Atypical genitalia in neonates

Information on the long-term outcome is lacking in many disorder of sex development (DSD) conditions.

46,XX DSD

Girls with congenital adrenal hyperplasia secondary to 21 hydroxylase deficiency may have a varied outcome. Fertility may be impaired. If glucocorticoids and fludrocortisone are taken as prescribed (lifelong treatment) and monitoring of growth in childhood continues, adequate height is often achieved and adrenal crises can usually be avoided.

46,XY DSD and sex chromosomal DSD

The outcome depends on the underlying disorder and the treatment and is the subject of ongoing study. Sex steroids may be required. Ongoing psychological support is helpful. Further surgical treatment may be indicated.