Development Of Mullerian Ducts Research Articles

Secondary dysmenorrhea Unicornuate uterus with rudimentary and non-communicating horn

Unicornuate uterus is a type of female genital malformation caused by abnormal development of Mullerian ducts during embryogenesis.

Amenorea Primer et Hematokolpos et Hematometra ec. Septum Vagina Transversal

Background: Amenorrhea is defined as the absence of menarche in females of reproductive age. Primary amenorrhea is the failure to reach menarche by age 13 with no secondary sexual characteristics if menarche has not occurred five years after initial breast development or if the patient is 15 years or older. A transverse vaginal septum is a rare congenital abnormality in Mullerian duct development that can cause primary amenorrhea. Transverse vaginal septum incidence ranges from 1:2.100 to 1:72.000. The surgical treatment should be carried out as early as possible to prevent vaginal stenosis. Objective: To describe a rare müllerian duct abnormality and its management. Methods: This study is a case report. Primary data was obtained through auto anamnesis, anamnesis, physical examination, and ultrasound workup. Result: A 15 years old girl complained of cyclical abdominal pain a year ago without a history of menstruation. Ultrasound examination showed signs of hematometra and hematocolpos. The septum location was approximately 3cm from the vaginal introitus. We performed simple excision of the septum, then the distal end of the vagina wall of the septum was sutured to the proximal end of the vaginal wall with a simple interrupted suture. The patient was then discharged in good condition. Conclusion: The transverse vaginal septum remains a rare anomaly of the female genital tract; the reasons for its discovery are highly variable according to its shape and location. Haematocolpos remains the main consequence of these septums. The management is based on surgery while considering the risks of postoperative stenosis and the repercussions on the upper genital tract.

A Common Path: Magnetic Resonance Imaging of Müllerian and Wolffian Duct Anomalies.

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. 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.

Pregnancies in anomalous uterus: series of three cases

Uterine malformations can result from various alterations in the normal development of Mullerian ducts that make up a diverse category of congenital anomalies. In most cases, the defect remains undiagnosed and exhibits the normal number of chromosomes. Very few malformations require any intervention after thorough investigations. The most frequently used classification by the American Society for Reproductive Medicine is comprehensive, but does not include cervical or vaginal malformations. The European Society of Human Reproduction and Embryology has postulated a consensus that precisely shows independent cervical malformations.

O-005 Mullerian anomalies overview

Abstract text Mullerian Duct Anomalies- An Overview Prof. Sudha Prasad President Indian Fertility Society, Director, Matritava Advanced IVF & Training Centre, New Delhi, India Congenital anomalies of the mullerian duct system is one of the complex disorder encountered in gynecological practice. Mullerian ducts are paired embryological structures which undergo fusion and resorption in utero to form the uterus, fallopian tubes, cervix and upper two-thirds of the vagina. Disruption in the mullerian duct development throughout embryogenesis could result a large spectrum of inherent abnormalities identified as mullerian duct anomalies (MDAs). There is a wide variation in the prevalence of MDAs across various studies, ranging from 1–10% in the general population to 2–8% among infertile women and 5–30% among women with a history of miscarriage. These discrepancies in the reported prevalence are mainly attributed to lack of a universal classification system. Different varieties of malformations can occur when this system is not well developed. It ranges from absence of uterus, cervix or vagina, septum/duplication of vagina to of the uterus and vagina to minor uterine cavity abnormalities. Mullerian malformations are frequently associated with abnormalities of the renal and axial skeletal systems. Therefore, on initial examination of MDAs patients, these points should be kept in mind. Most mullerian duct anomalies (MDAs) are associated with functioning ovaries and age-appropriate external genitalia. These abnormalities are often recognized after the onset of puberty. After the onset of puberty, young women often present to the gynecologist with menstrual disorders. Late presentations include infertility and obstetric complications. A meta-analysis of nine studies comprising 3805 women with congenital uterine anomalies reviewed the obstetric outcome. The study reported that canalization defects such as septate and partial septate uteri had reduce fertility and increase rates of miscarriage and preterm delivery. None of the unification defects (bicornuate, unicornuate and didelphic uteri) reduce fertility but some are related to miscarriage and prematurity. Arcuate uteri are specifically associated with second-trimester miscarriage. All uterine abnormalities increase the risk of fetal malformation during delivery1. A retrospective longitudinal study concluded that reproductive performance of the unicornuate and didelphys uteri was poor (20–30% chance of carrying a pregnancy to term), while that of the septate and bicornuate uteri (live birth rate of 62%) was better than expected. The arcuate uterus had no impact on reproductive performance of women2. Agenesis of uterus and vagina requires surgical techniques, such as the Vecchietti and McIndoe procedures, have enabled many women to have normal sexual relations. Uterine transplant has changed the perspective of all other surgical advances and assisted reproductive technologies to improve fertility and obstetric outcomes3, 4,5.

