True hermaphroditism: 10 years' experience.
True hermaphroditism (TH) is the rarest variant of intersex malformations. By definition, these patients have both ovarian and testicular tissue. Most present due to ambiguous genitalia and/or gynecomastia. From 1986 to 1996, we treated 97 patients with intersex disorders; there were 4 with TH. In all cases testicular and ovarian tissue was separate. In 3 patients the external genitalia were ambiguous and 1 had hypoplastic male genitalia. Three patients had a 46, XY and 1 a 46, XX karyotype. Three patients had been listed as males and 1 as a female. The number of operations required varied from 3 to 9 (mean 5.7 per patient). Two patients who had been raised as males finished puberty with hypogenitalism and hypoplastic testes. One married and could have erections, but no ejaculation. Two male patients have been on supplementary androgen therapy. The only patient who had been raised as a female after clitorovaginoplasty had a 6-cm-deep, blind-ending vagina that was deepened to 12 cm with bougienage, so that she was capable of having normal intercourse. It is felt at present that most patients with TH should be raised as females. This was confirmed in our patients as well, which underlines the significance of early diagnosis.