Agonadism

Classification according to ICD-10
Q50.0	Congenital absence of the ovary
Q55.0	Absence and aplasia of the testicle
ICD-10 online (WHO version 2019)

Agonadism describes the absence or complete loss of function of the gonads (gonads). Genetically male individuals are also referred to as anorchidia (missing testicles ), and to people with a female genotype as anovaria (missing ovaries ). The cause is almost always a congenital or in utero acquired malformation.

The malformation is very rare. In most cases, more or less rudimentary gonads are detectable. The Overzier syndrome , described by the Mainz internist Claus Overzier (born 1918), is a true congenital agonadism in women, i. That is, the embryonic anchoring of the gonads is already missing, presumably as a result of damage to both X chromosomes.

People with the comparatively common chromosomal disorders Turner syndrome (genotype X0) and Klinefelter syndrome (XXY, XXXY) usually have gonads with restricted function ( hypogonadism ).

This article is about a health issue. It is not used for self-diagnosis and does not replace a diagnosis by a doctor. Please note the information on health issues !