Efficacy of urethroplasty for disorders of sex development with concurrent severe hypospadias

Abstract

Objective 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. Key words: Child; Urethroplasty; Disorders/Differences of sex developemt