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患者,社会性别男,22岁。因会阴型尿道下裂于1989年4月21日入院。患者18岁开始有乳房发育,每月有月经来潮,量少,每次行经3天。有性冲动及“梦遗”。检查:男性装束,皮肤细白,无胡须及腋毛,喉结较明显。双侧乳房隆起,乳晕明显,阴毛分布呈倒三角形。阴茎短小、屈曲,尿道与阴道同一开口于会阴部,口径约2cm,右侧阴囊内触及2×1×1cm睾丸,质地正常,左侧阴囊内无内容物。B超检查发现幼稚子宫。实验室检查:FSH9.8mIU/ml,LH42mIU/ml,尿17-羟及17-酮正常范围,染色体核型为46XX。手术探查:右侧阴囊内有睾丸,盆腔内子宫大小4×3×2cm,左侧有卵巢和输卵管。切除子宫、卵巢及输卵管,阴茎屈曲矫正。病理切片证实为子宫、卵巢及卵泡组织。最后诊断为真两性畸形。同年11月21日再次入院行阴囊带蒂皮管尿道成形术,住院21天痊愈出院。术后尿道开口于阴茎头部,能直立排尿,有勃起及性冲动。讨论真两性畸形是性分化畸形中极为罕见的
Patient, male, 22 years old. Due to perineal hypospadias in April 21, 1989 admission. Patients 18 years of age have breast development, monthly menstrual cramps, less, each passing through 3 days. Sexual impulse and “dreams.” Check: men’s clothing, skin white, no beard and armpit hair, Adam’s apple more obvious. Bilateral breast bulge, wereola obvious, pubic hair was inverted triangle. Short penis, flexion, urethra and vagina the same opening in the perineum, diameter of about 2cm, right scrotum touch 2 × 1 × 1cm testis, normal texture, no content within the left scrotum. B-ultrasound found that childish uterus. Laboratory tests: FSH9.8mIU / ml, LH42mIU / ml, urinary 17-hydroxy and 17-ketones normal range, chromosome karyotype 46XX. Surgical exploration: There are testicles in the right scrotum, pelvic uterine size 4 × 3 × 2cm, the left ovary and fallopian tubes. Excision of the uterus, ovary and fallopian tubes, penile flexion correction. Pathological confirmed as uterine, ovarian and follicular tissue. The final diagnosis of true hermaphroditism. November 21 the same year again admitted to the hospital scrotum pedicle urethroplasty, hospitalized 21 days cured. Postoperative urethral opening in the penis head, can urinate upright, erectile and sexual impulses. Discussing true hermaphroditism is extremely rare in degenerative deformities