带蒂包皮岛状皮瓣阴道成形术在46,XX性发育异常患儿女性化手术中的应用  

Preliminary exploration of pedicled prepuce island flap vaginoplasty for 46,XX DSD feminization

作　　者：张正涛 刘国昌[1] 邓富铭 高晓峰 朱士博 赵天鑫 伏雯 Zhang Zhengtao;Liu Guochang;Deng Fuming;Gao Xiaofeng;Zhu Shibo;Zhao Tianxin;Fu Wen(Department of Pediatric Urology,Municipal Women&Children Medical Center,Guangzhou 510120,China)

机构地区：[1]广州医科大学附属广州市妇女儿童医疗中心小儿泌尿外科,广州510120

出　　处：《临床小儿外科杂志》2024年第10期976-980,共5页Journal of Clinical Pediatric Surgery

基　　金：国家自然科学基金青年项目(82200742)。

摘　　要：目的探讨带蒂包皮岛状皮瓣阴道成形术在46,XX性发育异常(46,XX differences of sex development/disorders of sex development,46,XX DSD)患儿女性化手术中的应用,并对其疗效进行评估。方法本研究为回顾性研究。2017年1月1日至2020年12月1日,广州医科大学附属广州市妇女儿童医疗中心小儿泌尿外科采用带蒂包皮岛状皮瓣阴道成形+阴蒂短缩、Y-V阴唇成形手术治疗46,XX DSD患儿共9例。此9例全部为21-羟化酶缺乏导致的先天性肾上腺皮质增生症(congenital adrenal hyperplasia,CAH),均为失盐型,外阴Prader分型为Ⅲ~Ⅴ型,泌尿生殖窦造影均为高位尿道阴道合流。平均年龄20.2个月(10~62个月)。观察术后外生殖器外观及术后尿道阴道瘘、阴道狭窄、尿失禁等并发症发生情况。结果9例中失访1例,8例获随访;术后3~4 d拆除敷料,术后5~7 d出院,术后2周拔除尿管及阴道支架管。8例术后外生殖器外观均得到患儿家属认可,仅1例出现阴道狭窄,经阴道扩张改善。8例均未出现尿失禁及尿频、排尿困难等下尿路症状,无一例出现尿道阴道瘘。随访12~60个月,术后外生殖器外观和早期排尿功能均达到满意效果。结论带蒂包皮岛状皮瓣阴道成形术是治疗46,XX DSD高位尿道阴道合流的有效方法,可单独或联合整体尿生殖窦游离(total urogenital mobilization,TUM)成形阴道。术后可获得开口于前庭的无狭窄阴道、良好的排尿控制能力和满意的外生殖器外观,适用于各种外阴女性化手术。Objective To explore the application of pedicled prepuce island flap vaginoplasty for femi-nized children with masculinized 46,XX sexual dysplasia and high vaginal confluence and evaluate its efficacy.Methods From January 1,2017 to December 1,2020,9 children with masculine 46,XX differences of sex de-velopment/disorders of sex development(46,XX DSD)underwent pedicled prepuce island flap vaginoplasty plus clitoris shortening and Y-V labioplasty.All of them belonged to salt loss type congenital adrenal hyperplasia(CAH)due to 21 hydroxylase deficiency.Prader type was II-V with high vaginal confluence.The average age was 20.2(10-62)month.Dressing was removed at Day 3-4 post-operation and discharge occurred at Day 5-7 post-operation.Urinary catheter and vaginal stent were removed at Week 2 post-operation.Results Among them,8 cases were followed up for(12-60)month.The postoperative appearance of external genitalia was rec-ognized by family members.One case of vaginal stenosis was improved through vaginal dilation.There were no onsets of such lower urinary tract symptoms as urinary incontinence,frequent urination and dysuria or urethro-vaginal fistula.Both postoperative appearance and early function were satisfactory.Conclusions As an effec-tive treatment for high vaginal confluence of masculine 46,XX DSD,vaginoplasty with pedicled prepuce island flap may be applied alone or in combination with total urogenital mobilization(TUM).After operation,a narrow vagina forms with an opening in vestibule,decent urinary control and satisfactory appearance of external genita-lia.It is suitable for all kinds of vulva feminization surgery.

关 键 词：阴道成形术 带蒂包皮岛状皮瓣 外科手术 儿童 

分 类 号：R69[医药卫生—泌尿科学]