Duplicated and dilated ovarian vein with coexistence of persistent mesonephric artery.

During standard cadaveric dissection we encountered multiple vascular variations in the retroperitoneum: duplicated and dilated left ovarian vein with the coexistence of a persistent right mesonephric artery.

Drosophila Mon1 constitutes a novel node in the brain-gonad axis that is essential for female germline maturation.

Monensin-sensitive 1 (Mon1) is an endocytic regulator that participates in the conversion of Rab5-positive early endosomes to Rab7-positive late endosomes. In Drosophila, loss of mon1 leads to sterility as the mon1 mutant females have extremely small ovaries with complete absence of late stage egg chambers - a phenotype reminiscent of mutations in the insulin pathway genes. Here, we show that expression of many Drosophila insulin-like peptides (ILPs) is reduced in mon1 mutants and feeding mon1 adults an insulin-rich diet can rescue the ovarian defects. Surprisingly, however, mon1 functions in the tyramine/octopaminergic neurons (OPNs) and not in the ovaries or the insulin-producing cells (IPCs). Consistently, knockdown of mon1 in only the OPNs is sufficient to mimic the ovarian phenotype, while expression of the gene in the OPNs alone can 'rescue' the mutant defect. Last, we have identified ilp3 and ilp5 as critical targets of mon1. This study thus identifies mon1 as a novel molecular player in the brain-gonad axis and underscores the significance of inter-organ systemic communication during development.

Identification of reproduction-related genes and pathways in the Culter alburnus H-P-G axis and characterization of their expression differences in malformed and normal gynogenetic ovaries.

This study applied RNA-seq technology to discover reproduction-related genes and pathways in female topmouth culter brain (including pituitary) and ovarian tissues. In functional analysis, 2479 and 2605 unigenes in the brain and ovary tissue were assigned to the "reproductive process" subcategory in addition to the 2660 and 2845 unigenes assigned to the "reproduction" subcategory. Twenty-three complete cDNA sequences were identified through the different gene expression (DGE) approach from five reproduction-related pathways (MAPK signaling pathway, neuroactive ligand-receptor interaction pathway, gonadotropin-releasing hormone signaling pathway, oocyte meiosis pathway, and steroid biosynthesis pathway). The expression levels of 16 candidate genes using qPCR in this study were in accordance with the results of transcriptome analysis. In addition, the expression levels of the FSH, 3ß-HSD, PGR, and NPYR genes in malformed gynogenetic ovaries were considerably low, which was consistent with the progress of oocytogenesis in the ovaries of topmouth culter. The high expression of these four genes in the ovaries of normal topmouth culter suggested they might involve in the preparation for the shift of oogenesis to ovulation. Hence, our work identified a set of annotated gene products that are candidate factors affecting reproduction in the topmouth culter H-P-G axis. These results could be essential for further research in functional genomics and genetic editing for topmouth culter reproduction.

MRI of female genital tract congenital anomalies: European Society of Urogenital Radiology (ESUR) guidelines.

OBJECTIVE: To develop imaging guidelines for the MR work-up of female genital tract congenital anomalies (FGTCA). METHODS: These guidelines were prepared based on a questionnaire sent to all members of the European Society of Urogenital Radiology (ESUR) Female Pelvic Imaging Working Group (FPI-WG), critical review of the literature and expert consensus decision. RESULTS: The returned questionnaires from 17 different institutions have shown reasonable homogeneity of practice. Recommendations with focus on patient preparation and MR protocol are proposed, as these are key to optimised examinations. Details on MR sequences and planning of uterus-orientated sequences are provided. CONCLUSIONS: The multiplanar capabilities and soft tissue resolution of MRI provide superb characterisation of the wide spectrum of findings in FGTCA. A standardised imaging protocol and method of reporting ensures that the salient features are recognised, contributing to a correct diagnosis and classification of FGTCA, associated anomalies and complications. These imaging guidelines are based on current practice among expert radiologists in the field and incorporate up to date information regarding MR protocols and essentials of recently published classification systems. KEY POINTS: • MRI allows comprehensive evaluation of female genital tract congenital anomalies, in a single examination. • A dedicated MRI protocol comprises uterus-orientated sequences and vaginal and renal evaluation. • Integration of classification systems and structured reporting helps in successful communication of the imaging findings.

Case report: a Chinese girl with dent disease 1 and turner syndrome due to a hemizygous CLCN5 gene mutation and Isochromosome (Xq).

