What is MRKH ?

       MRKH stands for Mayer-Rokitansky-Küster-Hauser syndrome. It is a congenital disorder that primarily affects the reproductive system in females. Individuals with MRKH syndrome are born with underdeveloped or absent reproductive organs, particularly the uterus and upper part of the vagina.

Picture 1 shows The reproductive system in females

       MRKH syndrome is usually diagnosed in adolescence when a young woman fails to menstruate or experiences difficulty with sexual intercourse due to the absence of a vaginal opening. The exact cause of MRKH syndrome is not fully understood, but it is believed to result from abnormal development of the Müllerian ducts during fetal development.

There are two main types of MRKH syndrome:

• Type I: In this type, only the uterus and upper two-thirds of the vagina are affected, while the ovaries and external genitalia (such as the labia and clitoris) develop normally.
• Type II: In addition to the absence of the uterus and upper two-thirds of the vagina, individuals with Type II MRKH syndrome may also have abnormalities in the kidneys, skeleton, or other organs.

       Treatment for MRKH syndrome typically focuses on addressing the functional and psychological needs of affected individuals. While there is currently no cure for MRKH syndrome, several treatment options are available to address its associated challenges:

• Vaginal dilation: Vaginal dilation therapy involves using progressively larger vaginal dilators to stretch and elongate the existing vaginal tissue. This can help create a functional vaginal canal that allows for sexual intercourse and reduces discomfort.
• Surgical procedures: Surgical options for MRKH syndrome include vaginal reconstruction surgery (vaginoplasty) to create or lengthen the vagina, as well as procedures to address associated abnormalities such as kidney or skeletal abnormalities.
• Assisted reproductive technologies (ART): In cases where the ovaries are present and functioning normally, individuals with MRKH syndrome may be able to undergo egg retrieval and in vitro fertilization (IVF) using a gestational carrier (surrogate) to carry a pregnancy to term.
• Psychological support: Coping with the diagnosis of MRKH syndrome and its associated challenges can be emotionally difficult. Psychological support, counseling, and support groups can help individuals and their families navigate the physical and emotional aspects of living with MRKH syndrome.

       MRKH syndrome is relatively rare, occurring in approximately 1 in 4,500 female births. It is named after the physicians who first described it: August Franz Joseph Karl Mayer, Carl Freiherr von Rokitansky, Hermann Küster, and Georges André Hauser.

       It's important for individuals with MRKH syndrome to work closely with a team of healthcare professionals, including gynecologists, reproductive endocrinologists, psychologists, and other specialists, to develop a personalized treatment plan that addresses their unique needs and goals. With appropriate treatment and support, many individuals with MRKH syndrome are able to lead fulfilling lives.