Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a congenital condition that affects the reproductive system in females. Among affected women, the uterus and vagina are either underdeveloped or entirely absent.
The first symptom of the condition that is usually noticed is a lack of menstruation by time the child reaches 16 years of age.
The condition can raise various psychological challenges for affected females, who may suffer from anxiety and depression as a result of failed attempts at intercourse and self esteem issues during adolescence.
The overall aim of treatment is to achieve sexual function for affected females.
Young girls or women who are diagnosed with this condition should receive adequate support from their family and doctor. Support should be available in the form of counselling as well as group programmes and support groups.
The idea of creating a functional vagina is introduced to affected females once they have reached sexual and emotional maturity. A number of techniques have been used over the years including surgical and non-surgical techniques.
These are considered the first-line approach and involve the use of vaginal dilators, which are plastic tubes that have been specifically designed to help create or enlarge the vagina.
The most common method is called Frank’s dilator method, which involves applying the dilator and stretching the vagina on a daily basis to gradually expand it over a period of six weeks to several months.
Various surgical techniques exist for creating an artificial vagina. These include the McIndoe technique, which involves the application of a skin graft to a space between the rectum and the bladder. Women are still likely to require the vaginal dilators, to enhance the success of the surgery. Another approach is autotransplant, meaning the vagina is created using the patient’s own cells from another part of their body to create a fully functional vagina.
Women with MRKH syndrome do have normal, functioning ovaries and can still have children with the help of assisted reproduction. For example, some women are able to have a child using in vitro fertilization of their own eggs and a surrogate mother. Affected females also have normal external genitalia and sexual characteristics. In some cases, other defects may also be present such as underdeveloped kidneys, skeletal abnormalities, impaired hearing and less commonly, heart disorders.
Reviewed by Sally Robertson, BSc