Mullerian Anomalies By Elizabeth Diaz .


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Mullerian Anomalies By Elizabeth Diaz. Mullerian Anomalies. Introduction Embryology and Development Anomalies of the Female Ductal System Clinical Presentation, Diagnosis and Treatment. Introduction.
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Mullerian Anomalies By Elizabeth Diaz

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Mullerian Anomalies Introduction Embryology and Development Anomalies of the Female Ductal System Clinical Presentation, Diagnosis and Treatment

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Introduction Congenital uterine irregularities are more regular than for the most part perceived by numerous rehearsing clinicians. Pervasiveness as a rule populace 1 in 201 (0.5%) According to "Acien," 2-3% of fruitful ladies and 3% of barren ladies and 5-10% of those with rehashed unnatural birth cycles. Appropriation : 7% arcuate, 34% septate, 39% bicornuate, 11% didelphic, 5% unicornuate, 4% hypoplastic/asplastic/strong and different structures

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Introduction True frequency and pervasiveness are hard to survey. Why? Determination predisposition: 0.4% nonobstetric, 8-10% repetitive pregnancy misfortune. Nonstandardized order frameworks Differences in analytic information securing brought about broadly divergent appraisals Overall commonness; ordinary fruitfulness/fruitlessness 1% and rehashed pregnancy misfortune 3%.

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Why is this vital? Lion\'s share have no issue considering, however have higher rates of: 1. Unconstrained Abortion 2. Unexpected labor 3. Barrenness 4. Irregular Fetal Lie 5. Dystocia at conveyance 6. Dysmenorrhea, endometriosis 7. Cervical ineptitude

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Embryology and Development

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Indifferent Embryo Genotype of incipient organism 46XX or 46XY is built up at treatment Weeks 1-6 sexually apathetic or undifferentiated stage; that is hereditarily female and male fetuses are phenotypically vague Week 7 starts phenotypic sexual separation Week 12 female or male attributes of outer genitalia can be perceived Week 20 phenotypic separation is finished.

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Indifferent Embryo Components which shape the grown-up female and male regenerative frameworks are: 1. Gonads ovaries or testicles 2. Genital Duct Systems Paramesonephric and Mesonephric Ducts 3. Outside Genitalia

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5 th week of pregnancy Swellings on either side of the dorsal mesentery Intermediate mesoderm frames the urogenital edge . Gonad creates from physical and germ cells. Substantial cells got from mesonephric cells Formation and Differentitation of the Gonads

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Coelomic epithelium and hidden mesoderm of the urogenital edge multiply to frame the gonadal edge Germ cells touch base in gonadal locale, empower coelomic epithelium and mesonephros to shape primitive sex ropes Formation and Differentiation of Gonads

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Primary sex ropes create from the gonadal edge and fuse primordial germ cells (XX or XY genotype) which move into the gonad from the mass of the yolk sac. Sex ropes and germ cells vital for typical improvement Gonad

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Y c\'some: TDF ( testis determing variable), SRY (sex deciding locale), critical for separation Primary sex ropes shape seminiferous ropes, tubuli recti, and rete testicles. Physical cells of essential sex ropes frame sertoli cells and germ cells shape spermatogonia Seminiferous strings: spermatogonia and sertoli cells, which discharge Anti-Mullerian Hormone (AMH) or MIS, and ABP (androgen restricting protein). Mesoderm b/w seminiferous ropes offers ascend to Leydig cells, which discharge testosterone. Leydig cells have receptors for HCG, prompted by HY antigen. Advancement of Testes

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Development of Testes Genital edge SRY Testes Primitive sex lines sertoli cells seminiferous strings Seminiferous ropes isolated from shallow epithelium by tunica albuguinea Seminiferous ropes seminiferous tubules Sertoli cells AMH/MIS AMH ipsilateral paramesonephric relapse Leydig cell testosterone virilization wolffian pipe epididymis, vas deferens, fundamental vesicle.

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Primary sex lines form into the rete ovarii, which deteriorate. Auxiliary sex ropes create and join primordial germ cells Secondary sex lines break separated and shape primordial follicles, which contain essential oocytes encompassed by a layer of follicular (granulosa) cells. Essential oocyte organize kept up until adulthood Formation of the Ovary

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Summary of Testes Development

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+ TDF medullary sex ropes separate into Sertoli Cells Sertoli cells create against mullerian hormone - TDF cortical sex lines separate into oocytes and follicular cells Lack of sertoli cells no AMH Genetic guys have testis deciding element on the sex deciding area SRY of the Y chromosome. Hereditary guys create Testis Determing Factor in the sex ropes

