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腹腔镜手术诊治卵睾型性发育异常13例分析

Laparoscopic Surgery for Ovotesticular Disorders of Sex Development:Analysis of 13 Cases
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摘要 目的总结卵睾型性发育异常的腹腔镜手术诊疗经验。方法回顾性分析2015年1月~2018年10月我院收治并确诊卵睾型性发育异常13例资料,年龄6个月~3岁9个月(中位年龄1岁8个月)。社会性别7例为男性,6例为女性。完善检查后行腹腔镜性腺探查,对双侧性腺包括盆腔、腹股沟、阴囊内性腺均予活检。结果13例腹腔镜性腺探查活检病理结果:双侧卵睾2例,一侧卵巢、一侧睾丸6例,一侧卵睾、一侧睾丸2例,一侧卵巢、一侧卵睾3例。再次手术行性腺切除及外生殖器整形手术。13例随访3~24个月,平均14.5月。外生殖器发育基本正常,排尿良好,无社会性别与生长发育不协调。结论卵睾型性发育异常患儿的腹腔镜手术诊疗包括性腺探查活检,性腺切除与固定等,手术方案应依据外生殖器表型、性腺优势、社会性别等综合评估,谨慎决定。 Objective To summarize the experience of laparoscopic therapy for ovotesticular disorders of sex development( DSD). Methods From January 2015 to October 2018,13 children with ovotesticular DSD admitted in our hospital underwent laparoscopic treatment. The age for first surgery ranged from 6 months to 3 years 9 months old( median,1 year 8 months). For 7 patients the social gender was male,and for the rest 6 patients the social gender was female. To determine the gonadal tissue components,biopsy of all gonads in the abdominal cavity,intra-scrotal and intra-groin was required after laparoscopic exploration.Results The pathological results were bilateral ovariotestis in 2 cases,unilateral ovary and testis in 6 cases,unilateral ovariotestis and testis in 2 cases,and unilateral ovariotestis and ovary in 3 cases. Based on diagnostic laparoscopic findings,the gonads and/or ductal structures incompatible with the social gender were removed by laparoscopy,and plastic reconstructions of external genitalia were performed. The patients recovered well during a 3-24 months of follow-up( average,14. 5 months) with good morphologic and cosmetic outcomes. No malignant inclination of the gonad or significant retraction of the testis was found. Conclusions Laparoscopic procedures for ovotesticular DSD include gonadal exploration and biopsy,gonadectomy and orchiopexy. The operation schemes are depended on external genitalia phenotype,gonads evaluation,social psychology,and so on.
作者 邱颖 白东升 叶辉 郝春生 李龙 牛志尚 宋晋秋 张晨捷 王宏扬 刘晓萌 Qiu Ying;Bai Dongsheng;Ye Hui(Department of Urology,The Affiliated Children’s Hospital,Capital Institute of Pediatrics,Beijing 100020,China)
出处 《中国微创外科杂志》 CSCD 北大核心 2019年第6期518-522,共5页 Chinese Journal of Minimally Invasive Surgery
基金 北京市医院管理局青年人才培养“青苗”计划(QML20171303) 北京市医院管理局重点医学专业发展计划(扬帆计划)(ZYLX201825)
关键词 卵睾 性发育异常 腹腔镜 性腺活检 Ovotestis Disorders of sex development Laparoscopy Gonadal biopsy
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