What is Mayer-Rokitansky-Küster-Hauser Syndrome?

Mayer-Rokitansky-Küster-Hauser (MRKH) Syndrome, also known as Müllerian agenesis or vaginal agenesis, is a rare congenital condition affecting the development of female reproductive organs.

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Types of MRKH Syndrome

Type 1 (MRKH Syndrome): Involves underdevelopment or absence of the uterus and upper part of the vagina, with normal ovaries.
Type 2 (MURCS Association): Includes additional anomalies like kidney abnormalities and spinal deformities, alongside underdeveloped or absent uterus and upper vagina.

Symptoms of MRKH Syndrome

Absence or underdevelopment of the uterus and upper two-thirds of the vagina.
Normal external genitalia and secondary sexual characteristics.
Functional ovaries enabling hormone production and egg release.

Causes of MRKH Syndrome

The condition likely stems from combined genetic and environmental factors affecting the development of Müllerian ducts, which form female reproductive organs.

Complications

Primary complications revolve around reproductive challenges, as affected women cannot conceive or carry pregnancies due to the absence of a functional uterus.

When to Consult a Doctor

Consult a doctor if you experience unusual symptoms or concerns about your health, including changes in your body or reproductive health issues.

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Diagnosis of MRKH Syndrome

Diagnosis typically occurs in teenage years when menstruation does not commence. Diagnostic procedures include medical history, physical exams, pelvic ultrasound, and sometimes MRI or laparoscopy for further evaluation of internal structures.

Treatment of MRKH Syndrome

Vaginal Dilation Therapy:

What it is: Using tube-like devices (dilators) to gradually stretch the vaginal canal.
Purpose: Helps create a functional vagina over about six months.
Success Rate: About 90% effective.
Process: Usually done at home under medical guidance.

Surgical Options:

When needed: If dilation therapy isn't effective or preferred.
Types: Vaginoplasty (creating a vaginal canal using tissue grafts).
Variety: Methods vary based on surgeon expertise and patient anatomy.

Reproductive Assistance:

Options: IVF and surrogacy are viable for biological children.
Emerging Possibility: Uterine transplantation may allow pregnancy in the future.

Routine Care:

Importance: Includes HPV vaccination and STD testing.
Management: Pain relief through medications like ibuprofen.
Hormone Therapy: May help manage symptoms like cramping.

Supportive Care:

Counseling: Includes sexual health and emotional support.
Family: Support for coping with reproductive challenges.

Mayer Rokitansky Kuster Hauser Dos and Don'ts:

Do's	Don'ts

Consult a specialist for accurate diagnosis	Isolate yourself due to your condition
Ask questions about your condition and treatments	Compare yourself to others
Consider treatment options like dilators or surgery	Ignore emotional challenges
Seek emotional support through counseling or groups	Rush decisions about your care

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Frequently Asked Questions

Is MRKH syndrome hereditary?

MRKH syndrome is typically not inherited and is considered sporadic, meaning it usually occurs by chance rather than being passed down from parents to children.

What are the psychological and emotional impacts of MRKH syndrome?

MRKH syndrome can have significant psychological and emotional impacts, including feelings of grief, loss, and anxiety about fertility and sexual function. Support and counseling can help individuals cope.

Can women with MRKH syndrome have a normal sex life?

Yes, with appropriate medical treatment and support, many women with MRKH syndrome can have a fulfilling sex life. Vaginal dilation therapy and surgical options can help address physical challenges.

Can individuals with MRKH syndrome have children?

While pregnancy is not possible naturally for most women with MRKH syndrome due to an absent or underdeveloped uterus, options like gestational surrogacy or adoption can allow individuals to become parents if they desire.

Can MRKH syndrome be detected before birth?

MRKH syndrome cannot be detected before birth through prenatal screening or testing. It is typically diagnosed during adolescence or early adulthood when menstrual cycles do not start or develop fully.