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Abstract Müllerian anomalies are congenital conditions characterized by the incomplete development of the female reproductive tract. Women affected by Müllerian anomalies often display additional malformations of the renal, skeletal, and cardiovascular system, and are at a higher risk for infertility and adverse pregnancy outcomes. Several Müllerian anomalies have been reported in association with endometriosis, but it is unclear if all classes or anatomical variations are associated with the disease. Most importantly, both Müllerian anomalies and endometriosis can manifest with a wide degree of variability, adding further complexity to their poorly defined relationship. Retrograde menstruation occurring in obstructive Müllerian anomalies is a well-accepted mechanism for the development of endometriosis. However, endometriosis can occur following surgical correction of the anomaly or in the absence of obstruction. This suggests that other mechanisms may be involved, although the specific pathogenesis remains elusive. This review provides a comprehensive summary of the current state of clinical research on endometriosis in Müllerian anomalies. This review also highlights research and knowledge gaps, informing the development of future experimental designs to address current limitations including heterogeneity of phenotypes, variable comorbidities, and lack of genetic information.

Müllerian anomalies are congenital conditions characterized by the incomplete development of the female reproductive tract. Women affected by Müllerian anomalies often display additional malformations of the renal, skeletal, and cardiovascular system, and are at a higher risk for infertility and adverse pregnancy outcomes. Several Müllerian anomalies have been reported in association with endometriosis, but it is unclear if all classes or anatomical variations are associated with the disease. Most importantly, both Müllerian anomalies and endometriosis can manifest with a wide degree of variability, adding further complexity to their poorly defined relationship. Retrograde menstruation occurring in obstructive Müllerian anomalies is a well-accepted mechanism for the development of endometriosis. However, endometriosis can occur following surgical correction of the anomaly or in the absence of obstruction. This suggests that other mechanisms may be involved, although the specific pathogenesis remains elusive. This review provides a comprehensive summary of the current state of clinical research on endometriosis in Müllerian anomalies. This review also highlights research and knowledge gaps, informing the development of future experimental designs to address current limitations including heterogeneity of phenotypes, variable comorbidities, and lack of genetic information. [ABSTRACT FROM AUTHOR]

Background: Women with an aplastic uterus (ESHRE/ESGE classification) or Müllerian agenesis (ASRM MAC 2021) might present with functional uterine remnants. Our study aimed to report the clinical course of symptomatic uterine rudiments in adolescents and adults with Mayer–Rokitansky–Küster–Hauser syndrome (MRKHS). Methods: This study involved 20 patients with MRKHS who, between 2012 and 2023, underwent surgery for symptomatic uterine horns at the mean age of 25.2 years in the Division of Gynaecology, Gynaecological and Obstetric Clinical Hospital, Poznan University of Medical Sciences, Poland. The records of the patients were retrospectively analysed. Results: The volume of the uterine horns ranged from 0.8 to 58.3 cm3, and the volume of the endometrial cavity within the horns ranged from 0.03 to 12 cm3, with no significant difference between adolescents and adults (p = 0.36). In five patients (25%), MRKHS was identified during the diagnosis of recurrent pelvic pain at the age of 12.6–14.8 years. In 19 patients, uterine rudiments were removed: unilaterally in 3 patients (16%), bilaterally in 16 patients (84%), and laparoscopically in 89% of cases. In one patient, the horn was preserved (horno-neovaginal anastomosis). Histopathology confirmed the presence of the endometrium in uterine rudiments ipsilateral to the pain location in 75% of cases. Four patients (20%) were diagnosed with endometriosis. Conclusions: Recurrent pelvic pain in patients with MRKHS should prompt the diagnosis of functional uterine rudiments. The resection of symptomatic uterine horns can result in the complete resolution of pain. Patients with endometriosis are at risk of pain recurrence. In some patients strongly desirous of menstruation, horno-neovaginal anastomosis can be cautiously attempted. [ABSTRACT FROM AUTHOR]

Müllerian duct anomalies (MDAs) refer to defects arising during the embryological development of the female reproductive system. They are characterized by the failure of vertical fusion of the bilateral paramesonephric ducts with the urogenital sinus. Patients usually present with primary amenorrhea, infertility, recurrent miscarriages, and abdominal pain. Here, we discuss 9 patients who had presented to our tertiary care teaching institute with varying symptoms and were diagnosed with Müllerian anomalies and their management at our hospital. [ABSTRACT FROM AUTHOR]

Purpose: The present systematic review aimed to assess the fecundity of women with congenital uterine anomalies (CUAs) undergoing assisted reproductive technology (ART). Methods: The present systematic review of the literature was reported according to the PRISMA guidelines. We systematically searched PubMed, MEDLINE, Embase and Scopus, from database inception to 17th October 2023. Studies were deemed eligible only if they included women with CUAs clearly fitting into one of the categories of the ASRM Müllerian anomalies classification 2021. Results: Data relevant to the reproductive outcomes of women with CUAs who underwent ART were extracted from 55 studies. Regarding Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome, studies on gestational surrogacy reported a live birth rate (LBR) ranging from 37 to 54%. Uterus transplant, although still experimental, showed promising results. Most studies reported a negative impact of unicornuate uterus and partial or complete septate uterus on both the miscarriage rate (MR) and the live birth rate (LBR). The reproductive prognosis of women with unicornuate uterus was shown to be particularly poor in case of twin pregnancy. Uterus didelphys, bicornuate and arcuate uterus seem not to negatively impact the ART reproductive outcomes. Uterus didelphys was associated with an increased risk of preterm birth (PTB), cesarean section and low birth weight (LBW). Conclusion: Women with CUAs should be informed regarding the impact (if any) of their congenital anomaly on both the chances of success of ART and on pregnancy-related complications. Elective single embryo transfer (eSET) should always be the first choice in patients with an increased baseline obstetric risk. [ABSTRACT FROM AUTHOR]

Uterine rupture is a rare but serious complication that predominantly occurs in the third trimester of pregnancy. It is exceptionally uncommon in the second trimester, particularly in the presence of uterine anomalies such as a bicornuate uterus or uterus didelphys. This case report presents a significant instance of second-trimester uterine rupture associated with a bicornuate uterus, resulting in a life-threatening intra-abdominal hemorrhage of approximately 4000 mL. The case report details the clinical presentation, diagnostic challenges, and management strategies used in this case, highlighting the critical importance of prompt recognition and intervention in similar scenarios to improve maternal outcomes.

Objective: To present the comprehensive methodology for generating personalized three-dimensional (3D) printed uterine models from 3D ultrasound (US) volumes in individuals diagnosed with Müllerian anomalies and discuss potential applications in the field of reproductive endocrinology and infertility. Design: Pilot study. Setting: Single large university-affiliated teaching hospital. Patient(s): Patients with the presence of a Müllerian anomaly between the ages of 18 and 45 years attending the maternal-fetal medicine as well as reproductive endocrinology and infertility outpatient offices from 2018 to 2023 were included in the study. Intervention(s): Subjects underwent 3D US transvaginal scanning for the collection of data. The 3D US volumes were acquired, edited, and exported from a US cart Voluson E10 system (GE Healthcare, Chicago, IL). High-definition virtual models were created and modified, making them suitable for printing using Materialise 3-Matic Medical (Materialise NV, Leuven, Belgium). The models were printed on a J5 MediJet 3D printer (Stratasys, Rehovot, Israel). Colors were set to mimic a realistic appearance, and shore values were set before printing. Main Outcome Measure(s): Successful creation and utilization of personalized 3D-printed uterine models for individuals with Müllerian anomalies. Results(s): Three-dimensional models were created for a uterus without anomalies, 2 variations of a partial septum, a unicornuate, and a didelphys uterus. Models were used as a tactile and customized tool for patient education, counseling, and medical student and resident teaching. This technique illustrates that the creation of personalized 3D-printed uterine models for utilization in the fields of reproductive endocrinology and infertility is feasible. Conclusion(s): We propose a novel use of individualized 3D-printed uterine models in the evaluation of individuals with Müllerian anomalies. These models may play a complementary role to standard imaging options in the assessment of these anomalies, with a special potential for application in highly complex or yet-to-be-determined types of anomalies.

