Mao Yu,Xia Meng,Cai Yongchuan,et al.Masculinizing plasty in 46,XY severely undervirilized genital[J].Journal of Clinical Pediatric Surgery,,18():196-201.[doi:10.3969/j.issn.1671-6353.2019.03.007]
Masculinizing plasty in 46,XY severely undervirilized genital
- Keywords:
- Gonadal Dysgenesis; 46; XY/Embryo; Vulva/Abnormalities; Plastic
- CLC:
- R726.9;R726.2
- 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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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