Abstract
Disorders of sex development are complex congenital anomalies, where chromosomal, gonadal and genital genders are not consistent with aech other. It may be manifested immediately after birth, when accurate determination of the disorder and gender assignment represents an acute condition.
Disorders associated with ambiguous genitalia are usually not difficult to detect early. Other forms are diagnosed in adolescence as part of early or delayed puberty, amenorrhea or failure of secondary sexual characteristics development.
In the case of ambiguous genitalia an early gender assignment and detection of congenital adrenal hyperplasia, which is associated with severe sodium and potassium metabolism disorder 75% of the cases, is esential. In patients with Y chromosome a germ cell tumors are more frequent.
The risk and type of tumor are associated with the degree of gonodal differentiation. Before the gonadectomy it is essential to assess the risk individually with respect to the particular type of disorder and lavel of risk.