Background: Accessory and cavitated uterine masses (ACUMs) are rare congenital mullerian anomalies where isolated cavitated lesions are located in the lateral myometrium with no communication with the uterine cavity or fallopian tube. The diagnostic criteria for the diagnosis of ACUM includes: (1) isolated mass in lateral myometrium/broad ligament, (2) no communication with a fallopian tube or uterine cavity, (3) cavity has myometrial mantle surrounding the endometrium, (4) cavity lined by functional endometrium, (5) contains dark brown hemorrhagic fluid. The woman usually presents at a younger age with dysmenorrhea. Even though there is an assumption that ACUM can be asymptomatic, there is a scarcity of data. Case description: We present a case of ACUM which was mistaken for a rudimentary horn in a woman who was evaluated for new-onset menstrual irregularity. Conclusion: Knowledge and awareness of this entity will help us to consider preoperative diagnosis of ACUM and provide better treatment options to women. Clinical significance: All women diagnosed to have ACUM may not require surgical intervention if it is an incidental finding when evaluated for other conditions. [ABSTRACT FROM AUTHOR]

Objective: This study aimed to evaluate whether a trial of labor after cesarean delivery (TOLAC) in women with a bicornuate uterus is associated with increased maternal and neonatal morbidity compared to women with a non-malformed uterus. Methods: A multicenter retrospective cohort study was conducted at two university-affiliated centers between 2005 and 2021. Parturients with a bicornuate uterus who attempted TOLAC following a single low-segment transverse cesarean delivery (CD) were included and compared to those with a non-malformed uterus. Failed TOLAC rates and the rate of adverse maternal and neonatal outcomes were compared using both univariate and multivariate analyses. Results: Among 20,844 eligible births following CD, 125 (0.6%) were identified as having a bicornuate uterus. The overall successful vaginal delivery rate following CD in the bicornuate uterus group was 77.4%. Failed TOLAC rates were significantly higher in the bicornuate group (22.4% vs. 10.5%, p < 0.01). Uterine rupture rates did not differ between the groups, but rates of placental abruption and retained placenta were significantly higher among parturients with a bicornuate uterus (9.8% vs. 4.4%, p < 0.01, and 9.8% vs. 4.4%, p < 0.01, respectively). Neonatal outcomes following TOLAC were less favorable in the bicornuate group, particularly in terms of neonatal intensive care unit admission and neonatal sepsis. Multivariate analysis revealed an independent association between the bicornuate uterus and failed TOLAC. Conclusions: This study found that parturients with a bicornuate uterus who attempted TOLAC have a relatively high overall rate of vaginal birth after cesarean (VBAC). However, their chances of achieving VBAC are significantly lower compared to those with a non-malformed uterus. Obstetricians should be aware of these findings when providing consultation to patients. [ABSTRACT FROM AUTHOR]

STUDY QUESTION Does endometriosis prevalence differ in patients with obstructive Müllerian anomalies (OMA) versus those with nonobstructive Müllerian anomalies (NOMA), and in patients with NOMA versus those without Müllerian anomalies? SUMMARY ANSWER The quantitative synthesis of published data demonstrates a substantially increased prevalence of endometriosis in patients with OMA compared with those with NOMA, and a similar prevalence in patients with NOMA and those without Müllerian anomalies. WHAT IS KNOWN ALREADY The pathogenesis of endometriosis has not been definitively clarified yet. A higher prevalence of endometriosis in patients with OMA than in those with NOMA would support the retrograde menstruation (RM)/implantation theory, whereas a higher prevalence of endometriosis in the NOMA group than in the group without Müllerian anomalies would support the embryonic remnants/celomic metaplasia hypothesis. STUDY DESIGN, SIZE, DURATION This systematic review with meta-analysis was restricted to full-length, English-language articles published in peer-reviewed journals between 1980 and 2023. The PubMed and EMBASE databases were searched using the keyword 'endometriosis' in combination with 'Müllerian anomalies', 'obstructive Müllerian anomalies', 'female genital malformations', 'retrograde menstruation', 'infertility', 'pelvic pain', and 'classification'. References from relevant publications were screened, and PubMed's 'similar articles' and 'cited by' functions were used. PARTICIPANTS/MATERIALS, SETTING, METHODS Studies were selected if they reported the prevalence of surgically confirmed endometriosis in either individuals with OMA compared to those with NOMA, or patients with NOMA compared to those without Müllerian anomalies. Cohort and case-control studies and case series were deemed eligible for inclusion. Noncomparative studies, studies not reporting both the number of individuals with endometriosis and the total number of those with Müllerian anomalies or with other gynecological conditions, those including exclusively data on patients with absent or uncertain menstrual function (e.g. complete Müllerian agenesis category), or with imperforate hymen were excluded. Two reviewers independently abstracted data. The risk of bias was assessed with the Risk of Bias In Non-randomized Studies of Exposures tool. The overall certainty of the evidence was graded according to the Grading of Recommendations Assessment, Development and Evaluation (GRADE) guidelines. MAIN RESULTS AND THE ROLE OF CHANCE Seven retrospective studies were included. The overall mean estimate of endometriosis prevalence was 47% (95% CI, 36–58%) in patients with OMA, and 19% (95% CI, 15–24%) in patients with NOMA, with a common odds ratio (OR) of 4.72 (95% CI, 2.54–8.77). The overall mean estimate of endometriosis prevalence in patients with NOMA was 23% (95% CI, 20–27%), and that in patients without Müllerian anomalies was 21% (95% CI, 20–22%), with a common OR of 0.95 (95% CI, 0.57–1.58). The overall certainty of the evidence according to GRADE guidelines was judged as low for both comparisons. LIMITATIONS, REASON FOR CAUTION Some NOMA subtypes may create a partial obstacle to menstrual efflux and/or generate dysfunctional myometrial contractions that favor transtubal reflux, thus increasing the risk of endometriosis and limiting the difference between OMA and NOMA. As infertility and pelvic pain are strongly associated with endometriosis, women with these symptoms are inappropriate controls. Confounding by indication could explain the lack of difference in endometriosis prevalence between patients with NOMA and those without Müllerian anomalies. WIDER IMPLICATIONS OF THE FINDINGS The results of this meta-analysis support the validity of the RM theory but do not definitively rule out alternative hypotheses. Thus, RM may be considered the initiator for the development of endometriotic lesions, while not excluding the contribution of both inheritable and tissue-specific genetic and epigenetic modifications as disease-promoting factors. STUDY FUNDING/COMPETING INTEREST(S) No funding was received for this review. P.Ve. is a member of the Editorial Board of Human Reproduction Open , the Journal of Obstetrics and Gynaecology Canada , and the International Editorial Board of Acta Obstetricia et Gynecologica Scandinavica ; has received royalties from Wolters Kluwer for chapters on endometriosis management in the clinical decision support resource UpToDate; and maintains both a public and private gynecological practice. E.S. discloses payments from Ferring for research grants and honoraria from Merck-Serono for lectures. All other authors declare they have no conflict of interest. REGISTRATION NUMBER N/A. [ABSTRACT FROM AUTHOR]

There is wide variation in the language used to describe Mullerian structures. To standardize terminology, the American Society of Reproductive Medicine (ASRM) created the Mullerian Anomalies Classification (MAC) in 2021. The objective of this study was to evaluate the applicability of the MAC nomenclature to pediatric patients with cloaca. A retrospective review of all patients with cloaca was performed at a single institution. Descriptions of Mullerian structures were evaluated and compared to the ASRM MAC categories. Descriptive statistics were used to report findings. 36 patients with cloaca were identified, 13 (36%) of whom had congenital Mullerian structures that could not be adequately described by the MAC terminology. All 13 patients had two hemiuteri that were not connected in the midline and were not accurately described as uterus didelphys. Additionally, 5 of these 13 patients had reproductive anatomy that was connected by a fistula or ectopic connection to other pelvic structures. Despite the ASRM expansion of the Mullerian anomalies nomenclature, more than a third of our patients with cloaca could not have their Mullerian structures accurately described. Describing anatomy with accurate and consistent language can improve communication between healthcare providers and may allow patients and families to better anticipate fertility options. Retrospective. IV. [ABSTRACT FROM AUTHOR]

Agenesis of the dorsal pancreas is a rare congenital anomaly that often remains asymptomatic and is requently discovered incidentally during imaging for other conditions. Its association with Müllerian anomalies, such as uterus didelphys, suggests that both conditions may stem from similar embryological disruptions during early fetal development. This report aims to raise awareness of Agenesis of the Dorsal Pancreas (ADP) and its potential associations with Müllerian anomalies. By presenting a detailed case study, we seek to address the diagnostic challenges faced by healthcare professionals and the implications for patient management, emphasizing the importance of early detection and appropriate intervention. We present a case involving a 24-year-old Iranian female who presented with a 10-day history of abdominal pain and vomiting. Initial ultrasound imaging revealed moderate hydroureteronephrosis in the right kidney, prompting further investigation. A contrast-enhanced CT scan was performed, which not only confirmed ureteropelvic junction obstruction but also unexpectedly revealed dorsal pancreatic agenesis. In addition to these findings, the patient was diagnosed with uterus didelphys, highlighting a significant developmental anomaly. This case underscores the necessity for increased awareness of ADP and its associations with Müllerian anomalies among healthcare providers. Early and accurate diagnosis through advanced imaging modalities significantly improves patient outcomes and facilitates better management strategies for associated complications.

To report a rare, misleading fibroid degeneration involving a nonfunctional, noncommunicating horn in a woman with a unicornuate uterus. Although the presence of a functional rudimentary horn may lead to signs and symptoms that recommend its removal, nonfunctional cases are rarely reported, and because of their apparent functional inactivity, the need for their removal has not yet been reported. No previous report showed the possibility of a degenerative process in a nonfunctional rudimentary horn causing patient discomfort. This is a step-by-step narrated video showing a unique case of fibroid degeneration and growth of a nonfunctional, noncommunicating rudimentary horn in a unicornuate uterus (American Society for Reproductive Medicine classification 2021) and its surgical management. University academic hospital. A 48-year-old White nulliparous premenopausal woman was referred to our institution because of abdominal pain and an enlarging adnexal mass. Her personal history showed primary infertility with a previous diagnosis of unicornuate uterus. Given the possibility of ectopic ureters in these occurrences, complete computed tomography was performed, and no genitourinary alterations were found. Preoperative imaging (ultrasound evaluation, computed tomography, and magnetic resonance imaging) provided a provisional diagnosis of a suspicious ovarian fibroma. Considering the patient's age, lack of desire for pregnancy, and volumetric increase in the adnexal mass, a laparoscopic intervention to perform mass removal and prophylactic bilateral salpingectomy was planned. The patient was counseled about the low risk of an underlying malignant transformation. Therefore, the decision to remove the intact mass via a minilaparotomy at the end of the surgery was shared. Once the abdominal cavity was entered, the right unicornuate uterus was found in anatomical continuity with the ipsilateral broad ligament, fallopian tube, and ovary. These structures were wholly attached to the right pelvic wall. On the other side, cranially compared with the right hemiuterus, a roundish myoma-like mass was detected in direct connection with the left broad ligament, fallopian tube, and ovary. In light of a changed intraoperative finding, amputation of the left rudimentary horn and prophylactic bilateral salpingectomy were performed. Showing the fibroid degeneration and growth of a nonfunctional, noncommunicating rudimentary horn in a unicornuate uterus (American Society for Reproductive Medicine classification 2021) treated laparoscopically. Laparoscopic removal of the uterine horn was successful, and no intraoperative and postoperative complications occurred. The patient was in good health at the 6-month follow-up visit. The histopathological examination confirmed the fibroid degeneration and absence of the endometrium. The lack of symptomatic cases of rudimentary nonfunctional horns reported in the literature led gynecologists to consider them a silent Müllerian anomaly. This unique case demonstrates that even nonfunctional rudimentary horns can undergo symptomatic transformation processes requiring surgery. This information may be helpful for more comprehensive counseling of women and for considering the possibility of this occurrence in the diagnostic workup of misleading Müllerian anomalies. With this in mind, surgical treatment can also be better planned as the technical aspects change compared with what is expected for an adnexal pathology. [ABSTRACT FROM AUTHOR]

Uterus didelphys is a rare Müllerian anomaly, often diagnosed during menarche or in women with a personal history of infertility and/or recurrent pregnancy loss. Its association with other genitourinary anomalies is frequent and may determine the existence of established syndromes. This case report refers to a 13‐year‐old female patient diagnosed with OHVIRA syndrome (Obstructed Hemivagina with Ipsilateral Renal Agenesis), a condition wherein the presence of a didelphic uterus is associated with hemivagina obstruction and ipsilateral renal agenesis. The patient presented with cyclic pelvic pain, related to the presence of hematocolpos and hematometra, which persisted despite several surgical approaches, including vaginal septum excision and correction of cervical stenosis. The recurrence of the condition indicated exploratory laparotomy, revealing two hemi‐uteri and two uterine cervixes, with hematometra on the right. A subtotal hemihysterectomy was performed on the right. Post‐procedure, the patient developed with regular menstrual cycles and improvement of pelvic pain complaints. Given the limited prevalence and low index of suspicion, the potential requirement for surgical intervention and its potential impact on reproductive future, diagnosing and treating OHVIRA syndrome and other Müllerian anomalies poses notable challenges in clinical practice. Hence, sharing different therapeutic approaches of a rare diagnosis with the scientific community is of paramount importance to aid in early diagnosis and effective management of similar clinical cases. Synopsis: A case report of a patient with obstructed hemivagina with ipsilateral renal agenesis syndrome supports hemihysterectomy as a potential therapeutic approach in refractory cases. [ABSTRACT FROM AUTHOR]

