Globally, one in 4,500 women does not have a uterus or vagina. This is Mayer-Rokitansky-Küster-Hauser syndrome (MRKH), simplified as Rokitansky syndrome. Patients affected by this rare congenital malformation face a real obstacle course to find a semblance of normal life.
Discovered in adolescence in 80% of cases
First of all, you should know that Rokitansky syndrome is a form of intersex. This term qualifies people born with sexual characteristics that do not meet the typical definitions of male and female. In fact, Rokitansky syndrome affects women born without vagina and uterus. Sometimes there may also be a partial congenital absence.
The syndrome appears in utero, but is discovered in adolescence in 80% of cases. This is particularly the case for the young Élisa, whose testimony was the subject of an article published in Slate March 5, 2021. At the age of 17, her gynecologist announced the presence of two ovaries, a partially formed vagina and above all, an absence of a uterus. A few months earlier, Élisa had undergone an MRI because of her absence of periods, despite the presence of developed sexual characteristics. The verdict was final: inability to be pregnant, or even having vaginal sex.
President ofMRKH Association in France, Amélie Victor has heard many testimonies from women with this syndrome. She explains act for a multiplication of studies on the subject. And for good reason, knowledge about MRKH syndrome is limited. Nevertheless, it was established that there was a question of a gene mutation occurring during the sixth week of the development of the embryo. However, this mutation stops the formation of Müllerian ducts before their term. In the world there would be approximately 4,500 women affected, but this number could be lower than reality.
Means exist, not without risks
Several methods exist to counter this syndrome. The first is the vaginal dilation (or Frank’s method). The goal? Create a neovagina through the use by the patient of dilators of different sizes. These dilators will gradually dilate the vaginal cup. The advantage of this method is that it uses fabric from the young woman. In six months, this allows the appearance of a vagina of normal size. However, this must be accompanied by therapeutic education of the patient. The other possibility is surgery, especially with vaginoplasty with a sigmoid graft and the Vecchietti technique. According to experts, the results of these operations are not necessarily better than those of vaginal dilation. They also imply a great psychosexual maturity of the patient as well as a good experience of the medical teams.
If successful, the means mentioned above allow a clear improved sex life patients. On the other hand, the absence of a uterus completely excludes the possibility of giving birth. However, the uterus transplant authorized in France in 2015 could be a game-changer. In 2019, Prof. Jean-Marc Ayoubi from the Foch hospital in Suresnes performed the first transplant of this kind in the country. This is obviously a particularly complicated procedure. Currently, researchers await the results regarding embryo implantation, pregnancy and childbirth to see if it is really a viable technique in all respects.