Mullerian Agenesis

Mullerian agenesis, a rare congenital condition –also referred to as MRKH syndrome or Müllerian aplasia, is caused by the embryologic growth failure of the müllerian duct. Most common presentations result in the congenital absence or underdevelopment  of the uterus or the vagina, or both. The vaginal canal is markedly shortened or absent. First-line treatment usually can be managed non-surgically with successive vaginal dilation.

The non-surgical process is an effective and safe method for the creation of a neo-vagina for patients with vaginal agenesis. In comparison with surgical methods, vaginal dilation has the advantage of low morbidity and no surgical scarring. This method uses progressively sized dilators to expand the vaginal opening.

If surgery is necessary to create a neo-vagina, post-operative dilation therapy is essential to help prevent significant skin graft contracture.  Dilators must be used intermittently until the woman is able to engage in regular sexual intercourse.

This condition is commonly discovered during puberty and the typical symptoms include:

• Having a shortened vagina
• Sexual pain
• Absence of the cervix, uterus and/or vagina
• Primary amenorreah (lack of menstrual periods)

Did you know?

• Müllerian agenesis occurs in 1 out of every 4,000–10,000 women
• 7-10% of women suffering from müllerian aplasia have a normal but obstructed uterus
• Incidence of urologic abnormalities associated with MRKH ranges between 15-40%
• Most gynecologists will only encounter MRKH once or twice during their careers