Primary Amenorrhea with Mayer-Rokitansky-Kuster-Hauser Syndrome

BACKGROUND: Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a rare disorder described as aplasia or hypoplasia of uterus and vagina due to an early arrest in the development of Mullerian ducts. The etiology of MRKH syndrome remains uncertain: Although at the beginning, it was mentioned that this syndrome was the result of sporadic abnormalities. It has recently been assumed the genetic background is the cause of the increasing number of familial cases. CASE REPORT: A 29-year-old female patient presented with amenorrhea. She also has normal pubic and axillary hair and breast development. Routine investigations were normal. Genitalia inspection shows that pubic hair was well developed, labia majora and vagina were seen. Inspeculo show that vaginal wall was smooth, but portio was not seen. Vaginal examination result: The uterus was unpalpable, both adnexa were normal, parametrium was laxed, and Douglas pouch was not protruded. Rectal examination shows that uterus and both adnexa were difficult to identify. Transvaginal sonography revealed absence of uterus, both ovaries within normal. Transvaginal sonography diagnosed a uterine aplasia. With this, the provisional diagnosis made as primary amenorrhea due to suspected MRKH syndrome, the patient was referred for follicle-stimulating hormone, estradiol, testosterone, and prolactin levels by enzyme-linked immunosorbent assay examination and the result was within normal limit followed by genetic karyotyping and the result was normal 46, XX. CONCLUSIONS: We report a 29-year-old woman with primary amenorrhea. From our examination, the patient was diagnosed with MRKH syndrome. Uterus transplant is an alternative for that woman to treat amenorrhea and possibility to get pregnant.

Oviduct Epithelial Cells Constitute Two Developmentally Distinct Lineages that are Spatially Separated Along the Distal-Proximal Axis

Owing to technical advances in single cell biology, the appreciation of cellular heterogeneity has increased, which has aided our understanding of organ function, homeostasis and disease progression. The oviduct (also known as the fallopian tube in humans) is the distal-most portion of the female reproductive tract. It is essential for reproduction and the proposed origin of high grade serous ovarian carcinoma (HGSOC). In mammals, the oviduct is morphologically segmented along the ovary-uterus axis into four evolutionally conserved regions. It is unknown however if there is a diversification of epithelial cell characteristics between these regions. In this study, we identified transcriptionally distinct populations of secretory and multiciliated cells restricted to the distal and proximal regions of the oviduct. We demonstrated that these distal and proximal populations are distinct lineages specified early in Mullerian duct development and are maintained separately. These results aid our understanding of epithelial development, homeostasis and initiation of disease from the oviduct.

Role of Imaging in Rare Obstructive Reproductive Tract Anomalies: an Enigma—a Case Report

Mullerian duct anomalies (MDAs) result from abnormalities occurring during the embryologic development of mullerian ducts. Among the various mullerian abnormalities, obstructive reproductive tract anomalies (ORTA) form an interesting and difficult group to diagnose. This case series emphasize the role of imaging, specifically magnetic resonance imaging (MRI) in evaluating such cases before surgery. The clinical presentation of ORTA is in two ways, either with primary amenorrhea and pain or with menstrual periods and progressive dysmenorrhea. In ORTA, it is important to identify the site of obstruction, as surgical management depends upon the site of obstruction. Hence, the role of imaging is important in such cases. When suspecting MDAs especially ORTA, an initial screening ultrasound should be followed by MRI because of its multiplanar capability, excellent soft tissue characterization, and absence of ionizing radiation. All MRI examinations in our study were performed using 3 T MRI system using a phased array body coil. Here we are reporting three rare cases of ORTA presented to our institute where MRI identified the exact site of obstruction before surgery, leading to better preoperative planning. Hence MRI should be mandatory part of workup of this group of patients.

