Mixed gonadal dysgenesis (MGD) is a rare disorder of sexual development (DSD). A seven-year-old child with vagina and an elongated clitoris looking like a penile part of male external genitalia, presented in the Paediatric Surgery Department. Ultrasound of pelvis revealed a streak ovary on the right side and ovotestis on the left side. Uterus was not visualized. Karyotyping revealed 46, XY/45, XO genotype. Laparoscopic gonadectomy with vaginoplasty and the clitoral reduction was performed. A thorough histopathological examination of the specimen revealed structure of ovotestis-consisting of the ovary with Graafian follicles, fallopian tube, infantile testis with Leydig and Sertoli cells, epididymis, vas deference, and pampiniform plexus. Presence of Graafian follicle in the ovary, Leydig and Sertoli cells in the testis, at this age makes this case extremely interesting. The child was rehabilitated to lead her life as a girl.

译文

:性腺混合发育不良(MGD)是一种罕见的性发育障碍(DSD)。小儿外科介绍了一个七岁的孩子,有阴道和细长的阴蒂,看起来像是男性外生殖器的阴茎部分。骨盆超声检查显示右侧有条状卵巢,左侧有卵睾丸。子宫不可见。核型分析显示46,XY / 45,XO基因型。进行腹腔镜性腺切除术,阴道成形术和阴蒂复位术。对该标本进行彻底的组织病理学检查,发现包含卵形睾丸的卵巢结构包括卵巢囊,输卵管,具有睾丸间质细胞和睾丸支持细胞的婴儿睾丸,附睾,输精管和绒毛状丛。在这个年龄,卵巢中存在Graafian卵泡,睾丸中存在Leydig和Sertoli细胞,这种情况非常有趣。这个孩子已经康复,过着小时候的生活。

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