Introduction: Accessory cavitated uterine malformation (ACUM) is a relatively recent term used to describe a noncommunicating, accessory uterine cavity. ACUM have been published under different terms ranging from juvenile cystic adenomyosis to "uterus‐like mass". The objective of this study was to systematically identify all cases of ACUM and definitions described in the literature, regardless of label, and identify morphological, epidemiological, and clinical characteristics as well as management, while also highlighting knowledge gaps. Material and methods: A systematic literature search of three databases was performed, reviewing all records of cystic myometrial lesions. Cases that fitted common definitions for ACUM were included and clinical and imaging characteristics were documented in detail. This work was registered to PROSPERO and reporting followed PRISMA guidelines for scoping reviews. Results: A total of 53 articles were included, comprising 115 cases that met the minimal criteria for ACUM. The median age at onset of symptoms was 17 years, presenting with dysmenorrhea soon after menarche. A total of 19 women were parous. On ultrasound, ACUM appears as unilocular myometrial cysts, usually with ground‐glass content. Hemorrhagic content is also observed on magnetic resonance imaging (MRI), with high signal intensity on both T2 and T1‐weighted images. Ninety‐five (83%) cases were managed surgically, with a trend towards primary nonsurgical options. Although no adverse outcomes were reported, long‐term follow‐up on subsequent fertility and pregnancy was rare. Conclusions: Despite its increasing recognition as a clinical entity, ACUM often remains underdiagnosed as it shares similarities with other myometrial masses. We propose a unified terminology and definition for ACUM based on the data in this review. ACUM presents as a cavitated lesion, surrounded by a myometrial mantle, in continuity with the anterolateral uterine wall and located beneath the insertion of the round ligament and the interstitial portion of the fallopian tube. In contrast to other uterine abnormalities, a normal uterine cavity is visualized. Future studies are needed, using a clear definition for ACUM, and prospectively investigating management strategies, including long‐term follow‐up of patient‐reported symptoms, fertility, and pregnancy outcomes. [ABSTRACT FROM AUTHOR]

Background: The prevalence of Müllerian anomalies (MA) among patients with congenital solitary functioning kidney (SFK) is not well defined. A delay in diagnosis of obstructive MA can increase the risk of poor clinical outcomes. This study describes the prevalence of MA in patients with congenital SFK. Methods: A retrospective review was performed of patients within the Nationwide Children's Hospital system with ICD9 or ICD10 diagnostic codes for congenital SFK defined as either unilateral renal agenesis (URA) or multicystic dysplastic kidney (MCDK) and confirmed by chart review. Patients with complex urogenital pathology were excluded. Renal anomaly, MA, reason for and type of pelvic evaluation, and age of diagnosis of anomalies were evaluated. Results: Congenital SFK occurred in 431 girls due to URA (209) or MCDK (222). Pelvic evaluation, most commonly by ultrasound for evaluation of abdominal pain or dysmenorrhea, occurred in 115 patients leading to MA diagnosis in 60 instances. Among 221 patients ages 10 years and older, 104 underwent pelvic evaluation and 52 were diagnosed with an MA of which 20 were obstructive. Isolated uterine or combined uterine and vaginal anomalies were the most common MA. MA were five-fold more common in patients with URA compared to MCDK. In 75% of patients, the SFK was diagnosed prior to the MA. Conclusions: The prevalence of MA in patients with congenital SFK was 24% among those age 10 years or older, and 38% were obstructive. This justifies routine screening pelvic ultrasound in girls with congenital SFK to improve early diagnosis. [ABSTRACT FROM AUTHOR]

In this article, we aim to highlight the importance of Müllerian duct anomalies by addressing concepts related to embryology, classification, symptomatology, and optimal imaging investigations, as well as associated renal malformations. Our study included 16 patients diagnosed at the “Sf. Maria” Emergency Clinical Hospital for Children in Iași, between June 2017 and June 2022, who received surgical treatment in the pediatric surgery departments. The average age at presentation was 12.2 years old, and the symptoms were present in 11 patients. All patients had associated renal malformations, and three patients presented with skeletal malformations. MRI was performed in only 10 of the patients included in the study, and the surgical treatment was carried out in 10 of the 16 patients. Psychological counseling was provided to four patients. Knowledge of genitourinary embryology is essential for the diagnosis and subsequent treatment of genital malformations, especially the complex ones that lead to gynecological and reproductive issues, particularly in young patients. We should consider all currently known concepts regarding imaging and therapeutic management, and perhaps it is time to unify all these efforts and knowledge to develop a therapeutic management plan that meets all prerequisites, both in the short and long term. [ABSTRACT FROM AUTHOR]

Purpose: This systematic review aims to provide a data synthesis about the risk of neovaginal cancer in women with Müllerian anomalies and to investigate the association between the adopted reconstructive technique and the cancer histotype. Methods: PubMed, MEDLINE, Embase, Scopus, ClinicalTrials.gov and Web of Science databases were searched from inception to March 1st, 2023. Studies were included if: (1) only women affected by Müllerian malformations were included, (2) the congenital defect and the vaginoplasty technique were clearly reported, (3) the type of malignancy was specified. Results: Literature search yielded 18 cases of squamous cell carcinoma and two cases of vaginal intraepithelial neoplasia 3 (VAIN 3). Of these, 3 had been operated on according to the Wharton technique, 8 according to the McIndoe technique, 3 with a split-skin graft vaginoplasty, 2 according to the Davydov technique, 2 with a simple cleavage technique, 1 according to the Vecchietti technique and 1 with a bladder flap vaginoplasty. A total of 17 cases of adenocarcinoma and 1 case of high-grade polypoid dysplasia were also described. Of these, 15 had undergone intestinal vaginoplasty, 1 had been operated on according to the McIndoe technique and 1 had undergone non-surgical vaginoplasty. Finally, 1 case of verrucous carcinoma in a woman who had undergone a split-skin graft vaginoplasty, was reported. Conclusion: Although rare, neovaginal carcinoma is a definite risk after vaginal reconstruction, regardless of the adopted technique. Gynaecologic visits including the speculum examination, the HPV DNA and/or the Pap smear tests should be scheduled on an annual basis. [ABSTRACT FROM AUTHOR]

It is unclear whether clinical background differs between endometriosis in adolescent patients with obstructive Müllerian anomalies and those without anomalies. The aim of the study is to identify the difference in clinical characteristics of endometriosis in patients with or without obstructive Müllerian anomalies. The study involved 12 patients aged under 24 years old who underwent primary surgery for obstructive Müllerian anomalies and 31 patients aged under 24 years old who underwent surgery for ovarian endometrioma. A total of 6 out of 12 cases with obstructive Müllerian anomalies developed endometriosis (4 Herlyn–Werner–Wunderlich syndrome, 2 non-communicating functional uterine horn, 2 cervical aplasia). The age at surgery was significantly younger in endometriosis with obstructive Müllerian anomalies, compared with those without obstructive Müllerian anomalies (17.8 ± 4.4 vs. 23.1 ± 1.3, p = 0.0007). The rate of endometrioma was 50.0% and the rate of hydrosalpinx was significantly higher (66.7% vs. 0%, p = 0.0002) in the group of obstructive Müllerian anomalies. The recurrence rate of endometriosis was 20.0% in the group of anomalies and 25.9% in the group of those without anomalies. Adolescent patients with obstructive Müllerian anomalies more easily developed endometriosis and co-occurred with higher rate of hematosalipinx. [ABSTRACT FROM AUTHOR]

