia should be used throughout the procedure to maintain a horizontal incision plane. For a complete uterine septum with a duplicated cervix, we additionally demonstrate how to make a window through the septum at the level of the internal os to incise the uterine body portion while preserving the tissue wall inferiorly that separates the duplicated cervices. CONCLUSION(S): Uterine septum incision is typically a short procedure that can be successfully performed with operative hysteroscopy. However, if a systematic approach is not followed, the surgeon can quickly and unknowingly become disoriented, resulting in inadvertent uterine perforation, incomplete septum incision, or excessive septum incision causing myometrial thinning, which has been shown to increase the risk of uterine rupture during pregnancy. In practice, the choice of technique used for septum incision should be made intraoperatively and will depend on the septum size and shape. Often, septum shortening, thinning, and residual measurement are best used in combination to achieve a successful result. Surgeons will find the use of these techniques helpful to maintain intraoperative orientation and provide a framework to guide adequate removal of either a partial or complete uterine septum.

Aqueous vaginal contrast and scheduled hematocolpos with magnetic resonance imaging to delineate complex müllerian anomalies.

OBJECTIVE: To demonstrate the advantage of using aqueous vaginal contrast and scheduled hematocolpos with magnetic resonance imaging (MRI) to improve the delineation of gynecologic anatomy and to recommend that this modality be considered in patients with complex müllerian anomalies. DESIGN: Video demonstration of MRI adjuncts to improve visualization of gynecologic anatomy. SETTING: Academic Hospital. PATIENT(S): A patient with obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) who presented for definitive surgical management. INTERVENTION(S): OHVIRA is a unilateral obstructed müllerian anomaly that presents typically after menarche with progressively worsening dysmenorrhea caused by progressive distension of the obstructed hemivagina and uterine horn. The definitive treatment for this anomaly is resection of the unilateral obstruction. When the obstructed hemivagina is within close proximity to the patent hemivagina, vaginal septum resection should be performed to relieve the obstruction successfully. However, when the obstructed hemivagina and uterine horn are not adjacent to the patent hemivagina, a simple septum resection is not feasible and there is a high rate of restenosis if anastomosis is attempted. In this case, laparoscopic removal of the obstructed uterine horn, fallopian tube, cervix, and vagina should be considered as an alternative approach to resolving the obstruction. A surgical approach can be recommended only once the surgeon has a clear understanding of the patient's pelvic anatomy and the magnitude of the obstruction. In the presented case, a 17-year-old patient with OHVIRA presented for definitive surgical management. While on hormonal suppression, a pelvic MRI was performed that identified a uterus didelphys with a left hemiuterus and cervix communicating with a patent vagina. The right hemiuterus and cervix were measured 2.5 cm from the patent vagina. However, because of hormonal suppression, the vaginal cavity was decompressed, making it very difficult to discern the relationship between the two uteri and vaginas. To better determine whether vaginal septum resection to relieve the obstruction was feasible, norethindrone was discontinued to allow menstrual blood to fill the obstructed hemivagina followed by a subsequent pelvic MRI with aqueous vaginal contrast to fill the patent vagina with contrast gel to improve the visualization of the decompressed vaginal cavities. MAIN OUTCOME MEASURE(S): Advantage of aqueous vaginal contrast and scheduled hematocolpos with MRI to image pelvic anatomy in a patient with a complex müllerian anomaly to guide surgical decision-making. RESULT(S): The addition of vaginal aqueous contrast clearly delineated the course and caliber of the patent vagina and its relationship to the obstructed hemivagina, now filled with blood. The inferior margin was in closer proximity to the patent vagina, but with only a very narrow segment (<1 cm) adjacent to the patent vagina and the obstructed cervix was displaced superiorly, now measuring 3.5 cm above the patent vagina. Surgical management options were discussed with the patient, and given the superior location of the obstructed uterus and cervix with only a narrow border of the vagina in continuity with the patent vagina, the risk of postoperative stenosis after vaginal septum resection was determined to be too high. The decision was made to proceed with a laparoscopic resection of the obstructed right side, and the patient underwent laparoscopic resection of the right hemiuterus, fallopian tube, cervix, and vagina. Intraoperatively, a survey of the pelvis again confirmed that the two vaginas were too far to reconnect safely without a high risk of stenosis. The patient recovered without complications postoperatively and her menses resumed without any pain. CONCLUSION(S): We highlight the use of two techniques to optimize MRI imaging of pelvic anatomy in a patient with a complex müllerian anomaly. First, the use of aqueous vaginal contrast with MRI is advantageous to clearly delineate the course and caliber of the patent vagina in patients with complex gynecologic anatomy. Second, cessation of hormonal suppression to allow menstruation to cause hematocolpos helped delineate the relationship between the obstructed vagina and patent vagina. In the presented case, these MRI adjuncts provided necessary detail that could not be appreciated with standard MRI to confirm that vaginal septum resection to preserve the right uterus would be too high a risk for postoperative stenosis in this patient. Aqueous vaginal contrast and scheduled hematocolpos should be considered as adjuncts to MRI when standard imaging modalities are unable to clearly describe the relationship between pelvic structures in cases of complex müllerian anomalies to help guide treatment recommendations.

