MRKH, or Mayer-Rokitansky-Küster-Hauser syndrome, is a rare disorder characterized by the failure of the uterus and the vagina to develop properly in women who have normal ovarian function and normal external genitalia.
Females who have MRKH syndrome urinate the same way as females who do not have the condition. MRKH mainly affects the reproductive organs.
In MRKH type II, other body parts are affected including the kidneys, but the urethra, the tube from which urine exits the body, is generally not.
What Are the Symptoms of MRKH?
MRKH (Mayer-Rokitansky Küster-Hauser) syndrome type 1 (also called Mullerian aplasia) affects the reproductive organs, though females with MRKH syndrome type 1 have normal secondary sexual development including breast development, armpit and pubic hair growth, increased body fat around the hips and other areas, sex steroid levels, female sexual identification, and sex drive.
Symptoms of MRKH syndrome type I include:
- Failure to menstruate
- Infertility due to absence of the uterus and underdeveloped fallopian tubes
- Difficulty having sexual intercourse due to the shortness of the vagina
- Pain during intercourse
Mayer-Rokitansky Küster-Hauser syndrome type II, also called (Mu)llerian duct aplasia, (R)enal dysplasia and (C)ervical (S)omite anomalies or MURCS association, affects other parts of the body in addition to reproductive organs. Symptoms of MRKH type II may include:
- Failure of the kidneys to develop properly that can cause:
- Kidney stones
- Growth deficiency
- Increased susceptibility to urinary tract infections (UTIs)
- Obstruction that can result in abnormal accumulation of urine in the kidney
- Skeletal malformations, mainly in the vertebrae, that can cause:
- Short neck
- Limited neck motion
- Abnormally low hairline
- Abnormal sideways curvature of the spine (scoliosis)
- Elevation of the shoulder blade
- Asymmetric, fused or wedge vertebra
- Malformed or missing ribs
- Abnormalities of the head and face
- Cleft lip
- Cleft palate
- Abnormally small jaw
- Underdevelopment of one side of the face causing facial asymmetry
- Hearing impairment/hearing loss
- Malformed ears
- Rare abnormalities
- Heart malformations
- Abnormalities of the extremities
What Is the Treatment for MRKH?
Treatment for MRKH (Mayer-Rokitansky-Küster-Hauser) syndrome is tailored to the specific symptoms each individual patient experiences.
Women with MRKH syndrome are usually advised to seek counseling after a diagnosis and before treatment because the diagnosis can cause anxiety and psychological distress.
Medical treatment for MRKH may include:
- Undeveloped vagina (vaginal aplasia) is treated by creating a new vagina for sexual intercourse
- Nonsurgical techniques include vaginal dilators
- Plastic surgery to create an artificial vagina (vaginoplasty)
- Treatment of kidney stones
- Antibiotics for urinary tract infections (UTIs)
- Reconstructive surgery for skeletal abnormalities
- Physical therapy for bone deformities
Health Solutions From Our Sponsors
