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Background: The prevalence of placenta accreta spectrum is on the rise, primarily as a consequence of an increasing number of Cesarean sections. Nevertheless, uterine anomalies, particularly uterine septum, pose a notable risk factor for its occurrence. While there are limited case reports documenting the association between uterine septum and placenta adherence, most of these cases have been linked to prior hysteroscopic treatment of the septum. Notably, this case represents the first-reported instance in which a uterine septum was preoperatively diagnosed in association with placenta increta adherent to it, and subsequently managed conservatively., Case Presentation: A 30-year-old pregnant Egyptian female patient, who had undergone two previous cesarean sections, was diagnosed with placenta increta and had an incomplete uterine septum. She was admitted to Elshatby University Hospital in December 2021. During her surgery, it was found that the placenta was adherent to right lower aspect of the septum, as well as the right anterior, lateral, and posterior uterine walls. A conservative surgical approach was scheduled and successfully performed, and 3 months later, an ultrasound examination revealed that the uterus had returned to its normal size, with no evidence of isthmocele or synechiae. The only abnormality noted was the presence of the septum., Conclusion: Ultrasound plays a crucial role in diagnosing placenta accreta and identifying any additional uterine abnormalities that may guide the planning of conservative treatment. Uterine septum represents a risk factor for placental adherence, as the placenta can indeed become adherent to it. Muscle defects may become apparent following placental separation and can often be effectively managed through the use of running sutures, obviating the need for resection of the affected portion. Trial registration clinicaltrials.gov, registered on 27 April 2021, registration number: NCT04866888, https://clinicaltrials.gov/ct2/show/NCT04866888 ., Competing Interests: Declarations Ethics approval and consent to participate Ethical committee of faculty of medicine, Alexandria university has approved the study; approval no. 0201462. Consent for publication Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal. Competing interests Authors report no conflict of interest., (© 2024. The Author(s).)

Objective: To determine the frequency and types of congenital anomalies of the kidney and urinary tract (CAKUT) in patients with Hirschsprung disease., Study Design: An observational cross-sectional study. Place and Duration of the Study: Department of Paediatric Surgery, National Institute of Child Health, Karachi, Pakistan, from June to December 2022., Methodology: All patients with biopsy-proven Hirschsprung disease were included. Ultrasound was done to find out the anatomical and structural anomalies of the kidney and urinary tract. Functional assessment was done by renal function tests and MAG3 radioisotope scan where indicated. Fisher's exact test was applied to find out the association. A p ≤0.05 was taken as significant., Results: Out of a total of 83, 15 (18.0%) patients had CAKUT. The mean age of the study population was 5.5 ± 2.3 years. It included 61 (73.5%) males and 22 (26.5%) females. No significant association was found between the type of Hirschsprung disease and CAKUT (p = 0.7). The gender distribution between CAKUT patients was also insignificant (p = 0.7). Renal hypoplasia was the most common anomaly found in six patients followed by hydronephrosis due to pelvi-ureteric junction obstruction in four children. All of these children were asymptomatic. Five male patients had undescended testis., Conclusion: Nearly a fifth of the children with Hirschsprung disease had CAKUT of whom renal dysplasia was the most common anomaly. There was no gender predilection and patients were asymptomatic regarding the urinary system. A routine ultrasound abdomen is a good screening investigation for identifying CAKUT., Key Words: Hirschsprung disease, Kidney and urinary tract, Congenital diseases of the urinary tract, Congenital anomalies.

Objectives: To investigate the diagnostic utility of copy-number variant (CNV) detection by chromosomal microarray analysis (CMA) and genotype-phenotype associations in prenatal congenital anomalies of the kidney and urinary tract (CAKUT)., Methods: This is a retrospective multi-center study of CNV analysis in 457 fetuses with ultrasound-detected CAKUT and normal karyotypes. Cohorts from published studies were included for further pooled analyses (N = 2746). A literature review of single-nucleotide variant (SNV) and small insertions and deletions (Indel) analysis by whole-exome sequencing was performed to investigate monogenic causes., Results: In our multi-center cohort, 5.3% (24/457) of fetuses had pathogenic CNVs (pCNV); 3.9% (14/359) and 10.2% (10/98) in isolated and non-isolated CAKUT, respectively. Fetuses with isolated hyperechogenic kidneys (HEK) had the highest incidence of having pCNVs. In the literature review, 6.6% (180/2746) of fetuses carried pCNVs; 6.1% and 7.5% in isolated and non-isolated CAKUT, respectively. SNV/Indel analysis provided at least 16.5% (63/381) additional diagnostic yield beyond CNV analysis; 12.8% and 23.8% in isolated and non-isolated CAKUT, respectively., Conclusion: pCNVs comprise a significant proportion of genetic diagnostic findings in prenatal CAKUT, most commonly detected in fetuses with isolated HEK, MCDK, renal agenesis, and non-isolated CAKUT. Monogenic causes should be considered when karyotyping and CMA are nondiagnostic., (© 2024 John Wiley & Sons Ltd.)

Purpose: To describe the clinical and imaging characteristics of Herlyn-Werner-Wunderlich syndrome (HWWS)., Methods: This study presented an observational case series involving consecutive patients diagnosed with HWWS, whose medical records were retrospectively reviewed. From June 2012 to December 2022, there were a total of 85 patients with HWWS enrolled in our study. We obtained the medical history, including demographic characteristics, clinical presentation, treatment, complications, and radiologic examinations performed. Patients > 18 years of age (n = 58) were recontacted., Result: In our analysis, 27 patients were categorised as having complete obstruction, and 58 were categorised as having incomplete obstruction. The mean age at the onset of symptoms and diagnosis of complete obstruction was significantly younger than incomplete obstruction (P < 0.05). For complete obstruction, the median time between menarche and the onset of symptoms was 2.1 years, while for incomplete obstruction, it was 5.3 years. There was a significantly lower incidence of intermittent mucopurulent discharge, irregular vaginal haemorrhage, and occasional examination findings of complete obstruction than incomplete obstruction (P < 0.05). Complete obstruction was significantly associated with dysmenorrhea and pelvic endometriosis compared with incomplete obstruction (P < 0.05)., Conclusions: There are distinct clinical differences between patients with complete obstruction of the hemivagina and those with incomplete obstruction. HWWS can manifest as various combinations of uterine anomalies, communications anomalies, and renal anomalies. Early recognition and treatment can avoid complications and preserve fertility., Keyswords: Herlyn-Werner-Wunderlich syndrome (HWWS); complete obstruction; incomplete obstruction; obstructed hemivagina; congenital malformation., (© 2024. The Author(s).)

