Mullerian ducts derivatives in abdominal cryptorchism in children

Disorder of sex determination is a condition associated with clinical and biochemical discrepancy between genetic, gonadal, and phenotypic sex of a child requiring detailed examination for final selection of sex. Indications for sex determination can arise both in infancy and during puberty. Several conditions pertaining to abnormal sex differentiation are manifested as normal male genitalia with Mullerian ducts derivatives.The study objective is to present clinical observations of children with persistent Mullerian duct syndrome.The study presents 2 clinical cases of children with disorders of sex determination. Both children were initially hospitalized at the surgical facility with cryptorchism diagnosis, Mullerian duct derivatives were found intraoperatively. During examination of the children (karyotyping, histological and immunohistochemical examination) in one case persistent Mullerian duct syndrome was diagnosed, in the other - chromosomal abnormality of sex formation (45,X/46,XY), mixed dysgenesis of the gonads.If Mullerian duct derivatives are found during diagnostic laparoscopy, resection biopsy of the ambiguous gonad and intraoperative single-step urethrocystoscopy for visualization of the seminal colliculus and catherization of the uterus for its identification from the abdominal side should be performed. Further examination includes karyotyping, hormonal examination, consultations with an endocrinologist and a geneticist, and genetic examination if necessary.

UTERUS DIDELPHYS IN NULLIPAROUS AND MULTIPAROUS WOMEN – A RARE ENTITY

Uterus didelphys is a rare congenital uterine abnormality in which the embryogenetic fusion of the Mullerian ducts fails to occur. It will lead to the formation of a double uterus with two separate cervices and most often a double vagina with a longitudinal septum as well. Here, we present two different cases of uterus didelphys with varied presentations. The first case is a nulliparous woman presented with post-coital bleeding. On examination, two cervical openings with a longitudinal complete vaginal septum were found, conservative management was done. Findings of didelphys uterus were confirmed on USG. The patient was counseled and discharged. The second case is a multiparous woman with previous cesarean delivery, rupture of membranes, and meconium in this pregnancy with term pregnancy taken up for emergency cesarean section. Dense adhesions and a mass on the right side of uterus were found intraoperatively, which on further inspection confirmed to be patent right horn of uterus. Diagnosis of uterus didelphys was made after doing per speculum and per vaginal examination post-cesarean.

The incidence of uterine abnormalities

Background: Congenital uterine abnormalities result from abnormal formation, fusion or resorption of the Mullerian ducts during fetal life. These abnormalities have been associated with an increased rate of miscarriage, preterm birth, and other fetal adverse outcomes. Material and methods: Was performed a clinical observational retrospective study of uterine malformations, diagnosed in the Republic of Moldova. Pelvic MRI (1.5-3 tesla) with contrast and without were examined, from 01.01.2016 to 20.11.2016. During this time, 190 MRIs were performed according to the program, 167 MRIs were included in the study, 23 MRIs were excluded, having total or partial hysterectomy performed. The age of the examined persons is between 81 years and 6 months. Results: 15 uterine malformations were detected, which represent 11.13%. Among the uterine abnormalities were detected 6 cases of bicorn uterus, 3 cases of didelph uterus, 2 cases of uterine agenesis and septate uterus and one case of arcuate uterus, and unicorn. Conclusions: In this study it was determined that the prevalence of uterine malformations in an unselected population in the Republic of Moldova is 11.13%, and that of the septate uterus is 1.2%, data that are similar and correspond to the international literature.

Intraovarian Gestation in Viviparous Teleosts: Unique Type of Gestation among Vertebrates

