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Müllerian agenesis

 

Müllerian agenesis, also known as MRKH syndrome or Müllerian aplasia, is a rare congenital condition caused by the failure of the müllerian duct to develop properly during embryonic growth. This condition typically results in the congenital absence or underdevelopment of the uterus, vagina, or both. In many cases, the vaginal canal is either severely shortened or absent. First-line treatment is often non-surgical, typically involving gradual vaginal dilation.

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 amenorrhea (lack of menstrual periods)

The non-surgical approach of vaginal dilation is an effective and safe method for creating a neo-vagina in patients with vaginal agenesis. Compared to surgical methods, vaginal dilation offers the advantages of low morbidity and no surgical scarring. This technique involves using progressively larger dilators to gradually expand the vaginal opening.

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

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