**4.9 Psychological aspects**

Sex assignment of children with DSD is a subject of intense debate. The early pioneers in this field coined the term 'optimal gender policy', which advocated for early corrective surgery to help the affected children and their parents to facilitate stable gender identity and appropriate gender role behavior (Money et al., 1955) . Opponents of early surgery argue for a 'full consent policy', in which surgery is not performed in non-emergency situations before full consent may be obtained from the child (Kipnis § Diamond, 1998). In 17-HSD3 deficiency, as in all situations characterized by severe undervirilization (Sinnecker stage 5 or 4), is not always feasible to wait the start of the virilization and/or the age for a reliable full consent for major intervention, because in this waiting period the patient could assume a female gender role and identity. According to the recent guidelines regarding ethical principles and recommendations for the medical management of DSD in children and adolescents, the parents take the first-line responsibility in defining what might be best for the child, and this might vary according to their individual experience and lifestyle, cultural expectations and religious beliefs (Wiesemann et al., 2010). The child, according to his or her developmental level, can express own preference. Each case must be weighed on its own merits. When there is a doubt, the psychological and social support of the child and the parent is to be ranked higher than the creation of biological normalcy.

## **4.10 Malignancy risk**

The external genitalia are mostly female in 17-HSD3 deficiency, but the internal structures are derivatives of wolffian structures. The testes are usually positioned in the inguinal canal, sometimes at the labia majora and rarely in the abdominal cavity (Mendonca et al., 2000). The consensus statement for management of DSD puts the risk of germ cell malignancy at 28% in 17-HSD3 deficiency (Houk et al., 2006; Hughes et al., 2006). This puts it in the intermediate risk group for malignancies and close monitoring is recommended for someone who is raised as a male rather than having gonadectomy at the time of diagnosis.
