Disorders of sexual development

Patients with ambiguous genitalia mostly present in the newborn period requiring a multidisciplinary team, which includes a pediatric surgeon/urologist to assign the sex of rearing as soon as possible after a thorough genetic, anatomic, functional, and socioeconomic workup. The Consensus Statement on Management of Intersex Disorders proposes the term disorders of sex development (DSDs), defined as congenital conditions in which development of chromosomal, gonadal, or anatomic sex is atypical. Sex differentiation refers to the anatomic development of the internal and external genitalia as male or female dependent on the presence or absence of functioning androgens. Placental human chorionic gonadotropin (HCG) is the one that ensures high concentrations of androgens during the first half of gestation to stabilize Wolffian duct development and differentiation of the external genital anlage. The term intersex was used till 2005, and subcategories included male pseudohermaphrodite, female pseudohermaphrodite, and true hermaphrodite.