Background: Congenital anomalies of the kidneys and urinary tract (CAKUT) are the most common cause of prenatally diagnosed developmental malformation. This study aimed to assess the relationship between maternal diseases and CAKUT in offspring., Methods: This retrospective study enrolled all pregnant women registered from January 2020 to December 2022 at one medical center. Medical information on maternal noncommunicable diseases, including obesity, hypertension, diabetes mellitus, kidney disease, hyperthyroidism, hypothyroidism, psychiatric disease, epilepsy, cancer, and autoimmune disease was collected. Based on the records of ultrasound scanning during the third trimester, the diagnosis was classified as isolated urinary tract dilation (UTD) or kidney anomalies. Multivariate logistic regression was performed to establish models to predict antenatal CAKUT., Results: Among the 19,656 pregnant women, perinatal ultrasound detected suspicious CAKUT in 114 (5.8/1000) fetuses, comprising 89 cases with isolated UTD and 25 cases with kidney anomalies. The risk of antenatal CAKUT was increased in the fetuses of mothers who experienced gestational diabetes, thyroid dysfunction, neuropsychiatric disease, anemia, ovarian and uterine disorders. A prediction model for isolated UTD was developed utilizing four confounding factors, namely gestational diabetes, gestational hypertension, maternal thyroid dysfunction, and hepatic disease. Similarly, a separate prediction model for kidney anomalies was established based on four distinct confounding factors, namely maternal thyroid dysfunction, gestational diabetes, disorders of ovarian/uterine, and kidney disease., Conclusions: Isolated UTD and kidney anomalies were associated with different maternal diseases. The results may inform the clinical management of pregnancy and highlight potential differences in the genesis of various subtypes of CAKUT., Competing Interests: Declarations. Ethical approval: Due to the retrospective nature of the study, informed consent was waived. All the procedures performed in studies involving human participants were in accordance with the ethical standards of the Ethical Committee for scientific research approval of the Tai’an Hospital (TA-2019-027). Conflict of interest: No financial or non-financial benefits have been received or will be received from any party related directly or indirectly to the subject of this article. The authors have no conflict of interest relevant to this article to declare., (© 2024. The Author(s).)

Objective: To describe the intraoperative and postoperative implications arising from the unexpected diagnosis of a Müllerian anomaly during the surgical management of an ectopic pregnancy., Design: Video article., Setting: Academic center., Patient(s): A 39-year-old nulligravid woman with anovulation and irregular menstrual cycles presented to the office. Her urine pregnancy test result was incidentally positive; the serum β-human chorionic gonadotropin level was 5,644 mIU/mL. Outpatient transvaginal ultrasonography demonstrated a 2.1 × 1.7 × 2.2-cm thick-walled structure in the left adnexa without an intrauterine pregnancy. These findings were highly suspicious for a left tubal ectopic pregnancy. The patient was consented for laparoscopy with planned left salpingectomy. The patient included in this video gave consent for publication of the video and posting of the video online including social media, the journal website, scientific literature websites (e.g., PubMed, ScienceDirect, and Scopus), and other applicable sites., Intervention(s): Diagnostic laparoscopy did not show an obvious left tubal ectopic pregnancy. Instead, a right unicornuate uterus with a dilated rudimentary left uterine horn was seen. Both fallopian tubes and ovaries appeared normal. These laparoscopic findings were consistent with an ectopic pregnancy in the rudimentary horn. However, in the absence of informed consent for a hemihysterectomy and no evidence of ectopic rupture or bleeding within the pelvis, we decided to proceed with excision of the ectopic pregnancy from the uterine horn. An incision was made over the anterior surface of the uterine horn, and the pregnancy sac was dissected from the underlying myometrium and excised in its entirety. Left salpingectomy was also performed. The patient was discharged home the same day, and her β-human chorionic gonadotropin levels decreased to <5 mIU/mL within 28 days of surgery., Main Outcome Measure(s): Complete resolution of a left rudimentary uterine horn ectopic pregnancy through surgical excision of the pregnancy sac without hemihysterectomy., Result(s): Postoperative hysterosalpingography demonstrated a right unicornuate uterus with normal fill and spill of the right fallopian tube. Magnetic resonance imaging of the pelvis confirmed the findings of a right unicornuate uterus with a noncommunicating left rudimentary uterine horn that did not contain any endometrial tissue. Thus, the patient did not require an interval hemihysterectomy. She underwent letrozole and intrauterine insemination treatment 5 months after the initial surgery, which resulted in a clinical intrauterine pregnancy. However, this pregnancy was terminated in the early second trimester because of findings of trisomy 18. She conceived naturally 1 year later, and this pregnancy resulted in a full-term vaginal birth at 39 weeks of gestation., Conclusion(s): Undiagnosed or unexpected Müllerian anomalies can impact the standard intraoperative and postoperative management of ectopic pregnancies., Competing Interests: Declaration of Interests S.T. has nothing to disclose. A.U. has nothing to disclose. S.R.L. has nothing to disclose. N.P. reports consulting fees and honoraria from BioSyent Pharma, Inc., outside the submitted work., (Copyright © 2024 American Society for Reproductive Medicine. Published by Elsevier Inc. All rights reserved.)

We reported a case of heterotopic twin pregnancy in a unicornuate uterus with a non-communicating rudimentary horn with survival of both fetuses. The diagnosis was made late at 28 weeks of gestation, with suspicion raised by ultrasound and confirmed by magnetic resonance imaging (MRI). During hospitalization, obstetric ultrasound with color Doppler was performed every 2 days to assess fetal well-being and myometrial thickness, which was determined by measurements of the uterine wall at the accessory horn. Elective cesarean section was performed at 33 + 5 weeks of gestation. Delivery started with the fetus in the rudimentary horn, with subsequent extraction of the fetus in the unicornate uterus. Three-dimensional virtual reconstruction allowed a spatial view of the both uterus and fetuses with better understanding of the obstetrical condition by the parents and interactive discussion by the multidisciplinary medical team., (© 2024 Wiley Periodicals LLC.)

Purpose: The eccentric implantation of pregnancies in the upper lateral aspect of the uterine cavity is poorly defined clinically. The aim of the current study was to investigate whether differentiating between uterine anomalies that can lead to cavitary distortion has implications for the management of these pregnancies., Methods: Eight cases of first-trimester eccentric pregnancy implantation within the endometrial cavity (study group) were retrospectively identified. For each woman in the study group, 10 women identified as having a first-trimester concentric pregnancy implantation during the first-trimester US examination were retrieved from our database (control group). After delivery or pregnancy demise, the presence of uterine anomalies was assessed by a 3D-US examination in all patients., Results: In the study group patients, an increased incidence of uterine anomalies (50.0% vs. 8.8%, p = 0.007) was found, compared to the controls. In the study group, the eccentric location persisted in half of the pregnancies (n = 4; 50%), whereas the other half migrated to a more centric location within the endometrial cavity (n = 4; 50%). The follow-up examination showed that all the early pregnancy demises occurred in cases where the pregnancy persisted at the eccentric location. Uterine malformations were also detected in all these cases., Conclusion: The data point to a significantly higher incidence of uterine anomalies in patients diagnosed with eccentric pregnancy implantation within the endometrial cavity. These results advocate for the value of differentiating between eccentric pregnancies in non-anomalous versus anomalous uteri., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: To assess the characteristics, additional structural anomalies and postnatal urinary outcome of the cases diagnosed with fetal ectopic kidneys in the prenatal period., Study Design: Cases having fetal ectopic kidneys, detected from a total of 14,617 pregnant women examined by routine detailed (Group 1) or indicated (Group 2) obstetric ultrasonography (USG) in a tertiary perinatology unit were analyzed. The prevalence of the cases, time of the diagnosis, sidedness of the affected kidney, anatomical location, origins of blood supply, additional urinary or extraurinary anomalies, and urinary complications during the postnatal follow-up period were investigated., Results: We have detected 33 fetuses with ectopic kidneys in our cohort. The prevalence of fetal ectopic kidney was 0.22 %, with a median (min.-max.) diagnosis time of 21.3 (17.6-34) weeks. In the group in whom indicated USG was performed, the time of diagnosis was later compared to routine detailed USG (p = 0.04) group. There was no difference in terms of gender [male, (n = 14), female (n = 19), p = 0.38] and the sidedness of the ectopic kidneys (p = 0.38). The location of ectopic kidneys was most frequent in the iliac fossa (n = 20, 60.6 %) and in the lateral pelvic areas (n = 13, 39.3 %). The blood supply origin of ectopic kidneys was the common iliac artery in 22 (66.6 %), whereas the aorta in 11 cases (33.3 %). There was an additional urinary anomaly in 8 cases (24 %), an extraurinary structural anomaly, most commonly cardiac, and/or a soft marker for aneuploidy were presented in 16 cases (48 %). The most common urinary complication in the postpartum period was vesicoureteral reflux (n = 5)., Conclusion: Ectopic kidney in the prenatal period is a rare structural anomaly that can equally affect both genders and both kidneys. Prenatal diagnosis is important for the diagnosis of additional anomalies and follow-up of postnatal complications., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 Elsevier B.V. All rights reserved.)

