Ovotesticular Differences of Sex Development: Surgery or Not Surgery? That Is the Question

The assignment of the sex of rearing for patients with precocious diagnosis of ovotesticular difference of sex development (OT-DSD) is a complex decision. Ovotesticular disorder or difference of sex development (OT-DSD) is a very rare congenital anomaly characterized by the simultaneous presence of both testis and ovary in the same individual. It occurs between 3 and 10% of the total DSD (1). The most common karyotype is 46 XX (60%), followed by chromosomal mosaicism/chimerism (33%) and 46 XY karyotype (7%) (2). Gonadal tumors occur between 2.6 and 4.6% of OT-DSD, more frequently in 46, XY cases (3). Therefore, a rigorous follow-up is needed. About Müllerian remnants; the possibility of degeneration is exceptional, although some authors describe it (4). There is no evidence that prophylactic removal of asymptomatic Müllerian remnants is required. If possible, we suggest leaving them in situ: they can theoretically be useful in sex-reverse genitoplasty. The timing of surgery for OT-DSD remains contentious, especially after the Consensus Statement on Management of Intersex Disorders held in Chicago in 2005. During this Meeting, international experts assembled to review this topic and to establish guidelines to help optimize and modernize the care given to patients with DSD. Gender assignment in newborns with OT-DSD represents a therapeutic challenge, the factors that influence gender assignment include: