DEVELOPMENT OF THE EXTERNAL GENITALIA

Influence of the Sex Glands on Further Sex Differentiation

Testis
Mullerian inhibiting substance (Sertoli cells) Paramesonephic ducts suppressed Testosterone (Leydig cells) Mesonephric ducts stimulated (vas deferans, epididymis) Dihydrotestosterone External genitalia stimulated Growth of penis, scrotum, and prostate

Ovary
Estrogens (including maternal and placental sources) Paramesonephric ducts stimulated (uterine tube, uterus, upper portion of vagina External genitalia stimulated (labia, clitoris, lower portion of vagina)

Development of Indifferent Genitalia

Third week of development, cell from development, primitive streak migrate around cloacal membrane and form a pair of elevated folds, cloacal folds. folds. Cloacal folds unite cranially to form genital tubercle Sixth week- cloacal folds divide to form weekurethral folds and anal folds Genital swelling - another pair of elevations on each side of urethral folds

Development of External Genitalia Indifferent Stage

Genital Tubercle Cloacal Fold Phallus Urogenital fold Anal fold Cloacal Membrane Urogenital Membrane Anal Membrane Genital Swelling Labioscrotal swelling

Formation of Female Genitalia (Estrogen Effect)

Genital tubercle becomes clitoris Urethral folds do not fuse and form the labia minora Urogenital groove becomes vestibule Genital swelling becomes the labia majora

Development of Male External Genitalia (Androgen effect)

Genital tubercle elongates to become phallus Urethral folds form the lateral walls of urogenital groove, fuses at 12th week to form penile urethra External urethral meatus is derived from invagination of ectodermal cells from tip of glans penis Genital swellings move caudally and fuse with each other to form scrotum

Hypospadias
Failure of urethral folds to fuse completely resulting in urethral openings at ventral surface of penile shaft

Epispadias
Urethral opening at the dorsum of penis due to abnormal location of genital tubercle

ABNORMALITIES OF SEXUAL DIFFERENTIATION

Klinefelters syndrome 47,XXY Gonadal dysgenesis ( Turners syndrome) 45,XO Pure Gonadal dysgenesis 46,XX or 46,XY Testicular Feminization syndrome 46,XY True Hermaphrodite - rare Pseudohermaphrodites male 46,XY insufficient androgens female 46, XX adrenogenital syndromsyndromexcessive androgens

Turner s Syndrome- 45 XO Syndrome-

Testicular Feminization Syndrome

Female PseudohermaphroditePseudohermaphroditeCongenital Adrenal Hyperplasia