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  1. 1. Embryology of the female genital tract
  2. 2. Embryology of the female genital tract <ul><li>The urogenital system (U.G.S) </li></ul><ul><li>develops from </li></ul>the mesoderm of the intermediate cell mass ( genital ridge ) urogenital sinus ( the anterior part of the cloaca ( give rise to the lower 1/4 of the vagina and vulva medial part give rise to the ovaries <ul><ul><li>middle part </li></ul></ul><ul><ul><li>undergo atrophy in female </li></ul></ul>lateral part give rise to fallopian tubes, uterus & upper 3/4 of vagina
  3. 3. Development of the ovaries <ul><li>The ovaries developed from the medial part of the genital ridge </li></ul><ul><li>At about 4 week gestation , primordial germ cells (that give rise to the ova) originate from the primitive hindgut ( yolk sac ) then migrate to the genital ridge </li></ul><ul><li>Failure of its migration give rise to streak gonads </li></ul><ul><li>At 12 weeks: Formation of the the mesentery of the ovary)-> as a result of projection of the ovary into coelomic cavity </li></ul>
  4. 4. Development of the ovaries (cont.) <ul><li>The ovary descends in the abdominal cavity with development of 2 ligaments </li></ul><ul><ul><li>The 1st is the suspensory ligament attached to the cephalic pole -> infundibulopelvic ligament . </li></ul></ul><ul><ul><li>The other ligament is called the gubernaculum (tractor) which is attached to the caudal end of the ovary and to the abdominal parities at the inguinal region.-> attached to the developing uterus forming the future: </li></ul></ul><ul><ul><ul><li>round ligament in its distal part </li></ul></ul></ul><ul><ul><ul><li>while its proximal part form the ovarian ligament . </li></ul></ul></ul>
  5. 5. Development of the fallopian tubes <ul><li>They develop from the upper parts of the mullarian ducts after their canalization </li></ul>
  6. 6. Development of the uterus <ul><li>The uterus develops from the middle part of the mullarian ducts after their fusion and canalization . </li></ul><ul><li>N.B: Failure of fusion or canalization or disappearance of the formed septum to become a single organ give rise to many of congenital uterine anomalies . </li></ul>
  7. 7. Development of the vagina <ul><li>The upper ¾ to 4/5 of vagina develops from the fused & canalized parts of the caudal ends of mullarian ducts while the lower ¼ or 1/5 formed from the urogenital sinus </li></ul><ul><li>N.B: </li></ul><ul><ul><li>Congenital malformation occurs when Mullerian ducts fail to develop -> (congenital absence of vagina) or fail to fuse ( septate vagina ) or fail to canalize ( atresia of vagina ). </li></ul></ul><ul><ul><li>The caudal ends are closed by a septum which is complete at 1st but later become excavated centrally to form the hymen . At least, the distal end of the vagina is formed from urogenital sinus </li></ul></ul><ul><li>The urogenital fold : is present in early embryo and contains the origins of the wolffian and Mullerian duct i.e. the primordia of both urinary and genital tract. </li></ul><ul><li>So, if there is genital tract malformation, look for associated malformation in the urinary tract and vice versa . </li></ul>
  8. 8. In summary <ul><li>In females , Mullerian ducts give: f. tubes + uterus (body + cx) + upper 3/4 to 4/5 of vagina . </li></ul><ul><li>In males , at 6 weeks, the future testis secretes Mullerian inhibitory factor (MIF), which inhibits Mullerian ducts development and secrets testosterone which stimulates differentiation of external genitalia </li></ul>
  9. 10. Development of the external genitalia <ul><li>Up to 10 weeks development is the same in male and female i . e . neutral primordium . Then differentiation occurs : </li></ul><ul><li>In female : testicular androgen is absent (androgen insensitivity), the following occur : </li></ul><ul><ul><li>The bladder and urethra from -> vesical portion of UGS </li></ul></ul><ul><ul><li>The vestibule -> from the pelvic and phallic potions of UGS </li></ul></ul><ul><ul><li>The clitoris -> from the genital tubercle which enlarges only slightly </li></ul></ul><ul><ul><li>The labia minora : develop from the genital folds which remain unfused </li></ul></ul><ul><ul><li>The labia majora : develop from the genital swellings </li></ul></ul><ul><ul><li>Greater vestibular gland& lesser vestibular gland ->arise from the vestibular endoderm </li></ul></ul>
