摘要
目的探讨卵睾型性发育异常临床特点。方法回顾性分析60例2007年1月至2017年12月因卵睾型性发育异常于首都医科大学附属北京儿童医院泌尿外科住院治疗患儿的临床资料,并对卵睾型性发育异常的临床特征进行归纳总结。结果60例中社会性别男42例,社会性别女18例;多以外生殖器异常就诊。46,XX DSD 52例;46,XY DSD 3例;性染色体DSD 5例。48例术前行盆腔及性腺超声检查,其中32例子宫发育欠佳,22例见阴道。60例均行性腺活检,性腺总数120个,其中卵睾74个,睾丸16个,卵巢30个。双侧型19例,单侧型36例,片侧型5例。33例行手术干预,其中按男性抚养者22例,均行阴茎矫直术和尿道重建术;按女性抚养者11例,均完成阴蒂矫形术。结论卵睾型DSD临床表现多样,容易漏诊,需提高警惕。如何进行性别、手术时机的选择目前仍然存在很大争议。
Objective To explore the clinical characteristics of ovotesticular disorders of sex development in children.Methods The clinical data were retrospectively reviewed for 60 hospitalized children with ovotesticular disorders of sex development from January 2007 to December 2017.Results Social gender:42 boys and 18 girls.Most of them had abnormal external genitalia.There were 46,XX DSD(n=52),46,XY DSD(n=3)and sex chromosome DSD(n=5).Forty-eight children were examined preoperatively by pelvic and gonadal ultrasonography.Underdeveloped uterus(n=32)and vagina(n=22)were detected.Gonadal biopsy was performed(n=60).The total number of gonads was 120,including ovotestes(n=74),testes(n=16)and ovaries(n=30).Exploration of gonads revealed bilateral type(n=19),ovotesticularplus ovary or testis(n=36),unilateral ovary and contralateral testis(n=5).Among 33 cases of surgical interventions,22 underwent penile straightening and urethroplasty.Clitoris correction was performed(n=11).Conclusion The clinical characteristics of ovotesticular disorders of sex development are diverse and easily missed.Thus clinicians should stay vigilant.Currently there is still a great controversy on choosing gender and operative timing.
作者
王冠男
宋宏程
梁海燕
许帅
孙宁
Wang Guannan;Song Hongcheng;Liang Haiyan;Xu Shuai;Sun Ning(Department of Urology,Beijing Children's Hospital,Capital Medical University,Beijing 100045,China)
出处
《临床小儿外科杂志》
CAS
2020年第11期1021-1025,共5页
Journal of Clinical Pediatric Surgery
关键词
卵睾型性发育异常
临床特点
Ovotesticular Disorders of Sex Development
Clinical Characteristics
