Müllerian Agenesis

Not to be confused with Müllerian.

Müllerian Agenesis or Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is an intersex condition that effects 1 in 4,500 AFAB/CTF individuals in which one is born without a uterus. They may or may not have lone ovaries, no ovaries, and/or an underdeveloped or incomplete vagina. One with this condition may or may not have oddly placed kidneys, a single kidney or undersized kidneys, spine abnormalities, hearing issues, or heart defects however this is not always the case, and only tends to be the case when ones condition is more severe.

Individuals with this condition are unable to reproduce as they have no uterus. Most with this condition are unaware of it until they hit puberty, due to the fact that they do not menstruate. Those with this condition may also have hyperandrogenism, due to a lack of estrogen production that may co-part this condition.

In some cases, they may have WNT4 Deficiency, however this is usually not the case.

Causes
The cause of this syndrome is unknown. Changes in several genes that are involved in development before birth have been identified in individuals with this syndrome, however, each of these cases has been found in only a few affected individuals, and it is unclear whether these changes cause the syndrome itself. Researchers are working to determine how genetic changes might lead to problems with reproductive system development.

Resources

 * https://en.wikipedia.org/wiki/M%C3%BCllerian_agenesis
 * https://medlineplus.gov/genetics/condition/mayer-rokitansky-kuster-hauser-syndrome/