Congenital abnormalities of the kidneys and urinary tract (CAKUT) encompass a spectrum of pathology ranging in severity from incidental findings to marked deviations of anatomic form and function. The imaging appearance of CAKUT generally reflects a deviation from normal embryologic development. Common and uncommon congenital abnormalities of the kidneys, ureters, bladder, and urethra are reviewed with an emphasis on the imaging appearance and embryologic origin so that they may be promptly identified and treated when encountered in clinical practice., Competing Interests: Disclosure None., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Introduction: Metroplasty is a procedure used by some teams to correct certain uterine anomalies to improve fertility outcomes. Our goal was to evaluate hysteroscopic metroplasty in the management of nulliparous and infertile patients with a uterine anomalies., Material and Methods: We conducted a single-center, retrospective, observational study of women who underwent hysteroscopic metroplasty for infertility between January 1, 2015 and December 31, 2019. The primary endpoint was the occurrence of a live birth at 18 months post-surgery. The secondary endpoint was to identify predictive factors for the success of the procedure, in particular ultrasound criteria, and live-birth rate during total follow up., Results: We included 43 nulliparous patients with an average of 5.2±-2.4 years of primary infertility, including 84.2 % patients who had at least one IVF cycle prior to the surgery. The mean age was 37±5 years. The post-surgery live-birth rate was 27.9 % at 18 months and 53.5 % during the total postoperative follow-up (mean follow-up 4.5 ± 1 years). Pregnancies were obtained spontaneously after surgery for 8/28 (28.6 %) patients who were undergoing assisted reproduction technology before surgery. No intra- or postoperative complications were recorded. We did not identify any predictive ultrasound factors, pre- or postoperatively, for a live birth at 18 months post-surgery., Discussion: Hysteroscopic metroplasty appears to improve the chances of a live birth in a population of nulliparous and infertile patients with at least one uterine pathology. Spontaneous pregnancy can occur after hysteroscopic metroplasty. The Congenital-Ultrasound-Malformation-by-Expert ultrasound criteria do not appear to be predictive of post-surgery outcomes., Competing Interests: Declaration of competing interest None., (Copyright © 2024. Published by Elsevier Masson SAS.)

Zinner syndrome is a rare congenital urological entity, secondary to an alteration in embryogenesis between 4th and 13th weeks of gestation, specifically because of abnormalities in the development of the distal mesonephric duct. It is characterized by the triad of unilateral renal agenesis, cystic dilatation of the ipsilateral seminal vesicle and ipsilateral ejaculatory duct obstruction. The aim of this article is to provide the reader with all the necessary information to be able to suspect the presence of this syndrome, reviewing its physiopathology, clinical manifestations and the imaging techniques that enable its diagnosis, emphasizing those radiological findings by MRI that should lead us to think about it. This work is illustrated with representative radiological images of cases belonging to our institution, including patients with different variants of Zinner syndrome. We also include an overview of the embryology of the male urogenital system, to remember the role of the mesonephric duct and the ureteral bud in the formation of the different urogenital structures, as well as a differential diagnosis that allows us to differentiate seminal vesicle cysts from other pelvic cystic lesions., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) is a rare congenital malformation of the female urogenital tract characterized by a triad of uterine didelphys, obstructed hemivagina, and ipsilateral renal anomaly. It was formerly known as Herlyn Werner Wunderlich Syndrome (HWWS). The syndrome usually presents with cyclic pelvic pain following menarche. Endometriosis is a prevalent complication. Magnetic resonance imaging (MRI) helps in diagnosing OHVIRA syndrome and endometriosis due to its high contrast resolution and objectivity. We reported a 13- year-old girl who was evaluated for cyclic pelvic pain after her menarche at 12 years of age. Magnetic resonance imaging (MRI) revealed two separate uterine cavities, services and vaginae, indicating didelphys. The left uterine cavity is filled with fluid, and the left hemivagina is dilated and filled with hyperintense and hypointense fluid on T1 and T2, respectively, indicating blood products. Left hemivagina dilatation implicated the presence of an obstructing vaginal septum. A single left adnexal cyst lesion with blood products was suggestive of an endometriotic cyst. Additionally, the left kidney was absent. A uterine didelphys with left hemivagina obstruction, hematometra, hematocolpos, and the ipsilateral ovarian endometriotic cyst was diagnosed. A final diagnosis of OHVIRA syndrome or HWWS was made, considering that she had no left kidney.MRI is a suitable diagnostic tool for precise anatomical delineation of the uterus, cervix, and vagina in uterovaginal disorders such as OHVIRA syndrome. MRI can also properly evaluate endometriosis and adhesion.