BACKGROUND: Female Dent disease 1 patients with low-molecular-weight proteinuria (LMWP) due to CLCN5 gene mutation were rarely reported, and these cases that the people were also with Turner syndrome (TS) were even hardly documented before. CASE PRESENTATION: Here we report a 3-year and 11-month old Chinese girl with short stature who had a karyotype of 46,X,i(X)(q10) and a de novo pathogenic variant in the CLCN5 gene on the short arm of X chromosome. Laboratory examinations showed that the patient had LMWP, hypercalciuria, hypophosphatemia, delayed bone age, and genital dysplasia. CONCLUSION: The combination of i(X)(q10) and CLCN5 mutation causes the deletion of the wild-type CLCN5 allele that results in Dent-1 and TS. To the best of our knowledge, this is the first case that a female CLCN5 mutation hemizygote is diagnosed with Dent-1 and Turner syndrome due to isochromosome X. Also, our case has indicated that the prevalence of the situation may be largely underestimated because of the mild signs of females with Dent-1.

Losartan ameliorates ovarian ischaemia/reperfusion injury in rats: an experimental study.

This study aimed to investigate the protective and antioxidant role of losartan in ovarian ischaemia and ischaemia/reperfusion injury in an experimental ovarian torsion model. Thirty adult female rats were used. Rats were separated randomly into five groups; Group 1: sham group (abdominal wall was only opened and closed), Group 2: torsion group with 3-hour ischaemia using atraumatic vascular clips. Group 3: torsion + losartan group with 3-hour ischaemia 30 minutes after the administration of 40 mg/kg of losartan via oral gavage. Group 4: torsion-detorsion group with 3-hour ischaemia and 3-hour reperfusion (vascular clips were removed). Group 5: torsion-detorsion + losartan group with 3-hour ischaemia followed by administration of 40 mg/kg of losartan 30 minutes prior to a 3-hour detorsion/reperfusion. Ovarian tissue damage was scored by histopathological analysis. Ovarian tissue malondialdehyde (MDA) and plasma pentraxin 3 (PTX 3) levels were measured biochemically. In comparison with the sham group, both the torsion and torsion-detorsion groups had significantly higher scores for follicular degeneration, vascular congestion, oedema, haemorrhage, and leukocyte infiltration (p < .05). The aforementioned parameters significantly decreased in the torsion-detorsion + losartan group (p < .01) compared to those in the torsion-detorsion group. MDA and plasma PTX 3 levels were notably higher both in the torsion and torsion-detorsion groups compared with those in the sham group (p < .01). The current experimental ovarian torsion study suggests a protective role for losartan upon ischaemia and ischaemia/reperfusion injury in rat ovaries. Losartan may be a novel agent for decreasing ovarian ischaemia/reperfusion injury in ovaries.Impact statementWhat is already known on this subject? Among gynaecological emergencies, the diagnosis of ovarian torsion is highly difficult. A delayed diagnosis may lead to ovarian necrosis and subsequent loss of ovaries if timely surgical intervention is not performed, which is essential for the fertility and protection of ovarian functions in young patients. However, reperfusion of the ischaemic tissue might leads to more serious damage to the tissue than the damage caused by ischaemia.What the results of this study add? This study found that losartan, an Ang II type 1 receptor blocker which has been currently used for regulation of blood pressure, could be used experimentally to alleviate I/R injury in ovary through improving histological parameters, reducing tissue MDA and plasma PTX3 levels. To date, there is no study regarding the usage of losartan for alleviating I/R on ovary due to torsion.What the implications are of these findings for clinical practice and/or further research? Losartan may be suggested to have therapeutic value in patients with ovarian torsion. Further large clinical studies are necessary to prove the beneficial effect of losartan to prevent I/R injury on human ovaries.

Adamts9 is necessary for ovarian development in zebrafish.