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.

Incidence of first trimester pregnancy loss in the infertile population during the first wave of the coronavirus disease 2019 pandemic in New York City.

OBJECTIVE: To describe the incidence of first trimester clinical pregnancy loss in the infertile population during the first wave of the COVID-19 pandemic in New York City. DESIGN: Web-based cross-sectional survey. SETTING: New York City-based academic reproductive medicine practice. PATIENTS: A total of 305 infertile patients with a confirmed intrauterine pregnancy in their first trimester between December 1, 2019, and April 1, 2020, were matched by age and treatment type to pregnant patients from the year prior. INTERVENTIONS: None. MAIN OUTCOME MEASURES: First trimester clinical pregnancy loss rate. RESULTS: In total, the first trimester pregnancy loss rate was lower in the COVID-19 era cohort compared with that in the pre-COVID-19 era cohort (11.9% vs. 20.1%). There was no difference between cohorts in the pregnancy loss rate of women conceiving via fresh embryo transfer (19.6% vs. 24.4%) or via frozen embryo transfer with preimplantation genetic testing (5.4% vs. 9.5%,). In women conceiving via frozen embryo transfer without preimplantation genetic testing, the pregnancy loss rate was statistically lower in the COVID-19 group (12.5% vs. 24.5%). There was no difference in the pregnancy loss rate by treatment type when stratifying by COVID-19 testing or symptom status. Of the 40 (13.1%) patients with a pregnancy loss, there was no difference in self-reported COVID-19 symptoms or symptom type compared with results in those who did not experience a pregnancy loss. CONCLUSION: Despite patients expressing significant worry about COVID-19 and pregnancy, our data provides reassuring information that the first trimester pregnancy loss rate is not elevated for women conceiving via assisted reproductive technology during the global COVID-19 pandemic.

Uterine Isthmus Agenesis with Inverted Uterine Body and Malrotated Kidney.

A 21-year-old with a history of cyclic abdominal pain beginning at age 13 years and a previous diagnosis of a complex müllerian anomaly presented with abdominal pain and a finding of a right distended hemiuterus, left hematosalpinx, and cervix separate from the uterine body. After laparoscopic decompression for symptomatic relief at that time, she presented to our center for definitive management. After a diagnostic vaginoscopy and laparoscopy confirmed the diagnosis of uterine isthmus agenesis, an abdominal approach to uterocervical anastomosis was planned and undertaken. At the postsurgical clinical follow-up, the patient reported normal menses and resolution of pain, and imaging confirmed maintenance of anastomotic patency. The diagnosis and classification system of müllerian anomalies are complex, and few reports detail the management of rare müllerian anomalies. In this case report, the successful anastomosis of the uterus and the cervix is reviewed as an approach that can restore normal menses successfully and safely and potentially allow for future fertility in patients with uterine isthmus agenesis.

Unilateral Obstructed Müllerian Anomalies: A Series of Unusual Variants of Known Anomalies.

BACKGROUND: Obstructed Müllerian anomalies in adolescents can be grouped into complete outflow obstruction or unilateral outflow obstruction. The challenge with unilateral obstructions is that diagnosis can be delayed for weeks to years, as menstruation occurs normally through the patent side and thus obstruction is often not initially considered in the differential diagnosis. CASES: In this case series, we present 3 unusual and challenging cases of unilateral Müllerian obstructions in adolescent female patients, along with strategies for diagnosis and management. Each case involves a unique variation of a recognized Müllerian anomaly that was initially misdiagnosed, leading to a significant delay in definitive diagnosis and treatment. SUMMARY AND CONCLUSION: These cases highlight that even among the well-described Müllerian anomalies, there can be unusual variations. Patients who do not respond to initial management or who develop new symptoms should be further evaluated to confirm the correct diagnosis Tools that may be helpful in making the correct diagnosis include imaging studies that use contrast dye to better delineate cavities and their connections, magnetic resonance imaging with a radiologist experienced in Müllerian anomalies, and an examination under anesthesia. A definitive diagnosis is critical to the successful management of these conditions, and individualized management plans are required for each patient depending on their specific anomaly and their preferences for treatment.

