Extreme virilization in patients with congenital adrenal hyperplasia fails to induce descent of the ovary

Abstract

The commonly held hypothesis that androgens cause testicular descent in the male [9] predicts that excess endogeous androgens in the female fetus might cause descent of the ovary. To re-evaluate this prediction from a clinical perspective, a retrospective review was made of genotypic females with congenital adrenal hyperplasia and severe virilization. Patients with maximal virilization were included, since this is the group with the earlies and highest production of adrenal androgens during fetal development. Records were reviewed of five children in whom the external genitalia were completely or almost completely masculinized, who had been regarded as “males with undescended testes” at birth. The position of the ovaries was determined where possible from operative or pathological reports. In four patients the ovaries had been identified at operation, and were found to be in their normal position. In none were the ovaries in or adjacent to the inguinal canal. In the most recent patient, who presented in 1979, laparotomy was not performed because of the belief that the female internal genitalia were “normal”. In addition, review of the literature revealed two other similar female children with maximal virilization but documented normal ovarian position. The significance of these well-known findings has been ignored in recent studies of testicular descent. Failure of endogenous androgens to affect ovarian position supports the view that initiation of gonadal descent is independent of androgens. Since Müllerian inhibiting substance is the other recognizable hormone in the fetal testis apart from testosterone, it is suggested that it may be responsible for initiating gonadal descent.