Genuine hermaphroditism

True hermanaphroditism is characterized by the presence of both ovarian and testicular tissue. Thus, the external genitalia may be female as well as male or ambiguous.

All combinations of testis and ovary are possible, but most of the gonads that have appeared are ovotestis. The labioscrotal, inguinal or abdominal gonads are crucial for the development of the inner sex. This differs depending on the adjacent gonad.

Common signs include the onset of menstruation, breast development, and feminization, an often malformed vagina ending in the urogenital sinus. A hypoplastic uterus is also possible in the case of an appearing uterus. The patients are mostly infertile.

The genetic cause is different: 60% of the cases have a female karyotype 46, XX, 20% a 46, XY karyotype, and in 20% there is a mosaic 46, XX / 46, XY.