Creation of a novel inflatable vaginal stent for McIndoe vaginoplasty.

OBJECTIVE: To describe how to create an inflatable vaginal stent for use in McIndoe vaginoplasty that can be constructed using standard operating room supplies. DESIGN: Step-by-step video instructions that demonstrate how to construct and use an inflatable vaginal stent. (This video article was exempt from institutional review board approval.) SETTING: Academic hospital. PATIENT(S): A woman presenting with vaginal agenesis requiring a McIndoe vaginoplasty for the creation of a neovagina. INTERVENTION(S): A novel inflatable vaginal stent compliant with operating room procedures that is radio-opaque, functional, and can be used for patients with or without a functional uterus. The device is modeled after the effective inflatable vaginal stent that was previously commercially available but is no longer produced. Although a vaginal stent may be created in the operating room by placing surgical sponges inside a sterilized condom, many operating rooms have restrictions on equipment that can be brought into the operating room and special criteria for how to sterilize this equipment, and there are also restrictions against leaving non-radio-opaque objects "inside" the patient. The novel inflatable vaginal stent we have developed has multiple advantages compared with a rigid dilator: it is deflatable, so it does not cause trauma or interrupt the delicate tissue graft during insertion, removal, or repositioning; it is firm enough to conform and circumferentially press the tissue graft against the dissected vaginal space but is soft enough to decrease the risk of pressure necrosis or damage to the urethra; and it has a drainage port to prevent the buildup of a fluid pocket that could interfere with graft adherence. Our stent incorporates all these unique properties and can be easily constructed using sterile operating room supplies. The construction of this device requires a silicone Foley catheter, sterile foam sponges from a vaginal prep kit, a sterile radio-opaque sponge, a sterile vaginal ultrasound probe cover, a long Kelly, a 60-cc catheter tip syringe, a ruler, scissors, 0-vicryl suture, and sterile gloves. MAIN OUTCOME MEASURE(S): Effectiveness of a self-made inflatable vaginal stent using standard operating room supplies that meets operating room protocol standards. RESULT(S): A standard dose of prophylactic antibiotics should be administered preoperatively to prevent surgical site infection. After placement of the tissue graft in the dissected neovagina cavity, the vaginal stent is placed and slowly inflated to circumferentially apply the tissue graft against the dissected vaginal space. The stent remains in place for 7 days and then should be removed in the operating room to allow for an examination under anesthesia, which provides the ideal setting to best evaluate the initial graft adherence. After careful inspection of the neovagina and tissue graft, a standard silicone vaginal mold is placed to maintain vaginal patency and prevent stricture of the tissue graft. The silicone mold should remain in place continuously until complete graft adherence occurs (approximately 4 to 6 weeks), and then it can be worn nightly until the patient is regularly sexually active. If at any point the patient discontinues nightly use of the silicone mold before regular sexual activity, vaginal stricture and a decrease in vaginal caliber will occur. CONCLUSION(S): Our novel inflatable vaginal stent is useful to surgeons performing a McIndoe vaginoplasty for vaginal agenesis with or without a uterus. It is compliant with operating room protocols and restrictions, as it is constructed from operating room supplies and is radio-opaque. Moreover, it is adjustable in size and effective in applying circumferential pressure for graft adherence. When used for segmental vaginal agenesis, the Foley catheter may be advanced through the cervix, then the balloon can be inflated, to stabilize the position of the stent during the first week postoperatively. The main limitation of this device is that it must be constructed by the surgeon, but the advantage of self-constructing the stent is that the size and shape can be tailored to conform to each individual patient. We prefer this inflatable vaginal stent to a rigid vaginal dilator in the first week of tissue healing to allow for easy insertion and removal of the stent without disrupting the tissue graft, to help prevent tissue necrosis, and to provide a fluid drainage port during graft adherence. We recommend this device as an ideal option for surgeons to consider when performing a McIndoe vaginoplasty.