Rubinstein-Taybi syndrome (RSTS) is a multi-system neurodevelopmental condition caused by deficiency of CREBBP (16p13.3) or EP300 (22q13.2). Müllerian agenesis, or Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, is defined as congenital agenesis of the uterus, cervix, and upper vagina without a definite genetic cause. We present a 14-year-old female with RSTS type 1 (CREBBP, c.4395-2A>C) and MRKH, the first documented in the literature. Following presentation to Gynecology for anticipatory guidance regarding future menstrual suppression and follow-up of previously diagnosed labial adhesions, exam under anesthesia revealed a single urogenital opening with cystoscopy demonstrating a normal urethra and bladder. Laboratory evaluation was consistent with peripubertal female gonadotropins and estradiol, 46,XX karyotype, and normal microarray, and a pelvic MRI confirmed Müllerian agenesis. Given this case, we assessed our cohort of females with RSTS and found that 4 of 12 individuals also had Müllerian anomalies. Gynecologic evaluation should be a part of medical care for females with RSTS, particularly in individuals with delayed menarche or abnormal menstrual history, on the basis of the observed association between RSTS and Müllerian anomalies in this case series. Although several candidate genes and copy number variants are associated with MRKH, no candidate genes in close proximity to the 16p13.3 region have been identified to explain both RSTS and MRKH in the index patient. Due to the regulatory nature of CREBBP during embryonic development, we theorize that CREBBP may play a role in the migration of Müllerian structures during embryogenesis. [ABSTRACT FROM AUTHOR]

The Mayer–Rokitansky–Küster–Hauser syndrome is characterized by aplasia of the uterus and upper two-thirds of the vagina. While it can appear as an isolated genital malformation, it is often associated with extragenital abnormalities, with little still known about the pathogenetic background. To provide an overview of associated malformations and syndromes as well as to examine possible ties between the rudimentary tissue and patient characteristics, we analyzed a cohort of 469 patients with MRKHS as well as 298 uterine rudiments removed during surgery. A total of 165 of our patients (35.2%) had associated malformations (MRKHS type II). Renal defects were the most common associated malformation followed by skeletal abnormalities. Several patients had atypical associated malformations or combined syndromes. Uterine rudiments were rarer in patients with associated malformations than in patients without them. Rudiment size ranged from 0.3 cm3 to 184.3 cm3 with a mean value of 7.9 cm3. Importantly, MRKHS subtype or concomitant malformations were associated with a different frequency of uterine tissue as well as a different rudiment size and incidence of endometrial tissue, thereby indicating a clear heterogeneity of the phenotype. Further research into the associated molecular pathways and potential differences between MRKHS subtypes is needed. [ABSTRACT FROM AUTHOR]

Background: Mullerian duct anomalies occur due to the abnormal fusion and canalisation of the mullerian (paramesonephric) duct. In mullerian anomalies uterine anomalies are the most common anomalies. Mullerian anomalies occur in 3-4% of population. Mullerian anomalies have low prevalence as they often remain asymptomatic and undiagnosed. Women with uterine anomalies are associated with a higher incidence of infertility, recurrent pregnancy loss (RPL), preterm labour, FGR, PROM, Malpresentations, retained placenta and increased caesarean section rate. This case series aimed to summarize the prevalence of mullerian anomalies, maternal and perinatal outcome of pregnancy in women with mullerian anomalies. Materials and Methods: This was a case series conducted in tertiary care centre, Government General Hospital, Guntur over a period of seven months (June 2023 to December 2023). Ethical approval was obtained from institutional ethical committee. Out of all the total deliveries, 14 cases of uterine anomalies were studied, either diagnosed before or during pregnancy or as an incidental finding during caesarean section. All detailed data such as demographic data, maternal age, parity, mode of delivery, obstetrical history, obstetric outcome, and foetal outcome were recorded. Results: In our hospital out of 3680 deliveries,14 pregnancies were identified to have mullerian anomalies. The prevalence of mullerian anomalies in our study was 0.3%. All the cases have undergone caesarean sections. Preterm labour, malpresentations especially breech presentation and foetal growth restriction were common in the study. Conclusion: Mullerian anomalies are asymptomatic or often have subtle gynaecological symptoms. Pregnancy with uterine anomalies have an adverse obstetric outcome and hence require proper counselling and close monitoring during antenatal period and during labour. Asymptomatic course, lack of MRI, and less preference for HSG as it is an invasive procedure are the causes of low rate of pre conceptional diagnosis. [ABSTRACT FROM AUTHOR]

Background: Metroplasty is a reconstructive surgery to repair congenital uterine anomalies. Anomalous uteri cause infertility and recurrent abortions due to defective implantation, restrictive fetal growth, and malpresentation. Aim: To discuss our experiences and surgical techniques with various cases of metroplasty in women with structural uterine anomalies with infertility or recurrent abortions at a tertiary care hospital in Mumbai. Case description: A descriptive study of case series of metroplasty conducted in a tertiary care hospital in women with bicornuate and didelphys uterus by opening the uterine cavities by incision on the medial aspect of the hemicolpos and approximating the myometrial edges and suturing them to create a single uterine cavity. Conclusion: By the unification of two smaller uterine cavities, metroplasty restores normal anatomy of the uterine cavity as is required for positive implantation and good obstetric outcomes. Clinical significance: After metroplasty, the chances of conception and probability of carrying conception to full term rise significantly. [ABSTRACT FROM AUTHOR]

Background: Mullerian Anomalies are associated with such reproductive disorders as infertility, recurrent pregnancy losses, primary amenorrhea, and others. Accurate diagnosis is essential for determining a woman's reproductive prognosis and planning certain types of interventions. The internal and external uterine contours evaluation is the key to the exact diagnosis and treatment. The hysterolaparoscopic procedure is considered the gold standard for evaluating Mullerian Anomalies, but the method is costly and invasive. 2D ultrasound sonography is a good screening method, but the uterus is not evaluated in the coronal plane. MRI is an expensive method and is used for the evaluation of complex anomalies. 3D ultrasound is a fast, cheap, non-invasive, and informative method for evaluating Mullerian Anomalies. Aim: To determine the effectiveness of 3D ultrasound in the diagnosis Mullerian Anomalies . Methods: We aimed to observe literature data to review the current understanding of the role of 3D ultrasound in the diagnosis of Mullerian anomalies. For this purpose following electronic databases were searched: Pubmed, Crossref, Mendeley, and Elsevier. Results: 90 articles were reviewed, and 43 of them attracted our attention due to their statistical significance and were analyzed and discussed in our article. Conclusion: Based on the literature review, we can conclude that 3D ultrasonography is a cheap, fast, non-invasive method for evaluating Mullerian anomalies. It showed high diagnostic concordance with the MRI method, virtually identical images are obtained by 3D ultrasound. Diagnostic difficulties arise in the case of cervical and vaginal anomalies, as well as in complex anomalies. Up to this date, it remains as a challenge, the correct diagnosis of the unclassifiable forms of Mullerian anomalies and the subsequent selection of their treatment methods, which is the basis for future research. [ABSTRACT FROM AUTHOR]

Complex congenital heart disease is often accompanied by extracardiac manifestations; , the significance of genitourinary involvement remains unclear. We present 3 patients with palliated complex congenital heart disease and with pregnancies complicated by premature delivery who were found to have congenital uterine anomalies that may have contributed to their obstetrical complications.