  10. 11. Development of the external genitalia <ul><li>In male : with androgen secretion, the following occur : </li></ul><ul><ul><li>Genital tubercle : greater enlargement to form the penis. </li></ul></ul><ul><ul><li>Genital folds : fuse over a deep groove formed between them to become the penile part of male urethra. </li></ul></ul><ul><ul><li>Genital swellings enlarge and fuse to form scrotum </li></ul></ul>
  11. 12. Genital homologues in males and females
  12. 13. The Wolffian remnants in the female <ul><li>in female, the wolffian ducts undergo atrophy leaving remnants between two layers of B.L. and in the anterolateral wall of the vagina </li></ul><ul><li>These remnants are: </li></ul><ul><ul><li>Hydated cyst of morgagni : lateral to the tube </li></ul></ul><ul><ul><li>Koblet's tubules : in the outer part of B.L. </li></ul></ul><ul><ul><li>Epoophoron: between the ovary and F.T </li></ul></ul><ul><ul><li>Paroophoron: between the ovary and uterus. </li></ul></ul><ul><ul><li>Gartener's duct : may undergo cystic dilatation in the B.L. gives rise to paraovarian cyst or in the anterolateral wall of the vagina, gives rise to Gartener's cyst </li></ul></ul>
  13. 14. Congenital anomalies of reproductive tract <ul><li>The Mullerian and wolffian ducts are closely related embryologically, so, the association between the genital & urinary system anomalies is common. </li></ul><ul><li>Malformations of genital tract may be represented by: aplasia ‑ atresia ‑ hypoplasia ­-fusion defects or duplication of accessory organs. </li></ul><ul><li>Complete formation and differentiation of the mullerian ducts into the segments of the female reproductive tract depend on completion of 3 phases of development as follows: </li></ul><ul><ul><ul><li>Organogenesis </li></ul></ul></ul><ul><ul><ul><li>Fusion </li></ul></ul></ul><ul><ul><ul><li>Septal resorption </li></ul></ul></ul>
  14. 15. Congenital anomalies of reproductive tract (cont.) <ul><li>Organogenesis : One or both Mulllerian ducts may not develop fully, resulting in abnormalities such as uterine agenesis or hypoplasia (bilateral) or unicornuate uterus </li></ul><ul><li>Fusion : </li></ul><ul><ul><li>The process during which the lower segments of the paired Mullerian ducts fuse to form the uterus, cervix, and upper vagina is termed lateral fusion. Failure of fusion ->bicornuate or didelphys uterus. </li></ul></ul><ul><ul><li>The term vertical fusion occasionally is used to refer to fusion of the ascending sinovaginal bulb with the descending Mullerian system (ie, fusion of the lower 1/4and upper ¾ of the vagina). Complete vertical fusion forms a normal patent vagina, while incomplete vertical fusion results in an imperforate hymen . </li></ul></ul><ul><li>Septal resorption : after the lower Mullerian ducts fuse, a central septum is present, which subsequently must be resorbed to form a single uterine cavity and cervix . </li></ul><ul><li>Failure of resorption is the cause of septate uterus . </li></ul>
  15. 16. Internal structures anomalies <ul><li>Mullerian duct anomalies are categorized most commonly into 7 classes according to the American Fertility Society (AFS) Classification Scheme (1988) as follows : </li></ul><ul><li>Class I (hypoplasia/agenesis): </li></ul><ul><ul><li>Uterine/Cervical agenesis or hypoplasia. </li></ul></ul><ul><ul><li>The most common form is the Mayer‑Rokitansky‑Kuster­ Hauser syndrome, which is combined agenesis of the uterus, cervix, and upper portion of the vagina. </li></ul></ul><ul><ul><li>Patients have no reproductive potential. </li></ul></ul>
  16. 17. Class II (unicornuate uterus) <ul><li>Complete, or almost complete, arrest of development of one Mullerian duct. If the arrest is incomplete, as in 90% of patients, a rudimentary horn with or without functioning endometrium is present. If the rudimentary horn is obstructed, it may come to surgical attention when presenting as an enlarging pelvic mass. If the contralateral healthy horn is almost fully developed, a full‑term pregnancy is believed to be possible </li></ul>
  17. 18. Class III (didelphys uterus) <ul><li>Results from complete non-fusion of both Mullerian ducts </li></ul><ul><li>The individual horns are fully developed and almost normal in size. </li></ul><ul><li>Two cervices are inevitably present . </li></ul><ul><li>A longitudinal or transverse vaginal septum may be noted as well. </li></ul><ul><li>Since each horn is almost a fully developed uterus, patients have been known to carry pregnancies to full term . </li></ul>