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Overview

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Genital Duct Systems

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6 th week, two sets of genital conduits. Mesonephric Duct stretching out from the mesonephros (Wolff\'s body) to the cloaca (urogenital sinus) alluded to as the Wolffian framework Second conduit emerges as a longitudinal invagination of coelomic epithelium on the anterolateral surface of the urogenital edge, known as Paramesonephric or Mullerian Duct. Genital Duct Development

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Cranial uterine tubes Caudal bits wire and frame the uterovaginal primordium and unite two peritoneal folds, the wide tendon. At first they stay isolated by a septum however later they breaker to shape the uterus. Paramesonephric Duct

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Paramesonephric channels don\'t reach the urogenital sinus. Paramesonephric channels combine with the average mass of the mesonephric pipes. Beneath the caudal tip of the uterine primordium or more the dorsal mass of the urogenital sinus, with the mesoneprhic pipes lying along the side, an accumulation of paramesonephric cells shapes constituting the Mullerian tubercle. Paramesonephric Duct

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Ureteral Buds : Mesonephric channels open caudally in urogenital sinus. At caudal tip ureteral bud grows. Becomes horizontally, anteriorly and cranially gets to be metanephros to shape the conclusive kidney

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Testis frames, the testosterone delivered by leydig cells and is conveyed along the tubules by ABP fortifies the advancement of the mesonephric channels to frame the efferent ductules, epididymis, ductus deferens, and fundamental vesicles. The AMH delivered in Sertoli cells represses paramesonephric channel advancement. Male Genital Duct Formation

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In ovary the nonappearance of testosterone hinders the improvement of the mesonephric channels. The atretic remains frame the epoophoron, paraoophoron and Gartner\'s conduits. Without AMH, paramesonephric pipes shape the female inside genital tract. Female Genital Duct Formation

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Embryology of Vagina Embryology disputable Derived paramesonephric channels versus mesonephric pipes versus urogenital sinus, or a mix. Most acknowledged, prevalent part determined combination paramesonephric , while second rate part emerges from urogenital sinus, BUT accept the "inductor" capacity of the mesonephric conduits invigorate sufficient mullerian/paramesonephric advancement.

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Mullerian tubercle cell buildup b/w second rate some portion of intertwined paramesonephric conduits and urogenital sinus. Sinovaginal globules create, constitute vaginal plate. Pit shaped fixed with paramesonephric epithelium, opens into the urogenital sinus and the metaplastic enlistment to polystratified plain epiithelium would deliver the vagina. Hypothesis of Mullerian Tubercle.

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Studies indicate sinovaginal knobs are caudal sections of mesonephric conduits. Between these knobs and caudally to the paramesonephric channels, a strong epithelial structure is found reaching the dorsal mass of the urogenital sinus… the mullerian tubercle. Inductor part of mesoneprhic channel on vagina

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Vagina got from melded mesonephric conduits and Mullerian tubercle. Paramesonephric channels frame uterus to outside cervical os and satisfactory development is initiated by mesonephric pipes. Mesonephric conduits relapse cranially however at cervical os, they expand and shape the sinovaginal globules. The paramesonephric cell buildup (mullerian tubercle ) joins itself in the vaginal plate framed by combination of the two knobs. Cavitation permits the paramesonephric cells to fix the primitive vaginal cavity with paramesonephric epithelium. Mesonephric conduit enlistment

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As ureteral bud grows from the opening of the mesonephric pipes in the urogenital sinus, the nonattendance or distal agenesis of a mesonephric pipe would bring about a nonappearance of its opening to the sinus ( the cause of the visually impaired vagina) and without the ureteral bud on that side. Accordingly, the conclusive kidney would neglect to create (ipsilateral renal agenesis) Agenesis of mesonephric conduit

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Embryology of External Genitalia

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Development outside genitalia Early, comparable in both genders 6 th wk, three outer projection encompass cloacal layer, the left and right genital swellings meet anteriorly to shape the genital tubercle. 12 th wk distinguish contrast. Genital swelling labioscrotal folds scrotum or labia major Genital tubercle phallus penis or clitoris

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Development of External Genitalia

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Female and Male outside genitalia

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Role of Dihydrotestosterone Differentiation depends in a roundabout way on gonadal emission in male embryo. Testosterone acts locally on the tubules however not specifically on the outer genitalia. Hormonal activity is applied by dihydotestsoterone (DHT) framed from testosterone by the 5 alpha reductase catalyst. DHT causes outside genitalia to wind up a penis and scrotum and incites advancement of prostate

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Anomalies of Female Ductal System

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Mullerian Anomalies

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Classification System: American Fertility Society

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Classification into 3 bunches: Based on comparable embryonic improvement surrenders and clinical presentation 1. Agenesis of uterus/vagina: Rokitansky-Kuster-Hauser Syndrome. 2. Def

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