Mao Yu,Xia Meng,Cai Yongchuan,et al.Masculinizing plasty in 46,XY severely undervirilized genital[J].Journal of Clinical Pediatric Surgery,2019,18(03):196-201.[doi:10.3969/j.issn.1671-6353.2019.03.007]
46,XY严重男性化不全外阴的男性化整形方式探讨
- Title:
- Masculinizing plasty in 46,XY severely undervirilized genital
- Keywords:
- Gonadal Dysgenesis; 46; XY/Embryo; Vulva/Abnormalities; Plastic
- 分类号:
- R726.9;R726.2
- 摘要:
- 目的 总结46，XY严重男性化不全的外阴男性化整形的临床经验，并初步评估本中心所采用的几种整形方式的治疗效果。方法 回顾性分析64例2014年1月至2018年11月间于电子科技大学附属医院儿童医学中心进行外生殖器男性化整形的严重外阴男性化不全患儿临床资料。64例染色体核型均为46，XY，外生殖器表型均为会阴型尿道下裂合并完全性阴囊对裂或阴茎阴囊转位。47例阴茎头横径<10 mm，双侧隐睾9例，单侧隐睾6例。47例采用雄激素治疗，患儿均进行阴茎下曲矫正术、尿道成形术、阴茎阴囊转位术和阴囊对裂矫正术，15例隐睾患儿同期进行睾丸下降固定术。尿道成形采取岛状包皮瓣卷管、尿道板重建卷管及其对应的一期或分期尿道成形术（我院在重型尿道下裂修复中常用的手术方式）。结果 雄激素治疗后阴茎头横径均≥10 mm，但有1例接受治疗后3个月血睾酮浓度增至12.7 nmol/L，并出现骨龄提前；隐睾均下降至阴囊内，未出现萎缩或回缩；阴茎未出现残留弯曲和复发；阴茎阴囊转位和阴囊对裂校正后外形良好。术后并发症总发生率为23.4%，34例一期接受尿道成形术者并发症发生率为29.4%，30例接受分期尿道成形术者并发症发生率为16.7%，差异无统计学意义（P>0.05）。岛状包皮瓣卷管尿道一期成形术、岛状包皮瓣卷管尿道分期成形术、尿道板重建卷管尿道一期成形术、尿道重建卷管尿道分期成形术的受术者术后并发症发生率分别为26.3%、15.0%、33.3%、20.0%，四组并发症发生率差异无统计学意义（P>0.05）。结论 46，XY严重男性化不全的外阴男性化整形应包括阴茎弯曲的矫正、长段尿道成形、阴茎阴囊转位的矫正、对裂阴囊的融合、睾丸下降至阴囊内和阴茎增粗增长。本中心采用的岛状包皮瓣卷管和尿道板重建卷管及其对应的一期或分期尿道成形术均适用于严重男性化不全外阴整形的尿道重建。
- Abstract:
- Objective To summarize the procedure of masculinizing plasty in 46,XY severely undervirilized genital and explore the efficacy of plastic surgery.Methods A retrospective analysis was conducted for 64 children with severely undervirilized genital undergoing external genital masculinizing plastic surgery from January 2014 to November 2018.The karyotype was 46,XY in all cases.All external genitalia were perineal hypospadias with complete bifid scrotum and penoscrotal transposition.The transverse diameter of glans were<10 mm in 47 cases.Cryptorchidism was bilateral (n=9) and unilateral (n=6).Androgen therapy was offered.All patients underwent correction of chordee,urethroplasty,correction of penoscrotal transposition and bifid scrotum.At the same time,15 children with cryptorchidism underwent orchidopexy.For repairing severe hypospadias,one-stage or staged island flap tubularization urethroplasty and one-stage or staged urethral plate reconstruction urethroplasty were performed.Results After androgen treatment,the transverse diameter of glans were ≥ 10 mm.However,in one case,serum testosterone level spiked to 12.7 nmol/L and bone age advanced after 3 months.All testes descended to scrotum without atrophy or retraction.There was no residual tethering or recurrent curvature.Penoscrotal transposition and bifid scrotum were properly corrected.The total incidence of complications after urethroplasty was 23.4%,29.4% in 34 cases of one-stage urethroplasty and 16.7% in 30 cases of staged urethroplasty.No significant difference existed in the incidence of complications between one-stage and staged urethroplasty.The incidence of complications after four types of urethroplasty was 26.3% for one-stage island flap tubularization urethroplasty,15.0% for staged island flap tubularization urethroplasty,33.3% for one-stage urethral plate reconstruction urethroplasty and 20.0% for staged urethral plate reconstruction urethroplasty.Conclusion The procedures of masculinizing plasty in 46,XY severely undervirilized genital include correction of chordee,long length of urethroplasty,correction of penoscrotal transposition and bifid scrotum,orchidopexy and growth of penis.Four types of urethroplasty are suitable for urethral reconstruction of genital masculinizing.
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备注/Memo
收稿日期:2018-12-21。
基金项目:四川省卫生和计划生育委员会科研基金项目（编号：150206）
通讯作者:唐耘熳,Email:tangyunman@126.com