  18. 19. Class IV ( bicornuate uterus) <ul><li>Results from partial non fusion of the Mullerian ducts . </li></ul><ul><li>The central myometrium may extend to the level of the internal cervical os ( bicornuate unicollis ) or ex ternal cervical os ( bicornuate bicollis ). The latter is distinguished from didelphys uterus because it demonstrates some degree of fusion between the two horns, while in classic didelphys uterus, the two horns and cervices are separated completely . </li></ul><ul><li>In addition, the horns of the bicornuate uteri are not fully developed; typically, they are smaller than those bicornuate ( bicollisunicollis ( </li></ul><ul><li>Some patients are surgical candidates for metroplasty of didelphys uteri </li></ul>
  19. 20. Class V ( septate uterus) <ul><li>A septate uterus results from failure of resorption of the septum between the two uterine horns . </li></ul><ul><li>The septum can be partial or complete , in which case it extends to the internal cervical os </li></ul><ul><li>Histologically , the septum may be composed of myometrium or fibrous tissue . </li></ul><ul><li>The uterine fundus is typically convex but may be flat or slightly concave ( <1cm fundal cleft ). </li></ul><ul><li>Women with septate uterus have the highest incidence of reproductive complications . </li></ul><ul><li>Differentiation between a septate and a bicornuate uterus is important because septate uteri are treated using transvaginal hysteroscopic resection of the septum, while if surgery is possible and/or indicated for the bicornuate uterus, an abdominal approach is required to perform metroplasty </li></ul>
  20. 21. Class VI (arcuate uterus) <ul><li>Arcuate uterus has a single uterine cavity with the outer contour of the uterus convex or flat. </li></ul><ul><li>This form is often considered a normal variant since it is not significantly associated with the increased risks of pregnancy loss and the other complications found in other subtypes . </li></ul>
  21. 22. Class VII ( diethylstilbestrol‑related anomaly) <ul><li>The uterine anomaly is seen in the female offspring of as many as 15% of women exposed to DES during pregnancy . </li></ul><ul><li>Female fetuses who are affected have a variety of abnormal findings that include uterine hypoplasia and a T‑shaped uterine cavity . </li></ul><ul><li>Patients also may have abnormal transverse ridges, hoods, stenosis of the cervix, and adenosis of the vagina with increased risk of vaginal clear cell carcinoma . Imaging findings are pathognomonic for this anomaly . </li></ul>
  22. 23. Fallopian tube anomalies <ul><li>Include: </li></ul><ul><ul><li>aplasia, </li></ul></ul><ul><ul><li>atresia, </li></ul></ul><ul><ul><li>hypoplasia ( very long & thin ) , </li></ul></ul><ul><ul><li>accessory horn or ostia and tubal diverticulum </li></ul></ul><ul><li>It may cause infertility or ectopic pregnancy </li></ul>
  23. 24. Vaginal anomalies <ul><li>Vaginal septa: </li></ul><ul><li>Longitudinal septa : </li></ul><ul><ul><li>Partial or complete septa extending antroposteriorly in the vagina results from failure of disappearance of the fused Mullerian structure, this may be associated with normal uterus or duplicate uterus </li></ul></ul><ul><ul><li>Occasionally the septum may not be in the midline and may not communicate externally leading to lateral pouch containing menstrual blood </li></ul></ul>
  24. 25. Transverse vaginal septum <ul><li>Complete or perforated </li></ul><ul><li>Rare occurs at the junction of upper and middle 1/3 of vagina </li></ul><ul><li>When there is no perforation in the septum retention of mucoid material may occur before puberty leading to mucocolpos </li></ul><ul><li>After puberty menstrual flow becomes hidden and accumulate in the vagina, uterus and tubes giving a picture like imperforate hymen </li></ul><ul><li>If the septum is perforated patient may complain of dyspareunia and it may cause dystocia in labor </li></ul><ul><li>Double or separate vagina . </li></ul><ul><li>Vaginal agenesis (absent ( </li></ul><ul><ul><li>This could be partial or complete </li></ul></ul><ul><ul><li>Complete vaginal agenesis may be a part of Mullerian agenesis, here the vagina is represented by a small pouch (part derived from U.G.S.) </li></ul></ul><ul><ul><li>Patient usually have normal external genitals and lower vagina derived from the urogenital S. </li></ul></ul><ul><ul><li>Ovaries are normally developed </li></ul></ul><ul><ul><li>Present usually with primary amenorrhea </li></ul></ul><ul><ul><li>In 50% urinary anomalies present e.g. absent or pelvic kidney </li></ul></ul><ul><ul><li>Anomalies of spinal cord is seen in some cases </li></ul></ul>
