Female Genital Tract Development

Development of Female Genital Tract

• Content covered includes the development of the female genital tract, congenital anomalies, imperforate hymen, and intersex conditions.

Early Embryonic Development

• By the 12th day after fertilization, the ectoderm completely covers the embryo.
• The endoderm differentiates to form the primitive gut, which attaches to the body's posterior wall via the mesentery.
• The body cavity is lined by the mesoderm structure.
• The coelomic epithelium bulges on the other side of the mesentery root, forming a ridge along the body cavity's length.
• The intermediate cell mass develops into the urogenital system.

Intermediate Cell Mass

• The intermediate cell mass divides into three parts: cephalic, middle, and caudal.
• The cephalic part becomes the pronephros, which typically atrophies in humans.
• The middle part develops into the mesonephros, giving rise to the genital ridge, mesonephric duct, and Mullerian duct.
• The caudal part develops into the metanephros, which forms the kidney.

Genital Ridge Formation

• Around the 5th week of intrauterine life, the genital ridge forms on the medial side of the mesonephros and can give rise to the ovary or testis.
• A canal forms in the lateral part of the mesonephros, creating the mesonephric or Wolffian duct, which develops into the ductus epididymis, epididymis, and vas deferens.
• The Mullerian groove forms lateral to the mesonephric duct, leading to the development of the Fallopian tubes, uterus, and upper 4/5 of the vagina in females.

Sexual Differentiation

• The development is identical in both male and female fetuses until the 7th week of intrauterine life.
• Development into female or male depends on the presence or absence of the Y chromosome.
• The presence of a Y chromosome leads to positive production of H-y gene or testicular determining factor (TDF), resulting in testes development and the production of androgenic steroids and the growth of the wolfian system.
• Mullerian inhibiting factor (MIF), produced by Sertoli cells, inhibits the development of the Mullerian duct in males.

Absence of Y Chromosome

• The female genital system develops in the absence of the Y chromosome, androgen, and MIF.

Female Genital System

• The medial part of the urogenital ridge forms the genital ridge, from which the ovary develops at the level of T A.11 T ,10.
• The gubernaculum attaches the ovary's lower pole to the inguinal region, later attaching to the uterus.
• The part of the gubernaculum between the ovary and uterus becomes the ovarian ligament.
• The part of the gubernaculum between the uterus and inguinal region becomes the round ligament.
• The descent of the ovary is due to traction by the gubernaculum.

Wolffian System in Females

• The Wolffian system (mesonephric) lies lateral to the genital ridge.
• In males, develops into Vasa efferentia, epididymis, vas deferens.
• In females, it atrophies, leaving remnants within the broad ligament layers, including koblt tubules, epoophoron, paroophoron, and Gartner’s duct.

Mullerian Duct

• The Mullerian duct (paramesonephric duct) lies lateral to the Wolffian system.
• The upper part canalizes to give the fallopian tubes.
• The lower part undergoes fusion and canalization to form the uterus, cervix, and upper 4/5 of the vagina.

Urogenital Sinus

• The urogenital sinus is solid condensation of tissues that canalize and fuse with the lower end of fused Mullerian ducts, forming the lower 1/5 of the vagina.
• The lower end of the vagina is bounded anteriorly by the genital tubercle, which forms the clitoris, and laterally by the outer and inner genital folds which form the labia Majora and minori respectively.

Congenital Abnormalities

• Congenital abnormalities can affect the ovaries, tubes, uterus, vagina, and external genitalia.

Ovaries Abnormalities

• Abnormalities include absent ovaries, extra ovaries, and improper development (as in Turner’s Syndrome).

Turner's Syndrome

• Ovaries are represented by fibrous tissue due to a genetic error (45 XO).
• Presents with varying degrees of mental retardation, ptosis, perceptive deafness, webbing of the neck, coarctation of the aorta, kyphosis, horseshoe kidney, hip dislocation, tibial exostosis, short stature, pigmented Navi, and DM.
• Other findings include amenorrhea and infertility.
• Management involves correcting any medical disorders, estrogen replacement therapy, and psychic therapy.

Tubes Abnormalities

• Aplasia (absence of tubes).
• Hypoplasia (underdevelopment of tubes).
• Congenital diverticula.

Uterus Abnormalities

• Imperfect development.
• Imperfect fusion.
• Imperfect canalization.
• Other abnormalities.

