Abstract
BACKGROUND: Partial androgen insensitivity syndrome (PAIS) is a rare cause of variations in sexual and genital development characterized by severe hypospadias, micropenis, and a bifid scrotum. Sex assignment and determination of gender are significant challenges both from societal and surgical considerations. This case report describes the surgical management of masculinizing genital reconstruction without urethroplasty in an adult patient with PAIS, demonstrating that achieving penile straightening and lengthening is possible as an initial surgery in adulthood. CASE DESCRIPTION: A 30-year-old patient born with PAIS exhibited genitalia variation with severe perineoscrotal hypospadias and a microphallus. Assigned female at birth, he developed gender dysphoria in adolescence and was referred to our institution for surgery in adulthood. The surgery consisted of cavernoplasty with a double-Y albugineal incision covered by a patch using Surgisis(®) and metoidioplasty implants to correct the penile curvature. The urethral plate was transected, a penoscrotal urethrostomy was performed and a scrotal flap was harvested. The patient reported a high level of satisfaction with sexual function. CONCLUSIONS: This case demonstrates that it is possible to delay genital reconstruction surgery and raises the question of the societal acceptability of the intersex situation during childhood. Following to its European neighbors, France has recently legislated on the management of these patients, promoting self-determination before any gender-affirming surgery.