Fetal MR imaging overcomes many of the technical barriers of ultrasound and is an important diagnostic tool for fetal genitourinary (GU) anomalies. It is suited for evaluation of GU anomalies because of the fluid-sensitive sequences and superior soft tissue contrast. Often GU malformations are part of a multisystem genetic or congenital condition, and imaging the entire fetus with MR adds additional clarity about the extent of disease. It adds confidence to diagnoses of renal agenesis, urinary tract dilation, cystic disease, and tumors. It is particularly useful to delineate anatomy in complex GU malformations. This additional information guides counseling., Competing Interests: Disclosure The author has nothing to disclose., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Study Question: What is the prevalence of congenital and acquired anomalies of the uterus in women with recurrent pregnancy loss (RPL) of unknown etiology examined using 3D transvaginal ultrasound (US)?, Summary Answer: Depending on the adopted diagnostic criteria, the prevalence of partial septate uterus varies between 7% and 14% and a T-shaped uterus is 3% or 4%, while adenomyosis is 23%, at least one of type 0, type 1 or type 2 myoma is 4%, and at least one endometrial polyp is 4%., What Is Known Already: ESHRE and the Royal College of Obstetricians and Gynaecologists guidelines on RPL recommend the adoption of the 3D transvaginal US to evaluate the 'uterine factor'. Nevertheless, there are no published studies reporting the prevalence of both congenital and acquired uterine anomalies as assessed by 3D transvaginal US and diagnosed according to the criteria proposed by the most authoritative panels of experts in a cohort of women with RPL., Study Design, Size, Duration: This was a retrospective cohort study including 442 women with at least two previous first-trimester spontaneous pregnancy losses (i.e. non-viable intrauterine pregnancies), who referred to the obstetrics and gynecology unit of two university hospitals between July 2020 and July 2023., Participants/materials, Setting, Methods: Records of eligible women were reviewed. Women could be included in the study if: they were between 25 and 42 years old; they had no relevant comorbidities; they were not affected by infertility, and they had never undergone ART; they and their partner tested negative to a comprehensive RPL diagnostic work-up; and they had never undergone metroplasty, myomectomy, minimally invasive treatments for uterine fibroids or adenomyomectomy. Expert sonographers independently re-analyzed the stored 2- and 3D transvaginal US images of all included patients. Congenital uterine anomalies (CUAs) were reported according to the American Society for Reproductive Medicine (ASRM) 2021, the ESHRE/European Society for Gynaecological Endoscopy (ESGE) and the Congenital Uterine Malformation by Experts (CUME) criteria. Acquired uterine anomalies were reported according to the International Federation of Gynecology and Obstetrics (FIGO) and the Morphological Uterus Sonographic Assessment (MUSA) criteria., Main Results and the Role of Chance: The partial septate uterus was diagnosed in 60 (14%; 95% CI: 11-17%), 29 (7%; 95% CI: 5-9%), and 47 (11%; 95% CI: 8-14%) subjects, according to the ESHRE/ESGE, the ASRM 2021, and the CUME criteria, respectively. The T-shaped uterus was diagnosed in 19 women (4%; 95% CI: 3-7%) according to the ESHRE/ESGE criteria and in 13 women (3%; 95% CI: 2-5%) according to the CUME criteria. The borderline T-shaped uterus (diagnosed when two out of three CUME criteria for T-shaped uterus were met) was observed in 16 women (4%; 95% CI: 2-6%). At least one of FIGO type 0, type 1, or type 2 myoma was detected in 4% of included subjects (95% CI: 3-6%). Adenomyosis was detected in 100 women (23%; 95% CI: 19-27%) and was significantly more prevalent in women with primary RPL and in those with three or more pregnancy losses. At least one endometrial polyp was detected in 4% of enrolled women (95% CI: 3-7%)., Limitations, Reasons for Caution: The absence of a control group prevented us from investigating the presence of an association between both congenital and acquired uterine anomalies and RPL. Second, the presence as well as the absence of both congenital and acquired uterine anomalies detected by 3D US was not confirmed by hysteroscopy. Finally, the results of the present study inevitably suffer from the intrinsic limitations of the adopted classification systems., Wider Implications of the Findings: The prevalence of CUAs in women with RPL varies depending on the classification system used. For reasons of clarity, the US reports should always state the name of the uterine anomaly as well as the adopted classification and diagnostic criteria. Adenomyosis seems to be associated with more severe forms of RPL. The prevalence rates estimated by our study as well as the replicability of the adopted diagnostic criteria provide a basis for the design and sample size calculation of prospective studies., Study Funding/competing Interest(s): No specific funding was used. The authors have no conflicts of interest to declare., Trial Registration Number: N/A., (© The Author(s) 2024. Published by Oxford University Press on behalf of European Society of Human Reproduction and Embryology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Transverse vaginal septum (TVS) is a rare obstructive genital tract anomaly. It is associated with primary amenorrhoea and typically presents with cyclical abdominal pain due to obstruction of the menstrual flow. Caudal regression syndrome (CRS) is also a rare congenital anomaly that is frequently associated with anomalies of the neurological, musculoskeletal, cardiac, genitourinary and gastrointestinal systems. Obstructive genital tract anomaly in CRS is exceptionally rare. This report describes the case of a girl in early adolescence with underlying CRS who presented with severe abdominal pain associated with primary amenorrhoea. Clinical and radiological assessment revealed 'haematocolpos' manifesting as a tender 20 weeks' size abdominal mass and an absent vaginal opening. TVS was identified during examination under anaesthesia. The patient subsequently underwent a successful vaginoplasty with no recurrence of symptoms after 2 years., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

This case report elucidates a scenario involving two sibling sisters born out of consanguineous marriage-one initially presenting with lower respiratory infection, concurrently exhibiting short stature and primary amenorrhoea. Investigation into the primary amenorrhoea unveiled hypergonadotropic hypogonadism, confirmed by the absence of ovaries and a hypoplastic uterus on pelvic MRI. Genetic analysis via whole exome sequencing identified a homozygous variant NM&#95;001282717.2: c.808C>T in the MCM8 gene, located on exon 8 of chromosome 20, inherited in an autosomal recessive manner. The scarcity of primary ovarian insufficiency cases linked to MCM8 highlights the necessity of thoroughly investigating the genetic and clinical consequences of such variants., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Objective: Duplex kidney is a relatively frequent form of urinary system abnormality. This study aimed to elucidate the value of chromosomal microarray analysis (CMA) and whole exome sequencing (WES) for duplex kidney and the perinatal outcomes of duplex kidney fetuses., Methods: This retrospective cohort study included 63 patients with duplex kidney diagnosed using antenatal ultrasound between August 2013 and January 2023. We reviewed the clinical characteristics, genetic test results, and pregnancy outcomes of the patients., Results: Among the 63 cases based on the inclusion criteria, the CMA detected seven (11.1%) clinically significant variants and nine variants of uncertain significance (VUS), and the pathogenic/likely pathogenic (P/LP) copy number variations (CNVs) in the recurrent region that were associated with prenatal duplex kidney included 17q12, 17p13.3, and 22q11.2. No significant disparity was observed in the CMA detection rate between the unilateral and bilateral groups, or between the isolated and non-isolated groups. WES identified three (50%) P/LP single-gene variants in six fetuses with duplex kidney. We detected the following pathogenic genes in the duplex kidney fetuses: KMT2D, SMPD4, and FANCI. Pregnancy termination in cases where clinically significant variants were detected by genetic testing was different in statistical significance from that in cases with negative results (9/10, 90.0% vs 8/48, 16.7%, P < 0.001)., Conclusion: This study elucidated the value of CMA and WES for fetal duplex kidney, proving that CMA and WES may be useful tools in prenatal diagnosis and genetic counseling., (© 2024 International Federation of Gynecology and Obstetrics.)

Background: 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., Methods: 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., Results: 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., Conclusion: 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., Study Type: Retrospective., Level of Evidence: IV., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Robert's uterus is a rare Müllerian duct anomaly (MDA) characterized by complete asymmetric septum dividing uterine cavity into two non-communicating parts. This study offers insights into diagnosing and management of this condition in a young patient, and a systematic literature review. The review included 19 studies from 2013 to 2023. 11.4% of women reported a history of miscarriage and 5.7% primary infertility. Dysmenorrhea was the most frequent symptom (54.3%), and 14.3% of patients had a coexisting endometriosis. The surgical interventions most commonly combine laparoscopic and hysteroscopic techniques. Robert's uterus requires heightened clinical awareness for early diagnosis, particularly in young women., (© 2024 Wiley Periodicals LLC.)

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., (© 2024 The Authors. Acta Obstetricia et Gynecologica Scandinavica published by John Wiley & Sons Ltd on behalf of Nordic Federation of Societies of Obstetrics and Gynecology (NFOG).)