Imperfect Uterus Development

• Aplasia (absence of uterus).
• Hypoplasia: Infantile uterus with body to cervix ratio of 2:1 (amenorrhea, infertility) or prepubertal 1:1 (hypomenorrhea, recurrent abortion).

Imperfect Uterus Fusion

• Uterus didelphys: two bodies, two cervices, longitudinal vaginal septum.
• Uterus pseudo didelphys: two bodies, two attached cervices, one vagina.
• Bicornate uterus: two bodies, one cervix.
• Bicornate uterus with rudimentary horn.
• Arcuate uterus: slight depression of the fundus.
• Septate uterus.
• Uterus unicornis: develops from one mullerian duct only.

Uterus Anomaly Differentiation

• HSG alone cannot differentiate between bicornuate and septate uterus.
• Hysteroscopy and laparoscopy are better tools to differentiate.

Imperfect Uterus Canalization

• Cervical atresia.
• Requires sounding of cervical canal to differentiate between CX atresia and CX Stenosis

Other Uterus Abnormalities

• Congenital elongation of portiovaginalis of CX.
• Cochleate uterus: C-shaped uterus with concavity forwards.

Clinical Presentation of Uterus Anomalies

• Gynecological presentation: menstrual disturbances (amenorrhea, hypomenorrhea, or menorrhagia in bicornuate uterus), dysmenorrhea, dyspareunia, and infertility.
• Obstetric presentation: abortion, recurrent abortion, preterm labor, obstructed labor, increased rate of C-section, and postpartum hemorrhage.

Investigations for Uterus abnormalities

• HSG is used for diagnoses.
• Imaging, including U/S, CT, and MRI
• Endoscopy, like Hysteroscope & laparoscope.

Treatment for Uterus Abnormalities

• Uteroplasty (metroplasty).
• Laparotomy.
• Hysteroscopic resection of septum like use of Diathermy or laser

Vaginal Abnormalities

• Imperfect development (aplasia): treated with neovagina formation using Mc-indoe’s operation.
• Imperfect fusion of mullerian ducts causes longitudinal vaginal septum.
• Imperfect canalization causes transverse vaginal septum obstructing drainage, or imperforate hymen.

External Genitalia Abnormalities

• Fused labia.
• Ectopic anus.
• Hypertrophy of clitoris.

Imperforate Hymen

• Complete closure of the vagina due to imperfect canalization leading to retained menstruation and is the most common cause of cryptomenorrhea.
• Pathology: distension of vagina with blood (hematocolpos), blood in the uterus (hematometra) , blood in tubes(hematosalpinx), and distended vagina pressing on nearby organs leads to retention of urine, and tenesmus.

Clinical Findings of Imperforate Hymen

• Delayed menstruation (primary amenorrhea).
• Abdominal pain, especially suprapubic, recurring every month.
• Difficulty then retention with urine.
• Pelviabdominal mass.
• Bulging bluish imperforate hymen.
• Rectal examination reveals a cystic swelling in front of the rectum.

Treatment for Imperforate Hymen

• Under general anaesthesia and asepsis cruciate incision in the hymen, and the retained blood should be allowed to escape slowly and spontaneously as well administering antibiotics to decrease incidence of infection.

Intersex

• Imperfect sexual differentiation between male and female.

Abnormal and Normal Types of Sex

• Normal types: chromosomal sex, gonadal sex, hormonal sex, and gender sex.
• Abnormal types: chromosomal intersex, gonadal intersex, pseudohermaphrodite, and social intersex.

Types of Intersex

• Chromosomal intersex: Turner’s syndrome (45 X0) and Klinefelter syndrome (47 XXY).
• Gonadal intersex: selective gonads and/or ovitestes on either or both sides.

Pseudohermaphrodite

• Hormonal intersex where:
  • Male type: gonads are male, but external genitalia are female (testicular Feminization syndrome = Androgen insensitivity Syndrome), requiring testes removal at puberty due to malignancy risk.
• Female type: gonads are female, but external genitalia are male (congenital adrenal hyperplasia), related to decreased 21 hydroxylase leading to increased ACTH and androgen, requiring cortisone replacement.

Social Intersex

• A social intersex is in homosexual patients.

Intersex Investigations

• Chromatin pattern from buccal smear (XX = positive Barr body as normal female.
• Turner’s syndrome = negative Barr body).
• Kleinfilter= positive Barr body.
• Chromosomal culture, hormonal assay, U/S, C/T, MRI, Laparoscopies.
• Investigation for Congenital anomalies especially skeletal , renal , CNS.