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Rare Disorders : Mayer Rokitansky Kuster Hauser (MRKH) Syndrome

About:

Mayer Rokitansky Kuster Hauser (MRKH) Syndrome is a disorder characterized by the partial or complete absence of uterus and vagina in women with normal ovarian function and external genitalia.

MRKH affects 1 in every 4500 females [1]

-Chronic : Can not be treated, lasts for a lifetime
-Surgical intervention can allow partial functioning
-Self Diagnosable

Women affected by MRKH generally do not have a functioning menstrual cycle. They are unable to carry a pregnancy, although they may be able to bear a child through assisted reproduction.

Symptoms:

MKRH is classified into two types,
- Type 1 is characterized by absence of reproductive organs and amenorrhea. Other aspects of sexual development are not affected, and the subject may develop breasts and pubic hair.
- Type 2 is the more severe form, where other abnormalities are also present like abnormal formation or positioning of the kidney and skeletal abnormalities affecting the spinal cord. Other symptoms may include hearing loss and heart defects.

Self-Care:
Women with MRKH syndrome are encouraged to seek counseling after a diagnosis and before treatment because the diagnosis can cause anxiety and extreme psychological distress

Treatment:

- Artificial vagina maybe grafted or vaginal dilators may be used to develop a functioning vagina for penetrative intercourse. However, in absence of a uterus pregnancy is not possible.
- Uterus transplantation are being performed in people with MKRH, although it is still in experimental stage. In 2014, the first successful uterine transplant was carried out.
- Other researches include lab grown structures developed using a person's own own cells. Recent developments have resulted in fully functioning vaginas capable of menstruation showing promises of fully correcting these conditions in a few sufferers.

Specialists:
MKRH can be a genetic defect and its effect may last a lifetime. At mfine, you can consult our doctors for the best alternatives and advanced medicines for complete resolution of your problems.

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Dr. Vidya Desai Mohan

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Dr. K Swarajya Lakshmi

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Ankura Hospital for Women & Children

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Dr. Rani Nandakumar

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Cloudnine Hospital - Chennai

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Dr. Gohar Shahean

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