MRKH Definition

Also called Mullerian agenesis, MRKH is the condition in a female where the mullerian ducts fail to develop and a uterus will not be present. MRKH stands for Mayer-Rokitansky-K?ster-Hauser Syndrome, derived from the names Augsut Franz Joseph Karl Mayer, Carl Freiherr von Rokitansky, Herman K?ster, and G.A. Hauser.

MRKH Diagnosis

Diagnosis of the disorder is often made either radiologically or laparoscopically among patients whom hormonal and karyotypic investigations for primary amenorrhea are normal.

MRKH Treatment

Treatments to lessen pain during sexual intercourse are available but women. Uterine transplant is being planned by UK and Swedish doctors that would allow women to carry their own child but no transplant has been seen successful. Certain women who have ovaries can have genetic children though IVF with embryo transfer to a gestational carrier. Developing a functioning vagina to make way for satisfactory sexual intercourse may require vaginal dilators. In a McIndoe procedure, a skin graft is done and applied to form an artificial vagina. Dilators are still needed though after the procedure to prevent stenosis. A laparoscopic procedure called Vecchieti has been shown to result in a vagina that is comparable to a normal vagina.

MRKH Symptoms and Signs

A woman with MRKH is hormonally normal, which means she will enter puberty with development of secondary sexual characteristics including thelarce and adrenarche. Ovulation generaly occurs and chromosome constellation will be 46,XX. Affected women typically have shortened vagina and intercourse will be difficult and painful. A fractional absence of the cervix, uterus, and vagina may be revealed in medical examinations, supported by gynecologic ultrasonography. Women with MRKH cannot get pregnant since there is no uterus. In vitro fertilization and surrogacy however are options for them to have a genetic offspring. The disease is commonly found out during a woman's puberty years, when the menstrual cycle does not start. It can also be discovered earlier in some cases when women undergo surgeries for other conditions such as hernia. Less common symptoms of the disorder include kidney problems, hearing loss, and bone malformations.

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