1329 Surgical Management of Longitudinal Vaginal Septum and Complete Uterine Septum

Video Objective To illustrate the steps for surgical correction of a longitudinal vaginal septum and complete uterine septum using narrative video footage. Setting A longitudinal vaginal septum and complete uterine septum are anomalies of Mullerian duct development that occur during embryological development. The exact incidence of Mullerian anomalies is difficult to determine, since women with them are often not diagnosed if asymptomatic, but is approximately 2-3%. In women who present with infertility, the incidence of uterine anomalies is often higher, approximately 5-10%. Although some women with vaginal anomalies are asymptomatic, some may note difficulty inserting tampons or dyspareunia. Reproductive outcomes in women with a complete uterine septum include possible decreased chance of implantation, and increased risk of spontaneous abortion and preterm labor/delivery. Benefits of surgical correction of a uterine septum include possible improved reproductive success and decreased poor obstetric outcomes. Interventions In this video, we illustrate the key surgical steps of excision of a longitudinal vaginal septum and hysteroscopic metroplasty for a complete uterine septum resection. Key surgical steps include: 1. Exam under anesthesia Lidocaine with epinephrine injection into vaginal septum. 2. Excision of septum with mayo scissors and re-approximation of vaginal mucosal edges. 3. Dilation of one side of cervical canal and placement of pediatric foley catheter to instill methylene blue-dyed fluid. 4. Hysteroscopic incision of septum using bipolar resectoscope until blue dye is visualized. 5. Complete incision of uterine septum. 6. Placement of uterine balloon for endometrial healing. 7. Vaginal packing placed to promote vaginal healing. Conclusion Surgical management of a longitudinal vaginal septum and complete uterine septum is a minimally invasive procedure to correct these Mullerian anomalies that could impact a woman's quality of life and possible reproductive success in the future.

Cervicovaginal agenesis: a case report

Introduction: Mullerian anomalies arise from the failure in the development of mullerian ducts and their associated structures during organogenesis that gives adverse impact in fertility and reproductive health.

Complete Müllerian agenesis, renal agenesis, and renal ectopia: MURCS association – A rare cause of primary amenorrhea

Mullerian duct aplasia–renal agenesis–cervicothoracic somite dysplasia (MURCS) association is a rare and unusual constellation of nonrandom findings that include Mullerian duct aplasia, renal aplasia, and cervicothoracic somite dysplasia. The agenesis of the Mullerian duct is the second-most common cause of primary amenorrhea after Turner syndrome. The abnormal development of Mullerian duct often associates with the urinary tract and skeletal abnormalities. MURCS association is a unique and rare developmental disorder with four common features of uterine hypoplasia or aplasia, renal agenesis or ectopy, vertebral anomalies, and short stature. Here, we report a 32-year-old female with aplasia of the Mullerian duct, unilateral renal agenesis, and anomalies of the cervicothoracic somites (MURCS association).

Prevalence of Uterine Anomalies in Relation with Radiological Anatomy among Patients Attending Tertiary Care Centre

Introduction: Uterine anomalies arise due to malformation in the Mullerian duct development. These anomalies were often asymptomatic and unrecognized, also increases the risk of adverse pregnancy outcome like infertility, recurrent pregnancy loss, preterm delivery, amenorrhea, pain and fetal malpresentation. Many of these malformations were detected by radiologic or sonographic studies. Materials and Methods: Observational study includes 150 women between 18-35 years with gynecological problem in a tertiary care hospital in Kancheepuram district over a period of 1 year from January 2017 to January 2018. Pelvic imaging of study subjects with transabdominal and transvaginal ultrasound was performed as per the standard procedure. Results: Among 150 patients 75 had the complaint of primary infertility, 71 had primary amenorrhea and 4 came with frequent miscarriage. Out of 150 study subjects who underwent 2D ultrasonography, 69 showed uterine anomalies. Overall prevalence of anomalies was 46%, of which frequency of septate form of anomalies was maximum (45%), followed by bicornuate uterus (17%), arcuate uterus (16%), didelphic uterus (13%), unicornuate uterus (4.3%), and subseptate uterus (4.3%). Conclusion: Actual prevalence with correct assessment of anomalies using radiological anatomy will help to differentiate uterine anomalies and thereby suggests a right therapeutic option.

Intraperitoneal Third Müllerian Duct Mimicking a Hydrosalpinx: Laparoscopic Diagnosis and Treatment

Abstract Background: The most frequent congenital anomalies of the genital tract in women are caused by irregular development of the Mullerian ducts. At present, known Mullerian anomalies include a...