Objective: To demonstrate the surgical approach for Müllerian agenesis with bilateral uterine remnants containing functional endometrium., Design: Stepwise demonstration of the technique with narrated video footage., Setting: Reproductive surgery unit of a tertiary university hospital., Patient: An 18-year-old adolescent was admitted to a tertiary university hospital with complaints of primary amenorrhea and cyclic pelvic pain. Physical examination and magnetic resonance imaging scans suggested a complex Müllerian abnormality. The patient had uterine remnants with bilateral functional endometrium and cervicovaginal agenesis., Intervention: An operation was planned to reconstruct her anatomy by providing a neovagina and anastomosing the uterine remnants. Gonadotropin-releasing hormone analogs were prescribed to suppress her menstruation until the procedure. The operation was performed in the third month after the initial diagnosis. A laparoscopy was conducted, revealing approximately 5 × 6-cm bilateral uterine horns with healthy adnexa. As the first step, a neovagina was created using a modified peritoneal pull-down technique, a standard approach in our clinic. A vaginal incision was made, and a blind vaginal dissection was performed to reach the peritoneum vaginally. Subsequently, an acrylic vaginal mold was inserted. The vaginal orifice was laparoscopically incised using ultrasonic energy with guidance from the inserted vaginal acrylic mold. The orifice was gradually dilated with larger molds. The entire pelvic peritoneum was dissected circularly, and the distal part of the dissected peritoneum was pulled down using four 2.0 Vicryl sutures at 0°, 90°, 180°, and 270° from the opened vaginal orifice. The uterine cavities of both remnants were incised, and two separate Foley catheters were placed in both cavities. A mold with a hole was used to insert the catheters through the vagina. Both catheters were secured in the cavities with Prolene sutures pulled up from the anterior abdominal wall. The next step involved uterine anastomosis. The uterine remnants were unified through continuous suturing, resulting in the formation of a normally shaped uterus. In the final step, the created uterus and neovagina were anastomosed. The patient received instructions on how to perform mold exercises and follow-up care., Main Outcome Measure: Description of laparoscopic management of a rare Müllerian abnormality., Results: The postoperative magnetic resonance imaging scan at 1 month revealed healed unified uterine cavities and vagina. The patient experienced spontaneous menstruation in the second month after surgery and now maintains regular menses with an approximately 9-10 cm functional vagina. Within 3 months after surgery, the visual analogue scale scores for chronic pelvic pain and dysmenorrhea decreased from 9 to 2-3., Conclusions: Müllerian abnormalities are exceptionally rare, and their spectrum is broad, making it challenging to identify an exact surgical method to restore functional anatomy. Therefore, a customized surgical approach should be designed for each patient on the basis of their unique condition., Competing Interests: Declaration of Interests G.U. has nothing to disclose. I.K. has nothing to disclose. K.A. has nothing to disclose., (Copyright © 2024. Published by Elsevier Inc.)

Study Objective: To define objective and readily applied diagnostic criteria for Y-shaped uteri using 3-dimensional transvaginal ultrasound (3D-TVUS) volume recordings., Design: A retrospective case-control diagnostic measurements study., Setting: A tertiary university hospital's reproductive health and research center., Patients: Two hundred thirteen patients who presented with infertility between January 2020 and December 2022., Interventions: Two blinded physicians re-evaluated the 3D-TVUS images of all patients for the presence of a uterine abnormality. The images of patients with a Y-shaped uterus were re-evaluated, and descriptive measurements were done., Measurements and Main Results: The most common uterine abnormality in this cohort was the Y-shaped uterus with a prevalence of 12.2%, followed by the arcuate, T-shaped, and septate uteri, respectively. Reviewers identified 26 and 28 Y-shaped uteri, respectively, and came to a consensus on 25 images. Diagnostic criteria for the Y-shaped uterus based on 3D-TVUS findings have been specified as: lateral indentation depths between 4 and 7 mm, fundal indentation depth between 5 and 9 mm, lateral indentation angles between 121° and 149°, fundal indentation angle between 121° and 145°, and Y-angles 25° to 46°., Conclusion: Some subtypes of dysmorphic uteri, such as the Y-shaped uterus, whose clinical significance is unknown and may be variants of the normal, may actually be more common than thought. Determining objective diagnostic criteria with 3D-TVUS will allow standard research on these anomalies and their clinical significance., (Copyright © 2024 AAGL. Published by Elsevier Inc. All rights reserved.)

Malformations of the fetal kidneys and urinary system are common and easily visualized and diagnosed on ultrasound. This article presents the typical sonographic findings of these abnormalities during the various stages of pregnancy. Because malformations of the urogenital tract often have an association with genetic diseases/ciliopathies, these are also discussed. To complete the article, we provide a brief overview of the normal anatomy of the kidneys and urinary system. The normal anatomy and malformations of the genitalia will not be discussed in this article due to their complexity., Competing Interests: Declaration of financial interestsReceipt of research funding: no; receipt of payment/financial advantage for providing services as a lecturer: no; paid consultant/internal trainer/salaried employee: no; patent/business interest/shares (author/partner, spouse, children) in company: no; patent/business interest/shares (author/partner, spouse, children) in sponsor of this CME article or in company whose interests are affected by the CME article: no.Declaration of non-financial interestsThe authors declare that there is no conflict of interest., (Thieme. All rights reserved.)

This pictorial essay focuses on ultrasound (US) and magnetic resonance imaging (MRI) features of fetal urogenital anomalies. Fetal urogenital malformations account for 30%-50% of all anomalies discovered during pregnancy or at birth. They are usually detected by fetal ultrasound exams. However, when ultrasound data on their characteristics is insufficient, MRI is the best option for detecting other associated anomalies. The prognosis highly depends on their type and whether they are associated with other fetal abnormalities., (© 2024 Japanese Teratology Society.)

Objective: To discuss several techniques of hysteroscopic surgery for complete septate uterus., Case Report: A 40-year-old female with unexplained primary infertility was diagnosed with complete septate uterus with septate cervix. Hysteroscopic incision of complete septate uterus was performed by using ballooning technique. The patient conceived naturally shortly after the operation and delivered a healthy, term infant., Conclusion: Hysteroscopic incision of complete septate uterus is a safe and prompt way of metroplasty. With the knowledge obtained from a pre-operative MRI, it can be completed without laparoscopy and the need for hospitalization., Competing Interests: Conflicts of interest The authors have no conflict of interest to declare., (Copyright © 2024. Published by Elsevier B.V.)

Objective: This study aimed to determine the effect of nonreassuring fetal heart rate (NRFHR) patterns in labor on the postnatal renal function of neonates with a prenatal diagnosis of congenital anomalies of the kidney and urinary tract (CAKUT)., Study Design: A retrospective cohort study was conducted in a single tertiary referral center between 2012 and 2020. All cases with a prenatal diagnosis of CAKUT were extracted, and their fetal, maternal, obstetrical, and neonatal characteristics were analyzed. Cases of multiple gestations, preterm delivery, small for gestational age, major associated malformations or genetic aberrations, and prelabor acute obstetrical events were excluded from the analysis. The study group was comprised of patients who experienced NRFHR during labor. The control groups included (1) patients who had a trial of labor with a normal fetal heart rate pattern and (2) patients who delivered by elective cesarean section (CS). The primary outcome was abnormal serum creatinine levels in the perinatal period. For statistical purposes, the CAKUT cases were classified into a low and high estimated risk for an abnormal postnatal renal outcome. A subgroup analysis of the results was performed accordingly., Results: Two hundred and fifty-six fetuses diagnosed prenatally with CAKUT comprised the study group. Among these, 214 women (83%) opted for a labor trial, while 42 (17%) chose elective CS. Within the labor trial group, 21/214 patients (9.8%) experienced NRFHR during labor. Analysis of maternal and fetal characteristics revealed no statistically significant disparities between the groups. NRFHR patterns were not associated with a deterioration in neonatal serum creatinine compared with those with normal fetal monitoring or those born by an elective CS., Conclusion: NRFHR patterns during labor and delivery did not impair neonatal renal function status in fetuses diagnosed prenatally with low- and high-risk CAKUT. Delivery can be managed according to standard obstetrical guidelines., Key Points: · Kidneys affected by CAKUT commonly display renal dysplasia and a reduction in nephron mass.. · Low Apgar scores and asphyxia are recognized as risk factors for perinatal acute kidney injury.. · Lack of research on NRFHR impact on perinatal renal function in prenatally diagnosed CAKUT.. · NRFHR patterns during labor did not impair neonatal renal function status in prediagnosed fetuses.., Competing Interests: None declared., (Thieme. All rights reserved.)