We present a simple surgical technique aiming to improve urine outflow through the common urogenital sinus in cloaca and facilitate drainage of existing hydrocolpos. The study included three cases of cloaca with associated hydrocolpos that were operated during the period 2022 through 2023. The patient is placed in the prone position for a standard posterior sagittal anorectoplasty. The distal rectal fistula is severed flush with the vagina/sinus leaving an open defect in the posterior wall of the vagina/sinus. The defect is then widened distally via a vertical incision (∼1 cm) through the posterior wall of the common urogenital sinus toward but not reaching the perineum. This vertical defect is then closed horizontally displacing the posterior vaginal wall downwards toward the perineum (posterior sinuplasty). The postoperative recovery was uneventful in the three cases. Adequate drainage of hydrocolpos was confirmed by imaging at follow-up, as well as improvement of upper urinary tract dilatation. In selected cases of cloaca, posterior sinuplasty is a simple procedure that can be applied during anorectoplasty to provide effective drainage of associated hydrocolpos.

Endometriosis is known to occur frequently in adolescents with obstructed Müllerian anomalies. Our cases emphasize that endometriosis can rapidly progress to a severe stage in obstructed hemivagina and ipsilateral renal anomaly syndrome, one of the completely obstructed Müllerian anomalies. The first patient was a 14-year-old girl who complained of cyclic abdominal pain. Imaging revealed a uterine didelphys with unilateral hematocolpos and a left adnexal endometrioma. The second, an 11-year-old girl, visited the hospital complaining of cyclic abdominal pain, had a unicornuate uterus with a functioning horn and left adnexal endometrioma. Also, both patients had unilateral renal agenesis. The surgery in both cases revealed Stage IV endometriosis. Adjuvant hormone therapy was administered for 1 year, and there was no recurrence until 3 years after surgery. We emphasize that patients diagnosed with renal agenesis should be screened to check for gynecological anomalies when menstrual cramps occur after menarche. [ABSTRACT FROM AUTHOR]

Recent advances in surgical technology and innovative techniques have revolutionized surgical gynecology, including transcervical hysteroscopic procedures. Surgical lasers (Nd-Yag, Argon, diode, and CO2 lasers) have been promoted to remove a variety of gynecological pathologies. For hysteroscopic surgery, the diode laser represents the most versatile and feasible innovation, with simultaneous cut and coagulate action, providing improved hemostasis compared with CO2 laser. The newest diode laser devices exhibit increased power and a dual wavelength, to work precisely with reduced thermal dispersion and minimal damage to surrounding tissues. Their efficacy and safety have been validated both in the hospitals as well as in the office setting. Updated evidence reports that several hysteroscopic procedures, including endometrial polypectomies, myomectomies and metroplasties can be successfully performed with a diode laser. Therefore, this review aimed to give a deeper understanding of the role of laser energy in gynecology and subsequently in hysteroscopy in order to safely incorporate this technology into clinical practice. [ABSTRACT FROM AUTHOR]

A quiz is presented related to a case where a 14 year old girl who was suffering from chronic, severe abdominal pain with a renal anomaly.

Background: In unicornuate uterus cases, when the rudimentary horn is very close to the uterus and is firmly attached, laparoscopic surgery can be very challenging due to the danger of massive bleeding and the possiblity of damaging the healthy hemiuterus. The aim of study is to verify if the laparoscopic resection of the horn site of hematometra, when solidly attached to the unicornuate uterus, is safe and effective.Materials and Methods: This is a retrospective analysis of prospectively collected data in a tertiary referral centre. From 2005 to 2021, a total of 19 women were diagnosed with unicornuate uterus with cavitated noncommunicating horn (class II B). We reviewed the original documentations of the patients and created a database. The follow-up results were assessed by questionnaires answered by the patients. In all cases, the chosen treatment was: laparoscopic removal of the rudimentary horn together with the ipsilateral salpinx and reconstruction of the myometrium of the hemiuterus. Statistical Package for Social Science (SPSS) version 21.0 was used to perform data analysis. We decided to calculate continuous variables in terms of mean and standard deviation (SD) or as median and interquartile range (IQR), as appropriate. Instead, categorical variables were expressed in terms of percentage.Results: Five patients (12-18 years old) with unicornuate uterus and rudimentary horn with hematometra and broadly connected to the hemiuterus were operated laparoscopically. The surgical procedure was successfull in all cases. No major complications were recorded. Postoperative course was uneventfull. In the follow-up in all cases dysmenorrhea and pelvic pain disappeared. Three patients sought to become pregnant and have children. They had in total 4 pregnancies with 2 abortions in the 1st trimester and two pregnancies with premature births at the 34th and 36th weeks. No serious gestational complications were recorded and the pregnancies ended with caesarean sections due to breech presentation.Conclusion: Overall, for the rudimentary horn solidly attached to the unicornuate uterus, the laparoscopic resection of the horn site of hematometra seems to be safe and effective.

Abstract Ovarian absence is an uncommon condition that most frequently presents unilaterally. Several etiologies for the condition have been proposed, including torsion, vascular accident, and embryological defect. A systematic review was conducted to describe the clinical presentation of ovarian absence, as well as its associations with other congenital anomalies, through a systematic search of Cochrane Library, ClinicalTrials.gov, Google Scholar, Ovid Embase, Ovid Medline, PubMed, Scopus, and Web of Science. Exclusion criteria included cases with suspicion for Differences of Sex Development, lack of surgically-confirmed ovarian absence, and karyotypes other than 46XX. Our search yielded 12,120 citations, of which 79 studies were included. 10 additional studies were found by citation chasing resulting in a total 113 cases including two unpublished cases presented in this review. Abdominal/pelvic pain (30%) and infertility/subfertility (19%) were the most frequent presentations. Ovarian abnormalities were not noted in 28% of cases with pre-operative ovarian imaging results. Approximately 17% of cases had concomitant uterine abnormalities, while 22% had renal abnormalities. Renal abnormalities were more likely in patients with uterine abnormalities (p

Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome is a rare congenital defect of the Müllerian ducts characterized by uterus didelphys, unilateral obstructed hemivagina, and ipsilateral renal agenesis. The aim of this systematic review is to summarize the main symptoms and presentation of the OHVIRA syndrome, as well as the different types of management, fertility, and obstetrical outcomes. A comprehensive search was performed in PubMed, EMBASE, SCOPUS, and Web of Science databases since inception to May 1, 2022, according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement. After duplicate records removed, the search strategy retrieved 103 articles. The full texts of 73 articles further were assessed for eligibility, and 44 studies were finally included in the systematic review. The mainstay surgical treatment of OHVIRA syndrome is usually a minimally invasive vaginal approach to remove the septum. Ultrasound-guided hysteroscopic resection and laparoscopic resection of the septum have been described as alternatives. Considering the feasibility of minimally invasive approach for the management of the syndrome, laparotomy should be avoided as much as possible and considered only in selected cases.

