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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.)

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.

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.)

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.)

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_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.)

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.)

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.)

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).)

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.)

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.)

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.)

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.)

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.)

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.)

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: 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.)

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.)

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).)

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.)

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.

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).)

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.)

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.)

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.)

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.)

Background: Zinner syndrome (ZS), the association of congenital seminal vesicle cyst (SVC) and ipsilateral kidney anomalies, is rarely diagnosed in childhood. This study aimed to assess presentation, imaging findings, management, and outcome of pediatric ZS., Methods: Sixteen children with ZS were diagnosed and managed at our hospital from 2003 to 2021. We reviewed the medical records to collect data on initial symptoms, results of imaging studies, complications, operation, and follow-up., Results: Ultrasound was used in all 16 cases as initial diagnostic tool. Fourteen patients were asymptomatic at diagnosis: these were transferred from obstetricians or pediatricians for evaluation of the prenatally or postnatally detected ultrasonic kidney anomalies. SVCs were incidentally noted on ultrasonography. The other two cases initially presented with urinary tract infection (UTI). Kidney anomalies included multicystic dysplastic kidney in 3 and kidney agenesis in 13 patients. Eleven (68.7%) patients had ipsilateral ectopic ureters entering SVC. Four (36.4%) patients had a reflux from urethra into SVC (urethro-cystic reflux) on voiding cystourethrography. Ten (62.5%) patients remained asymptomatic over a mean of 58 months (range, 7-216 months), two patients developed lower urinary tract dysfunction, and five patients had UTIs. Two boys needed SVC removal, and SVC had disappeared in two patients after 2.5-4 years of follow-up., Conclusions: Unilateral kidney hypodysplasia with ectopic ureter inserting into the ipsilateral SVC is a characteristic sign for diagnosis of ZS. In our case series, ZS was mainly asymptomatic. Urethro-cystic reflux was associated with UTIs in young infants. SVC removal was rarely required. A higher resolution version of the Graphical abstract is available as Supplementary information., (© 2022. The Author(s), under exclusive licence to International Pediatric Nephrology Association.)

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare complex female urogenital anomaly, with diverse anatomical presentations. Due to obstruction, most patients with HWW syndrome need to be addressed surgically. The treatment strategy should be tailored to the different anatomical variants of each patient. Therefore, a detailed and comprehensive preoperative evaluation is needed. In this review, we describe the embryology and clinical manifestations of HWW syndrome and discuss and illustrate its diverse preoperative magnetic resonance imaging presentations to guide clinical treatment., (© 2022 Japanese Teratology Society.)

Objectives: To determine the detection rates of hysterosalpingogram (HSG), transvaginal 2D ultrasound (TV 2D US), transvaginal 3D ultrasound (TV 3D US) in diagnosing of incomplete uterine septum (IUS) and significant arcuate uterine anomaly (AUA)., Methods: This retrospective cohort study included patients with infertility or recurrent pregnancy loss (516) that were found to have IUS (317) or significant AUA (199) on diagnostic hysteroscopy (DHS). We compared the detection rate of the various radiological tests in all patients with documented IUS or AUA on DHS., Results: The diagnosis of IUS and AUA was made in 49.7% on HSG, 54.2% on TV 2D US and 69.5% on TV 3D US. When both HSG and TV 2D US or HSG and TV3D US were used, the diagnosis was correct in 67.7% and 82.6% of patients, respectively., Conclusion: Although HSG, TV 2D US, TV 3D US are useful in the screening for IUS/AUA, none of these imaging tests alone or in combination are accurate enough to rule out the diagnosis of such congenital uterine anomalies. In patients with a history of infertility or RPL and negative radiologic tests, DHS is the only reliable method to rule out IUS or significant AUA., (© 2022 International Federation of Gynecology and Obstetrics.)

