Consensus and controversy in management of undervirilized 46, XY disorders of sex development

Abstract

Objective To explore the consensus and controversy of managing 46, XY perineal hypospadias with a questionnaire survey of 16 specialists from 15 domestic pediatric urological centers. Methods The questionnaire contained 13 domains including disorders of sex development, crypotorchidism, gonad, gender assignment, hormonal therapy for micropenis, staged urethral reconstruction, replacement material of urethra, chordee repair, and scrotal plasty similar experiences.The outcomes were analyzed. Results Consensus of most specialists is that evaluations of disorders of sex development should be completed by a multidisciplinary team, Hypospadias with inguinal cryptorchidism should be co-repaired.Intra-abdominal testis may be repaired in I/II stage depending on the situation.Intra-abdominal gonad is routinely explored with laparoscopy.For staged repair, orchidopexy and cordee repairing are arranged in the first stage and urethral reconstruction in the second stage.Micropenis requires hormonal treatment prior to operation.If hypospadias is repaired in one stage, Duckett plus Duply is recommended.If urethral plate appears healthy, Snodgrass or Onlay is preferred; If non-healthy, transsection is required.Tunica albuginea plication needs freeing of neurovascular band.For severe cordee, ventral lengthening is necessary.Scrotal plasty is recommended.Controversy is common in many other aspects. Conclusions Perineal hypospadias should be evaluated by a multidisciplinary team.Still many controversies persist in the management of similar cases.Further prospective and well-designed case control studies are warranted. Key words: Disorders of sex development; Hypospadias; Cryptorchidism