Intersex disorders in the newborn. A brief summary of current views.

FEW congenital abnormalities can be so distressing for parents than the discovery of ambiguous genitalia in their newborn infant. Intersex is the term used to describe such patients, of which there are 3 groups: (1) The Female Pseudohermaphrodite who has normal ovaries, but masculinized external genitalia; (2) The Male Pseudohermaphrodite who despite testicular tissue, has external genitalia which are incompletely masculinized, or even completely feminized, and (3) The True Hermaphrodite in whom gonadal tissue of both sexes is associated with varying degrees of masculinization of the external genitalia. The term hermaphrodite comes from Greek mythology. Hermaphroditus was the offspring of Hermes and Aphrodite. A water nymph became infatuated with this youth and prayed to be united with him for ever. Immediately their two bodies were joined and they became as one. "In their double form they are neither man nor woman; they seem to have no sex yet to be of both sexes". (Larousse, 1959). An understanding of the genesis of these disorders requires a brief review of the embryological events which control the formation of the genitalia (Fig. 1). Prior to the seventh week the fetal gonads are bi-potential and each is associated with both a Mullerian and a Wolffian duct. If the individual's karyotype is 46XX (female) the cortex of the primitive gonad become an ovary. The Wolffian ducts then atrophy and the Muillerian ducts persist to form the fallopian tubes, uterus and upper vagina. On the other hand, if the karyotype is 46XY (male) the medulla of the gonad becomes a testis. Although much is not understood of the factors