Wang Jing-qi, Wang Dong-wen, Liu Chun, Yang Hua, Xu Ji-xiu
Zhonghua Nan Ke Xue. 2015 Oct;21(10):917-20.
To investigate the characteristics, diagnosis, and treatment of ovotesticular disorder of sex development (OT-DSD).
We retrospectively analyzed 2 cases of OT-DSD treated in our hospital. The patients were 19 and 15 years old, respectively, and both received systematic physical examination and examinations of the karyotype, sex hormone, adrenocorticotropic hormone (ACTH), color Doppler ultrasonography, urethrocystoscopy, and human chorionic gonadotropin (HCG) test. Under the laparoscope, we performed surgical gonad exploration, gonadectomy, and vulvar orthopedics. Intraoperative exploration and pathology confirmed true hermaphroditism in both cases, with sex selection as female. One underwent laparoscopic resection of the ovotestis, and the other removal of the testis with the ovarian tissue reserved.
The patients were followed up for 12 months postoperatively, which found no abnormality in either the vulvas or the genital glands.
Surgical exploration of the gonad is the only method for the diagnosis of OT-DSD and sex selection is the key to treatment. Laparoscopic surgical exploration of the gonad and vulvar orthopedics are the first treatment options.
探讨卵睾性发育障碍（OT-DSD）的特点、诊断及治疗方法。
回顾性分析我院收治的2例OT-DSD患者。患者分别为19岁和15岁，均接受了系统的体格检查、染色体核型、性激素、促肾上腺皮质激素（ACTH）、彩色多普勒超声、尿道膀胱镜检查及人绒毛膜促性腺激素（HCG）试验。在腹腔镜下，我们进行了性腺探查手术、性腺切除术及外阴整形术。术中探查及病理证实2例均为真两性畸形，性别选择为女性。1例行腹腔镜下卵睾切除术，另1例切除睾丸并保留卵巢组织。
术后对患者进行了12个月的随访，发现外阴及性腺均无异常。
性腺手术探查是诊断OT-DSD的唯一方法，性别选择是治疗的关键。腹腔镜下性腺手术探查及外阴整形术是首选的治疗方式。