Background: Müllerian anomalies are rare developmental abnormalities. In the pediatric population, they manifest clinically during puberty and may require surgical treatment for symptom management. Methods: Retrospective case series study of children with Müllerian anomalies who were taken to diagnostic or therapeutic procedures by the pediatric surgery service at the Fundación HOMI from January 2018 to October 2022. Statistical analysis was performed calculating measures of central tendency and dispersion. Results: Fifteen patients were included. The most common reason for consultation was abdominal pain in 80 % of the cases. Associated comorbidities were found in 73.3 % of the patients, the most frequent being anorectal malformations (40 %) and renal agenesis (33.3 %). Pelvis MRI was performed in all cases. the most commonly observed finding was imperforate hymen, accounting for 19.8 % of cases. The anomaly was surgically corrected in 86 % of the patients. The median age at the time of surgery was 13 years (IQR 10–14). Reoperation was required in 33.3 %, of which all required vaginoscopy and dilatation. Conclusions: Müllerian anomalies are rare and usually present with nonspecific symptoms, the most frequent being pain. They are usually associated with renal and anorectal anomalies. In this case series, the use of magnetic resonance imaging and vaginoscopy were essential for diagnosis.

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. [ABSTRACT FROM AUTHOR]

To investigate the possibility of conservative management of rudimentary uterine horns associated with vaginal agenesis. Observational study on cohort of consecutive cases treated with the same criteria from 2008 to 2021. Two academic institutions and teaching hospitals in Milan, Italy. Eight patients with vaginal agenesis associated with rudimentary cavitated uterine horns treated by the same team and postoperatively followed. All the subjects underwent the same standardized surgical procedure: laparoscopy, intraoperative ultrasound, and horn-vestibular direct anastomosis. Postoperatively vaginoscopy was performed every 6 months. The postoperative course was generally uneventful and the mean hospital stay was 4.3 ± 2.5 (SD) days. All the patients began to menstruate a few months after the operation. Menstrual flows were light but regular. All patients had a neovaginal length > 4 cm at 1 year postoperatively, reaching approximately 6 cm at 2 years. During the follow-up period, 5 patients were sexually active without dyspareunia. In all cases, surgery restored the continuity of the neovagina and uterine horn through the creation of a "vaginal-horn fistula tract." In patients with vaginal agenesis associated with the presence of a uterine cavitary horn, it is possible to recover not only sexual activity but also menstrual function. The horn-vestibular anastomosis may be considered a valid, safe, and effective therapeutic option but requires accurate preoperative and intraoperative evaluation of rudimentary uterine structures. [ABSTRACT FROM AUTHOR]

Objectives: The purpose of this study is to report nine patients of young women who underwent a surgical treatment of an accessory and cavitated uterine mass (ACUM) in our hospital between 2014 and 2022 and review all cases described in the literature. Material and methods: The principal outcomes measured are the imaging techniques used to determine the diagnosis, the type of surgery used and the post-operative evolution of symptoms. We also report and analyse the 79 patients found in the literature since 1996 in addition to our 9 patients. Results: Surgical excision is the only long-lasting treatment. Small invasive surgery with laparoscopic access is the gold standard and most widely used (83.0%). Some new therapeutic procedures have been recently described of which ethanol sclerotherapy seems very promising. Post-operatively, 54.5% of patients have a complete relief of symptoms. MRI is the best imaging technique to identify ACUM. Finally, we refine the description of this pathology and give a more precise definition of it. Conclusion: Through our literature review and the analysis of our cases, we want to underline an important diagnostic criterion of this pathology: the fallopian tube on the homolateral side of the ACUM never communicates with the latter. It is a capital element for differential diagnosis. [ABSTRACT FROM AUTHOR]

Didelphic uterus are caused by Müllerian tube fusion failure that eventually form two uterine cavities, which is one of the rarest uterine malformations. Women with Müllerian anomalies may increase the incidence of perinatal complications and adverse outcomes of pregnancy. If Müllerian anomalies are found during pregnancy, the type of abnormality should be identified promptly, the impact on the mother and fetus should be assessed, and the frequency of prenatal care should be appropriately increased. Ovarian pregnancy as a rare ectopic gestation is often difficult to diagnose before surgery. Ovarian pregnancy seemingly has no logical relationship with the didelphic uterus. The high risk factors of ovarian pregnancy may include intrauterine device, assisted reproductive technology (ART) and intrauterine surgery history. A case of ovarian pregnancy in a woman with didelphic uterus after ART was reviewed, and the possible causes of ovarian pregnancy were summarized and analyzed. Single embryo transfer should be considered as the best transplantation strategy for those patients with didelphic uterus. Women of childbearing age should undergo a thorough pelvic examination before ART in order to make early diagnosis and proper management of congenital uterine abnormalities. [ABSTRACT FROM AUTHOR]

ObjectivesThe purpose of this study is to report nine patients of young women who underwent a surgical treatment of an accessory and cavitated uterine mass (ACUM) in our hospital between 2014 and 2022 and review all cases described in the literature.Material and methodsThe principal outcomes measured are the imaging techniques used to determine the diagnosis, the type of surgery used and the post-operative evolution of symptoms. We also report and analyse the 79 patients found in the literature since 1996 in addition to our 9 patients.ResultsSurgical excision is the only long-lasting treatment. Small invasive surgery with laparoscopic access is the gold standard and most widely used (83.0%). Some new therapeutic procedures have been recently described of which ethanol sclerotherapy seems very promising. Post-operatively, 54.5% of patients have a complete relief of symptoms. MRI is the best imaging technique to identify ACUM. Finally, we refine the description of this pathology and give a more precise definition of it.ConclusionThrough our literature review and the analysis of our cases, we want to underline an important diagnostic criterion of this pathology: the fallopian tube on the homolateral side of the ACUM never communicates with the latter. It is a capital element for differential diagnosis.

Purpose: To investigate the perinatal outcomes of singleton pregnant women with Müllerian anomalies (MuAs). Methods: A retrospective cohort study was conducted on singleton pregnant women with MuAs who delivered at the West China Second University Hospital between January 1, 2009 and December 31, 2020. Results: Four hundred fifty-seven cases of MuAs were identified, with an incidence of 0.40%. The most common anomaly was a septate uterus (38.7%). Compared to the control group, the MuAs group had significantly higher incidences of perinatal complications, including preterm deliveries (PTDs) (27.4 vs. 9.8%, P < 0.001), preterm premature rupture of membranes (PPROM) (29.1 vs. 22.5%, P = 0.001), malpresentation (34.4 vs. 5.6%, P < 0.001), abruptio placentae (4.6 vs. 1.2%, P < 0.001), placental accreta/increta (19.7 vs. 11.8%, P < 0.001), and uterine rupture (2.8 vs. 1.6%, P = 0.035). The rates of in vitro fertilization and embryo transfer (IVF–ET), foetal growth restriction (FGR), and low birth weight were also significantly higher in the MuAs group (8.3 vs. 4.5%, P < 0.001; 2.6 vs. 0.9%, P = 0.001; 3.1 vs. 1.7%, P = 0.033, respectively). In the MuAs group, the incidence of PPROM was high in cases with unicornuate uterus (31.5%), and malpresentation was as high as 42.4 and 37.0% in cases with septate and didelphys uteri, respectively. Conclusion: The data suggest that pregnancy with MuAs may increase adverse perinatal outcomes, which calls for intensive supervision during pregnancy and delivery to reduce maternal and foetal complications. Individualized considerations should be emphasized according to the different categories of MuAs in pregnancies. [ABSTRACT FROM AUTHOR]

