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El hipospadias es la anomalia congenita mas frecuente de la uretra peniana. Su gravedad depende del nivel de division del cuerpo esponjoso y del grado de la hipoplasia resultante de los tejidos que forman la cara ventral del pene. La posicion del meato uretral es un criterio insuficiente para definir la gravedad del hipospadias. La curvatura ventral, asociada a menudo al hipospadias, es la consecuencia de la hipoplasia ventral del pene. Las valvulas de la uretra posterior son la anomalia mas grave del flujo de la orina y constituyen una urgencia uronefrologica del periodo neonatal. Obligan a realizar un drenaje vesical de urgencia mediante la colocacion de una sonda uretral por el cirujano o con un cateter suprapubico. En el primer dia de vida, el diagnostico de las valvulas de la uretra posterior se establece mediante la ecografia y la cistografia retrograda, que muestran una dilatacion de la uretra posterior y la repercusion sobre la vejiga y las vias urinarias. Es esencial realizar un seguimiento de la funcion renal mediante pruebas de laboratorio a partir de las primeras horas de vida. Las anomalias adquiridas de la uretra masculina en la infancia son traumaticas en la mayoria de los casos, por la realizacion incorrecta de un sondeo o de la circuncision. Las consecuencias son las falsas vias uretrales, fistulas o estenosis. La uretrocistoscopia permite confirmar estos diagnosticos.

Background There is a wide difference of opinion between the medical–surgical community and advocacy group regarding Disorders of Sexual Development (DSD) secondary to congenital adrenal hyperplasia (CAH) being ranked in the intersex category. This rupture is even more evident when the issue of genitoplasty is brought up. For physicians it is obvious and unequivocal that a person with CAH and an XX karyotype has a female gender identity, whereas associations tend to rank persons with CAH in the intersex category and advocate holding-off on surgical management. Material/Methods A retrospective case study vs. control group, spanning over 40years, included 21 patients who were treated in 3 different centers. Each patient and their parents were contacted independently and interviewed regarding interpersonal relationships, psychological impact of genitoplasty, gender identity and opinion on optimal care management for this disorder. Three couples controls (parent–child) per CAH patients were used and matched according to age, sex assigned at birth and ethnic origin. Results Sex assigned at birth seemed to concord with the gender identity perceived by the patients in 85.7% of cases. In fact, 89.7% of patients and 100% of parents felt that feminizing genitoplasty should be performed within the first year of life. There is however a significant difference compared to controls who felt that surgical management should occur later on in life. No difference was highlighted during childhood regarding parents–child relationships or social integration. However, during adolescence, the parents–child relationship tended to be significantly more painful for the CAH group. Integrating their parenting role was significantly harder for patients in the CAH-DSD group. In the population of CAH-DSD patients who had genitoplasty the level of sexual fulfillment was not lower to the one reported by the control group. Conclusion Female sex assignment seems legitimate according to this study and the development of gender identity in these patients matches the sex assigned at birth. Resolving early on the adequacy of the genital anatomy with the sex assigned is promoted by patients as well as their parents. Proper psychomotor development and sexual satisfaction underline the absence of complications related to the surgical technique and the relevance of early surgical management.