  25. 26. Treatment of vaginal agenesis (absent) : <ul><li>Construction of new vagina ( McIndoe’s operation ): involves the development of a space between the bladder and rectum by blunt dissection and inserting a skin - graft - covered mold ( from thigh ) into the cavity allowing epithelialization . This undergoes metaplasia and loss its keratinization . </li></ul><ul><li>Gradual dilatation ( Frank’s method) Frank’s non - operative procedure used progressive larger dilators inserted by the patient to gradually deepen and widen the canal </li></ul>
  26. 27. External genital anomalies <ul><li>Aplasia: obliteration of perineum ( absence of anal, urethral or genital orifices ). This may be combined with limb anomalies . </li></ul><ul><li>Fusion : agglutination of labia ( postnatal inflammation ( </li></ul>
  27. 28. Imperforate hymen <ul><li>Cryptomenorrhea (haematocolpos ‑ haematometra ‑ haemotosalpinx ( </li></ul><ul><li>The vaginal plate develops near the junction of the lower part of the vagina and the vestibule. Canalization of this plate is generally completed by the 6th. Month of fetal life. Failure of the final process of canalization occurs and results in imperforate hymen which obstruct the menstrual outflow </li></ul><ul><li>Presentation: Usually present with primary amenorrhea with monthly pain </li></ul><ul><li>Sometimes present with retention of urine due to collection of blood in vagina (hematocolpos), uterus (hematometra) and tubes (hematosalpinx) </li></ul><ul><li>Treated by cruciate incision or excision of hymen </li></ul>
  28. 29. Ovarian anomalies <ul><li>Accessory or supernumerary ovaries </li></ul><ul><li>Failure of descent </li></ul><ul><li>Gonadal dysgenesis (Turner' s syndrome ( streak gonads, chromosomal pattern is XO, sex chromatin negative, in. 3/ 100.000 full term births, vagina and uterus develop. The patient complaining of primary amenorrhea . Examination revealed no secondary sex characters, short stature, webbing of the neck, cubitus vulgus and coarctation of aorta </li></ul><ul><li>Androgen insensitivity ( testicular feminization syndrome ): male but Mullarian inhibitory factor ( MIF ) is present . Testosterone receptors absent . Phenotypically female : no pubic or axillary hair ‑ no vagina or tubes or uterus, small vaginal pouch, external genitalia female type ‑ well develop breast . X‑linked recessive . </li></ul>
  29. 30. Ovarian anomalies (cont.) <ul><li>Super female(XXX): may be normal female & give birth to children. Others, may be amenorrheic with mental defects </li></ul><ul><li>True Hermaphrodites : presence of both ovarian & testicular tissues in the gonads </li></ul><ul><li>Female pseudohermaphrodites : Partially masculinized female – XX, presented by ambiguous genitalia </li></ul><ul><ul><li>Causes : ‑ </li></ul></ul><ul><ul><ul><li>Maternal intake of androgens . </li></ul></ul></ul><ul><ul><ul><li>Androgenic tumors </li></ul></ul></ul><ul><ul><ul><li>Congenital adrenal hyperplasia . </li></ul></ul></ul><ul><li>Male pseudo hermaphrodites: </li></ul><ul><ul><li>Incompletely masculinized male XY </li></ul></ul><ul><ul><li>Partial androgen insensitivity </li></ul></ul><ul><ul><li>Inadequate androgen . </li></ul></ul><ul><ul><li>No MIF . </li></ul></ul>
  30. 31. Clinical presentation of congenital malformation of the female genital tract <ul><li>Nothing ( asymptomatic </li></ul><ul><li>Menstrual problems : </li></ul><ul><ul><li>Primary amenorrhea </li></ul></ul><ul><ul><li>Menorrhagia </li></ul></ul><ul><ul><li>Hypomenorrhea </li></ul></ul><ul><ul><li>Dysmenorrhea </li></ul></ul><ul><ul><li>Premature ovarian failure </li></ul></ul><ul><li>Dyspareunia </li></ul><ul><li>Infertility </li></ul><ul><li>Pelvic endometriosis </li></ul><ul><li>Pelvic / abdominal mass </li></ul><ul><li>Urinary complication & malformation (pelvic kidney) </li></ul><ul><li>Pregnancy complication (abortion, premature labour, malpresentation and obstructed labour) </li></ul><ul><li>IUCD cannot be inserted in abnormal shaped uterus </li></ul><ul><li>Malformation of the external genitalia </li></ul>