Magnetic resonance imaging in diagnosis and classification of uterovaginal congenital anomalies

Uterine congenital anomalies are clinically relevant because these are associated with increased incidences of infertility and menstrual disorder. These are frequently not diagnosed at birth. These anomalies usually diagnosed at child-bearing age when reproductive malfunction arise. When normal mullerian duct development interrupted at any stage, it results in mullerian abnormality. Magnetic resonance imaging (MRI) has an excellent role in evaluation of mullerian duct anomalies. Ultrasonography is the primary investigation; however, MRI is an excellent noninvasive investigation for accurate evaluation of uterine congenital anomalies. MRI is a very good modality to evaluate the vaginal malformation, which usually difficult to evaluate by ultrasound.

Rudimentary horn of Uterus : A diagnostic challenge in pregnancy

When the development of the mullerian duct is normal on one side, but imperfect on the other side , a lateral fusion defect often with obstruction ,described as rudimentary horn,is produced. Most rudimentary horn are non functional and some are attached to the functioning contralateral horn by means of fibrous bands. If the endometrium lining the cavity of the non communicating rudimentary horn is is functional ,retention of menstrual blood causes cyclic abdominal pain and spillage of blood into the abdominal cavity via the tubal ostium may lead to endometriosis.Sometimes a narrow communicating channel exists between the rudimentary horn and the opposite uterine cavity.Under these circumstances pregnancy is possible,most of the patients present with symptoms suggestive of an ectopic pregnancy including of uterine rupture causing catastrophic bleeding and circulatory collapse.We present a similar case where laparotomy was done thinking of ruptured ectopic pregnancy.

Tata Laksana Kasus Perempuan dengan Pure Gonadal Dysgenesis 46, XY (Sindrom Swyer)

Disorders of sex development (DSD) are medical conditions in which the development of chromosomal, gonadal or anatomic sex varies from normal and may be incongruent with each other. Swyer syndrome is a condition caused by pure gonadal dysgenesis 46,XY, which followed by inadequate anti-mullerian (AMH) production results in maintenance and further development of mullerian duct into uterus. Therefore, many patients who suffer from this condition initially come with chief complaint of primary amenorrhea with insufficient development of secondary sexual characteristics. Patient’s gender orientation commonly is a female, since her brain was not sufficiently exposed with androgen hormone. Management of DSD patients should be based on patient-centered approach. Therefore, overall management of DSD patients should follow according to patient’s perception regarding to her gender orientation. Herein, we reported a case of Swyer syndrome in female aged 29 years with chief complain primary amenorrhea. Chromosomal analysis was 46,XY (20 mataphase), FSH level was 31.5miu/ml, LH 10.8miu/ml, estradiol (E2) <5pg/ml, testosteron total (T) <0.0025ng/ml. Medical management for this patient has the purpose to drive her feminization process in order to improve her physical appearance. However, since there is a great tisk for having gonadal tumor development from intra-abdominal dysgenetic gonad with Y chromosome, this patient has been strongly suggested to have bilateral gonadectomy. Normal 0 false false false IN X-NONE X-NONE

New theory of uterovaginal embryogenesis

ABSTRACTBackground: The explanation of uterine and vaginal embryogenesis in humans still poses many controversies, because it is difficult to assess early stages of an embryo. The literature review revealed many disagreements in Mullerian theory, inciting some authors to propose new embryological hypotheses. In the original Mullerian theory: the paramesonephral ducts form the Fallopian tubes, uterus and vagina; the mesonephral ducts regress in female embryos. Aims: The aim of this article is to investigate the development of Mullerian ducts in humans, using comparative analysis of fundamental embryological theory and various utero-vaginal anomalies. Material and methods: Between 1998 and 2015, 434 patients with various uterovaginal malformations had been operated on at the Scientific Centre of Obstetrics Gynaecology and Perynatology in Moscow. The anatomies of the uterovaginal malformations in these patients were diagnosed with ultrasound and MRI and then verified during surgical correction by laparoscopy. Results: A systematic comparison of uterovaginal malformations to those in the literature has allowed us to formulate a new theory of embryonic morphogenesis. The new theory is significantly different: ovary, ovarian ligamentum proprium, and ligamentum teres uteri derive from gonadal ridges; Fallopian tubes and vagina completely develop from mesonephral ducts. The uterus develops in the area of intersection between the mesonephral ducts with gonadal ridges by the fusion of the two. Conclusions: The new theory may to induce future embryological studies. The hypothetic possibility that the ovary and endometrium derive from the gonadal ridges could be the key to understanding the enigmatic aetiologies of extragenital and ovarian endometriosis.

