Fuse H, Minagawa H, Ito H, Shimazaki J
Hinyokika Kiyo. 1983 Apr;29(4):451-7.
The patient was a two years and seven months old, who was legally a female. She visited our University Hospital with the chief complaint of ambiguous genitalia. The hypertrophied clitoris was 13 mm in length and 11 mm in diameter. The external urethral orifice and vagina were observed separately. The testis-like mass was palpable in the right labium. She had a karyotype of 46XX . The Urinary excretion of 17 KS and pregnanetriol and serum 17 alpha-hydroxyprogesterone were within the normal range. Vaginography showed the vagina, uterus and left fallopian tube. At exploratory laparotomy, we found the uterus and left fallopian tube; the left and right gonads were in the intraperitoneal cavity and the inguinal canal, respectively. To build female characteristics, clitoroplasty was performed, the right gonad was removed the testis-like portion of the left gonad was resected, and the ovary-like portion in the left gonad was biopsied. Both gonads were confirmed to be ovotestis histologically.
该患者为两岁七个月大的法定女性。她因生殖器模糊为主诉前来我院就诊。肥大的阴蒂长13毫米，直径11毫米。可见尿道口和阴道分开。右侧阴唇可触及睾丸样肿物。她的核型为46XX。17酮类固醇和孕三醇的尿排泄量以及血清17α-羟孕酮均在正常范围内。阴道造影显示了阴道、子宫和左侧输卵管。在探查性剖腹手术中，我们发现了子宫和左侧输卵管；左右性腺分别位于腹腔和腹股沟管内。为塑造女性特征，进行了阴蒂成形术，切除了右侧性腺，切除了左侧性腺的睾丸样部分，并对左侧性腺中的卵巢样部分进行了活检。组织学检查证实双侧性腺均为卵睾。
