Ovotesticular Disorders of Sex Development

**Definition:** Conditions of sexual ambiguity in which the individual possesses gonadal tissues of both sexes, tissues from the OVARY and the TESTIS. There can be a testis on one side and an ovary on the other (lateral), or there may be combined ovarian and testicular tissue (ovotestes) on each side (bilateral). The karyotype may be 46,XX; 46,XY; or a mosaic of 46,XX/46,XY. These disorders have historically been called true hermaphroditism.

**Tree numbers:** - C12.050.351.875.253.343 - C12.200.706.316.343 - C12.800.316.343 - C16.131.939.316.343 - C19.391.119.343

**Synonyms:** - Ovotesticular DSD - Ovotesticular Disorder Of Sex Development - 46,XX Gonadal Dysgenesis, Complete, Sry-Positive - 46,XX True Hermaphroditism, Sry-Positive - XX Male Syndrome, Sry-Positive