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Mayer-Rokitansky-Kuster-Hauser Syndrome (MRKH)

Mayer-Rokitansky-Kuster-Hauser Syndrome (MRKH): Understanding the Causes and Symptoms

What is Mayer-Rokitansky-Kuster-Hauser Syndrome (MRKH)?

  • A rare congenital disorder affecting the female reproductive system.

  • Characterized by the underdevelopment or absence of the uterus and vagina, while external genitalia appear normal.

  • Affects approximately 1 in 4,500 females.

What are the causes?

  • Exact cause unknown, but believed to be related to abnormal development during fetal life.

  • Two main types:

  • MRKH Type 1 (Isolated MRKH): No other organ abnormalities, often linked to genetic mutations or environmental factors.

  • MRKH Type 2 (MURCS Association):Associated with other malformations, such as kidney or skeletal abnormalities, often associated with genetic mutations.

What are the symptoms?

  • Primary amenorrhea (absence of menstrual periods) by age 16.

  • Pain during intercourse (due to vaginal shortening or absence).

  • Difficulty getting pregnant (due to absent or underdeveloped uterus).

  • Other symptoms may include:

  • Absence of cervix or fallopian tubes

  • Kidney problems

  • Hearing loss

  • Spinal malformations

Diagnosis and Treatment

  • Diagnosed through pelvic examinations, imaging tests, and genetic testing.

  • No cure for MRKH, but various treatment options exist:

  • Vaginal reconstruction surgery: Creates a functional vagina using tissue from other body parts.

  • Fertility treatments: Egg donation and surrogacy can help achieve pregnancy.

  • Psychological support: Crucial for managing emotional challenges associated with the condition.

Living with MRKH

  • MRKH doesn't prevent normal sexual function or relationships.

  • Early diagnosis and treatment can improve quality of life.

  • Support groups and resources are available for women with MRKH.

Additional Points:

  • MRKH is not a life-threatening condition, but its impact on fertility and emotional well-being necessitates proper management.

  • Ongoing research is exploring potential causes and treatment options

Mayer-Rokitansky-Kuster-Hauser Syndrome (MRKH)

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