Expression of adamts9 (A disintegrin and metalloprotease with thrombospondin type-1 motif, member 9) increases dramatically in the somatic cells surrounding oocytes during ovulation in vertebrates from zebrafish to human. However, the function of Adamts9 during ovulation has not been determined due to the embryonic lethality of knockouts in mice and Drosophila. To identify the role of Adamts9 during ovulation we generated knockout (adamts9-/-) zebrafish using CRISPR/Cas9 and characterized the effects of the mutation. From 1047 fish generated by crossing adamts9+/- pairs, we found significantly fewer adult adamts9-/- fish (4%) than predicted by Mendelian ratios (25%). Of the mutants found, there was a significant male bias (82%). Only 3 female mutants were identified (7%), and they had small ovaries with few stage III and IV oocytes compared to wildtype (wt) counterparts of comparable size and age. Astoundingly, the remaining mutants (11%) did not appear to have normal testis or ovaries. Instead there was a pair of transparent, ovarian-like membranous shells that filled the abdominal cavity. Histological examination confirmed that shells were largely empty with no internal structure. Surprisingly, seminiferous tubules and various spermatocytes including mature spermatozoa were observed on the periphery of these transparent shells. No female or female like knockouts were observed to release eggs, and no ovulated oocytes were observed in histological sections. To our knowledge, this is the first report of an adamts9 global knockout model in any adult vertebrates and the first description of how gonadal sex and structure are affected- highlighting the importance of Adamts9 during gonadal development and the value of zebrafish as a model organism.

Prepared for the Unexpected: Accessory Ovaries with Abernethy Malformation.

Supernumerary and accessory ovaries are collectively coined ectopic ovaries. These are rarely encountered by the benign gynecologist and are often discovered incidentally during evaluation for other gynecologic, gastrointestinal, or urologic pathologies. We report the presentation of a patient with multiple accessory ovaries in addition to a rare congenital anomaly of the splanchnic vasculature called an Abernethy malformation. Incidental identification of ectopic ovaries necessitates a search for additional malformations outside of the genitourinary tract that can have larger implications for long-term health.

Management of inguinal hernia with prolapsed ovary in very low birthweight infants during neonatal intensive care unit hospitalisation.

AIM: Prolapsed ovary (PO) in an inguinal hernia (IH) may cause torsion and infarction; however, the management of IH with PO in very low birthweight (VLBW) infants during neonatal intensive care unit (NICU) hospitalisation remains inconsistent. METHODS: The medical records of 47 IHs in 30 VLBW infants during NICU hospitalisation between 2008 and 2017 were reviewed retrospectively. RESULTS: Of the 47 IHs, PO was diagnosed in 18 (38.3%). Post-natal age and body weight at diagnosis of IH with PO were 71 (44-172) days and 2120 (1305-2965) g, respectively. Seventeen IHs with PO underwent surgery for correction electively just before discharge from the NICU at the age and body weight of 94 (51-187) days and 2645 (2340-2945) g, respectively. Therefore, the time interval between diagnosis and surgery was 15 (7-90) days. There was no torsion of ovary before surgery and no postoperative complications, including apnoea and recurrence. CONCLUSIONS: The high incidence of IH with PO in VLBW infants during NICU hospitalisation was confirmed in this study. Under careful observation, elective IH repair just before discharge from the NICU could be one acceptable option for the management of IH with PO in VLBW infants during NICU hospitalisation to prevent torsion and infarction of ovary.

Female Reproductive Performance in the Mouse: Effect of Oral Melatonin.

Although melatonin has some of the broadest ranges of actions on the physiology of vertebrates, especially on their reproductive processes, the mechanism by which melatonin regulates animal reproduction is still incompletely understood. This study was designed to determine the effect of oral melatonin on the reproductive performance of female mice. Female ICR mice (7 weeks old) were given melatonin-containing water (3, 30 and 300 µg/mL; melatonin) or water only (control) until 10 weeks of age. Then, some of the mice were successfully mated (confirmed by vaginal plugs), and the number of live births and their weights were recorded. Some mice were used for a histological analysis of the number of follicles in the ovaries. Others were used for oocyte collection after superovulation, and in vitro fertilization (IVF) was performed. The mRNA expression of the apopotosis-related genes (BAX, BCL2) in the IVF embryos were analyzed. After melatonin administration, the mice showed similar serum melatonin levels to that of the control. The number of antral follicles per mm² unit area in the 30 µg/mL melatonin-treated group (14.60) was significantly higher than that of the control (7.78), which was lower than that of the 3 µg/mL melatonin-treated group (12.29). The litter size was significantly higher in the 3 µg/mL melatonin-treated group (15.5) than in the control (14.3). After IVF, the hatched blastocyst formation rate in the 30 µg/mL melatonin-treated group (85.70%) was significantly higher than that of the control (72.10%), and it was the same for the BCL2/BAX expression ratio. Although oral melatonin did not appear to have an effect on the serum melatonin rhythm in the mouse, melatonin did increase litter size at the 3 µg/mL dose level, and improved the developmental competency of IVF embryos at the 30 µg/mL level.

