Absence of uterus: Mayer-Rokitansky-Küster-Hauser syndrome

The Mayer-Rokitansky-Küster-Hauser is also known as MRKHS, müllerian agenesis or vaginal agenesis.

It’s a congenital disorder in women, in which the embryonic Müller conducts don’t develop, and as a consequence the woman suffers from primary amenorrhea and absence of uterus and vagina, but functional uterus.

It’s estimated that 1 out of every 5,000 female suffer from it, even though the affectation degree of the illness may vary, hence being the symptoms also different.

It’s been linked to a lack of sexual hormones receptors in the Müller conducts, as well as a lack of the enzyme galactose-1-phosphate uridylyltransferase.

Vaginal agenesis: absence of uterus

Diagnosis

Women with this syndrome present a normal production of their female hormones, which means that they develop their female sexual characteristics when they reaches puberty. They ovulate normally.

It’s common that the vagina is shorter than normally, which makes it difficult and paintful to have sexual intercourse. It’s also linked to a higher probability of diseases of bones and kidneys.

The diagnosis can be performed before the woman reaches 20, due to primary amenorrhea and incapability of vaginal penetration. It can be carried out through ultrasound, where the complete or partial lack of cervix, uterus or vagina will be proved. A magnetic resonance can also be carried out to confirm the diagnosis.

Solution for the Mayer-Rokitansky-Küster-Hauser syndrome

Treatment

This malformation can be corrected with vaginal dilation techniques. A neovagina of 8-10cm with lubrication and good compilance can be obtained.

Surgical techniques that consist on constructing a channel between the rectus-bladder space can be performed.

Women with this syndrome can’t get pregnant but can have biological descendants thanks to IVF and uterine transplants.

Currently, there have been great advances in the technique of uterine transplant, since the first baby from a transplanted uterus has been born. Even though this is not a routine technique, it’s experimental. A woman with this syndrome takes part in the study of this technique, although no results have been published yet.

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