Clinical Issue: Congenital anomalies of the urogenital tract are common, whereas isolated developmental disorders of the genital organs are rare and varied. The type and timing of imaging to characterize these developmental disorders and the planning of any treatment are the subject of discussion. The aim of this article is to provide a brief overview of embryology, diagnostic procedures, and examples of findings., Standard Radiological Methods: The primary procedure for imaging the genitals is sonography, which must be performed with appropriate empathy. In addition to the genitals, the general situation of the kidneys and urinary tract should be examined, as urogenital malformations are often combined. Dedicated magnetic resonance imaging (MRI) is indicated as advanced imaging in interdisciplinary consultations. Invasive procedures, such as genitography combined with micturition cystourethrography (MCU) using X‑rays or contrast-enhanced sonography, are rarely required. Computed tomography (CT) or excretory urography play no role., Conclusion: Imaging plays an important role in the assessment of genital anomalies. Knowledge of embryology facilitates anatomical understanding and the assessment of pathologies., (© 2024. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Objective: To demonstrate the surgical techniques for improving safety in robotic-assisted abdominal cerclage in patients with bicornuate uteri complicated by recurrent pregnancy loss and cervical insufficiency., Design: Stepwise demonstration with narrated video footage., Setting: An academic tertiary care hospital., Patients: Our patient is a 22-year-old G2P0020 with a history of recurrent pregnancy loss. During her first pregnancy, she was asymptomatic until 19 weeks and delivered because of a preterm premature rupture of membranes. A transvaginal cerclage was performed for her second pregnancy at 14 weeks, which ended at 16 weeks because of preterm premature rupture of membranes. The final magnetic resonance imaging report noted a "bicorporeal uterus with duplication of the uterine body, resulting in two markedly divergent uterine horns that are fused at the isthmus... unlike a typical didelphic uterus, a single, non septated cervix is noted, which shows normal appearances, measuring 3.8 cm in length." Given her history of a uterine anomaly and recurrent pregnancy loss in the absence of other biochemical factors, her maternal-fetal medicine specialist referred her to us as the patient strongly desired future viable pregnancies. The patient was counseled on multiple alternatives, including different methods of performing the cerclage, and ultimately decided on the robotic-assisted (Da Vinci Xi) prophylactic abdominal cerclage., Interventions: The bicornuate uterus is a rare class IV mullerian duct anomaly caused by the impaired fusion of the mullerian ducts in the uterus, classically appearing in imaging studies as a heart-shaped uterus. This patient demographic reports a high incidence of obstetric complications. Pregnancy in such a uterus causes complications like first- and second-trimester pregnancy loss, preterm labor, low-birthweight infants, and malpresentation at delivery. 1 Researchers have postulated that there is an abnormal ratio of muscle fibers to connective tissue in a congenitally abnormal cervix. During pregnancy, an inadequate uterine volume may lead to increased intrauterine pressure and stress on the lower uterine segment, which can lead to cervical incompetence. 2 To address cervical incompetence, cervical cerclages are a commonly utilized procedure, as recent studies demonstrate that the incidence of term pregnancies in the group with documented cervical incompetence treated with cerclage placement increased from 26% to 63%. 3 One observational study noted improved obstetrical outcomes occurred with interval placement, a cerclage placed in between pregnancies in the nongravid uterus, compared with cerclage placement between 9 and 10 weeks gestation, with the mean gestational age for delivery at 32.9 weeks and 34.5 weeks when a cerclage was placed in gravid and nongravid women, respectively. 4 In addition, another retrospective study was done, which demonstrated a lower incidence of neonatal death with prophylactic cerclages. 5 Operating on a nonpregnant uterus offers several benefits, including its reduced size, fewer and smaller blood vessels, and simplified handling. Moreover, there are clearly no concerns regarding the fetus. In the decision to use a robotic-assisted platform vs. laparoscopic, a systematic review showed the rates of third-trimester delivery and live birth (LB) using laparoscopy during pregnancy were found to be 70% and 70%-100%, respectively. The same review demonstrated slightly improved outcomes via the robotic route regarding gestational age at delivery (median, 37 weeks), rates of LB (90%), and third-trimester delivery (90%). 6 Additional factors contributing to the preference for robotics in surgical procedures include incorporating advanced tools, which can enhance the robotic system's advantages compared with traditional laparoscopy. An invaluable tool in this context is the simultaneous utilization of Firefly mode, which employs a near-infrared camera system, achieved through injecting indocyanine green dye or integrating other light sources concurrently. The intravenous administration of indocyanine green is acknowledged widely for its safety and efficacy as a contrast agent in the evaluation of microvascular circulation and organ vascularization. This property equips surgeons with heightened precision when guiding the needle, proving especially advantageous when faced with challenges in visualizing vascular anatomy. In our specific case, we harnessed the capabilities of Firefly mode in conjunction with hysteroscopic light, enabling us to vividly illustrate the contours of a bicornuate uterus from both external and internal perspectives. We demonstrate a simplified technique of the abdominal cerclage, one cerclage around the internal cervical os of the uterus, using a robotic-assisted platform in a nongravid patient. The surgery began with the eversion of the umbilicus, and a 15-mm skin incision was made in the umbilicus. A Gelpoint mini advanced access site laparoscopy device was inserted into the incision, and CO 2 was allowed to insufflate the abdominal cavity with careful attention given to intraabdominal pressure. Once the DaVinci was docked, the surgeon began the creation of a bladder flap. The bladder was carefully dissected from the lower uterine segment and both uteri using monopolar scissors. The anatomical differences of a bicornuate uterus prompted the surgeon to dissect a wider circumference for safety reasons, where a wider dissection offers a better view of the uterine vessels and ease of introducing the Mersilene tape later on. Bilateral uterine vessels were further skeletonized and exposed anteriorly using blunt dissection and monopolar scissors. After further dissection and lateralization, the final result creates a landmark medial to the right uterine vessels at the level of the internal cervical os with which the needle of the Mersilene tape will be able to pass through. The Mersilene tape was guided from anterior to posterior via a previously straightened needle. Similarly, a landmark was created on the left, and the Mersilene tape was directed from anterior to posterior. The Mersilene tape was placed circumferentially around the internal cervical os of the bicornuate uterus, medial to the uterine vessels. Both ends of the Mersilene tape were then gently pulled, ensuring that the tape was lying flat on the anterior of the internal cervical os with no bowels or uterine vessels within it. The tape was then tied posteriorly at the 6 o'clock position with appropriate tension. A 2-0 silk was then sutured to the tails of the tape using the purse-string technique to ensure that it would remain securely tied and in the correct position. Hemostasis was assured. Both a hysteroscopy and a cystoscopy were done after the completion of the cerclage to ensure that no tape or sutures were seen within the cervical canal or the uterine cavity. None were observed., Main Outcomes Measures: The success criteria for the surgery were identified as the patient's ability to attain a viable pregnancy after the cerclage placement, along with achieving LB., Results: Subsequently, a spontaneous pregnancy was achieved. An infant weighing 3 pounds and 16 ounces was delivered by cesarean section at 36 weeks because of an oligohydramnios. The infant is currently healthy at 13 pounds., Conclusion: Robotic-assisted abdominal cerclage around the internal cervical os in a bicornuate uterus offers a possibly feasible and straightforward technique for surgeons seeking to reduce risks, although further research is needed., (Copyright © 2024 American Society for Reproductive Medicine. Published by Elsevier Inc. All rights reserved.)