[Pathologic features on gonadal changes of sexual developmental disorders in children].

Objective: To investigate the pathologic features of gonadal tissues of disorders of sexual development (DSD) in children. Methods: Fifty-three cases of gonadal developmental disorders were collected from July 2015 to August 2017 at Guangzhou Women and Children's Medical Center. Clinical manifestations, karyotypes, sex hormone levels, ultrasound imaging, histology and immunophenotype of gonadal tissues were analyzed. Results: The age of patients ranged from 7 months to 17 years with an average of (50.7 ± 47.1) months. Social genders of the patients included 32 males and 21 females. Forty-eight patients had abnormal sex hormone levels. Clinical presentations included: toward female genitalia in 25 cases, male genitalia tendency in 17 cases and ambiguous external genitalia in 11 cases. Hypospadias was seen in 31 cases and short stature was seen in 8 cases. Chromosomal karyotyping of peripheral blood revealed 23 cases of sex chromosome disorders, 22 cases of 46 XY disorders, of which 3 cases were 5α-reductase deficiency and 8 cases of 46 XX disorders. Ultrasound examination showed cryptorchidism in 30 cases, including 16 cases of unilateral, 14 cases of bilateral and 1 case presenting a huge pelvic tumor. A total of 97 gonadal tissues from 53 cases of DSD were examined, including 9 cases of unilateral and 44 cases of bilateral gonads. Microscopically, 55 gonads (56.7%) showed dysplastic testes including 17 unilateral and 19 bilateral gonads. Fourteen were streak gonads (14.4%) including 8 unilateral and 3 bilateral gonadal tissues. Nine streak gonad with epithelial cord-like structures (9.3%) were found, of which 5 were unilateral and 2 were bilateral lesions. Seven gonads were ovotestis (7.2%), unilateral in 5 cases (the other side of the gonads of ovary in 4 cases, 1 case of dysplastic testes) and bilateral in 1 case. Seven gonads showed follicular-rich ovarian tissue (7.2%). One case showed bilateral dysplastic testes with gonadoblastoma and ectopic adrenal cortex. One case of streak gonad showed epithelial cord-like structures and undifferentiated glandular tissue embedded in malignant mixed germ cell tumors (mixed gonadoblastoma, dysgerminoma, mature teratoma and yolk sac tumor). One case had testicular microlithiasis. Uterus and fallopian tube structures were found in 11 cases. Immunohistochemical stains were performed in 15 cases. D2-40, PLAP and CKIT were expressed in germ cells and Calretinin, WT1 and inhibin were positive in Setoli cells. SALL4 and OCT3/4 were positive in 3 cases. Inhibin highlighted interstitial Leydig cells in 2 cases. GPC3 was positive in yolk sac tumor component. Conclusions: Gonadal dysgenesis presents a broad spectrum of gonadal phenotypes with variable degrees of differentiation. The development of bilateral gonadal tissues has certain variability. Chromosomal karyotypes have no correlation with gonadal phenotypes. Accurate histopathologic diagnosis of gonadal dysgenesis plays an important role in the treatment and prognosis of the patient.

Zfrp8 forms a complex with fragile-X mental retardation protein and regulates its localization and function.

Fragile-X syndrome is the most commonly inherited cause of autism and mental disabilities. The Fmr1 (Fragile-X Mental Retardation 1) gene is essential in humans and Drosophila for the maintenance of neural stem cells, and Fmr1 loss results in neurological and reproductive developmental defects in humans and flies. FMRP (Fragile-X Mental Retardation Protein) is a nucleo-cytoplasmic shuttling protein, involved in mRNA silencing and translational repression. Both Zfrp8 and Fmr1 have essential functions in the Drosophila ovary. In this study, we identified FMRP, Nufip (Nuclear Fragile-X Mental Retardation Protein-interacting Protein) and Tral (Trailer Hitch) as components of a Zfrp8 protein complex. We show that Zfrp8 is required in the nucleus, and controls localization of FMRP in the cytoplasm. In addition, we demonstrate that Zfrp8 genetically interacts with Fmr1 and tral in an antagonistic manner. Zfrp8 and FMRP both control heterochromatin packaging, also in opposite ways. We propose that Zfrp8 functions as a chaperone, controlling protein complexes involved in RNA processing in the nucleus.

