1. Development of female genital system
Dr. Lubna Nazli

2. Objectives Devp of ovaries, uterus and fallopian tubes
Devp of external genitalia
Congenital malformations of FGS
Hermaphrodites: types

3. The primordial germ cells arise from the yolk sac/endoderm and migrate into the intermediate mesoderm

4. Development of ovaries
Is from the same source as the testis.
The intermediate mesoderm/ genital ridge, is invaded by the sex cells arising from the yolk sac endoderm.
These sex cells are having XX chromosomal complement, in absence of SRY gene, the anti-mullerian factor is not produced.

5. Migration of sex cells
Along dorsal mesentery

7. The primary sex cords grows into the medulla.
They give rise to secondary sex cords.
Later they form the primordial ovarian follicles.
The mullerian duct system develops parallel to the mesonephric duct.

8. The paramesonephric ducts are formed by infolding of the coelomic epithelium.
Parallel to the mesonephric ducts, paramesonephric ducts grows/ Mullerian ducts.

9. Female fetus
In the absence of TDF, medulla regresses and cortical sex cords break up into isolated cell clusters(primordial follicles)
in the absence of AMH & testosterone,
Mesonephric duct system degenerates
Then, paramesonephric duct system develops
Inf. fused portion
Uterovaginal canal -> uterus and upper vagina
Cranial unfused portions
Open into celomic cavity(future peritoneal cavity)
Fallopian tubes

10. The PMN duct form the fallopian tubes from the upper 1/3rd, fundus, body and cervix from the fused middle 1/3rd and lower 1/3rd will form the upper part of vagina.

11. The lower end of the duct opens up in the urogenital sinus/ the posterior wall of cloaca.
The vagina develops from the sinovaginal bulb.

14. The female external genitalia development
At the lower end of the embryo, around the cloacal membrane a median genital tubercle grows and it is called as phallus.
On either side of the cloacal membrane, a labioscrotal swellings develop, the fore runner of the labia majora.
In female fetus, the labioscrotal swelling will give rise to labia majora.
The labia minora is developing from the urethral folds that has not united.
The undeveloped phallus remains as clitoris.

15. The embryological derivatives
Clitoris = median genital tubercle
Labia majora = Labioscrotal swellings/lateral genital swellings
Labia minora = Urethral groove
Vagina = sinovaginal bulb
Uterus/tubes = Paramesonephric ducts
Ovary = Genital ridge/Intermediate mesoderm

16. Congenital anomalies of uterus development

17. Unicornuate - Where one of the Müllerian duct fails to develop resulting in a single uterine horn.
Bicornuate - Both ducts form but one duct develops to a greater extent than the other.
Consequently a small "rudimentary horn" exists, which may end blind or be continuous with the dominant horn.
There is usually only one cervix.

18. Double vaginas
Double - Complete failure of fusion of the Müllerian ducts. As a result of the independence of the ducts, two uteri develop, each with their own cervix. In addition one or two vaginas may be found.
Septate - Both ducts develop but they only partially fuse. This leaves a septum at the fundus

19. Imperforate hymen If the cloacal membrane does not rupture
It will give rise to imperforate hymen
A congenital anomaly

20. Other Congenital Malformations
Ovarian hypoplasia
Pure gonadal dysgenesis
Testicular feminization syndrome
Hermaphroditism
true
Female pseudohermaphroditism
Male pseudohermaphroditism

21. Pure Gonadal Dysgenesis
Normal karyotypes (46, XX or 46, XY)
Primordial germ cells do not migrate from the yolk sac
No development of the ovaries or testes

22. Testicular Feminization Syndrome
Occurs at rate of 1:50,000
Appears to be a normal female despite the presence of testes in either abdomen or inguinal region
Testes produce normal levels of testosterone
Tissues unresponsive to androgens
External genitalia are normal
Shallow blind ending vagina

23. Hermaphroditism True hermaphrodite
Both ovaries and testicular tissue present
Masculine form
Feminine form
Intermediate form (more common)
46, XX/ 46, XY or 46, XX/ 47, XXY

24. Hermaphroditism Female pseudohermaphroditism (46, XX)
AKA congenital adrenogenital syndrome
Masculinization due to high level of androgens from adrenal cortex
Male pseudohermaphroditism
Testes and ambiguous female genitalia
Many types, most common is of unknown etiology
Often considered females at a young age because penis is absent
Raised as girls until puberty when male secondary sex characteristics appear via endocrine activities of testes

25. Genital system abnormalities
Congenital defects in sexual development, usually arising from a variety of chromosomal abnormalities, tend to present clinically with ambiguous external genitalia
Known as intersex conditions or hermaphroditism
Classified according to the histologic appearance of the gonads

26. (1) True hermaphroditism
Individuals with true hermaphroditism
Have both ovarian and testicular tissue
Most commonly as composite ovotestes
Occasionally with an ovary on one side and a testis on the other
In the latter case, a fallopian tube and single uterine horn may develop on the side with the ovary
     ∵ absence of local AMH
Extremely rare condition

27. (2) Pseudohermaphroditism
In individuals with pseudohermaphroditism,
Genetic sex indicates one gender
External genitalia has characteristics of the other gender
Caused either by abnormal levels of sex hormones or abnormalities in the sex hormone receptors

28. (2) Pseudohermaphroditism
Males with pseudohermaphroditism
Genetic males with feminized external genitalia
Hypospadias(urethral opening on the ventral surface of the penis)
Incomplete fusion of the urogenital or labioscrotal folds ; m/c manifesting sx.
Females with pseudohermaphroditism
Genetic females with virilized external genitalia
Clitoral hypertrophy
Some degree of fusion of the urogenital or labioscrotal folds