Disorders of Sexual Differentiation

In the context of sex development, sex differentiation defines the phenotype of the internal and external genitalia that logically follows sex determination. In 2006, a consensus statement was published summarizing results of a worldwide expert meeting that proposed, among other things, alternative and more precise nomenclature on management of disorders of sex development coupled with a new diagnostic classification. Key principles for management of DSD are: (1) avoid instantaneous gender assignment before expert evaluation, (2) assessment and longer-term management must be performed in a centre with an experienced multidisciplinary team, and (3) full and open communication with affected families is mandatory, with the families encouraged to participate in the decision-making.