Journal of pediatric urology · 2026
Long-term surgical results and satisfaction in patients born with 46, XX, SRY-negative, ovotesticular-DSD and raised as male.
СтатьяНазвание и аннотация: Перевод в очереди
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Ibrahim Hussein, El-Ghoneimi Alaa, Pierucci Ugo Maria, Lachkar Amane-Allah, Bidault-Jourdainne Valeska, Amar Mahmoud Salah, Josset-Raffet Éliane, Carel Jean-Claude, Paye Annabel, Peycelon Matthieu
Аннотация
OBJECTIVES: SRY-negative 46, XX,ovotesticular disorders of sex development (DSD) are considered a unique condition characterized by the development of testicular tissue in the absence of a Y chromosome. This study aims to report the clinical and surgical management of these patients raised as males.
PATIENTS AND METHODS: Patients with a 46, XX, SRY-negative,ovotesticular-DSD raised as males who were referred to our multidisciplinary DSD center were identified (2006-2020). We retrospectively analyzed clinical and histological data, with special emphasis on surgical management (gonadal and reconstructive procedures) and postoperative complications. We also evaluated surgical and cosmetic outcomes, sexual activity and voiding symptoms in adulthood.
STATISTICAL ANALYSIS: descriptive.
RESULTS: Four patients were evaluated in our multidisciplinary DSD center for the first time at a median (IQR) age of 7.5 years (1 month-11 years) and followed-up for a median (IQR) time of 14 (12-16) years. Gonadal frozen section biopsies were performed in all of them. Ovotestis was found bilaterally (N = 1) or unilaterally (N = 3). Gonadal surgery was performed in all patients (left total gonadectomy (N = 1), bilateral total gonadectomy (N = 2), bilateral selective gonadectomy (N = 1)) at a median age of 9 years (2-11). Mullerian structures were removed laparoscopically (N = 2). Genital reconstructive surgery was performed at a median age of 8 years (2-12) (N = 4): one-stage (N = 2) (urethroplasty with double-face tubularized preputial island flap) and staged procedure (curvature correction, urethroplasty with preputial (N = 1) or bladder mucosal graft (N = 1)). Urethrocutanous fistula occurred in three (75%) patients and was successfully managed by elective closure. All patients received intramuscular testosterone therapy to increase growth velocity and pubertal progression. At the last follow-up, all patients reported cosmetic satisfaction, spontaneous erections, and no residual penile curvature. Gender affirmation was male for all patients. Two patients opted for bilateral testicular prosthesis.
CONCLUSION: When managed by a dedicated multidisciplinary DSD center, this specific cohort of patients has acceptable surgical and cosmetic outcomes on long-term follow-up.
PATIENTS AND METHODS: Patients with a 46, XX, SRY-negative,ovotesticular-DSD raised as males who were referred to our multidisciplinary DSD center were identified (2006-2020). We retrospectively analyzed clinical and histological data, with special emphasis on surgical management (gonadal and reconstructive procedures) and postoperative complications. We also evaluated surgical and cosmetic outcomes, sexual activity and voiding symptoms in adulthood.
STATISTICAL ANALYSIS: descriptive.
RESULTS: Four patients were evaluated in our multidisciplinary DSD center for the first time at a median (IQR) age of 7.5 years (1 month-11 years) and followed-up for a median (IQR) time of 14 (12-16) years. Gonadal frozen section biopsies were performed in all of them. Ovotestis was found bilaterally (N = 1) or unilaterally (N = 3). Gonadal surgery was performed in all patients (left total gonadectomy (N = 1), bilateral total gonadectomy (N = 2), bilateral selective gonadectomy (N = 1)) at a median age of 9 years (2-11). Mullerian structures were removed laparoscopically (N = 2). Genital reconstructive surgery was performed at a median age of 8 years (2-12) (N = 4): one-stage (N = 2) (urethroplasty with double-face tubularized preputial island flap) and staged procedure (curvature correction, urethroplasty with preputial (N = 1) or bladder mucosal graft (N = 1)). Urethrocutanous fistula occurred in three (75%) patients and was successfully managed by elective closure. All patients received intramuscular testosterone therapy to increase growth velocity and pubertal progression. At the last follow-up, all patients reported cosmetic satisfaction, spontaneous erections, and no residual penile curvature. Gender affirmation was male for all patients. Two patients opted for bilateral testicular prosthesis.
CONCLUSION: When managed by a dedicated multidisciplinary DSD center, this specific cohort of patients has acceptable surgical and cosmetic outcomes on long-term follow-up.