Congenital anomalies of the kidney and urinary tract are collectively one of the most commonly diagnosed antenatal conditions. Clinicians have several tools available to diagnose anomalies, including imaging, biomarkers, family history and genetic studies. In certain cases, antenatal interventions such as vesico-amniotic shunting may be considered to improve postnatal outcomes.Congenital kidney anomalies detected antenatally can vary in clinical significance from almost no impact postnatally to significant morbidity and perinatal mortality. Prognosis broadly depends on kidney size, structure and amount of amniotic fluid, alongside genetics and family history, and progression on subsequent scans. It is important to counsel parents appropriately using a parent-focused and personalised approach. The use of a multidisciplinary team should always be considered., (© 2023. The Author(s).)

Objective: Study the incidence of genitourinary malformations combined with microtia, find the regularity of the disease, and analyze the correlation between them., Methods: There is a total of 3143 microtia patients from January 2015 to December 2019 in Plastic Surgery Hospital. We examined their renal ultrasound, counted cases with genitourinary system deformities, and calculated the incidence of the disease., Results: Among 3143 patients with congenital microtia, there were 163 patients with congenital genitourinary system malformations, accounting for 5.2%. The side of congenital microtia is associated with the side of congenital genitourinary malformations., Conclusions: Genitourinary malformations occur more frequently in patients with microtia than in the general population. The 2 malformations are more likely to be ipsilateral. We believe it is necessary to complete the systemic physical and renal ultrasound examination for patients with microtia., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by Mutaz B. Habal, MD.)

We present a rare case report of a patient with a congenital uterine anomaly - uterus didelphys. Who spontaneously became pregnant and completed the pregnancy successfully after peripartum hysterectomy of one uterus. The reason for the peripartum hysterectomy was a life-threatening hemorrhage with hemoperitoneum after cesarean delivery.

Fetal genitourinary anomalies can present a diagnostic challenge for the radiologist. The absence of a normally located kidney may represent agenesis or be secondary to a fusion or migration abnormality. A dilated renal pelvis should prompt evaluation for a specific cause, including ureteropelvic junction obstruction, reflux, or an obstructed duplicated system. Cystic parenchymal changes are characteristic of a multicystic dysplastic kidney but may also be seen in obstructive cystic dysplasia. There are numerous causes of megacystis including chromosomal (trisomy 18 syndrome), obstruction (posterior urethral valves, urethral atresia), or muscular dysfunction (prune belly syndrome, megacystis microcolon intestinal hypoperistalsis syndrome). Important mimics of a large bladder include hydrocolpos and urogenital sinus or cloacal malformation. Complications of genitourinary malformations are common and include oligohydramnios, urinary ascites, and urinoma. Making an accurate diagnosis often requires additional US views beyond those obtained in the standard fetal survey and occasionally performing fetal MRI. The appropriate use of orthogonal T2-weighted sequences, in conjunction with diffusion-weighted images for evaluation of the kidneys and gradient-recalled-echo sequences for evaluation of T1-hyperintense meconium in the colon, can play an integral role in diagnosis. Accurate diagnosis of fetal genitourinary malformations is vital to direct patient counseling and pregnancy management as outcomes are highly variable. Some conditions can be surgically corrected quite simply, some require multiple complex procedures, and some are lethal. The authors offer troubleshooting tips to narrow the differential diagnosis for four observations: unilateral absent kidney, dilated renal pelvis, cystic renal parenchyma, and megacystis and its mimics. © RSNA, 2023 Test Your Knowledge questions are available in the Online Learning Center.

Abstract: Primary vaginal calculi are uncommon in children. Urethral duplication in females is seen to occur in association with complex congenital malformations. We report the case of perianal persistent urogenital sinus with a hypertrophied clitoris with phallic urethra, scrotum-like pouch, uterus didelphys with obstructed hemivagina, and giant colpolithiasis in 46XX female. A 16-year-old presented with pain abdomen and cyclic passage of blood clots per rectum. She had a tender lump in left iliac region, a phallus like protrusion and a ruggous sac below it. Vaginal opening was absent. Computed tomography showed two uterine horns with a separate cervix and distended non-communicating hemivaginas with a large calcified oval mass in the left hemivagina. On exploration, calculus was extracted from the left hemivagina. The large calculus found in the left hemivagina appears to be the cause of all presenting symptoms. It obstructed the left hemivagina, filling the left uterine horn with menstrual blood causing its gradual enlargement and secondary infection. The early diagnosis and prompt referral of such an anomaly can only be ensured in institutional deliveries. For a significant proportion of newborns in the developing world, the ability to afford or even be referred to institutes which deal with such cases is a luxurious affair. We hope to bridge bridging the knowledge, attitude and practice gap that exists in our health-care system with this report., (Copyright © 2023 Copyright: © 2023 African Journal of Paediatric Surgery.)

Genital anomalies are a heterogeneous group of congenital pathologies that have become increasingly relevant since the Chicago Consensus of 2005. Their postnatal diagnosis has developed significantly in the last two decades, while prenatal diagnosis seems to be underdeveloped, with few protocols available, fragmented scientific literature, and low diagnostic rates. This review aims to examine the current status of this subspecialty from the perspective of prenatal imaging. Indications for the evaluation of fetal genitalia can be divided into medical and non-medical reasons. Medical reasons include sex-linked disorders, detection of other anomalies, relevant family history, or multiple pregnancy. Non-medical reasons include parental request for sex disclosure. Disclosure of fetal sex may be associated with ethical, legal, and medical issues. The main imaging technology used is 2D ultrasound, although there are other complementary techniques such as 3D, MRI, or Color Doppler. Regarding working methodology, several authors have drawn attention to the lack of standardized protocols and guidelines. Most guidelines tend to limit their recommendations to study indications and ethical issues. Technical proposals, measurements, or working methods have not yet been standardized. Fetal sex determination is usually divided into early and late gestation. Early gestation is based on the sagittal sign. Late gestation is based on direct visualization. There are several measurements to describe male and female genitalia, such as penile length, bilabial diameter, or scrotal diameter. Prenatal diagnosis of genital pathologies presents some particularities such as the wide spectrum of phenotypes, the high frequency of associated deformities, or the time of diagnosis. Some of the most frequent pathologies are ambiguous genitalia, fetal sex discordance, hypospadias, micropenis, clitoromegaly, ovarian cysts, hydro(metro)colpos, and cloacal anomalies. Higher-quality studies and direction from scientific societies through the implementation of clinical guidelines are needed., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Background: Robert's uterus is a rare congenital anomaly, characterized as an asymmetric septate uterus that has a blind hemicavity with unilateral menstrual fluid retention and a unicornuate hemicavity connecting to the cervix unimpededly. Patients with Robert's uterus generally present with menstrual disorders and dysmenorrhea, and some may have reproductive problems as well, including infertility, recurrent miscarriage, preterm labor and obstetric complications. In this case, we describe a successful pregnancy implanted on the obstructed hemicavity and delivered a liveborn girl. Meanwhile, we highlight diagnostic and therapeutic difficulties in patients with atypical symptoms of Robert's uterus., Case Presentation: A 30-year-old Chinese primigravida sought for emergency treatment at 26 weeks and 2 days of gestation because of preterm premature rupture of membranes (PPROM). At the age of 19, the patient was misdiagnosed with hyperprolactinemia and pituitary microadenoma for showing symptom of hypomenorrhea and was suspected to have a uterine septum in the first trimester. She was diagnosed with Robert's uterus at 22 weeks of gestation by repetitious prenatal transvaginal ultrasonography, which was subsequently confirmed by magnetic resonance imaging. At 26 weeks and 3 days of gestation, the patient was suspected to have oligohydramnion, irregular uterine contraction, and umbilical cord prolapse, and she expressed a strong will of saving the baby. Emergency cesarean delivery was performed and a small hole, together with several weak spots, was found at the lower and back wall of the septum of the patient. The treatment was effective and both the mother and the infant, who had an extremely low birth weight, were discharged in good health conditions., Conclusions: Pregnancy in the blind cavity of Robert's uterus with living neonates is incredibly rare. In our case, the favorable outcome may result from the unusual hole found at the septum, which may play a role in communicating amniotic fluid between the two hemicavities so to keep the neonate alive. we highlight the importance of early diagnosis and pre-pregnancy treatment of this uterine malformation, and the timely termination of pregnancy, for improving birth quality and reducing mortality., (© 2023. The Author(s).)

