Disorder of gender development refers to the spectrum of rare congenital conditions where there is an atypical development of chromosomal, gonadal, or anatomic sex.
They can be classified broadly into four categories on the basis of gonadal histologic features which include:
- female pseudohermaphroditism: 46XX with two ovaries
- male pseudohermaphroditism: 46XY with two testes
- true hermaphroditism: both ovarian and testicular tissues, 46XX and 46XY mosaic
-
gonadal dysgenesis
- mixed gonadal dysgenesis (MGD): a testis and a streak gonad, 45XO/46XY
- pure gonadal dysgenesis (PGD): bilateral streak gonads, 46XO/46XX/46XY
- complete gonadal dysgenesis (CGD)
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