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Disorders of Sexual Differentiation
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==== Female development ==== * '''Testosterone is not secreted by the ovaries and therefore the Wolffian ducts regress''' ** '''Wolffian duct remnants in the female (2):''' **# '''Gartner duct''' **#* '''Can form cysts near the vaginal introitus and anterolateral vaginal wall''' **# '''Epoophoron and paroophoron: in the mesentery of the ovary''' * '''Estrogen synthesis by the ovary is detectable in the female embryo just after 8 weeks of gestation.''' ** '''The rate-limiting enzyme is aromatase, which is higher in the fetal ovary than in the fetal testis.''' * '''Estrogens are not required for normal female differentiation of the reproductive tract, but they can interfere with male differentiation.''' ** '''Estrogen can block the effect of AMH/MIS on Müllerian ducts''' ** '''Prenatal estrogen treatment of mothers has been associated with male reproductive tract abnormalities''' * '''Because the ovary does not produce AMH/MIS, the Müllerian ducts are maintained and develop into the female internal reproductive tract (4):''' *# '''Fallopian tubes''' *# '''Uterus''' *# '''Cervix''' *# '''Proximal 2/3rd of the vagina''' *#* '''The distal 1/3rd of the vagina is derived from the urogenital sinus''' * '''In females and males with abnormalities in testosterone and/or DHT production, 5α-reductase deficiency, or androgen-receptor insufficiency, the primitive perineum does not lengthen and the''' *# '''Genital tubercle bends inferiorly, passively become the clitoris''' *# '''Urethral folds passively become the''' '''labia minora''' *# '''Labioscrotal swellings passively become the labia majora''' ** '''The definitive urogenital sinus becomes the vestibule of the vagina''' ** '''5α-reductase deficiency more likely associated with ambiguous genitalia whereas complete androgen-receptor insufficiency have near-normal female external phenotype'''
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