摘要目的 探讨性别发育异常合并重度尿道下裂的手术治疗.方法 回顾性分析2012年1月至2016年1月收治的87例性别发育异常合并重度及复杂尿道下裂患儿的临床资料.本组患儿 年龄10个月至15岁2个月,平均40个月.87例均为初治病例(阴茎体型1例,阴囊型8例,会阴型78例),其中卵睾性别发育异常28例、混合性腺发育不良8例、5α-还原酶缺乏2例、Klinefelter综合征2例、雄激素不敏感3例,未找到具体病因的46,XY性别发育异常44例.本组一期矫治35例,其中Duckett尿道成形术14例,Duckett+Duplay尿道成形术21例;Duckett分期尿道成形术52例.结果 术后随访1年4个月至5年6个月,平均2年7个月.Duckett一期尿道成形术矫治患儿,并发症发生情况:尿瘘2例,尿道狭窄1例,尿道憩室1例.Duckett+Duplay一期尿道成形术矫治患儿,并发症发生率:尿瘘28.6%(6/21),尿道憩室4.8%(1/21),尿道狭窄9.5%(2/21).Duckett分期尿道成形术矫治患儿,并发症发生率:尿瘘7.6%(4/52),尿道狭窄1.9%(1/52),尿道憩室3.8%(2/52).结论 Duckett分期尿道成形术手术适用于矫治性别发育异常的重度尿道下裂,有效降低术后尿瘘、尿道狭窄和尿道憩室并发症的发生.

abstractsObjective To evaluate the therapeutic efficacy of two-stage tubularized transverse preputial island flapping for repairing disorders of sex development(DSD)with concurrent severe hypospadias.Methods From January 2012 to January 2016,retrospective analysis was performed for 87 DSD cases with concurrent severe hypospadias.The mean age of initial operation was 40(10-182)months.Hypospadias types were shaft(n=1),penoscrotal(n=8)and perineal(n=78),including ovotesticular DSD(n=28),mixed gonadal dysgenesis(n=8),5α-reductase deficiency(n=2),Klinefelter's syndrome(n=2),androgen insensitivity syndrome(n=3)and perineal hypospadias(n=44).The procedures were single-stage Duckett alone(n=14),single-stage Duckett plus Duplay(n=21)and two-stage tubularized transverse preputial island flapping(n=52).Results The mean follow-up period was 2.7(1.4-5.6)years.The complications of single-stage Duckett repair included fistula(2/14,14.2%),stricture(1/14,7.1%),urethral diverticulum(1/14,7.1%);single-stage Duckett plus Duplay with fistula(6/21,28.6%),urethral diverticulum(1/21,4.8%)and stricture(2/21,9.5%);two-stage tubularized transverse preputial island flap had fistula(4/52,7.6%),stricture(1/52,1.9%)and urethral diverticulum(2/52,3.8%).Conclusions Two-stage tubularized transverse preputial island flapping is indicated for repairing DSD with concurrent severe hypospadias.There are low postoperative complication rates of urinary fistula,urethral stricture and diverticulum.