An Indian wonen at her second pregnancy, with a previous preterm labour at 34.5 weeks, presented to the emergency room at 15.6 weeks, shocked due to massive hemoperitoneum. During the urgent surgery, a Mullerian malformation was detected, and a uterine rupture was diagnosed as the cause of the hemoperitoneum. Ultrasound and pelvic magnetic resonance results confirmed the malformation, determining that a unicornuate uterus with a rudimental horn within the pregnancy had taken place. We decided to report our case to underline that Mullerian malformation must be taken into consideration and evaluated at each routinary gynaecological visit or, at least, at the first pregnancy appointment, especially in the cases of women with previous adverse obstetric outcomes. The 2D abdominal ultrasound associated with trans-vaginal evaluation is an adequate procedure to test for suspected uterine malformations (if the operator keeps it in mind and is trained to check this kind of alteration), which is fundamental to reducing the risk of life-threatening events., (© 2022. Società Italiana di Ultrasonologia in Medicina e Biologia (SIUMB).)

Objectives: To study the reproductive outcomes of women with a unicornuate uterus and compare them to those of women with no congenital uterine anomaly., Methods: This was a single-center, retrospective cohort study. Cases were women aged at least 16 years who were diagnosed with a unicornuate uterus on transvaginal/transrectal ultrasound between January 2008 and September 2021. Controls were women with no congenital uterine anomaly matched 1:1 by age and body mass index. The primary outcome was live-birth rate. Secondary outcomes were pregnancy loss (miscarriage, ectopic pregnancy, termination of pregnancy), preterm delivery, mode of delivery and concomitant gynecological abnormalities (endometriosis, adenomyosis, fibroids)., Results: Included in the study were 326 cases and 326 controls. Women with a unicornuate uterus had a significantly lower live-birth rate (184/388 (47.4%) vs 229/396 (57.8%); P = 0.004) and higher rates of overall miscarriage (178/424 (42.0%) vs 155/465 (33.3%); adjusted odds ratio (aOR), 2.21 (95% CI, 1.42-3.42), P < 0.001), ectopic pregnancy (26/424 (6.1%) vs 11/465 (2.4%); aOR, 2.52 (95% CI, 1.22-5.22), P = 0.01), preterm delivery (45/184 (24.5%) vs 17/229 (7.4%); aOR, 3.04 (95% CI, 1.52-5.97), P = 0.001) and Cesarean delivery (116/184 (63.0%) vs 70/229 (30.6%); aOR, 2.54 (95% CI, 1.67-3.88), P < 0.001). Rudimentary-horn pregnancies accounted for 7/26 (26.9%) ectopic pregnancies in the study group. Women with a unicornuate uterus were more likely to have endometriosis (17.5% vs 10.7%; P = 0.018) and adenomyosis (26.7% vs 15.6%; P = 0.001), but were not more likely to have fibroids compared with controls. Women with a functional rudimentary horn were more likely to have pelvic endometriosis compared to those without (odds ratio, 2.4 (95% CI, 1.4-4.1), P = 0.002)., Conclusions: Pregnant women with a unicornuate uterus should be classified as high risk. Removal of a functional rudimentary horn should be discussed with the patient to prevent a rudimentary-horn ectopic pregnancy. © 2022 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology., (© 2022 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.)

Complex female genitourinary system anomalies include a wide spectrum of uncommon pathologies, caused from the abnormal separation of the urorectal septum and the urogenital sinus in early embryonic life. The resulting fusion of the distal urinary, genital and intestinal tracts increases the risk of death in utero and alters the normal organ functionality and the quality of life in survivors. An accurate prenatal identification of these pathologies depends mainly on prior suspicion at ultrasound screening, but also requires a solid knowledge of embryology and familiarity with the different patterns of malformation. Prenatal MRI provides an excellent anatomic evaluation of the fetal anatomy that may improve the diagnosis in complex cases with inconclusive echographic findings. The additional information can help both families and medical teams to better evaluate the severity of the pathology and the postnatal prognosis and therefore to better orientate the management during pregnancy, at delivery and after birth. This review article describes the embryological basis and the clinical findings of the most relevant pathologies included in the spectrum. It also describes the imaging signs on prenatal MRI studies in a series of confirmed cases and proposes a diagnostic algorithm based on imaging findings for guiding prenatal diagnosis., (© 2022 John Wiley & Sons Ltd.)

Purpose of Review: This review summarizes the pathway of Mullerian and Wolffian duct development, anomalies that result from disruptions to this pathway, and the characteristics on advanced imaging that identify them., Recent Findings: In-office evaluation for reproductive anomalies is usually inadequate for the diagnosis of congenital reproductive anomalies. Magnetic resonance imaging (MRI) has usurped invasive diagnostic methods including laparoscopy, hysteroscopy, and vasography as the new gold standard. Because of its superior soft-tissue delineation and the availability of advanced functional sequences, MRI offers a sophisticated method of distinguishing reproductive anomalies from one another, characterizing the degree of defect severity, and evaluating for concomitant urogenital anomalies non-invasively and without radiation exposure to the patient. Congenital anomalies of the Mullerian and Wolffian duct can be incredibly nuanced, requiring prompt and accurate diagnosis for management of infertility. Definitive diagnosis should be made early with MRI., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Background: Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital Mullerian duct anomaly disease that is characterized by a triad of symptoms, didelphys uterus, blind hemivagina, and ipsilateral renal agenesis. Herein, we reported a case from China., Case Presentation: An 11-year-old patient presented to our hospital with lower abdominal pain and frequent urination. Computed tomography and magnetic resonance imaging revealed hematocolpos, uterine hemorrhage, didelphys uterus, and renal agenesis on the right side. Thus, the patient was diagnosed with HWW syndrome. Laparoscopic combined with transvaginal surgery to remove the vaginal septum, the symptoms of the lesion disappeared after the blood was discharged., Conclusion: Abnormal urination and other symptoms should be carefully examined in adolescent girls with abdominal pain not menarche, since they may be related to reproductive organ development disorders and other diseases. We recommend laparoscopy combined with transvaginal surgery to remove the oblique septum in HWW syndrome, which is rarely reported., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.)