Background: In unicornuate uterus cases, when the rudimentary horn is very close to the uterus and is firmly attached, laparoscopic surgery can be very challenging due to the danger of massive bleeding and the possiblity of damaging the healthy hemiuterus. The aim of study is to verify if the laparoscopic resection of the horn site of hematometra, when solidly attached to the unicornuate uterus, is safe and effective. Materials and Methods: This is a retrospective analysis of prospectively collected data in a tertiary referral centre. From 2005 to 2021, a total of 19 women were diagnosed with unicornuate uterus with cavitated noncommunicating horn (class II B). We reviewed the original documentations of the patients and created a database. The follow-up results were assessed by questionnaires answered by the patients. In all cases, the chosen treatment was: laparoscopic removal of the rudimentary horn together with the ipsilateral salpinx and reconstruction of the myometrium of the hemiuterus. Statistical Package for Social Science (SPSS) version 21.0 was used to perform data analysis. We decided to calculate continuous variables in terms of mean and standard deviation (SD) or as median and interquartile range (IQR), as appropriate. Instead, categorical variables were expressed in terms of percentage. Results: Five patients (12-18 years old) with unicornuate uterus and rudimentary horn with hematometra and broadly connected to the hemiuterus were operated laparoscopically. The surgical procedure was successful in all cases. No major complications were recorded. Postoperative course was uneventful. In the follow-up in all cases dysmenorrhea and pelvic pain disappeared. Three patients sought to become pregnant and have children. They had in total 4 pregnancies with 2 abortions in the 1st trimester and two pregnancies with premature births at the 34th and 36th weeks. No serious gestational complications were recorded and the pregnancies ended with caesarean sections due to breech presentation. Conclusion: Overall, for the rudimentary horn solidly attached to the unicornuate uterus, the laparoscopic resection of the horn site of hematometra seems to be safe and effective. [ABSTRACT FROM AUTHOR]

It is unclear whether clinical background differs between endometriosis in adolescent patients with obstructive Müllerian anomalies and those without anomalies. The aim of the study is to identify the difference in clinical characteristics of endometriosis in patients with or without obstructive Müllerian anomalies. The study involved 12 patients aged under 24 years old who underwent primary surgery for obstructive Müllerian anomalies and 31 patients aged under 24 years old who underwent surgery for ovarian endometrioma. A total of 6 out of 12 cases with obstructive Müllerian anomalies developed endometriosis (4 Herlyn–Werner–Wunderlich syndrome, 2 non-communicating functional uterine horn, 2 cervical aplasia). The age at surgery was significantly younger in endometriosis with obstructive Müllerian anomalies, compared with those without obstructive Müllerian anomalies (17.8 ± 4.4 vs. 23.1 ± 1.3, p = 0.0007). The rate of endometrioma was 50.0% and the rate of hydrosalpinx was significantly higher (66.7% vs. 0%, p = 0.0002) in the group of obstructive Müllerian anomalies. The recurrence rate of endometriosis was 20.0% in the group of anomalies and 25.9% in the group of those without anomalies. Adolescent patients with obstructive Müllerian anomalies more easily developed endometriosis and co-occurred with higher rate of hematosalipinx.

Background: This study aimed to assess the prevalence and course of endometriosis in adolescents with obstructive Müllerian anomalies. Methods: The study group involved 50 adolescents undergoing surgeries (median age 13.5 (range 11.1–18.5)) for rare obstructive malformations of the genital tract: 15 girls had anomalies associated with cryptomenorrhea and 35 were menstruating. The median follow-up period was 2.4 (ranging from 0.1 to 9.5) years. Results: We diagnosed endometriosis in 23 of the 50 subjects (46%), including 10 of the 23 patients (43.5%) with obstructed hemivagina ipsilateral renal anomaly syndrome (OHVIRAS), six of eight patients (75%) with a unicornuate uterus with a non-communicating functional horn, two of three patients (66.7%) with distal vaginal aplasia, and five of five patients (100%) with cervicovaginal aplasia. Persistent dysmenorrhea, following treatment, affected 14 of the 50 adolescents (28%), including 8 of the 17 subjects (47.1%) diagnosed with endometriosis at the time of surgical correction and six adolescents diagnosed with endometriosis during the follow-up. Conclusions: Endometriosis affects about half of young adolescents undergoing surgical treatment of obstructive Müllerian anomalies after menarche. The incidence of endometriosis is highest in girls with cervical aplasia. The risk of developing endometriosis decreases after surgical correction of obstruction but is still significant in patients with uterine anomalies. [ABSTRACT FROM AUTHOR]

• What does this study add to the literature (required). • What is currently known about this topic? A large number of patients with cloacal malformation have associated gynecologic anomalies. • What new information is contained in this article? Girls with less complex ARM subtypes (such as rectoperineal and rectovestibular fistulas) have a high incidence of gynecologic anomalies. This incidence is significantly increased in the presence of a VACTERL association. VACTERL association is defined by the presence of 3 or more anomalies in any of the following systems: vertebral, anorectal, cardiac, trachea-esophageal, renal, or limb. This study hypothesized that the presence of VACTERL association would correlate with an increased risk of gynecologic anomalies in patients with anorectal malformation (ARM). This study is a cross-sectional, retrospective analysis from the prospectively collected, multicenter registry of the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC). The 834 female patients with ARM who were enrolled in the registry by January 1, 2020 were included in this study. The relationship of VACTERL association with presence of a gynecologic anomaly was evaluated with Fisher's exact test. The relationship of each VACTERL system with presence of a gynecologic anomaly was assessed in patients with cloaca, rectovestibular fistulas and rectoperineal fistulas. P-values reported were based on a 2-sided alternative and considered significant when less than 0.05. 834 patients with ARM underwent VACTERL screening and gynecologic evaluation with the three most common subtypes being cloaca (n = 215, 25.8%), rectovestibular fistula (n = 191, 22.9%) and rectoperineal fistula (n = 194, 23.3%). A total of 223 (26.7%) patients with ARM had gynecologic anomalies. VACTERL association was seen in 380 (45.6%) of patients with ARM. Gynecologic anomalies were present in 149 (39.1%) vs. 74 (16.3%) of subjects with vs. without VACTERL association (p < 0.001). VACTERL association did not significantly increase the risk of gynecologic anomaly in patients with cloaca and VACTERL (n = 88, 61.5%) vs. cloaca without VACTERL (n = 39, 54.2% p = 0.308). VACTERL association increased the risk of gynecologic anomalies in patients with rectoperineal fistulas (n = 7, 14.9% vs n = 9, 6.1% p = 0.014) and rectovestibular fistulas (n = 19, 31.1% vs. n = 13, 10.0% p <0.001). In patients with ARM who had a VACTERL association, when one of the associated anomalies was renal, there was an even higher risk of having an associated gynecologic anomaly (n = 138, 44.2% vs. n = 85, 16.3% p <0.001). VACTERL association in patients with rectoperineal and rectovestibular fistulas correlates with an increased risk of gynecologic anomalies. The presence of VACTERL associated findings, especially renal, should prompt a thorough evaluation of the gynecologic system. III. Retrospective comparative study [ABSTRACT FROM AUTHOR]