Unilateral ovarian absence in a Black-headed Squirrel Monkey (Saimiri vanzolinii Ayres, 1985), a threatened neotropical primate species.

Ovarian agenesis is an unusual anomaly with traumatic or congenital origin. In the present case report, we describe our findings in a senile S. vanzolinii female. As this neotropical primate species is listed as vulnerable, with limited geographic distribution in the Brazilian Amazonia, ovarian agenesis may be an important finding to be reported.

Laparoscopic Management of Autoamputated Ovary in Newborns: A Report of 2 Cases.

Intrauterine autoamputation of the ovary is an extremely rare diagnosis in the pediatric population. The current literature is limited to contradictory recommendations, while a standard management protocol for autoamputated adnexa secondary to intrauterine ovarian torsion is yet to be established. We report 2 cases of auto-amputation of the ovary, leading to a free-floating intra-abdominal cyst in the newborn. Laparoscopic management was successful in both cases.

European Society of Paediatric Radiology abdominal imaging task force recommendations in paediatric uroradiology, part IX: Imaging in anorectal and cloacal malformation, imaging in childhood ovarian torsion, and efforts in standardising paediatric uroradiology terminology.

At the occasion of the European Society of Paediatric Radiology (ESPR) annual meeting 2015 in Graz, Austria, the newly termed ESPR abdominal (gastrointestinal and genitourinary) imaging task force set out to complete the suggestions for paediatric urogenital imaging and procedural recommendations. Some of the last missing topics were addressed and proposals on imaging of children with anorectal and cloacal malformations and suspected ovarian torsion were issued after intense discussions and a consensus finding process that considered all evidence. Additionally, the terminology was adapted to fit new developments introducing the term pelvicalyceal dilatation/distension (PCD) instead of the sometimes misunderstood hydronephrosis. The present state of paediatric urogenital radiology was discussed in a dedicated minisymposium, including an attempt to adapt terminology to create a standardised glossary.

Abdominal Pain in the Post-menopausal Female: Is Ovarian Torsion in the Differential?

BACKGROUND: Ovarian torsion is often thought of as a disease process of reproductive-aged women; however, it is also seen in the extremes of age. In post-menopausal women, it can be both a challenge to diagnose and associated with increased morbidity. CASE REPORT: We present a case of a 68-year-old woman with sudden onset of lower abdominal pain 6 h before arrival at the emergency department (ED). She was diagnosed with ovarian torsion, secondary to an ovarian mass, and underwent a full malignancy evaluation. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Given the higher risk of malignancy in post-menopausal women, ovarian mass-related torsion is an uncommon but important cause of acute-onset lower abdominal pain. Due to the lower prevalence of ovarian torsion in the post-menopausal group, delayed or missed diagnosis is common and may increase associated morbidity. This morbidity is due to the increased likelihood of malignancy and complications of associated medical and surgical treatment of the mass. This report highlights the increased malignancy risk, difficulty with diagnosing torsion in the post-menopausal age group, and the rationale for different management strategies when compared to premenopausal women.

Isolated left descended inguinal ovary with ipsilateral ectopic pelvic kidney: a case report and review of literature.

Isolatd descended inguinal ovary is a rarity, but it should be considered as a differential diagnosis of an inguinal mass at any age; in particular during female infancy and up to adulthood. Herein the authors report a case of asymptomatic isolated left descended inguinal ovary, with ipsilateral left ectopic pelvic kidney that was accidentally discovered a few years after menarche.

Bilateral ovarian maldescent: Unusual cause of infertility - A case report and literature review.

Infertility due to ovarian maldescent is extremely rare and diagnosis can be challenging in the absence of uterine anomalies. We present a case of infertility due to bilateral ovarian maldescent with normal uterine anatomy who conceived after in vitro fertilization and did not require removal of the ovaries. A 19-year-old woman presented with primary infertility. After three failed cycles of ovulation induction/intrauterine insemination and one failed cycle of fresh embryo transfer, the patient underwent laparoscopy, which showed bilateral ovaries above the pelvic brim. The patient conceived after second in vitro fertilization and delivered at 34 weeks. Accurate diagnosis of ovarian maldescent in a patient of unexplained infertility and normal uterus is crucial for successful follicular aspiration and infertility treatment. It is important to counsel patients about the benign nature of this rare entity and future fertility.