Gonadal Agenesis

Gonadal Agenesis, Agonadal, Anorchia or Agonadism is a rare form of intersex in which one does not develop gonads (testes, ovaries, or ovotestes) during fetal development. In true agonadism one is born without testes, ovaries, ovotestes, and does not have any gonadal tissue at all. In most cases on agonadism the person has XY chromosomes (though cases with XX chromosomes have been reported) and always has female internal genitals. They may or may not have a uterus or other internal structures, these may or may not be fully developed. Due to the lack of gonads people with this condition will not go through puberty without hormone replacement therapy.

Subsets of it can be penile agenesis (testicular agenesis/hypogenesis), vaginal agenesis (Müllerian agenesis, genital aplasia/hypoplasia or Mayer–Rokitansky–Küster–Hauser syndrome (MRKH)). Not to be confused with aphallia.