Congenital anomalies of the kidneys and urinary tract (CAKUT) are some of the most common abnormalities detected on prenatal imaging assessment. It is estimated that CAKUT comprises 20% to 30% of all major birth defects. More than 200 clinical syndromes currently include CAKUT as a component of the phenotype. This chapter outlines the evaluation and management of the most common forms of CAKUT., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Objective: To examine the reliability of three-dimensional ultrasound (3 D-US) in the differentiation between subseptate and arcuate uteri, due to the different associated pregnancy outcomes; refine the existing 3 D-US parameters and evaluate the concordance between 3 D-US and MRI in diagnosing these anomalies., Study Design: This was a prospective cohort study of 455 women suspected of having a Mullerian anomaly. The diagnosis of subseptate, bicornuate or arcuate uterus was made by 3 D-US in 55 women. Two independent examiners manipulated the 3 D-US volume datasets and recorded the internal intercornual distance, indentation length, indentation tip angle, and myometrial wall thickness in the coronal plane of the uterus. Subsequently, 48 women underwent MRI which was used as the reference test for diagnosis. We calculated the degree of correlation between the two ultrasound assessors' 3 D-US measurements using interclass correlation coefficient and as well as a Bland-Altman plot. The mean values of the four parameters were used to create receiver operating characteristic curves for determining the best cutoff values for differentiation between subseptate and arcuate uterui. We used the Cohen's Kappa test to measure the level of agreement between 3 D-US and MRI., Results: There was good interobserver agreement between the two 3 D-US assessors for all four parameters. There was a substantial level of agreement between 3 D-US and MRI in differentiating between bicornuate, subseptate and arcuate uteri with a kappa value of 0.727 (95% CI 0.443-0.856). Distinction between subseptate and arcuate uterus was improved when using an indentation length ≥12.5 mm (AUC 0.99) and indentation tip angle ≤89.25 degrees (AUC 0.97) as cutoffs for diagnosis but not the internal intercornual distance or myometrial wall thickness., Conclusion: 3 D-US evaluation of the coronal view of the uterus can be relied upon to make a noninvasive, accurate differentiation between subseptate and arcuate uteri. The fundal indentation length and indentation tip angle cut offs of ≥12.5 mm and ≤88 mm, respectively were found to be most accurate for distinction. Thus, allowing for individualizing pre-pregnancy management plans and patient-informed healthcare choices.HighlightsThere are no agreed upon criteria for differentiating arcuate from subseptate uteri. Such differentiation is critical for counseling and management due to the substantial difference in pregnancy outcome.We aimed to propose cut off values for ultrasound measurements standardized against MRI diagnostic criteria for accurate differentiation between arcuate and subseptate uteri.We demonstrated substantial agreement between 3D-US and MRI in differentiating between bicornuate, subseptate and arcuate uteri.3D-US evaluation of the coronal view of the uterus is reliable to make an accurate differentiation between subseptate and arcuate uteri.Using the indentation length ≥12.5 mm and indentation tip angle ≤89.25 degrees as parameters to be measured on the coronal view by 3D-US increases its diagnostic accuracy for distinction between arcuate and subseptate uteri.

Congenital genitourinary anomalies are among the most frequent types of birth defects in neonates. Some anomalies can be a significant cause of morbidity in infancy, while others remain asymptomatic even until adulthood and can be at times the only manifestation of a complex systemic disease. The spectrum of these anomalies results from the developmental insults that can occur at various embryologic stages, and an understanding of the formation of the genitourinary system is helpful in the evaluation and treatment of a child with a congenital genitourinary anomaly. Imaging plays an essential role in the diagnosis of congenital genitourinary anomalies and treatment planning. In this article, we highlight the embryologic and characteristic imaging features of various congenital genitourinary anomalies, demonstrate the utility of different imaging modalities in management, and review specific imaging modalities and protocols for image optimization., (© 2021. This is a U.S. government work and not under copyright protection in the U.S.; foreign copyright protection may apply.)

Research Question: The aim of our study was the impact of T-shaped uterine anomaly on in vitro fertilization (per 1 IVF cycle per one embryo transfer) results and pregnancy outcomes., Design: Prospective cohort study included 388 somatically healthy patients at the age of 20-40 years old who underwent 3-dimensional ultrasound of uterine cavity before embryo transfer. The uterine cavities were classified based on morphometric criteria according to the CUME study. The comparison was performed between patients with normal uterine cavity (n = 266), patients with T-shaped anomaly of uterine cavity (n = 27), intermediate forms of T-shaped anomaly (n = 73), and patients with other uterine anomalies (n = 22)., Results: The rates of biochemical pregnancy in Normal, T-shaped, Intermediate and Other groups were 58.6%, 51.9% 52.1% and 45.4%, respectively, but there weren't detected statistically significant differences (P > 0.05)., Conclusions: T-shaped uterus did not affect the percentage of occurring pregnancy, but was associated with increased rate of preterm deliveries, miscarriages and ectopic pregnancy. No differences in occurrence of pregnancy rates in cases of intermediate types, but an increased number of preterm deliveries and miscarriages in cases with increased myometrial thickness in lateral angle area., Competing Interests: Declaration of Competing Interest The authors declare that there is no conflict of interest, (Copyright © 2021. Published by Elsevier Masson SAS.)

