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 a ll cases testicular and ovarian tissue was separate. In 3 patients the exte rnal genitalia were ambiguous and 1 had hypoplastic male genitalia. Three p atients had a 46, XY and 1 a 46, XX karyotype. Three patients had been list ed as males and 1 as a female. The number of operations required varied fro m 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 an d could have erections, but no ejaculation. Two male patients have been on supplementary androgen therapy. The only patient who had been raised as a f emale after clitorovaginoplasty had a 6-cm-deep, blind-ending vagina that w as deepened to 12 cm with bougienage, so that she was capable of having nor mal 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 under lines the significance of early diagnosis.