Two-Stage Urethroplasty with Buccal Mucosa for Penoscrotal Hypospadias Reconstruction in a Male with a 46,XX Karyotype

Pieter D'hulst; Jochen Darras; Steven Joniau; Pieter Mattelaer; Linsey Winne; Diederik Ponette

doi:10.1016/j.eucr.2017.06.016

Two-Stage Urethroplasty with Buccal Mucosa for Penoscrotal Hypospadias Reconstruction in a Male with a 46,XX Karyotype

Urol Case Rep. 2017 Jul 18:14:45-47. doi: 10.1016/j.eucr.2017.06.016. eCollection 2017 Sep.

Authors

Pieter D'hulst¹, Jochen Darras¹, Steven Joniau², Pieter Mattelaer¹, Linsey Winne³, Diederik Ponette¹

Affiliations

¹ Dept. of Urology Az Damiaan Oostende, Gouwelozestraat 100, 8400 Oostende, Belgium.
² Dept. of Urology UZ Leuven, Herestraat 49, 3000 Leuven, Belgium.
³ Dept. of Endocrinology Az Damiaan Oostende, Gouwelozestraat 100, 8400 Oostende, Belgium.

Abstract

We present a case regarding a 32-year old African male with penoscrotal hypospadias, left cryptorchidism and a left inguinal hernia. There were moderate masculinization characteristics. He underwent a Lichtenstein hernia repair with perioperative biopsies of the left inguinal testis and epididymis. Microscopic examination showed a Sertoli-only left testis with Leydig-cell hyperplasia and the left epididymis consisted of ovarian tissue with corpora albicantia and maturing follicles. Endocrinological evaluation showed a sex-determining region Y (SRY) negative 46,XX karyotype. We successfully performed a two-stage urethroplasty with buccal mucosa graft to reconstruct his penoscrotal hypospadias.

Keywords: 46,XX testicular disorder of sex development; Cryptorchidism; Hypospadias; Urethroplasty.

Publication types

Case Reports