Congenital uterine malformations occur as a result of a defective uterine development. The formation of the uterus is a dynamic process that culminates after completing four phases (differentiation, fusion, migration and canalization). Because is an evolutionary process, the resulting uterine malformation is different depending on the uterine formation phase in which the failure occurs. To establish a correct diagnosis of uterine malformations, it is necessary to evaluate both the shape of the uterine cavity and the external contour of the uterus. In this review we will describe the imaging modalities used in current clinical practice for the evaluation of the patient with suspected congenital uterine anomalies. The current role of each modality is described.

Competing Interests: Competing interests: None declared.

Several congenital heart diseases (CHD) are present in children with congenital anomalies of the kidney and the urinary tract (CAKUT) in syndromic or nonsyndromic patterns. The purpose of this study was to identify nonsyndromic children with CAKUT who required echocardiographic evaluation and to find the prevalence of CHD in these patients. Retrospective chart review of nonsyndromic patients with CAKUT between 2012 and 2018 was conducted. Types of congenital anomalies of the kidney and urinary tract and echocardiogram reports of the patients, age, gender, prematurity, prenatal diagnosis of CAKUT, and family history were noted. Among 806 children with CAKUT, 135 patients underwent transthoracic echocardiographic evaluation and CHD was detected in 91 (11.2%). The most common type of CHD was secundum type atrial septal defect that was found in 73 (80.2%) patients. On the other hand, the most frequent types of congenital anomalies of the kidney and the urinary tract were isolated hydronephrosis and primary vesicoureteral reflux (61.4% and 12.8%, respectively). Our results showed that 75.8% of the patients with congenital heart diseases had isolated hydronephrosis. Finally, only 18 (2.2%) cases out of 806 patients that were evaluated via transthoracic echocardiography due to the clinical findings and family history had a CHD that required close (<6 months) follow-up. Congenital heart diseases that required close follow-up are rarely observed in nonsyndromic patients with CAKUT. Therefore, we suggest echocardiography should be performed in cases of clinical suspicion and family history rather than routinely in this patient group., (© 2021 Japanese Teratology Society.)

Objective: To assess the prevalence and morphological appearance of deep endometriosis and ovarian endometrioma using pelvic ultrasound examination in women attending for an early pregnancy assessment., Methods: This was a prospective observational study set within a dedicated early pregnancy unit. The study included 1341 consecutive women who attended for an early pregnancy assessment for reassurance or because of suspected early pregnancy complications. All women underwent a transvaginal scan to assess the location and viability of their pregnancy. In addition, a detailed examination of pelvic organs was carried out to detect the presence of endometriosis and other gynecological abnormalities. Data analysis was performed using logistic regression and multivariable analysis., Results: The prevalence of deep endometriosis and/or ovarian endometrioma in women attending our early pregnancy unit was 4.9% (95% CI, 3.8-6.2%). In 33/66 (50.0% (95% CI, 37.9-62.1%)) women with endometriosis, this was a new diagnosis that was made during their early pregnancy scan. On multivariable analysis, the presence of endometriosis was strongly associated with a history of subfertility (odds ratio (OR), 3.15 (95% CI, 1.63-6.07)) and presence of a congenital uterine anomaly (OR, 5.69 (95% CI, 2.17-14.9)) and uterine fibroids (OR, 2.37 (95% CI, 1.31-4.28)). Morphological changes typical of decidualization were seen in 11/33 (33.3% (95% CI, 17.2-49.4%)) women with ovarian endometrioma and 18/57 (31.6% (95% CI, 19.5-43.7%)) women with deep endometriotic nodules., Conclusions: Deep endometriosis and ovarian endometrioma were present in a significant proportion of women attending for early pregnancy assessment. The prevalence varied depending on a history of subfertility, and therefore is likely to differ significantly among populations, depending on their characteristics. Ultrasound is a useful tool for the detection of endometriosis in early pregnancy and the identification of women who may benefit from specialist antenatal care. © 2021 International Society of Ultrasound in Obstetrics and Gynecology., (© 2021 International Society of Ultrasound in Obstetrics and Gynecology.)