The intraovarian gestation, occurring in teleosts, makes this type of reproduction a such complex and unique condition among vertebrates. This type of gestation of teleosts is expressed in special morphological and physiological characteristic where occurs the viviparity and it is an essential component in the analysis of the evolutionary process of viviparity in vertebrates. In viviparous teleosts, during embryogenesis, there are not development of Müllerian ducts, which form the oviducts in the rest of vertebrates, as a result, exclusively in teleosts, there are not oviducts and the caudal region of the ovary, the gonoduct, connects the ovary to the exterior. The lack of oviducts defines that the embryos develop into the ovary, as intraovarian gestation. The ovary forms the oocytes which may develop different type of oogenesis, according with the storage of diverse amount of yolk, variation observed corresponding to the species. The viviparous gestation is characterized by the possible intimate contact between maternal and embryonic tissues, process that permits their metabolic interchanges. So, the nutrients obtained by the embryos could be deposited in the oocyte before fertilization, contained in the yolk (lecithotrophy), and may be completed during gestation by additional provisioning from maternal tissues to the embryo (matrotrophy). Then, essential requirements for viviparity in poeciliids and goodeids are characterized by: a) the diversification of oogenesis, with the deposition of different amount of yolk in the oocyte; b) the insemination, by the transfer of sperm to the female gonoduct and their transportation from the gonoduct to the germinal region of the ovary where the follicles develop; c) the intrafollicular fertilization; d) the intraovarian gestation with the development of embryos in intrafollicular gestation (as in poeciliids), or intraluminal gestation (as in goodeids); and, e) the origin of embryonic nutrition may be by lecithotrophy and matrotrophy. The focus of this revision compares the general and specific structural characteristics of the viviparity occurring into the intraovarian gestation in teleosts, defining this reproductive strategy, illustrated in this review with histological material in a poeciliid, of the species Poecilia latipinna (Lesueur, 1821) (Poeciliidae), and in a goodeid, of the species Xenotoca eiseni (Rutter, 1896) (Goodeidae).

Unicornuate Uterus with Rudimentary Horn as a Rare Etiology of Secondary Dysmenorrhea: A Case Report

Congenital uterine anomalies are an uncommon type of female genital malformations caused by abnormal development of müllerian ducts during embryogenesis. Patients with an obstructive uterine anomaly have a higher risk of developing gynecological and obstetric complications that may present at menarche or later in life. We present a case of severe dysmenorrhea in a young teenager caused by obstructive hematometra in a noncommunicating horn of the unicornuate uterus. A differential diagnosis of a possible anomaly was made using 2-dimensional pelvic ultrasonography, which was later confirmed using MRI that revealed an anomalous uterine cavity with a single left-sided cornua communicating with the cervix and a distended right-sided rudimentary horn. She underwent a right salpingectomy with rudimentary horn excision, which was successfully managed laparoscopically. This case emphasizes the importance of physicians being cognizant in identifying patients with uterine anomaly to provide appropriate treatment and prevent adverse reproductive outcomes.

Case of successfully delivered dicavitary twins in uterus didelphus

Uterus didelphus is a congenital abnormality arising from failure of fusion of Mullerian ducts, creating two separate uterine horns, two cervices and, in some cases, a vagina divided by a longitudinal septum. In this case, a 26-year-old woman with previously undiagnosed uterus didelphus spontaneously conceived dicavitary twins. Although initially wanting a vaginal birth, when both twins were in a breech presentation, a caesarean section was performed at 36 weeks, delivering two healthy babies. We will discuss the risk of obstetric complications in uterus didelphus and the challenges surrounding a vaginal delivery.

Functional Activity of Recombinant Forms of Amh and Synergistic Action with Fsh in European Sea Bass Ovary

Although anti-Müllerian hormone (AMH) has classically been correlated with the regression of Müllerian ducts in male mammals, involvement of this growth factor in other reproductive processes only recently come to light. Teleost is the only gnathostomes that lack Müllerian ducts despite having amh orthologous genes. In adult teleost gonads, Amh exerts a role in the early stages of germ cell development in both males and females. Mechanisms involving the interaction of Amh with gonadotropin- and growth factor-induced functions have been proposed, but our overall knowledge regarding Amh function in fish gonads remains modest. In this study, we report on Amh actions in the European sea bass ovary. Amh and type 2 Amh receptor (Amhr2) are present in granulosa and theca cells of both early and late-vitellogenic follicles and cannot be detected in previtellogenic ovaries. Using the Pichia pastoris system a recombinant sea bass Amh has been produced that is endogenously processed to generate a 12–15 kDa bioactive mature protein. Contrary to previous evidence in lower vertebrates, in explants of previtellogenic sea bass ovaries, mature Amh has a synergistic effect on steroidogenesis induced by the follicle-stimulating hormone (Fsh), increasing E2 and cyp19a1a levels.

