Mullerian agenesis is a rare condition in which the vagina does not form and the uterus (womb) may only form partially or not at all.
Underdevelopment of the Mullerian duct during embryogenesis (formation of fetus inside a woman’s womb) leads to agenesis or atresia of the vagina, uterus, or both in patients with vaginal agenesis. The absence of menstruation at puberty is a common symptom of vaginal agenesis.
Approximately 1 in every 4,500-5,000 girls is born with Müllerian agenesis, also known as Müllerian aplasia, Mayer-Rokitansky-Küster-Hauser syndrome, or vaginal agenesis.
Females with Mullerian agenesis do not menstruate until their teens (amenorrhea). Female growth normally leads to puberty.
Mullerian agenesis features:
May be linked to:
Diagnosis relies on the detection of classic signs, the compilation of a comprehensive patient history, an exhaustive clinical evaluation, and the results of many specialized tests, including imaging techniques.
The first test must be transabdominal ultrasonography. It might be used with magnetic resonance imaging (MRI). The uterus and vagina can be seen on an ultrasound. It can also be used to check how the kidneys are working.
Karyotyping can be used to rule out other health problems. With karyotyping, the chromosomes in a group of cells are looked at. The karyotype of women with MRKH syndrome is a normal 46, XX.
Self-dilation is usually recommended as the first choice. Self-dilation is an alternative to surgical vaginal enlargement. The goal is to make the vagina long enough so that it can be used for sexual activity.
Self-dilation involves pressing a small, round rod (dilator) on the skin for 10 to 30 minutes, once to three times daily. This dilator resembles a hard tampon and is used in the same way. As time goes on, you switch to dilators that are bigger. It could take a few months to get the result you want.
When self-dilation fails, vaginoplasty—surgery to reconstruct a vagina. Different kinds of vaginoplasty surgery are:
Unfortunately, Mullerian agenesis is unpreventable. But it may be best to seek advice from a counselor in dealing with the problem of having an incomplete vagina and especially when dealing with a potential life partner.
American College of Obstetrics and Gynecologist (2022). Müllerian Agenesis: Diagnosis, Management, and Treatment. Retrieved November 25, 2022, from https://www.acog.org/clinical/clinical-guidance/committee-opinion/articles/2018/01/mullerian-agenesis-diagnosis-management-and-treatment
Mayo Clinic (2022). Vaginal Agenesis. Retrieved November 25, 2022, from https://www.mayoclinic.org/diseases-conditions/vaginal-agenesis/diagnosis-treatment/drc-20355741
National Organization for Rare Disorders, Inc. (2022 Mayer-Rokitansky-Küster-Hauser Syndrome. Retrieved November 25, 2022, from https://rarediseases.org/rare-diseases/mayer-rokitansky-kuster-hauser-syndrome/