Krstić Z D, Smoljanić Z, Vukanić D, Varinac D, Janjić G
University Children's Hospital, Belgrade, Yugoslavia.
Pediatr Surg Int. 2000;16(8):580-3. doi: 10.1007/s003830000415.
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.
真两性畸形（TH）是两性畸形中最罕见的一种变异类型。根据定义，这些患者同时具有卵巢和睾丸组织。大多数患者因生殖器模糊和/或男性乳房发育而就诊。1986年至1996年，我们共治疗了97例两性畸形患者，其中4例为真两性畸形。在所有病例中，睾丸和卵巢组织是分开的。3例患者的外生殖器模糊，1例男性生殖器发育不全。3例患者核型为46，XY，1例为46，XX。3例患者登记为男性，1例登记为女性。所需手术次数从3次到9次不等（平均每位患者5.7次）。2例自幼被当作男性抚养的患者青春期后出现生殖器发育不全和睾丸发育不良。1例结婚后能勃起，但无射精。2例男性患者接受了雄激素补充治疗。唯一1例在阴蒂阴道成形术后被当作女性抚养的患者，其阴道深6cm，盲端，经扩张术加深至12cm后能够进行正常性交。目前认为，大多数真两性畸形患者应被当作女性抚养。这一点在我们的患者中也得到了证实，强调了早期诊断的重要性。