An evo-devo perspective of the female reproductive tract

The vertebrate female reproductive tract has undergone considerable diversification over evolution, having become physiologically adapted to different reproductive strategies. This review considers the female reproductive tract from the perspective of evolutionary developmental biology (evo-devo). Very little is known about how the evolution of this organ system has been driven at the molecular level. In most vertebrates, the female reproductive tract develops from paired embryonic tubes, the Müllerian ducts. We propose that formation of the Müllerian duct is a conserved process that has involved co-option of genes and molecular pathways involved in tubulogenesis in the adjacent mesonephric kidney and Wolffian duct. Downstream of this conservation, genetic regulatory divergence has occurred, generating diversity in duct structure. Plasticity of the Hox gene code and wnt signaling, in particular, may underlie morphological variation of the uterus in mammals, and evolution of the vagina. This developmental plasticity in Hox and Wnt activity may also apply to other vertebrates, generating the morphological diversity of female reproductive tracts evident today.

AMH and AMHR2 Involvement in Congenital Disorders of Sex Development

Anti-müllerian hormone (AMH) is 1 of the 2 testicular hormones involved in male development of the genitalia during fetal life. When the testes differentiate, AMH is secreted by Sertoli cells and binds to its specific receptor type II (AMHR2) on the müllerian ducts, inducing their regression. In the female fetus, the lack of AMH allows the müllerian ducts to form the fallopian tubes, the uterus, and the upper part of the vagina. The human <i>AMH</i> gene maps to 19p13.3 and consists of 5 exons and 4 introns spanning 2,764 bp. The <i>AMHR2</i> gene maps to 12q13.13, consists of 11 exons, and is 7,817 bp long. Defects in the AMH pathway are the underlying etiology of a subgroup of disorders of sex development (DSD) in 46,XY patients. The condition is known as the persistent müllerian duct syndrome (PMDS), characterized by the existence of a uterus and fallopian tubes in a boy with normally virilized external genitalia. Approximately 200 cases of patients with PMDS have been reported to date with clinical, biochemical, and molecular genetic characterization. An updated review is provided in this paper. With highly sensitive techniques, AMH and AMHR2 expression has also been detected in other tissues, and massive sequencing technologies have unveiled variants in <i>AMH</i> and <i>AMHR2</i> genes in hitherto unsuspected conditions.

Morphology of the genital organs of male and female giant anteaters (Myrmecophaga tridactyla)

Background The giant anteater belongs to the supraorder Xenarthra which occupies a systematically isolated position among placental mammals. The species is categorized as Vulnerable by the International Union for Conservation of Nature, and understanding its reproductive characteristics is critical for future conservation efforts. Methods Gross and microscopic anatomy of the genital organs of 23 male and 21 female adult and young roadkill giant anteaters in Brazil were studied. Results Male giant anteaters presented a short conical penis, intraabdominal testes, and prostate, vesicular and bulbourethral glands. A tubular remnant of the partially fused Müllerian ducts extended from the seminal colliculus through the prostate gland, continued cranially in the genital fold, bifurcated, and attached with one elongation each to the left and right epididymal corpus. The structure presented a total length of up to 10 cm and contained a yellowish liquid in its lumen. Histologically, the caudal section of this structure resembled the female vagina, the middle portion corresponded to the uterus, and the extensions showed characteristics of uterine tubes. In adult female giant anteaters, ovoid ovaries with occasional seminiferous cord-like structures were observed. The animals possessed a simple uterus, which was directly continuous with the vaginal canal. The caudal portion of the vagina had two lumina, separated by a longitudinal septum and opening into two apertures into the vaginal vestibule, cranial to the urethral opening. In the urethral and the lateral vestibular wall, glandular structures with characteristics of male prostate and bulbourethral glands, respectively, were found. The vestibule opened through a vertical vulvar cleft to the exterior. A pair of well-differentiated Wolffian ducts with a central lumen originated ventrally at the vaginal opening into the vestibule and passed in a cranial direction through the ventral vaginal and uterine wall. Each duct extended highly coiled along the ipsilateral uterine tube until the lateral pole of the ovaries where it merged with the rete ovarii. Discussion The reproductive morphology of giant anteaters reveals characteristics shared with other Xenarthrans: intraabdominal testes, a simple uterus, and a double caudal vagina. The persistence of well-differentiated genital ducts of the opposite sex in both males and females, however, singles them out among other species. These structures are the results of an aberration during fetal sexual differentiation and possess secretory functions. The possibility of a pathological degeneration of these organs should be considered in reproductive medicine of the species. Conclusion Knowledge of the unique reproductive characteristics of the giant anteater is essential for future reproductive management of the species. Additionally, further research on the peculiarities of the persisting genital duct structures might help to understand sexual differentiation in placental mammals in general.