English

INTRODUCTION: Pregnancy in a rudimentary horn of a unicornuate uterus is rare.1 An incidence of 1 in 76,000-150,000 pregnancies is reported in the literature.2,3 We present a case report of rupture of a 25 weeks pregnancy in the non-communicating rudimentary horn of a unicornuate uterus. Congenital malformations of the uterus, also known as Mullerian duct anomalies, are rare in general population (Approx. 1%). These abnormalities result from arrested development, abnormal formation or incomplete fusion of mesonephric ducts. Unicornuate uterus results from unilateral arrested mullerian duct development. Rarely unicornuate uterus may also have a rudimentary horn, more on the right than on the left side. The incidence of unicornuate uterus is estimated to be 1:250 and its occurrence with rudimentary horn is 1:100,000. Such anomalies are reported to result in increased rate of miscarriages, recurrent pregnancy losses, preterm labor, infertility and other obstetric complications. Conception in rudimentary horn arises either from a small communication with the uterine cavity (Communicating) or by Trans peritoneal migration of the fertilized ovum from the contra-lateral side (Non-communicating). The proportion of non-communicating rudimentary horns is 70-90%. The frequency of pregnancy in rudimentary horn is reported to be 1:76000. The clinical presentations vary from being asymptomatic to vague complaints of mild lower abdominal pain with gastrointestinal upset to its severest form of acute abdomen with hemorrhagic shock. The most significant threat of a rudimentary horn pregnancy is the risk of rupture because of poorly developed musculature. In view of the paucity of literature on rare observation of pregnancy in rudimentary horn of uterus, the case reported here is of crucial importance.

Gonadal morphogenesis and sex differentiation in the oviparous lizard, Sceloporus aeneus (Squamata: Phrynosomatidae)

Among reptiles, morphological events involving differentiation of the gonads are similar, and their development is closely related to development of the Wolffian and Mullerian reproductive ducts and adrenal tissue. These structures have been described for some Squamata during their embryonic development. This study shows morphological characteristics of gonadal, adrenal tissue, and reproductive ducts development, during the embryonic development of oviparous lizard Sceloporus aeneus. The morphology of the urogenital complexes was examined using light microscopy, and ultrastructure of gonads was analyzed by transmission electron microscopy. The undifferentiated gonad was apparent from stage (st) 30 to st33; ovaries and testes became morphologically distinct from st34. In ovaries, the formation of the cortical and medullary region is evident, and germinal beds can be observed from st34 and synaptonemal complexes as evidence of meiosis was detected at st36. In testes, formation of testicular cords containing germ cells is observed; immature Leydig and Sertoli cells were identified from st38. Females showed evident Mullerian duct development, whereas in males, the Mullerian duct degenerated and the Wolffian duct remained. The adrenal tissue was observed as a cord of compact cells associated with the dorsal region of developing gonad from st31. In general, gonadal development of S. aeneus is similar to that observed in Sceloporus undulatus. Particularly, the presence of immature Leydig cells containing lipid droplets in testes and structures similar to germinal beds in ovaries is specific characteristics of S. aeneus.

Embryological basis of malformed female genital tract and various classifications

AbstractDevelopmental anomalies of the Mullerian duct system represent some of the most fascinating disorders that obstetricians and gynaecologists encounter. The uterus is formed during embryogenesis by the fusion of the two paramesonephric ducts (Mullerian ducts). This process usually fuses the two Mullerian ducts into a single uterine body, but fails to take place in the affected women who maintain their double Mullerian systems. A bicornuate uterus is a type of a uterine malformation where upper part of uterus forms two horns. The fusion process of upper part of Mullerian duct is altered. As a result, cranial part of the uterus becomes bifurcated. As Mullerian duct anomalies are of anatomic interest, this article discusses epidemiology, embryological development, genetics of development and malformation and lastly various classifications based on Mullerian duct development. Pregnancy in bicornuate uterus is usually of high risk with reproductive outcomes like recurrent abortions, preterm delivery or malpresentation. Classifying Mullerian duct anomalies bears merit because it correlates anatomic anomalies with arrests in morphogenesis. Establishing an accurate diagnosis is essential for planning treatment and management strategies.